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in the previous issue of critical care , osuka and colleagues from the osaka university hospital and school of medicine examine fecal ph as a proxy for composition and functional alterations in the gut flora that predict outcome in critically ill patients . this work builds on and extends their previous work examining the role of alterations in the intestinal microflora on the systemic immune response syndrome ( sirs ) and mortality during critical illness . in the present study the authors demonstrate that when the ph level is increased or decreased by one , the incidence of bacteremia and mortality is increased . as an explanation for this finding , the authors measured various levels of organic acids including lactic acid , succinic acid , acetic acid , and formic acid . propionic acid and butyric acid , cytoprotective short - chain fatty acids produced by intestinal bacteria , were also examined . the data seem to show that the various levels of these acids in feces play a major role in determining fecal ph . although somewhat noisy , the fecal bacterial counts seemed to suggest that microbial composition was a determinant of organic fecal acid levels and hence ph . as might be expected , bacteroidaceae were decreased in patient groups and there was a proliferation of pseudomonas and a decrease in enterobacteriaceae . the use of multiple antibiotics among these patient populations probably explains these findings , as pseudomonas spp . the authors imply that perturbations in the intestinal microflora from the use of antibiotics , as well as due to the catabolic stress itself , have a major impact on fecal ph that may then be used as a global marker to predict outcome . this finding is important and is the first of its kind to suggest that it may be possible to identify biomarkers within the complex ecology of the gut that predict outcome . the gut represents the most diverse and fragile microenvironment and ecosystem in the human body and can become dramatically altered by critical illness and its invariable treatment with powerful antibiotics and vasoactive agents [ 3 - 5 ] . precisely how this perturbed ecosystem shifts the various microbes or microbial population into evolutionary trajectories that cause sirs and mortality remains a mystery . this outstanding group of investigators has been working diligently to characterize these changes , and in this report provides the practicing clinician with a possible tool to use as a guide that will signal when the patient 's evolving microbiome may be entering the danger zone . whether changes in ph are a cause of or a consequence of the resulting sirs and mortality in this study can not be inferred from the data presented . although the authors imply that bacteremia was more frequent in the patients with extreme ph values and that mortality , on average , was higher among these groups , we can not infer causality between the two events . although the data were not presented , many patients probably died without bacteremia and conversely many patients with bacteremia probably survived . while the authors speculate that ph might shift bacteria to translocate as a mechanism of provoking sirs and mortality , the pathophysiology of gut - derived sepsis is emerging to be much more complex than previously hypothesized [ 3 - 5 ] . in this context there are regional and spatio - temporal constraints that have prevented in - depth high - resolution tracking of the microbe , their genes , and their gene products through the course of critical illness . even in the current era of metagenomic , metatranscriptomic , and proteomic analysis of fecal samples , as the authors point out , microbes spatially nested within clinically unreachable sites may have profound influences on the systemic immune system that remain unknown . the promise of meta - omics is that we will be able to reach these sites and gain a more comprehensive readout of how the disappearing microbiome and the emerging pathobiome affect the inflammatory response and the ultimate outcome of the patient . the study by osuka and colleagues is limited because of the use of culture - based interrogation of the microflora and because the ph was not specified by region . yet what is very clear is that there is a gold mine in front of us . the authors have shown us a glimpse of what we can learn by mining and tracking key physiologic variables in feces , which while limited in their ability to provide molecular details , are clear biological beacons pointing us toward recovery or deterioration . osuka and colleagues have taught us a lesson ; we must pay attention to the effect of our therapies on the most fragile and perhaps most important biological determinant for survival - the composition and function of the growing microbial community in the gut .

the composition and function of intestinal microflora are emerging as integral to both health and disease . during critical illness the normal microbiota are rapidly replaced by pathogenic species as a result of both the physiologic stress itself and the use of antibiotics . in this report , the authors use fecal ph as a surrogate marker to determine the predictive value of the functional output of the intestinal microflora during critical illness . fecal ph appears to be highly predictive of outcome from critical illness , and may reflect the output of key organic acids such as the short - chain fatty acids , lactic acid , and other important products of the gut microflora .

supratentorial metastasis is found in 14.6% of cases , intraspinal mets in 12.5% , and systemic metastasis in 9.7% . the management of patients who present with diffuse leptomeningeal spread preoperatively has not been clearly defined . an 11year - old boy presented with progressive headache and gait ataxia for 1 year . on examination he was found to have only cerebellar ataxia with no signs suggestive of spinal cord involvement . he was investigated with cranio - spinal contrast mri , which showed three lesions , one in the region of the vermis and the other in the suprasellar region and long segment cervico - dorsal region [ figures 1 , 2a b and 3a b ] . the lesions were hypointense on t1w , and hyperintense on t2 with heterogenous contrast enhancement . the right ventriculo peritoneal shunt was inserted as the patient had features of raised icp with hydrocephalus . he was operated in prone position with flexion at atlanto - occipital joint , thereby opening up the operative site , taking care not to overflex to avoid blocking the venous drainage or airway . there was minimal improvement in sensory loss but none in motor power in 1 week . in view of his repeat mri spine showed no hemorrhage in the spinal lesion or in the tumor bed . there was minimal improvement in sensory loss but none in motor power in 1 week . in view of his lower cranial nerve palsies mid - sagittal mri showing fourth ventricular mass and sellar mass ( a ) mid - sagittal mri of cervico - dorsal showing dorsally placed lesion extending from c3 below . ( b ) mid - sagittal mri of cervico - dorsal showing dorsally placed lesion , expanding the spinal subarachnoid space above and below the lesion ( a ) axial t2 mri showing mass in dorsal posterior aspect of the cord . ( b ) another axial t2 mri showing mass in dorsal posterior aspect of the cord drop metastasis from the primary location to anatomically lower sites including whole - spinal cord is known as a potential way for tumors like medulloblastoma to spread and are seen in approximately 40% of patients . the high incidence of involvement of the lumbosacral region shows the effect of gravity on csf borne metastases . disseminated plaques and nodules are also more common in the dorsal region , supporting the theory of spread along the csf pathways as the normal caudal flow of csf is along the dorsal aspect of the cord . by far the most common presentation is that of the intradural , extramedullary type though intramedullary metastases are also reported . the presence of leptomeningeal metastasis in medulloblastoma is used for clinical staging and is already demonstrated as an independent prognostic factor . early drop metastasis is used to describe the group of patients with positive preoperative or immediate postoperative ( less than 1 month ) mri findings . late drop metastasis has been defined by new image evidence of leptomeningeal enhancement found after the primary surgical procedure with at least one negative prior whole - spine mri examination . leptomeningeal metastases found at presentation seem not to be a prognostic factor though late drop metastasis reflects the relative resistance of adjuvant therapy and may be viewed as a possible poor prognostic factor in this small patient group . the uniqueness of this case lies in the fact that the spinal drop metastases were sheetlike extending from the c3 to d8 region . the causes of postoperative paraplegia following posterior fossa surgery could be the following : intraoperative patient position , overflexion of neck and/or imbalance of the subarachnoid pressure between rostral and caudal thoracic spine , and by removing csf at the operative site . another possibility was that the contamination of csf with blood after posterior fossa surgery could lead to the development of a symptomatic spinal hematoma being explained by the movement of blood from the tumor bed into the spinal canal under the effect of gravity , during or after the surgery . meticulous homeostasis and isolation of the surgical area from the spinal space with avoidance of over drainage of csf have been stressed upon . the hydrodynamic changes that occur following a shunt placement can be speculated as another cause of paraplegia following surgery for nonspinal lesions . the current staging stratifies patients harboring these tumors in two groups , a high risk and a low risk group , with the mainstay of treatment being surgery , craniospinal irradiation , and chemotherapy . however , the exact management for patients presenting with disseminated leptomeningeal spread prior to surgery ( high risk ) , like in our case , is not clear . we would like to discuss two new options regarding the management of medulloblastoma with disseminated metastases . the first option is the administration of neoadjuvant chemotherapy in order to downstage the tumor , followed by surgery and radiotherapy . reported on three cases of presumed medulloblastomas who were preoperatively treated with chemotherapy consisting of high doses of carboplatin alone . the treatment improved the clinical condition prior to surgery and facilitated tumor removal , resulting in partial regression of tumor . the preoperative single - drug therapy did not affect the histological diagnosis or prevent the presence of an effective degree of tumor sensitivity to the drug and all the three children were disease - free for a mean period of 42 months . multidrug chemotherapy after a endoscopic / small biopsy has shown to reduce the tumor bulk significantly , downstaging the tumor and making total excision feasible . another option would be to operate on the drop metastases , especially those with long segment involvement , in the same sitting . the literature search did not reveal any studies which had dealt with this kind of management . besides , it may not be feasible in cases with long segment involvement like in our case . the possibility of neoadjuvant chemotherapy and/or single sitting surgical intervention for drop metastases needs to be explored in order to improve the outcome for such lesions .

management of patients with medulloblastoma presenting with multiple metastasis in subarachnoid space preoperatively is unclear . an 11year - old boy presented with vermian medulloblastoma with an unusually long segment cervico - dorsal lesion and suprasellar lesion . the child underwent a posterior fossa craniotomy ( prone position ) and excision of vermian mass . he developed paraplegia in the immediate postoperative period , the possible causes for which are being discussed . besides , treatment options for patients presenting with disseminated disease preoperatively have been highlighted . preoperative chemotherapy to downstage such lesions may be tried and such complications could be avoided .

percutaneous dilatational tracheostomy ( pdt ) is a simple , safe , low cost , minimally invasive bedside procedure with several documented advantages over surgical tracheostomy such as less local tissue damage , lower risk of bleeding , wound infection , and mortality . pdt is routinely performed with the aid of bronchoscope to further enhance the safety and accuracy of the procedure . recently , portable ultrasound has been employed for preintubation assessment of upper airway , postintubation verification of endotracheal tube position , and for predicting extubation outcome . until now , however , there are only a few reports and observational data that support the safety and efficacy of real - time ultrasound guided ( usg ) pdt . we performed real time usg pdt in our intensive care unit ( icu ) patients with the aim of acquiring a safe and cost - effective technique for point - of - care management . the objective of this retrospective case series was to report a feasibility study of real time usg pdt where bronchoscopy is unavailable . the study was exempt from review by the institutional ethics committee since it was a retrospective review of medical records . between july 2012 and february 2013 , we ( icu consultants with minimum 3 years of experience of performing pdt ) performed real time usg pdt on 12 patients admitted in our 10-bedded level 4 icu who required prolonged ventilatory support . all patients were hemodynamically stable with normal coagulation profile and were mechanically ventilated with positive end - expiratory pressure ( peep ) less than 10 . pdt , real time usg guided pdt was used . after obtaining written informed consent an ultrasound machine ( esoate , esoate inc . philipsweg , netherland , europe ) was used with sterile , 8 - 3 mhz linear array probe ( sl 3323 ) . imaging mode ( 2d mode ) was set to highest resolution to visualize superficial structures . the endotracheal tube was withdrawn to subglottic position after cuff deflation under direct laryngoscopic vision . following local anesthetic infiltration , ultrasound probe was placed on the tracheal ring just above the incision to continuously visualize the trachea . we used griggs forceps technique kits ( smiths medical , portex , london , uk ) with tracheotomy tubes ( blue line ultra portex tracheostomy tubes ) for performing pdt . to determine the depth of the puncture by cannula , the distance from the probe to the echo of the anterior wall of tracheal wall puncture of tracheal cartilage was done with 14 g cannula - on - needle while observing the needle path and tracheal wall insertion by real time sonography . a j - shaped guide wire was threaded into the trachea and an introducer dilator was then passed over the guide wire to facilitate the stoma formation [ figure 1a ] . subsequently , blunt dilatation was done and griggs dilatation forceps was advanced over the guide wire under ultrasound guidance [ figure 1b ] . an appropriate size tracheostomy tube with obturator was then threaded over the guide wire into the trachea lumen . after tracheal placement of tracheostomy tube , cuff was inflated and proper position confirmed by bilateral expansion of chest with positive pressure ventilation and auscultation of bilateral equal air entry . it was further confirmed by ultrasound intercostal view by observing positive lung sliding sign . ( a ) th ( lateral lobe of thyroid ) , is ( isthmus ) ; arrow indicating puncture of tracheal cartilage with 14 g cannula . ( b ) arrow indicating blunt dilatation with grigg 's dilatation forceps patients were monitored for complications such as hypotension , decreased oxygen saturation , bleeding and injury to posterior tracheal wall , atelectasis , or pneumothorax during the observation period ( during the procedure and for 1 week postprocedure ) . out of the 12 patients , four had chronic obstructive pulmonary disease , four were post neurosurgical , two had guillain - barr syndrome , and two had adult respiratory distress syndrome , all requiring prolonged ventilatory support . they were aged between 30 and 66 years ( median 48 years , interquartile range ( iqr ) 36 - 60 ) . none was morbidly obese ( median body mass index 22 kg / m , iqr 20 - 25 ) . follow - up data on patients were noted from the case files for up to 1 week postprocedure ( median 5 days , iqr 4 - 6 ) , during which no new complications were noticed . blind pdt procedure had many concerns such as inability to identify correct point of insertion and positioning of tracheostomy tube , false passage , and inadvertent injury to surrounding structures . however , bronchoscopy has the disadvantages of compromising ventilation especially in patients with high levels of peep and causing significant hypercarbia with risk of raised intracranial pressure and hence poorly tolerated by patients with head or spinal cord injury . ultrasonography has several advantages such as being easily available , pain - free , inexpensive , portable , safe , and repeatable . real - time ultrasonography has the additional advantage of enabling the clinicians to define the needle path by showing displacement of tissues ahead of needle . acute indentation of anterior tracheal wall with penetration of needle and subsequent straightening could be well - visualized with this technique . we applied the concept of real - time sonography to correctly locate the insertion point , to continuously visualize the needle path till it penetrates the anterior tracheal wall while preventing injury to the posterior tracheal wall and finally to confirm the correct position of the tube by the lung sliding sign . though it has been suggested by some that the longitudinal ( in - plane ) view could help better localization of endotracheal tube position while withdrawing and visualisation of the needle path , we preferred the transverse view to prevent associated injury , as the former approach may make viewing of the needle and structures of interest more difficult . the limitations of this case series include small sample size , retrospective design , absence of comparator group , limited follow - up , and inability to additionally assess with bronchoscopy . the disadvantages of not using bronchoscopy include diminished control of the airway , inability to detect posterior wall injury and ring fractures , decreased ability to perform pulmonary toilet around the procedure , diminished ability to detect false passage of the tube especially if the guidewire is accidentally pulled back too far . . however , in developing countries , prohibitive cost of the fiberoptic bronchoscope is a major limitation at many institutions . this case series shows the feasibility of real time ultrasonography - guided pdt where bronchoscopic guidance is unavailable . because of the absence of a comparator group in this case series however , real time ultrasonography guided pdt holds promise as a cost - effective and safe bedside procedure for larger application in critically ill patients especially in the morbidly obese and in those with cervical spine injury .

there are few reports and observational data that support the safety and efficacy of real - time ultrasound guided ( usg ) percutaneous dilatational tracheostomy ( pdt ) when compared with unguided tracheostomy . we performed real - time usg pdt in our intensive care unit ( icu ) patients with the aim of providing safe and cost effective point - of - care management . real - time ultrasonography has the potential advantage of enabling the clinicians to define the needle path by showing displacement of tissues ahead of needle .

cardiovascular disease is the leading cause of death for both men and women . in the united states , roughly 600,000 people die of heart disease every year . the most common type of heart disease is coronary heart disease , which itself costs the united states around $ 110 billion each year . aggressive risk factor management is recommended , which has improved patient survival , reduced recurrent events and the need for interventional procedures , and improved the quality of life of these patients . many trials have studied optimal management of lipids , diabetes , blood pressure , weight , and pharmacotherapy such as the importance of ace inhibitors or arbs , beta - blockers , and antiplatelet agents . however , there remains one lifestyle component , which has not received the attention it deserves for preventing cardiovascular disease : mental health . as we strive to further improve cardiovascular outcomes , the next bridge to cross may be one of offering patients nonpharmacologic means for combating daily mental stress and promoting mental health . indeed , the best preventive cardiovascular medicine may be a blend of both western and eastern medicine . depression and anxiety lead to a worse prognosis and outcome in patients with cardiovascular disease . while depression and anxiety increase the risk of developing cardiovascular disease , cardiovascular disease also increases the risk of developing anxiety and depression . thus , promoting optimal mental health may be important for both primary and secondary prevention of cardiovascular disease . anger and hostility in response to daily life events and stress may also be associated with an increased risk of cardiovascular disease . higher hostility levels may be associated with increased carotid artery medial thickness and thus a more rapid rate of progression of atherosclerosis . yoga is an ancient indian discipline with the goal of bringing balance and health to the physical , mental , emotional , and spiritual dimensions of an individual . it consists of postures ( asanas ) , concentration ( meditation ) , slow breathing ( pranayama ) , and recital of phrases ( called mantras ) . while there are 84,000 postures , performing yoga a few times per week , with each session lasting roughly 20 min , is effective in treating hypertension , reducing angina episodes per week , improving exercise capacity , and decreasing body weight and waist circumference . furthermore , it can decrease the frequency of revascularization , suggesting that yoga may facilitate regression and prevent progression of atherosclerosis , with a mechanism similar to that of statins . pranayama is a component of yoga and a form of slow breathing consisting of a respiratory rate of roughly six breaths per minute . several studies have been conducted both in india and the united states which have shown that pranayama , which takes 5 min to perform , immediately lowers resting blood pressure and permanently decrease resting blood pressure after several weeks of consistent practice . several studies in the united states have replicated these findings by using a slow breathing device which guides patients to breath at a rate of six breaths per minute . one form of pranayama is to close the left nostril with the finger , slowly inhale through the right nostril for 10 s , then close the right nostril and exhale through the left nostril for 10 s. this is repeated by alternating which nostril does the inhalation and exhalation . we live in a fast - paced society with prevalent deadlines and pressures . like lowering blood pressure , lipids , and body weight , lowering anger and hostility and improving depression and anxiety may also be an important intervention in preventive cardiology . similar to how antihypertensives are prescribed to lower blood pressure and statins to lower lipids , it may also be beneficial to prescribe nonpharmacological interventions such as yoga and pranayama to lower mental stress and improve mood . while the effects of medications are temporary in the sense that stopping antihypertensives will again elevate resting blood pressure , the effects of consistent practice of yoga and pranayama are permanent , resetting the autonomic nervous system to one of parasympathetic dominance . this decreases arousal to external stimuli and thus decreases anger and hostility and improves mood . furthermore , impaired baroreflex sensitivity has been proposed as one of the major causes of essential hypertension . yoga is equivalent to head - up or head - down tilt and restores baroreflex sensitivity . and last , yoga and pranayama also offer some of the effects of pharmacotherapy as well , such as lowering blood pressure , lipids , hemoglobin a1c , and weight . yoga and pranayama are free , easy to learn , and take only 20 or 5 min per day to perform , respectively . they have no side effects but still offer the potential to reduce cardiovascular disease , thus improving long - term outcomes and reducing overall health care costs . the field of psychoneuroimmunology continues to develop and we are learning more and more about the mind - body connection , especially how it relates to cardiovascular disease . while we have done well conducting large - scale randomized trials for optimizing medical and interventional management of cardiovascular disease , the next steps may entail conducting large - scale randomized trials to evaluate nonpharmacologic interventions to promote mental health and well - being , and to study the effects of these interventions longitudinally on cardiovascular disease prevention .

cardiovascular disease is the leading cause of death for both men and women worldwide . much emphasis has been placed on the primary and secondary prevention of cardiovascular disease . while depression and anxiety increase the risk of developing cardiovascular disease , cardiovascular disease also increases the risk of developing anxiety and depression . thus , promoting optimal mental health may be important for both primary and secondary prevention of cardiovascular disease . like lowering blood pressure , lipids , and body weight , lowering anger and hostility and improving depression and anxiety may also be an important intervention in preventive cardiology . as we strive to further improve cardiovascular outcomes , the next bridge to cross may be one of offering patients nonpharmacologic means for combating daily mental stress and promoting mental health , such as yoga and pranayama . indeed , the best preventive cardiovascular medicine may be a blend of both western and eastern medicine .

choriocarcinoma is a highly aggressive malignant human chorionic gonadoprotein - producing neoplasm with usually an intrauterine location and can be gestational or non - gestational in origin . extrauterine choriocarcinomas are very rare and can be seen in other parts of the female genital tract like fallopian tube , ovary and cervix . the estimated incidence of gestational ovarian choriocarcinoma is one in 369 million pregnancies , associated with coincident or antecedent gestation . we report a case of primary gestational ovarian choriocarcinoma in a young lady where the diagnosis was rendered by intra - operative cytology and the proliferative potential was estimated by ki67 immunohistochemistry . a 25-year - old female presented with pain abdomen and bleeding per vaginum for 25 days . abdominal examination revealed a soft , non - tender palpable lump in the right iliac fossa . her urine tested negative for hcg and the serum level was slightly increased to 3080 miu . on laparotomy , the right ovary was replaced by a hemorrhagic , friable and necrotic mass . imprint cytosmears were prepared from the mass and fixed with absolute alcohol and stained with papanicolaou stain and hematoxylin and eosin ( h and e ) stain . two cytopathologists examined the smears , which showed small clusters of pleomorphic and hyperchromatic cells with clear to pale eosinophilic cytoplasm over a hemorrhagic and necrotic background [ figure 1a and b ] . we received the gross specimen as cut - opened uterus with cervix and a large hemorrhagic and friable mass . sections were taken from different areas of the ovarian mass along with uterine wall and cervix and stained with h&e stain after processing . histopathology revealed mostly areas of hemorrhage and necrosis with a biphasic tumor tissue comprising of pleomorphic cytotrophoblasts and syncytiotrophoblasts . the sections were also subjected toki67 immunostain and were found to be positive with diffuse and intense staining ( in more than 50% of cytotrophoblasts ) showing high proliferative index of the tumor cells [ figure 2b ] . in contrast , the syncytiotrophoblasts were negative for ki67 . ( a ) large cells with pale cytoplasm , centrally placed nuclei against a hemorrhagic and dirty background ( h and e , 100 ) . ( b ) cytotrophoblasts and syncytiotrophoblasts in the cytosmear , ( h and e , 400 ) ( a ) variegated friable mass with hemorrhage and necrosis and normal - appearing uterus . ( b ) diffuse and strong positivity of tumor cells with ki67 immunostain ( ihc , 400 ) ovarian choricarcinoma is a highly malignant tumor characterized by the presence of malignant trophoblastic cells histopathologically and a raised serum hcg level biochemically the distinction between the two is difficult , but necessary , as the non - gestational type has bad prognosis . the patients are 40 years or older , present with abnormal uterine bleeding and have a very high serum hcg level . the above - said patient was a young female of 25 years with a past history of ectopic gestation 1 year back , had a mild increase in the serum hcg level and had no abnormality in the uterus or fallopian tube . she was treated with methotrexate - based chemotherapy ( methotrexate , etoposide and actinmycin - d ) and her hcg level came down to normal after two cycles of chemotherapy , and is doing well till date . ki67 was applied here to study the proliferative index of choriocarcinoma , which was high ( more than 50% of cytotrophoblasts ) and also strong in intensity of staining . also , it can be used to determine the therapeutic response after treatment by comparing with the present index . but , in other situations where the diagnosis is not so straightforward , it can be used to differentiate from other gestational trophoblastic diseases like hydatidiform mole ( complete type ) and placental site trophoblastic reaction and tumor . there are no unique ultra - structural or immunohistochemical features to distinguish either of them . dna polymorphism analysis can be utilized to distinguish pure non - gestational choriocarcinoma of the ovary from gestational choriocarcinoma . genetic analysis showing whether a paternal contribution is present in the genome or not can be a useful modality in determining the origin of the choriocarcinoma . human leukocyte antigen typing for paternal antigen in trophoblastic elements can also be used to determine the gestational etiology . markedly elevated levels of serum human chorionic gonadoprotein levels are commonly found with gestational tumors but are not very reliable . the majority of patients with non - gestational ovarian choriocarcinoma are younger than 20 years . while most gestational choriocarcinomas show hematogenous spread , the non - gestational choriocarcinoma tend to follow the lymphatic system . the staging depends on the original site of the tumor , like for gestational ( metastatic or primary ) choriocarcinoma standard choriocarcinoma staging applies and for non - gestational choriocarcinoma staging is performed by ovarian cancer staging system . because this patient was a young female presenting with gestational choriocarcinoma of the ovary , she was treated with radical surgery with preservation of contralateral ovary and followed by methotraxate - based chemotherapy . we are reporting this case as it is rare and also because one should be aware of the cytologic findings of choriocarcinoma so as to give a correct diagnosis during operation to help the surgeons in the proper management of such lesions .

primary ovarian choriocarcinoma is a rare neoplasm that can be gestational and non - gestational in origin . it accounts for one in 369 million pregnancies . both types present with similar clinical , histomorphological and ultrastructural findings . but , it is essential to differentiate the two because the gestational type has a better clinical course and responds to single - agent chemotherapy . usually , the gestational ovarian choriocarcinoma is metastatic from uterine choriocarcinoma and follows antecedent pregnancy and is seen in females of 40 years or older . dna polymorphism analysis showing the presence of paternal genes in the tumor establishes the gestational origin of choriocarcinoma . we present the intra - operative cytological findings of a case of primary ovarian choriocarcinoma in a 25-year - old lady arising from ectopic pregnancy with ki67 immunostain .

functional hallucinations are an unusual form of perceptual disorder , in which hallucinations are triggered by a stimulus in the same modality , and co - occur with it . for example , a patient may report hearing voices criticizing him every time he hears the sound of a rotating fan , and which stop when the fan is not running . their exact significance is unknown , but they have been reported in schizophrenia and other functional psychotic disorders . the treatment of positive symptoms of schizophrenia , including hallucinations , generally involves the use of antipsychotics . the following is the report of a patient diagnosed with schizophrenia , with persistent functional hallucinosis , who responded to the addition of sodium valproate . a single man , aged 30 , employed in a factory , presented to our outpatient department , in 2007 , with two years continuous illness , characterized by persistent auditory hallucinations , secondary delusions of reference , social withdrawal , and impaired occupational functioning . he also reported obsessive doubts about routine activities , such as closing doors or taps , and a compulsion to check whether he had done these properly , despite knowing that this was unnecessary . he was diagnosed to have paranoid schizophrenia and obsessive - compulsive disorder , and was treated with risperidone ( titrated up to 8 mg / day ) and fluoxetine ( titrated up to 80 mg / day ) . on the above - mentioned medications , he improved significantly , and was able to return to his job . his job involved frequent contact with machinery and motors . whenever he heard these machines running he found these distressing , and this led him to frequently avoid his work or leave it incomplete . he did not hear these voices at any other time , and did not report any recurrence of his other symptoms . his body weight was 62 kg . due to financial difficulties ( the patient was receiving the above medications free of cost from the hospital ) and his overall good response to risperidone therefore , he was given a trial of adjunctive sodium valproate , which was freely available in the hospital and had some evidence of efficacy in reducing positive symptoms , as an adjunct to antipsychotics . after obtaining the patient 's consent , sodium valproate was initiated at a dose of 600 mg / day , and gradually increased by 200 mg every week based on his response and adverse effects . at 1000 mg / day of sodium valproate , the patient reported a significant reduction in his hallucinations , and he felt that he could carry out most of his work . hence , valproate was further increased at the same rate , up to 1800 mg / day . at this dose , the patient reported feeling near - normal , and was in line for a promotion at his workplace . however , he developed significant postural tremors , which interfered with his ability to work , and valproate was reduced to 1700 mg / day . at this dose , he reported that the voices had decreased by 75% , and he scored between 2 and 3 ( mild symptoms ) on item p3 ( hallucinations ) of the positive and negative symptom scale for schizophrenia . he has remained on valproate 1700 mg / day , along with risperidone 8 mg / day and fluoxetine 80 mg / day , for the past three months , and has remained stable . his functional hallucinations still occur from time to time at work , but he is less bothered by them and does not experience any impairment . the management of patients with schizophrenia , who have a single persistent symptom , is a challenging situation . evidence - based treatments , such as changes in medication or cognitive - behavioral therapy , may not always be feasible . in this patient , time and economic constraints made it unlikely that the patient would comply with either . hence , an alternative that would be safe , affordable , and with some evidence of effectiveness was required . on account of the rarity of functional hallucinations however , a single case report suggests that carbamazepine may be helpful . in psychiatry , a cochrane review suggests that it may be useful in reducing aggression and tardive dyskinesia , but has little effect on other symptoms . however , one trial has suggested that adjunctive divalproex reduces positive symptoms , and a case series found the addition of valproic acid useful in difficult - to - treat schizophrenia patients . the effect of adjunctive valproate in schizophrenia is probably not due to the elevation of risperidone levels . valproate acts primarily through gamma - amino butyric acid ( gaba)-ergic mechanisms , which may modulate the actions of dopamine . alternately , it may act through an epigenetic mechanism involving the demethylation of relevant sections of the gaba - related genes . finally , given the paroxysmal , event - triggered nature of the patient 's hallucinations , it is possible that valproate 's ability to block sodium channels in a use - dependent fashion , may have contributed to symptom amelioration , as carbamazepine did in the earlier case . although it can not be recommended in all patients , this case suggests that valproate may be beneficial in selected cases , particularly as an add - on to antipsychotics .

functional hallucinations are a rare phenomenon , wherein hallucinations are triggered by a stimulus in the same modality , and co - occur with it . although hallucinations in schizophrenia are normally treated using antipsychotics , not all patients respond to them . the following is the report of a patient with paranoid schizophrenia who experienced persistent functional hallucinations , triggered by the sound of machines in his factory , in the absence of other psychotic symptoms . these occurred despite adequate doses of risperidone , which had controlled his other symptoms . the addition of sodium valproate , titrated up to 1700 mg / day based on response and tolerability , resulted in a marked improvement in this phenomenon and enabled him to return to work . the implications and possible mechanisms of the patient 's response are discussed .

currently anti - tnf agents are widely used for cutaneous and systemic autoimmune diseases . for dermatologists most common indications for anti - tnf use are psoriasis and psoriatic arthritis . we describe a case of scedosporium apiospermum infection in a patient receiving long - term treatment with etanercept , a tnf- inhibitor . s. apiospermum is a ubiquitous filamentous fungus found in soil , polluted water , and contaminated ambient air in hospital isolation rooms . in immunocompromised patients , increasing reports of s. apiospermum infection in the past few years suggests that s. apiospermum is an emergent opportunistic pathogen . infectious diseases including tuberculosis , atypical mycobacteria , histoplasmosis , coccidioidomycosis , and various opportunistic infections in patients receiving tnf - a inhibitors have been reported in the literature and postmarketing surveillance . a pubmed search resulted in one report of scedosporium infection as a complication of infliximab therapy for ankylosing spondylitis . to the best of our knowledge , this is the first reported case of s. apiospermum infection in a patient receiving etanercept . neither he had active psoriasis lesions , but he had pitting and ridging on nails . after extensive workup , we narrowed our differential diagnosis to ankylosing spondylitis and psoriatic arthritis . he was started on methotrexate because of continued neck and lower back pain with morning stiffness . in 2004 , etanercept was initiated at the dose of 50 mg / week and sulfasalazine was discontinued . he did remarkably well with etanercept in respect to back pain and morning stiffness . in 2008 as he was receiving an anti - tnf agent , we actively pursued investigation for opportunistic and rare infectious agents . a flexible endoscopic examination of the sinuses demonstrated mucopurulent ethmoid disease . tissue specimens of the right and left nasal turbinates and nasal washes were sent for evaluation and culture . the tissue isolates showed chronic inflammatory edematous respiratory tissue without any significant pathology in the underlying bones and gms stains showed no evidence of fungus . however , cultures of the specimen from the sphenoid sinuses returned positive for s. apiospermum . a mri of head and sinuses did not demonstrate any evidence for invasive rhinocerebral fungal disease . we consulted our infectious disease colleagues and the patient was started on voriconazole 200 mg bid . follow - up mri and ct of the brain and sinuses did not show any evidence for sinusitis or invasion of the adjacent anatomical structures . as there was no histopathological or radiological evidence for invasive fungal infection we felt comfortable to continue etanercept . the patient has continued to take both etanercept and methotrexate for more than 1 year with no signs of re - infection or complication . scedosporium apiospermum has been associated with mycetoma , keratitis , endophthalmitis , osteomyelitis , and brain abscesses . a wide range of pulmonary manifestations exists , ranging from simple colonization as seen in patients with cystic fibrosis to fungus ball formation in patients with cavitary lesions and invasive disease , simulating aspergillosis . as the clinical presentation of scedosporium infection , including fever , cough , and dyspnea is nonspecific , diagnosis can be based on cytology , histopathology , and isolation of the fungus in culture . culture confirmation is important as the histological appearance and clinical presentation of s. apiospermum are difficult to distinguish from that of the aspergillus species on pathological examination . in a case report of three lung transplant patients , mean - time from specimen collection to positive - culture identification for sputum culture was 4.5 days compared to 9.5 days with bronchoalveolar lavage culture . in our patient , positive culture was identified in 10 days . s. apiospermum has been shown to have intrinsic resistance to many anti - fungal agents , including fluconazole and amphotericin b , the traditional antifungal of choice for disseminated hyalohyphomycoses . much of the data on the treatment of s. apiospermum pertains to the use of voriconazole . a retrospective review of 107 patients treated for scedosporium infection with voriconazole showed 57% of patients achieved a successful response after a median of 103 days of therapy . in vitro studies have demonstrated that voriconazole is more active against s. apiospermum than either itraconazole or amphotericin b. the optimal choice and duration of therapy remain unknown . surgical debridement or drainage for limited disease in combination with antifungal therapy , as was done successfully with our patient , is recommended now .

patients on anti - tnf therapy are at increased risk for rare opportunistic infections . here we are reporting a case of scedosporium apiospermum infection in a patient treated with anti - tnf for 5 years . patients on anti - tnf need close follow - up and clinicians should be suspicious for atypical infections in these immunocompromised hosts .

neuroendocrine tumors are derived from neuroendocrine cells and the most common site is the bronchopulmonary system . theoretically , primary neuroendocrine tumors do not occur in the breast due to the lack of endocrine cells . although there are many published studies regarding this rare pathological entity , there are a few reports that also include radiology findings . however , most of the published studies of patients with radiology findings are of the small cell type of tumor , and the characteristic imaging features are not described well ( 1 - 7 ) . we report the imaging findings in a case of primary neuroendocrine tumor ( large cell neuroendocrine carcinoma with atypical carcinoid features ) found in the breast . a 42-year - old female was presented with a lump in her right breast , which she had been aware of for several weeks . on physical examination , an approximately 5-cm , firm , movable lump was palpated in the upper outer subareolar region of her right breast . mammography revealed a high - density mass with an ill defined margin in the 11 o'clock direction of the right breast subareolar area . targeted sonography of the right breast revealed an irregularly shaped , lobulated marginated , heterogeneous echotextured mass with posterior enhancement . heterogeneous echotexture was noted , especially in the center of the mass , and increased vascular flow was noted in the mass on color doppler scanning . there was also lymphadenopathy in the right axilla with cortical thickening and loss of fatty hilum ( fig . the mass was categorized as breast imaging reporting and data system category 5 , highly suggestive of malignancy . fine needle aspiration cytology revealed highly cellular smears with monotonous hyperchromatic atypical small cells , suggesting high - grade invasive ductal carcinoma . the mass appeared to be of heterogeneously low signal intensity on t1 weighted images and a few parts of the mass showed intermediate to high signal intensity on t2 weighted images . the t2 non - high signal area showed rapid enhancement within the first two minutes of gadolinium injection , and washout was noted ( fig . the patient underwent a modified radical mastectomy and axillary dissection of the right breast . on the cut surface , the tumor appeared as a fairly well - defined but partially infiltrative , gray - white , solid mass with morphology similar to that of commonly seen , invasive ductal carcinoma . histopathologically , several , large , mucoid , necrotic areas with foamy macrophages were surrounded by dark , solid , and trabecular pattern tumor cells . the tumor was composed of a mostly solid growth of large to intermediate , polygonal or occasionally spindle cells , which showed organoid , nesting , trabecular , rosette - like , and palisading patterns . immunohistochemically , the tumor cells were diffusely positive for synaptophysin and were focally positive for chromograin a , although they were negative for cd 56 . , we considered this tumor to be a large cell neuroendocrine carcinoma with atypical carcinoid features ( histologic grade 3 , t2n1m0 , estrogen receptor [ er ] [ + ] , progesterone receptor [ pr ] [ + ] , human epidermal growth factor receptor 2 [ - ] ) . positron emission tomography - computed tomography after surgery showed an absence of abnormal uptake , suggesting that the breast was the only primary site . neuroendocrine tumors are derived from the neuroendocrine cells and the most common site is the bronchopulmonary system , where the endocrine cells are constantly present . in the breast , hyperplastic or benign neoplastic neuroendocrine lesions have never been reported , and this lead to the hypothesis that neuroendocrine differentiated breast tumors probably do not arise from the pre - existing endocrine cells , but are rather the result of the differentiation process within breast carcinoma ( 8) . focal neuroendocrine differentiation can be found in diverse histological types of breast carcinoma . in one case series , most neuroendocrine cells were found with invasive ductal carcinoma or with mixed carcinoma showing mucinous differentiation or signet - ring cells ( 9 ) . however , the term , neuroendocrine tumor , is defined by the diffuse expression of neuroendocrine markers , such as chromogranin - a , neuron - specific enolase , synaptophysin , and cd56 , in more than 50% of the cell population . neuroendocrine breast tumors are very rare , comprising of approximately 2 - 5% of all breast cancers , and most of these patients are in the sixth or seventh decade of life ( 10 ) . different classifications , according to the morphological features of histopathology , have been proposed for neuroendocrine tumors , arising within the breast . perhaps the most pragmatic is the who classification , which defines three principle tumors , i.e. , solid neuroendocrine carcinoma , small cell / oat cell carcinoma , and large cell neuroendocrine carcinoma ( 10 ) . it should be noted , however , that not all breast tumors that exhibit neuroendocrine features completely fit into the who classification categories . there are no specific differences in the clinical features of neuroendocrine tumors from those of the other breast malignancy , and endocrine hormone - related syndromes are extremely rare . of interest is the increase in neuroendocrine markers , such as chromogranin a , in the blood ( 10 ) . as metastatic neuroendocrine tumors of the breast are more common than that of primary neuroendocrine tumors of the breast , it is , therefore , important to differentiate primary breast neuroendocrine tumor from metastatic disease to the breast because of the differences in treatment focus . primary neuroendocrine tumor of the breast can be diagnosed if the presence of a non - mammary primary site can be clinically ruled out or if an in situ component is histologically detected , or both . the treatment of primary neuroendocrine tumors is highly variable , although surgery should always be considered as the first line of treatment . recent reports , which only deal with small cell neuroendocrine carcinoma , indicate that the size , stage of disease at the time of diagnosis , expression of the er and pr , and the ki-67 index are important determinants of the prognosis ( 11 - 13 ) . there is no relevant report about the prognosis of primary large cell neuroendocrine carcinoma seen in our case . however , the prognosis of our case would be poor , considering tumor size , axillary metastasis , and histologic grade . previously published case reports with radiology findings ( 1 - 7 ) have also described the imaging features of neuroendocrine tumors . ( 1 ) described that the common radiological features of breast neuroendocrine tumor include , a high - density mass with predominantly spiculated or lobulated margins on mammography and mostly irregular or microlobulated , homogeneously hypoechoic masses with normal sound transmission on sonography . , the number of cases with radiology findings has been too small to allow generalization of the imaging features , and there is no case report with radiology and pathology findings similar to ours . in this report we present a case of primary neuroendocrine tumor ( large cell neuroendocrine carcinoma with atypical carcinoid features ) of the breast , together with its mammographic , sonographic , and mri features . however , the radiologic findings are hard to differentiate from those of much more commonly seen invasive ductal carcinoma . reports of new cases will be necessary in order to determine the radiologic presentation of primary neuroendocrine tumor of the breast .

focal neuroendocrine differentiation can be found in diverse histological types of breast tumors . however , the term , neuroendocrine breast tumor , indicates the diffuse expression of neuroendocrine markers in more than 50% of the tumor cell population . the imaging features of neuroendocrine breast tumor have not been accurately described due to extreme rarity of this tumor type . we present a case of a pathologically confirmed , primary neuroendocrine breast tumor in a 42-year - old woman , with imaging findings difficult to be differentiated from that of invasive ductal carcinoma .

we present the case of a patient who dissolved the tablets and then injected intravenously oxymorphone extended release tablets ( opana er ; endo pharmaceuticals inc . , malvern , pa , usa ) and developed unexplained thrombotic thrombocytopenic purpura ( ttp ) . the patient was a 30-year - old , single , caucasian man who presented for opioid dependence to a substance use disorders inpatient rehabilitation program . prior to admission , he was hospitalized for ttp - like illness after injecting opana . the patient reported that his opiate dependence evolved over time , finding that injecting opana became his only method of use , injecting a 20 mg tablet of opana every 8 hours . for both admissions for ttp , the patient presented complaining of feeling sick , tired , confused , and had difficulty breathing . upon admission , he was found to have a mild fever and generalized purpura without mucosal involvement . his comprehensive metabolic panel was within normal limits except for a platelet count of 30,000 per l . a chest x - ray was unremarkable , and electrocardiogram ( ekg ) sinus tachycardia was 120 bpm . for both admissions , his treatment was uneventful and consisted of 3 days of glucocorticoids ( prednisone ) . his platelets continued to trend upward throughout his rehabilitation admission , but he left prior to completing the program . opana is one of the trade names of oxymorphone , a semi - synthetic opioid used to treat moderate to severe chronic pain.1 ttp is a rare blood coagulation disorder that causes microscopic clots to form in small blood vessels throughout the body that is typically associated with an infectious or inflammatory process , yet it is only seen in approximately one in 100,000 people.1 a case controlled study found 15 cases of ttp - like illness associated with intravenous opana er abuse in tennessee.2 our case is the only example , to our knowledge , where ttp reemerged following a re - challenge with iv oral opana . using iv oral opana is a growing concern . on a google search , there were 119,000 results for opana er iv , including tutorials on how to prepare opana tablets as an injectable , suggesting that its misuse is significant . this case report and others suggest that health care professionals should be aware of the possibility of developing ttp in those who misuse opana .

we present the case of a patient who developed thrombotic thrombocytopenic purpura ( ttp ) following intravenous injection of opana er . ttp reemerged after three months of abstinence with opana misuse . this case report brings awareness to the possibility of developing ttp in those who misuse opana , which is a growing concern .

posterior reversible encephalopathy syndrome ( pres ) , first described by hinchey et al . in 1996 , is a clinical condition presenting with neurological symptoms including headache , seizures , altered sensorium , and loss of vision , and accompanied by characteristic magnetic resonance imaging ( mri ) findings which are potentially reversible . over the years , this condition has been described by various names including reversible posterior leukoencephalopathy , reversible posterior cerebral oedema , reversible occipitoparietal encephalopathy , and hypertensive encephalopathy . the disease has been more commonly described in adult population , especially in the setting of eclampsia and organ transplantation . postulated underlying causes include sudden rise in blood pressure , immunosuppression , chemotherapeutic agents for lymphoma and leukemia , severe hypercalcemia , thrombocytopenic syndromes , henoch schnlein purpura , vasculitis , and renal failure , of which sudden rise in blood pressure and renal failure appear to be the most common . at neuroimaging , vasogenic oedema is seen involving the white matter of bilateral cerebral hemispheres posteriorly , most commonly parietal and occipital lobes . the prevalence of pres among children is not well established ; however , there have been reports of pres in children following chemotherapy and tumor lysis syndrome , as well as with hypertension . an 11-year - old boy , a known case of chronic kidney disease ( stage v ) on maintenance hemodialysis , presented with one episode of generalized tonic there was history of high - grade fever ( upto 103f ) for 34 days . on examination , he was found to be drowsy ; however , he was moving all four limbs . laboratory tests revealed a serum creatinine of 4.8 mg% and blood urea of 121 mg% . serum calcium , sodium , and potassium were 7.8 mg% , 135 meq / l , and 5.3 meq / l , respectively . mri brain at admission showed t2w / t2 flair hyperintensities , s / o oedema , involving the subcortical white matter of bilateral frontal , parietal , and occipital lobes , with few areas of cortical involvement [ figure 1 ] . similar lesions were also noted involving bilateral basal ganglia , left thalamus , splenium of corpus callosum , as well as brainstem [ figure 2 ] . diffusion - weighted imaging ( dwi ) showed increased signal intensity in the lesions in bilateral occipital lobes and in the splenium of corpus callosum . mri axial t2 flair section showing symmetrical hyperintense lesions involving subcortical and cortical locations of the bilateral frontal and parietal lobes mri axial t2 flair section showing similar lesions involving the basal ganglia and the splenium of corpus callosum the patient was started on antiepileptics , antihypertensives , and antibiotics , with daily hemodialysis . he had four more episodes of seizures during his hospital stay ; however , he slowly improved clinically and was discharged on antiepileptics and antihypertensives on biweekly hemodialysis . follow - up mri brain was done after 2 weeks , which showed near - total resolution of the brain lesions [ figure 3 ] . repeat mri axial t2 flair sections after 2 weeks show almost complete resolution of the lesions in the brain although significant elevation of the blood pressure may not always be demonstrated , pres is considered to be a variant of hypertensive encephalopathy with sudden rise in blood pressure being the most commonly associated feature although other causative factors have also been implicated . two theories are considered in the pathophysiology of pres , the first being sudden increase in blood pressure causing vasospasm and the other being failure of autoregulatory mechanism . with sudden elevation in systolic blood pressure , the autoregulatory capacity of brain vasculature is exceeded which results in a region of vasodilatation and vasoconstriction , especially in the arterial boundary zone . the preferential involvement of the posterior circulation has been postulated as being due to the sympathetic innervation protecting the brain from sudden increase in blood pressure being relatively less in the arterioles supplied by the vertebrobasilar system than in the anterior circulation . in the setting of end - stage renal disease ( esrd ) , as in our patient , both the rise in pressure beyond the ability of autoregulation of cerebral flow as well as the uremia by itself the first was the holohemispheric watershed pattern with a linear involvement of the frontal , parietal , and occipital lobes predominantly , along a watershed distribution . the second was the superior frontal sulcus pattern with predominant involvement of the frontal lobes , and the third was the dominant parietal other than these patterns , asymmetrical and/or partial manifestations of the primary patterns were also described . in our patient , the pattern seen could be best described as holohemispheric watershed pattern . atypical sites of involvement that have been described include brain stem , cerebellum , basal ganglia , thalami , internal capsule , and splenium of corpus callosum , with uremic encephalopathy sometimes described as having a propensity for central distribution . in our patient , lesions were noted in the brainstem , bilateral basal ganglia , left thalamus , and the splenium of corpus callosum . dwi and apparent diffusion coefficient ( adc ) have been found to be helpful in differentiating atypical presentations of pres from conditions like central pontine / extrapontine myelinolysis , non - hemorrhagic infarcts , and hypoglycemic or hypoxic encephalopathy . due to vasogenic oedema in pres , adc shows increased values with slightly increased signal intensity on dwi , whereas the other conditions show reduced adc values due to cytotoxic edema . our patient demonstrated increased intensity on dwi with increased adc values in the splenium and occipital lobes . the symptoms and lesions of pres may resolve completely if the diagnosis and treatment is prompt , as was seen in our patient ; however , failure to diagnose may lead to irreversible infarction and death . recurrence of pres is rare and may be associated with infections and rapid rise in blood pressure . the diagnosis may be overlooked , especially in children , unless a high index of suspicion and precise clinical history is maintained . we maintain that pres should be kept as a possibility in children presenting with encephalopathy and seizures in the setting of raised blood pressure or renal disease as delay in diagnosis and treatment may result in permanent neurological deficit .

posterior reversible encephalopathy syndrome presents with neurological and imaging features that are reversible , if prompt diagnosis and treatment is undertaken . however , the disease has been more commonly described in adult population , especially in eclampsia . in the background of predisposing factors like renal disease or chemotherapy , the pediatric population is also at equal risk for this condition , as we would like to present through this case and also demonstrate the potential for complete reversal of symptoms and imaging findings if diagnosed without delay .

in the previous issue of critical care , takala and colleagues published a multicenter randomized controlled trial ( rct ) investigating the use of less invasive cardiac output monitoring in hemodynamically unstable patients admitted to the intensive care unit ( icu ) . in this study , patients were randomly assigned to be treated with ( mico group ) or without ( control group ) a minimally invasive cardiac output monitor based on pulse pressure analysis ( flotrac vigileo , version 1.07 ; edwards lifesciences , irvine , ca , usa ) . the hypothesis of the study was that if clinicians were given extra information on cardiac output they would be able to manage hemodynamic instability in a more effective way . the primary outcome of the investigation was the achievement of hemodynamic stability within 6 hours of entering the study . icu mortality and hospital mortality were considered secondary outcomes . there were no differences in any of these outcomes between the control and protocol groups . the conclusion from these results is that early monitoring of cardiac output in patients admitted with hemodynamic instability to the icu is not associated with an improved outcome . the literature on the use of hemodynamic monitoring in improving the outcome of either septic or surgical patients provides two constant features : the use of treatment protocols with specific hemodynamic targets and very early application of these protocols . the use of such hemodynamic monitoring is often referred to as early goal - directed therapy ( egdt ) . in this study the authors acknowledge that this is one of the limitations of the study and that it may explain the lack of a positive result . interestingly , this lack of outcome benefit when hemodynamic monitoring is used without a protocol is consistent with data in the literature . rcts in which pulmonary artery catheters ( pacs ) have been used without a protocol have not shown any outcome benefit . in contrast , when the pac has been coupled with an egdt approach , there have been improved outcomes [ 4 - 8 ] . importantly , the targets and the intervention in the protocol need to be very specific while the use of guidelines is not associated with an improved outcome . however , there are rcts in which the use of pacs coupled with treatment protocols has not shown any outcome benefit . in these studies , the protocols were not applied ' early ' and patients were enrolled even if organ failure had developed . it is clear , therefore , that timing of entry into a protocol is fundamental to its success or failure . the present study was started promptly : in the mico group , arterial pressure monitoring was achieved 20 minutes after admission to the icu and entry into the study occurred less than 1 hour later . it is probably significant that the median time from hospital to icu admission is 13 hours and has a very substantial range . it may be , therefore , that the patients were already too sick to benefit from any hemodynamic intervention . in septic patients , goal - directed therapy started in the emergency department has demonstrated great benefits in survival whereas it makes no difference in survival if applied late . this monitor was used successfully in two recently published trials : one by benes and colleagues with vigileo version 1.10 and one by cecconi and colleagues with version 1.07 . it is important to note that , in these two studies , the monitor was used in a different population ( elective surgical patients ) . it may be that the monitor provides more accurate information in relatively stable , elective surgery patients , in contrast to the heterogeneous hemodynamically unstable population of patients in the present study , in which the data provided by the monitor may be less accurate . we concur with the authors that the main limitation of this study is the lack of a protocol in the mico group . no study in the literature demonstrates a benefit from the use of hemodynamic monitoring in the absence of a protocol , a finding that this study seems to confirm . the authors must be congratulated for studying the use of less invasive hemodynamic monitoring in such a challenging population . the role of egdt in hemodynamically unstable patients in the icu is , therefore , still unproven . a study that proves this role would be a major contribution to the management of critically ill , unstable patients . egdt : early goal - directed therapy ; icu : intensive care unit ; mico : minimally invasive cardiac output monitor ; pac : pulmonary artery catheter ; rct : randomized controlled trial . mc has received honoraria , travel expenses , or research support from lidco ltd ( cambridge , uk ) , edwards lifesciences llc ( irvine , ca , usa ) , cheetah medical , inc . ( vancouver , wa , usa ) , masimo corporation ( irvine , ca , usa ) , and bmeye ( amsterdam , the netherlands ) . db has received honoraria and travel expenses from lidco ltd and deltex medical ( chichester , uk ) .

in the previous issue of critical care , takala and colleagues presented the results of a multicenter study to investigate whether the early presence of less invasive hemodynamic monitoring improves outcome in patients admitted with hemodynamic instability to the intensive care unit . the authors ' results suggest that it makes no difference . we discuss these findings and compare them to the literature on early goal - directed therapy in which monitors are used early but with a protocol .

the campus health service at the university of arizona is located on campus in pima county . the average yearly enrollment during 19982006 of 36,000 was used for calculating case rates for all students ( 5 ) . campus health medical charts for january 1 , 1998 , through december 31 , 2006 , were reviewed for serologic testing for coccidioidomycosis . charts were also reviewed for international classification of diseases , 9th edition ( icd-9 ) , codes for coccidioidomycosis ( 114.0114.5 , 114.9 ) to identify students in whom the diagnosis was made by testing elsewhere . most serologic testing , identified by its current procedural terminology ( cpt ) code , was performed by the campus health laboratory , which used premier coccidioides enzyme immunoassay ( meridian diagnostics , cincinnati oh , usa ) . occasionally , 10% of the time , serum samples were tested by a single commercial laboratory , which used immunodiffusion testing for precipitin - type or complement fixing type antibodies ( 6 ) . students tested multiple times for coccidioidomycosis were counted once , either in the first year for which results were positive or in the year first tested if all results were negative . individual charts were reviewed for those students who did not have coccidioidal testing done at campus health and were included only if the diagnosis could be corroborated by outside laboratory documentation . a case of coccidioidomycosis was defined as one in which the diagnosis could be corroborated by positive coccidioidal serologic results , fungal culture , or histologic identification of spherules in biopsy specimens . in addition , charts of all athletes who had coccidioidal infection were reviewed for demographic information , type of sport ( indoors vs. outdoors ) , and extent of disease . all data were analyzed by using spss 11.0 for windows ( spss inc , chicago , il , usa ) . significance tests used the fisher exact . this study was approved by the university of arizona institutional review board and by the campus health service departmental review committee . of 2,754 student medical charts selected for review based on routine serologic testing for coccidioidomycosis or an icd-9 code for coccidioidomycosis , 305 students were found to have coccidioidomycosis . of these 305 , a total of 297 had positive coccidioidomycosis results by serologic testing done at campus health and 8 were identified with diagnostic results from other laboratories . annual rates for college students are 34 higher than available county and state rates for various age ranges ( table ) . because pima county statistics includes cases reported to the state by campus health , actual differences between student rates and nonstudent pima county rates for persons of similar ages would be even greater than represented here . * the university of arizona campus is located in pima county . the composite case rate , or annual incidence of coccidioidomycosis , was 374/100,000 ( 95% confidence interval [ ci ] 192639 ) for scholarship athletes compared with 90/100,000 ( 95% ci 79103 ) for other students ( p<0.00001 ) . in parallel , a much greater proportion ( 4.6% , 95% ci 3.95.4 ) of scholarship athletes were tested for coccidioidal antibodies than were other students ( 0.8% , 95% ci 0.770.84% ) ( p<0.000001 ) . infection for all but 1 athlete with disseminated infection was limited to the chest and/or skin , which resolved either untreated or after several months of antifungal therapy . the age distribution of scholarship athletes was the same as for other students , and only 1 of 16 athletes was from arizona . female athletes with coccidioidal infections comprised 56% of the total compared with 41% of all uninfected athletes ( p = 0.31 ) . for 81% of infected athletes , sports competition took place outdoors , whereas 88% of uninfected athletes competed outdoors ( p = 0.43 ) . a recent study in arizona found that < 13% of patients with community acquired pneumonia were tested for coccidioidomycosis ( 7 ) . in contrast , campus health service at the university of arizona tested 50% of students with community acquired pneumonia for coccidioidomycosis ( 8,9 ) . similarly , the case rate of 374/100,000 for scholarship athletes , > 4 higher than that for other students , is associated with a > 5-fold increase in coccidioidal testing . therefore , it would seem that most , if not all , of the increased case - rate for athletes can be accounted for by increased testing ; little , if any , increased susceptibility can be attributed to increased exercise or athletic training . we speculate that etiologic diagnoses for any illness that reduced physical performance were sought more frequently for scholarship athletes than for other students . however , we can not entirely exclude the possibility that the higher rate of testing was to some extent a consequence of higher rates of pneumonia in scholarship athletes . in any instance , it would appear that more complete testing for community acquired pneumonia associated with valley fever does result in considerably higher estimates of case rates for this fungal infection . in the past , skin test conversion rates have suggested an annual infection rate of 3,000/100,000 ( 10 ) . a 2007 telephone survey conducted by the arizona department of health services of persons reported with new diagnoses of coccidioidomycosis found that the median length of residence in an endemic area was 17 years ( 11 ) , further suggesting an average annual infection rate of 3% . in this context , case rates among scholarship athletes were actually underrepresented in outdoor sports , and the spectrum of disease severity was in line with that found in past studies of the student population as a whole ( 8) . many scholarship athletes come from regions where coccidioidomycosis is not endemic . because fewer of these students had prior coccidioidal infection , the annual risk for the group as a whole would be slightly increased as shown in a previous report from the same college health group ( 9 ) . arizona state statistics show a marked increase with age in case rates , e.g. , 163 cases/100,000 for persons > 65 years of age . our findings do not discount the possibility that immune responses of elderly patients differs from those of younger patients ; disease in the elderly is more severe ( 12 ) . however , our study suggests that at least an equal proportion of young adults have clinical illness from coccidioidal infection sufficient to seek medical evaluation . our findings address infection among college students , particularly scholarship athletes , and support a recommendation that student health clinicians and team physicians consider coccidioidomycosis as a possible etiology of illness in students who live in or have traveled recently to disease - endemic regions . more generally , our findings reinforce practice guidelines revised by the infectious diseases society of america for management of community - acquired pneumonia to assist clinicians in evaluating patients with endemic exposure ( 13 ) .

to compare coccidioidomycosis case rates among groups of young adults in a disease - endemic region , we reviewed medical charts for serologic testing and coding . case rates were higher for scholarship athletes than for other students and paralleled 5 more serologic testing . our findings underscore the need to routinely test patients for coccidioidomycosis .

a 41-year - old man presented with terminal gross hematuria that was noted one month earlier . his past medical history was unremarkable except that he had suffered with prostatitis . the serum markers for prostate cancer , prostate - specific antigen ( psa ) and prostatic acid phosphatase ( pap ) were normal . transrectal ultrasonography showed a well - demarcated , pear - shaped cystic lesion that included an echogenic papillary solid mass in his left seminal vesicle ( fig . contrast - enhanced pelvic ct showed that the papillary solid mass originated from the wall of the left seminal vesicle cyst and it was mildly enhanced ( fig . 1b ) . a dilated ectopic ureter opening into the dilated left seminal vesicle was also seen . contrast enhanced abdominal ct at the level of the l4 vertebra demonstrated a small abnormal soft tissue density in the aorta 's left lateral aspect , suggesting a dysgenetic or atrophic kidney ( fig . the axial t1-weighted ( tr / te : 540/12 ms ) mr image showed high signal - intensity fluid in the seminal vesicle cyst ( fig . the coronal t2-weighted ( tr / te : 5500/136 ms ) mr image demonstrated an approximately 7.8 6 5.2 cm papillary mass in the left seminal vesicle cyst ( fig . the sagittal t2-weighted image showed a markedly dilated ectopic ureter draining into the cyst ( fig . the cystoscopic findings showed bulging of the left hemitrigone on the left side of the bladder . radical excision of the left agenetic kidney , left ureter and seminal vesicle cyst and a partial cystectomy were done . photomicrography showed a papillary glandular configuration covered with carcinoma cells and mucinous materials in the cystic space without any muscular invasion ( fig . the histopathologic diagnosis was a well - differentiated primary mucinous adenocarcinoma of the left seminal vesicle cyst ( fig . the specimen we labeled " agenetic kidney " showed only a vestigial remnant of the ureteric buds . five years later , the follow - up ct and whole body bone scanning revealed no evidence of tumor recurrence or distant metastasis . primary tumors of the seminal vesicles , such as adenocarcinoma , sarcoma or lymphoma , are rare findings , and secondary tumors are more common . most often , the patients present late in their disease course with nonspecific symptoms ( 2 ) , so a late diagnosis of primary adenocarcinoma in the seminal vesicle is rather typical . our patient had a previous history of prostatitis seven years ago . elevated serum levels of psa and pap helped us to identify the prostate as the site of primary malignancy . on the other hand , the serum psa and pap levels are normal in patients with primary seminal vesicle adenocarcinoma , but the serum cea may be elevated ( 3 ) . in our patient , all of these serum markers were normal . the mesonephric duct extends caudally to the cloaca and it gives raise to the ureteric bud . if the ureteric bud originates more cranially than normal in the mesonephric duct , then the ureter may insert into seminal vesicle , the posterior urethra , the ejaculatory duct and the vas deferens ( 2 ) . ectopia in these areas may be associated with reflux or obstruction , and this is the suggested cause of renal functional impairment or absence ( 4 ) . so , seminal vesicle cysts are commonly associated with renal agenesis or dysgenesis on the ipsilateral side ( 6 ) . incomplete development between wolffian 's duct and the urogenital sinus in males results in an accumulation of secretions and the subsequent formation of seminal vesicle cysts during puberty ( 7 ) . we suggest that the cause of large seminal vesicle cysts is not only the ectopic ureter opening into the cyst , but also a mucin producing tumor . secondary involvement of the seminal vesicle is more common than primary seminal vesicle cancer for carcinoma in situ of the bladder , adenocarcinoma of the prostate , lymphoma or rectal carcinoma . the diagnosis of primary adenocarcinoma within the seminal vesicle is difficult to arrive at with using just the image findings . but in our patient , the intraluminal protruding papillary mass in the seminal vesicle cyst was easily identified on imaging study . we suggest that primary seminal vesicle cancer should be considered in the differential diagnosis of papillary mass in the cyst . okada et al . have reported a case of papillary adenocarcinoma in a seminal vesicle cyst associated with ipsilateral renal agenesis ; however , an ectopic ureter opening was not described ( 8) . a few cases of ectopic ureter associated with cancer have been previously reported ( 8 , 9 ) . most patients with seminal vesicle cysts combined with ipsilateral renal agenesis or dysgenesis are asymptomatic until the condition 's second or third decade ( 1 , 2 ) . our patient was younger than the patients in the previously reported cases who had primary seminal vesicle adenocarcinoma discovered , and he was older than the patients who had seminal vesicle cysts combined with ipsilateral renal agenesis or dysgenesis . so , we suggest the possibility that the development of seminal vesicle adenocarcinoma is due to chronic stimulation of the ectopic ureter 's secretion . in this clinical setting with a papillary mass of a seminal vesicle cyst with an ectopic ureter , primary mucinous adenocarcinoma arising from the seminal vesicle cyst should be considered as part of the differential diagnosis , even though the patient was young . in conclusion , we report here on a case of primary mucinous adenocarcinoma arising from a seminal vesicle cyst that was associated with an ectopic ureter opening and ipsilateral renal agenesis , which is a very rare condition indeed . the lesion was depicted on transrectal ultrasonography , contrast enhanced ct and mri as a papillary solid mass originating from the wall of the left seminal vesicle cyst .

primary adenocarcinoma of the seminal vesicles is a rare neoplasm . congenital seminal vesicle cysts are commonly associated with unilateral renal agenesis or dysgenesis . to the best of our knowledge , mucinous adenocarcinoma of the seminal vesicle cyst that 's associated with an ectopic ureter opening into the seminal vesicle and ipsilateral renal agenesis has not been described in the radiological literature . we report here on the radiological findings of a primary adenocarcinoma of a seminal vesicle cyst in this condition .

filariasis is a unusual parasitic disease in romania , last known report ( a case of lymphatic filariasis ) dating from 2005 , and in the entire world is rare too , excluding western africa [ 1 ] . therefore , suspicion of a such disease , especially in the emergency department is extraordinary and may seem to require a very specialized assessment , including the specific circulating antigens determination [ 2 ] and extended performance imaging examination . we present a male patient case ( 32 years old ) admitted in ed - county hospital craiova ( s- w romania region ) with migratory burning pain , initially in the right cervical area , then on the left frontal and upper orbital area , temporal , and the left upper eyelid , irritation , pruritus and swelling . apparently , the onset of symptoms was a few day , but six months ago he accused sudden , transient , both knee swelling , local pain , functional stiffness , with spontaneously remission after three days . the patient was classified as a green emergency triage priority , being assigned on the consultation area . at the starting point , the first diagnostic suspicion was represented by trigeminal neuritis , being also consider a conjunctivitis . the local examination provided by the emergency physician identified an inflammatory trace of 10 cm . it can feel an elastic and renitent cord with spontaneous and induced by palpation mobility . at that moment the eye examination was normal , and seeing was not affected . by surgical excision on the left upper eyelid a microfilaraemic parasite of 17 cm . length has been extracted ( fig . 1 ) and that seemed to be filaria . biological material was sent to the parasitological laboratory for specific identification of type , suspicion beings loa considering the dimensions of the parasite , the estimated age of the infection has been approximate at 1 year . the patient was subsequently taken over by the department of infectious diseases , for further investigation and therapy ( doxicicline , nonsteroidal anti - inflammatory therapy and albendazol ) no other subcutaneous determination or any clinical element identified as lymphatic damages has found , given that this type of damage is common in filariasis . later , the patient reported a trip to antalya ( turkey ) 1 year ago , where he was stung by mosquitoes on the back , neck and arms , but the area is not know as a specific one for this type of parasite . no others members of the family or touristic group has reported symptoms . in this situation , a detailed investigation detected that at the approximately moment of the contact , the patient worked as a member in a building team , developing activities at colentina hospital ( bucharest ) in a specific parasitological compartment for tropical diseases . it is very possible that the contamination occurred in this context , that involves a lots of aspects regarding work security and environment protection in this circumstances . the peculiarities of the case was the simplicity of the diagnosis due to the parasite mobility finding ( the key element ) for a disease that is basically found only in tropical areas , and the uncertain moment and specific place of the contamination which is not determined yet . in the same time we suspected that the first inflammatory reaction ( the knee oedema ) has been in context , though any node or joint inflammation were not observed after , and inflammatory tests remains normal . however , the complexity of the subject is related to the possibility that this localization is not the only one ( even if multiple affectations are uncommon but it remain possible to identify microfilaria into the soft structures of the knee ) , and the investigation and medical surveillance has to be systematic and long term extended ( minimum 2 years has been recommended by the parasitology specialist because of the further medical and epidemiological implications , and the medical history of the patient ) . a knee mri , specific blood tests and systemic inflammatory reaction study are in progress at this moment , and all the workers involved in operations at the colentina hospital are evaluated too . subcutaneous filariasis is a variant expression of infestation spinning relatively easier than lymphatic form revealed , especially if specified epidemiologic element . parasite mobility finding was the key element of an extremely quick and easy diagnosis for a disease that is basically found only in tropical areas . the peculiarity of the case is the simplicity of the emergency diagnosis and the complexity of the further medical and epidemiological implications .

a 32 year old male patient presented to the ed craiova with migratory , burning pain , to the right cervical and left upper eyelid . sudden , transient , bilateral knee swelling six months ago . an inflammatory trace and a renitent cord can feel at this level , with spontaneous mobility . a microfilaraemic parasite was surgically extracted from upper eyelid . mobility of the parasite was the key element of an extremely quick and easy diagnosis for a very rare disease but the specific circumstances of the contamination remains unclear . even uncommon , multiple determinations are possible requiring systematic and long term investigation and medical surveillance .

dermatofibrosarcoma protuberans ( dfsp ) is a rare skin sarcoma known for its indolent and low metastatic potential . recently , dfsp has been shown to be characterized by a reciprocal translocation , t(17;22)(q22;q13 ) , and a supernumerary ring chromosome derived from the translocation r(17;22 ) . the col1a1 gene is located at 17q2122 and encodes the a1 ( l ) chain of type i collagen . the pdgfb gene is located at 22q13 and encodes the b chain of the platelet - derived growth factor ( pdgf ) ligand . the fusion causes a deregulation of the pdgfb gene by deleting its exon 1 and placing it under the direct control of the col1a1 gene . this rearrangement leads to an unregulated production of the growth factor , which seems to play an important role in the development of dfsp . epidemiological studies revealed that more than 95% of dfsp present anomalies on the 17q22 and 22q13 chromosomal regions , leading to the fusion of the col1a1 and pdgfb genes [ 4 , 5 ] . in approximately 5% of cases , no col1a1-pdgfb fusion was found , suggesting that other genes might be involved in the dfsp pathogenesis . cushing 's syndrome is characterized by a chronic and excessive exposure to endogenous glucocorticoids ( gcs ) . chronic gc excess has detrimental effects on the skin , leading to atrophy and impaired wound healing . several in vitro studies have shown that gcs induce alterations in extracellular matrix homeostasis , such as collagen synthesis and the expression of matrix metalloproteinases , leading to a reduction in the collagen mass [ 8 , 9 , 10 ] . in other words , increased doses of gcs induce a catabolic phenotype in skin fibroblasts in vivo and in vitro . in this paper , we describe a patient with a rapid onset of dfsp associated with bilateral adrenalectomy for cushing 's syndrome . to our knowledge , there is no english report of dfsp associated with bilateral adrenalectomy for cushing 's syndrome . a 50-year - old japanese woman visited our outpatient clinic with a 20-year history of an asymptomatic , about 3 mm in diameter , subcutaneous nodule on her breast . one year before , this nodule rapidly enlarged after she underwent bilateral adrenalectomy for cushing 's syndrome . on her initial visit , physical examination revealed a brown - colored , elastic hard , well - demarcated nodule on the left side of her breast ( fig . an excisional biopsy showed densely packed , monomorphic , plump , spindle cells arranged in a storiform pattern in the central area of the tumor nodule and spindle cells infiltrating into the subcutis of the peripheral lesion ( fig . the tumor cells were positive for cd34 and negative for factor xiiia ( fig . 2c , d ) , sma , and s100 ( data not shown ) . based on the above - mentioned findings , we diagnosed this patient as having dfsp . interestingly , the serum levels of cortisol before and after bilateral adrenalectomy were 17.1 and 0.8 mg / dl , respectively . we excised the tumor with a 3-cm surgical margin of mammary gland tissue . a half year after surgical treatment this case is unique in suggesting an association between dfsp and cushing 's syndrome , and , to the best of our knowledge , this is the first case report of dfsp coexisting with cushing 's syndrome . gcs bind to receptors in the cytoplasm to form complexes that translocate into the nucleus to regulate downstream targets by binding to glucocorticoid response elements . previously , mahonen et al . reported that dexamethasone treatment decreased the transcription of the col1a1 gene . schoepe et al . reported that , in a skin model , the inhibition of col1a1 mrna expression correlated with the dose and type of gcs ( e.g. hydrocortisone , prednicarbate , mometasone-17-furoate , and clobetasol-17-propionate ) . thus , the increase of cortisol in cushing 's syndrome may suppress the transcription of the col1a1/pdgfb fusion gene in dfsp . in our case , the tumor mass dramatically enlarged 1 month after bilateral adrenalectomy for cushing 's syndrome , which was coincident with the sudden reduction of serum cortisol ( from 17.1 to 0.8 mg / dl ) . assessed the treatment of 25 cases of nonresectable dfsp patients with imatinib mesylate . in their report , although imatinib mesylate targets pdgf- and can be effective for dfsp , a clinical response was achieved in only 36% of cases . our present report sheds light on the suppressive effect of gcs on the development of dfsp , which could lead to therapeutic use of gcs , although in vitro studies regarding the effect of gcs on the growth of cultured dfsp are a prerequisite .

we describe a 50-year - old japanese patient with dermatofibrosarcoma protuberans ( dfsp ) rapidly growing after bilateral adrenalectomy for cushing 's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg / dl . it is known that glucocorticoids decrease the transcriptions of the col1a1 gene and the pdgfb gene , which is under the direct control of the col1a1 gene in most dfsp . therefore , the hypersecretion of glucocorticoids in cushing 's syndrome might suppress the development of dfsp . to the best of our knowledge , this is the first case of rapid growth of dfsp that may be associated with bilateral adrenalectomy for cushing 's syndrome .

we selected eight genes known to be involved in the ras pathway , namely cdk5 , hras , mapk1 , mapk3 , map2k1 , map2k2 , ngfr and raf1 . core genes due to their relevance in the ras pathway and their consequential importance in colorectal cancer . the knock - down experiments were performed on the eight core genes using short hairpin rna ( shrna ) in two colorectal cancer cell lines sw480 and sw620 . for each knock - down there are six replicates ( except cdk5 in sw620 with five replicates ) with three different types of controls ( empty vector , nontarget and non transduced ) , totaling 125 samples . we used the affymetrix genechip hg - u133plus2 platform to profile the gene expression of each sample . we used the simpleaffy bioconductor package to check the quality of each individual cel file . 1 shows that a majority of files contains a sufficiently large percentage of present calls ( > 40% , except for biological replicates one and two and hras biological replicate three with 39.58% ) and all scale factors lie within a 3-fold range which complies with the good quality guidelines from affymetrix . in more detail , we can observe that those cel files that were generated in the early stages of the data generation are of lower quality than the rest of the files , namely the biological replicates one and two ( fig . 2 and table 1 ) . the raw and normalized data are available from ncbi gene expression omnibus repository with accession number gse53091 . a successful knock - down experiment should result in significantly lower expression compared to the unperturbed samples . here , we assess the quality of a knock - down experiments by testing the difference between matched knock - down samples versus nontarget control samples using a wilcoxon signed rank test . in fig . 3 , we show the difference between knock - down and control sample expression for each of the eight knock - downs for both cell - lines together . in each plot , the knocked - down gene is highlighted in blue and the obtained p - values are represented by symbols in the bottom of each plot . the significance levels are represented as follows : * * * for p < 0.001 , * * for p < 0.01 , * for p < 0.05 and - for p < 0.1 . from the eight plots in fig . 3 , we can observe that the difference in expression between knock - down and control samples is significant for all of the eight knock - downs ( the differential expression of the blue boxes is significantly lower than zero ) . in our study , we determined the set of significantly affected genes for each of the eight knock - downs . for example , the knock - down of raf1 significantly changes the expression of map2k2 and ngfr with p - values < 0.001 ( only considering the eight core genes ) . we then used the set of significantly affected genes to quantitatively validate inferred gene regulatory interactions . we selected eight genes known to be involved in the ras pathway , namely cdk5 , hras , mapk1 , mapk3 , map2k1 , map2k2 , ngfr and raf1 . core genes due to their relevance in the ras pathway and their consequential importance in colorectal cancer . the knock - down experiments were performed on the eight core genes using short hairpin rna ( shrna ) in two colorectal cancer cell lines sw480 and sw620 . for each knock - down there are six replicates ( except cdk5 in sw620 with five replicates ) with three different types of controls ( empty vector , nontarget and non transduced ) , totaling 125 samples . we used the affymetrix genechip hg - u133plus2 platform to profile the gene expression of each sample . we used the simpleaffy bioconductor package to check the quality of each individual cel file . 1 shows that a majority of files contains a sufficiently large percentage of present calls ( > 40% , except for biological replicates one and two and hras biological replicate three with 39.58% ) and all scale factors lie within a 3-fold range which complies with the good quality guidelines from affymetrix . in more detail , we can observe that those cel files that were generated in the early stages of the data generation are of lower quality than the rest of the files , namely the biological replicates one and two ( fig . 2 and table 1 ) . the raw and normalized data are available from ncbi gene expression omnibus repository with accession number gse53091 . a successful knock - down experiment should result in significantly lower expression compared to the unperturbed samples . here , we assess the quality of a knock - down experiments by testing the difference between matched knock - down samples versus nontarget control samples using a wilcoxon signed rank test . in fig . 3 , we show the difference between knock - down and control sample expression for each of the eight knock - downs for both cell - lines together . in each plot , the knocked - down gene is highlighted in blue and the obtained p - values are represented by symbols in the bottom of each plot . the significance levels are represented as follows : * * * for p < 0.001 , * * for p < 0.01 , * for p < 0.05 and - for p < 0.1 . from the eight plots in fig . 3 , we can observe that the difference in expression between knock - down and control samples is significant for all of the eight knock - downs ( the differential expression of the blue boxes is significantly lower than zero ) . in our study , we determined the set of significantly affected genes for each of the eight knock - downs . for example , the knock - down of raf1 significantly changes the expression of map2k2 and ngfr with p - values < 0.001 ( only considering the eight core genes ) . we then used the set of significantly affected genes to quantitatively validate inferred gene regulatory interactions . in this article we described a unique data set containing ras pathway - related gene knock - down experiments in two different colon cancer cell - lines . it contains the expression values from the knock - down of eight genes as well as three different controls in six biological replicates . the genome - wide gene expression was measured using the human genome u133 plus 2.0 array . this data was recently used in a published study on the validation of regulatory gene networks . r code enabling to programmatically access the data and to fully reproduce the results presented in the paper . r code enabling to programmatically access the data and to fully reproduce the results presented in the paper .

quantitative validation of gene regulatory networks ( grns ) inferred from observational expression data is a difficult task usually involving time intensive and costly laboratory experiments . we were able to show that gene knock - down experiments can be used to quantitatively assess the quality of large - scale grns via a purely data - driven approach ( olsen et al . 2014 ) . our new validation framework also enables the statistical comparison of multiple network inference techniques , which was a long - standing challenge in the field.in this data in brief we detail the contents and quality controls for the gene expression data ( available from ncbi gene expression omnibus repository with accession number gse53091 ) associated with our study published in genomics ( olsen et al . 2014 ) . we also provide r code to access the data and reproduce the analysis presented in this article .

a 44-year - old nonsmoking male presented with a tender neck mass of several weeks duration . ct neck revealed two enlarged right cervical lymph nodes . a fine needle aspiration of the neck mass was felt to be most consistent with squamous cell carcinoma . pet / ct revealed three fdg - avid lymph nodes in the right neck and no evidence of distant metastases . he underwent a right selective neck dissection with bilateral tonsillectomies and directed biopsies of the nasopharynx , base of the tongue , and pyriform sinuses . pathology revealed no evidence of a primary tumor , and eight of 10 right cervical lymph nodes positive for poorly differentiated carcinoma most consistent with squamous cell carcinoma . the tumor was strongly positive for p16 on ihc staining , supporting the diagnosis of a squamous cell carcinoma of the head and neck region with unknown primary site . post treatment pet / ct revealed recurrence in a single cervical lymph node and new hepatic metastases . biopsy of the hepatic metastasis revealed mucoepidermoid carcinoma similar in morphology to the prior biopsy specimens . pcr testing was performed on both the original lymph node specimen and the liver metastasis ; both were negative for hpv . p16 , a cyclin - dependent kinase inhibitor , activates the cyclin d1 ckd4 and 6 complex , which prevents phosphorylation of the retinoblastoma protein ( prb ) and leads to cell cycle arrest 1,2 . hpv infection can lead to overexpression of p16 by virally - expressed oncoprotein e7 binding to and inactivating prb , thus leading to overexpression of p16 1,2 . in this case , the expression of p16 in the setting of a metastatic carcinoma to cervical lymph nodes with squamous differentiation was taken as supporting evidence of an unknown primary squamous cell carcinoma of the head and neck region . the subsequent biopsy revealed mucoepidermoid salivary gland carcinoma , which has been shown to overexpress p16 in the absence of hpv infection 412 . thus , it is important to recognize that although ihc staining for p16 is a useful surrogate for hpv infection , discordance both due to testing inaccuracy as well as true p16 positivity without hpv infection exists and should be considered when evaluating a carcinoma originating in the head and neck region 1,415,1720 .

key clinical messagethe prognostic utility of hpv in oropharyngeal squamous cell carcinoma ( opscc ) and non - opscc as has been well documented . currently , a standardized ihc scoring system does not exist and is needed to define hpv positivity . we have recently seen a patient that provides a caution in using p16 status as a diagnostic aid .

the agenesis of dorsal pancreas , also known as congenital short pancreas , is a rare congenital anomaly characterized by the absence of body and tail of pancreas , resulting from failure of development of the dorsal pancreatic bud . adp was first described in 1911 in an autopsy study and was associated with diabetes mellitus . till now less than 100 cases of adp have been reported in literature . we report a case of incidentally detected adp on contrast enhanced computed tomography ( cect ) component of fdg pet / ct in a non - diabetic patient . a 49 year old male , non - diabetic , case of carcinoma of tongue was treated with radiation therapy and concurrent chemotherapy till dec 2015 . after 4 months , the patient presented with suspicious lesion involving left lateral border of tongue and was referred for fdg pet / ct scan for further evaluation . pet / ct revealed increased fdg uptake in heterogeneously enhancing lesion involving the mid - third of the left lateral border of the tongue and few left cervical nodes . on examination of cect , the uncinate process and head of pancreas were seen , while the body and tail of pancreas were not visualized anterior to the splenic vein . on correlative t2 weighted mri images of the abdomen , the body and tail of pancreas were not visualized , confirming the diagnosis of congenital agenesis of dorsal pancreas . the embryological development of pancreas begins at the fourth week of gestation from ventral and dorsal buds , which form as outpouchings from the endodermal lining at the junction of foregut and midgut . the ventral pancreatic bud rotates clockwise and fuses with the dorsal bud at the seventh week of gestation . the dorsal pancreatic bud forms the upper part of head , body , and tail of the pancreas , while the ventral bud forms the inferior part of head and uncinate process . the duct of ventral bud fuses with the distal part of duct of dorsal bud to form the main pancreatic duct ( duct of wirsung ) , while if the proximal part of the duct of the ventral bud persists , it forms the accessory pancreatic duct ( duct of santorini ) . the most common pancreatic congenital anomaly is pancreas divisum , which results due to failure of fusion of dorsal and ventral buds . other pancreatic anomalies include annular pancreas and agenesis of either the ventral or the dorsal bud . serial axial cect and corresponding fused fdg pet / ct images demonstrating presence of pancreatic head and uncinate process ( green arrow , image e ) with no visualization of pancreatic body and tail . the potential space in the pancreatic bed is filled up with splenic flexure of colon , stomach ( yellow arrow , image a ) and small bowel loops ( red arrow , image c ) demonstrating dependent stomach sign and dependent intestine sign respectively confirming the diagnosis of adp . the agenesis of dorsal pancreas is a rare anomaly with less than 100 cases reported in literature . a primary dysgenesis of the dorsal pancreatic bud and an ischemic insult to the developing pancreas are possible explanations . in complete agenesis , the pancreatic neck , body , tail , and duct of santorini are absent . a minor papilla is not present in the duodenum . in partial agenesis or hypoplasia of the dorsal pancreas , pancreatic body , accessory papilla , terminal end of the duct of santorini it is most commonly associated with diabetes mellitus followed by pancreatitis and sometimes exocrine pancreatic insufficiency . however , with development of new imaging technologies , this congenital anomaly is often incidentally detected in asymptomatic patients undergoing imaging for an unrelated reason , similar to our case . other associations of adp reported in literature include polysplenia , bicornuate uterus , adenocarcinoma , etc . t2 weighted mri images of abdomen showing absence of pancreatic tail near the splenic hilum . the potential space is filled with intestinal loops correlating with ct images ( yellow arrow , image a ) . the pancreatic head and uncinate process is seen in the image b ( white arrow ) . ercp or mrcp demonstrating absence of the dorsal pancreatic duct is considered as confirmatory for diagnosis of adp . however , ercp is an invasive procedure . this is because air - filled stomach and intestinal loops appear as an echogenic structure in the pancreatic bed , and may be misinterpreted as normal body and tail of pancreas on ultrasound examination . on cect , adp shows presence of pancreatic head with non -visualization of pancreatic body and tail anterior to the splenic vein . this potential space in the pancreatic bed is filled up with stomach and bowel loops called as dependent stomach sign and dependent intestine sign , respectively . these signs are not seen in pancreatic lipomatosis as the pancreatic bed is filled up with fat and there is no space in the pancreatic bed to be occupied . therefore , demonstration of these signs is considered as hallmark for diagnosis of adp on cect imaging , obviating the need for further radiological confirmation . zhou et al . have also reported a case of congenital adp confirmed by three - dimensional reconstruction ct when no pancreatic tail and splenic artery branches to the pancreas were observed . in our patient , the space in the pancreatic bed was filled up with the splenic flexure of colon and small bowel loops ( dependent intestine sign ) . hence , the diagnosis of adp was confirmed in our case . also , our patient was non - diabetic , which is uncommon in adp . as ct has become an integral part of pet studies , nuclear medicine physician should be able to recognize incidental congenital anomalies and report the same . this case aims to make the nuclear medicine physician aware of this rare congenital anomaly . this case aims to make the reader aware of recognizing adp , a rare pancreatic congenital anomaly , which can be incidentally detected on cross - sectional imaging , its common associations , differential diagnosis on imaging and confirmatory methods .

agenesis of dorsal pancreas ( adp ) is a rare congenital anomaly characterized by the absence of body and tail of pancreas . we report a case of incidentally detected adp on contrast enhanced computed tomography ( cect ) component of fluorodeoxyglucose positron emission tomography ( fdg pet / ct ) in a treated case of carcinoma ( ca ) tongue with suspected local recurrence . dependent intestine sign , hallmark of adp on cect imaging was noted in our patient .

hepatocellular carcinoma ( hcc ) is the fifth most common malignancy and the third leading cause of cancer - related mortality globally . the usual aetiology is either due to gastro - oesophageal varices or ulcers . in rare instances , hcc invading into gi tract may be the cause of gi haemorrhage . we present a case of a 76-year - old lady with gi haemorrhage due to gastric invasion by hcc who was successfully managed with trans - arterial embolization . a 76-years - old lady with background history of cryptogenic child 's b liver cirrhosis presented with intermittent epigastric pain of 2 weeks duration associated with abdominal distension and nausea . on examination , vital signs were stable but marked pallor was noted in her conjunctiva . coagulation profile was mildly deranged with a prothrombin time of 11.3 s and a normal partial thromboplastin time . she was admitted under the gastroenterology service with the preliminary diagnosis of dyspepsia with anaemia for investigation . repeat blood investigations showed that haemoglobin had fallen from 6.8 to 5.3 g / dl despite blood transfusion . after attempted removal of the blood clots with flushing , the patient developed torrential bleeding necessitating intubation for airway protection . the bleeding eventually ceased spontaneously and the source of bleeding was identified to be originating from a large ulcer crater in the greater curve with an overlying clot ( fig . 1 ) . endoscopic ultrasound was performed which showed a 10 cm mass lesion abutting the stomach wall and extending into the peritoneal cavity ( fig . the preliminary impression was that of a bleeding gi stromal tumour and care was transferred to the surgical team for consideration of surgical intervention . a computed tomography scan was performed which showed multifocal hcc with a large exophytic lesion invading into the greater curvature of the stomach ( figs . 3 and 4 ) . angiography identified a replaced left hepatic artery as the dominant vessel supplying the lesion of concern in the left lateral hepatic segment ( fig . figure 3:computed tomography image showing hcc invading into greater curvature of stomach . figure 4:computed tomography image showing tumour originating from the liver . figure 5:pre- and post - trans - arterial embolization images of hcc leading to successful haemostasis . computed tomography image showing hcc invading into greater curvature of stomach . computed tomography image showing tumour originating from the liver pre- and post - trans - arterial embolization images of hcc leading to successful haemostasis . she was eventually discharged and will be reviewed by the medical oncologists for consideration of palliative chemotherapy . patients with hcc are predisposed to upper gi haemorrhage as they commonly have portal hypertension either due to underlying liver cirrhosis or due to portal vein involvement by tumour thrombus . common causes of upper gi haemorrhage in hcc patients include bleeding gastro - oesophageal varices , peptic ulcers , congestive gastropathy and gastric erosions . on rare occasions , direct invasion of hcc into the upper gi tract worldwide , < 20 cases of gi bleeding from upper gi invasion by hcc have been reported . although uncommon , it is important to recognize hepatic tumour invasion as a possible aetiology of upper gi haemorrhage as management strategies differ significantly from the common aetiologies . diagnosis of upper gi invasion by hcc causing haemorrhage is difficult and can be easily missed . radiological imaging may show a bulky hcc invading into the stomach or duodenum , but there have been numerous cases of upper gi tract involvement by hcc which were not demonstrated on imaging [ 46 ] . hence , the absence of typical features of tumour invasion in imaging studies does not exclude the pathology . compared with imaging , endoscopy may better visualize these lesions but hcc invasion of upper gi tract can adopt a myriad of appearances ranging from an ulcerative bleeding mass to a polypoidal or submucosal mass with or without features of extrinsic compression . thus , it requires a combination of imaging , suggestive endoscopic findings and a high index of suspicion before a diagnosis of hcc invasion causing upper gi haemorrhage can be confidently made . it identified the lesion to be originating from the peritoneal cavity which on hindsight should have led us to consider hcc invading into the stomach as a differential diagnosis in this patient with long - standing liver cirrhosis . optimal management of hcc invasion causing upper gi haemorrhage hinges on making the correct diagnosis . biopsy of the lesion may lead to torrential bleeding and should be avoided if clinical suspicion of upper gi invasion by hcc is high . the options available in the acute setting include endoscopic interventions , trans - arterial angioembolization and surgery . in contrast to the other more common causes of upper gi haemorrhage , the chance of successful endoscopic haemostasis is low in these cases [ 5 , 7 , 8 ] . to the best of our knowledge , there has only been one case report whereby successful endoscopic haemostasis was achieved . trans - arterial embolization offers better results and should be considered first - line therapy to cease bleeding and stabilize the patient in the acute setting before considering definitive surgical resection in suitable candidates [ 5 , 7 , 9 ] . although surgical resection may control bleeding , majority of patients with hcc invading into the gi tract have extensive tumours not amenable to curative resection . as palliative resection confers a dismal survival , surgery in the acute setting should only be considered as a last resort when other measures of achieving haemostasis have failed .

patients with hepatocellular carcinoma ( hcc ) are predisposed to upper gastrointestinal ( gi ) haemorrhage with bleeding gastro - oesophageal varices and peptic ulcers being the common aetiologies . on rare occasions , hcc with direct invasion into the upper gi tract can lead to haemorrhage . recognizing the possibility of invasive hcc causing upper gi haemorrhage is of paramount importance as acute management differs from the usual aetiologies . we describe a 76-year - old lady with long - standing liver cirrhosis who presented with upper gi haemorrhage due to an hcc invading into the greater curvature of the stomach . trans - arterial embolization was performed which led to successful cessation of bleeding . direct invasion of the gi tract by hcc causing haemorrhage is an extremely rare condition . compared with endoscopic therapy , trans - arterial embolization offers the best chance of successful haemostasis and should be considered first - line therapy in these patients .

the complexity of choosing a particular treatment for an individual patient while keeping her informed about the relevant options and considerations keeps increasing as personal genetic information becomes more commonly available . this is leading clinicians and patients to question their role in the decision - making process . for example , what role should patients take in choosing between alternative treatment options , in particular when the benefits and risks of each option are not crystal clear ? to what extent should clinicians share their own hesitations about the best treatment choice , exposing their patients to the incomplete knowledge about each alternative ? how should clinicians take such decisions without exposing patients to superfluous stress when current knowledge about the advantages and drawbacks of available therapy options is far from complete ? these questions , and the doctor 's dilemma , have long been the topic of public discourse . over 800 years ago , maimonides , a prominent jewish philosopher and practicing physician , wrote that ' the risk of a wrong decision is preferable to the terror of indecision ' . although this remains as true as ever , should we not be asking what role patients have in taking a treatment decision - even when current knowledge is incomplete ? such questions seem to be more pertinent as we enter the age of personal genomes , when an individual 's pharmacogenomic data may affect their choice between treatment options [ 1 - 3 ] . can patients comprehend complex diagnostic information and act on it when they face a choice between alternative therapeutic options , based on their personal genomic data ? in other words , should patients be made aware of the fine details of current medical knowledge , including the gaps in it , when crucial treatment decisions have to be made ? inevitably , some of those decisions may later turn out to have been the wrong ones for them . wendy lorizio and colleagues have examined this charged issue in a real - world personalized medicine scenario by following the treatment choices of 235 breast cancer patients currently taking or planning to take tamoxifen for prevention of cancer recurrence and who were offered the cyp2d6 genotyping test . their study is a fine example of our current knowledge limitations : at the time of conducting their cyp2d6 genotyping and follow - up patients survey ( march 2008 to may 2010 ) , most published studies , based on retrospective data , indicated that individuals having a cyp2d6 poor metabolizer genotype ( predictive of complete lack of the enzyme activity ) were less likely to benefit from tamoxifen for the prevention of breast cancer recurrence . however , more recent meta - analysis and studies cast doubt about the relevance of cyp2d6 genotypes for breast cancer recurrence in tamoxifen - treated patients . thus , it could well be that a similar study taking place today would find other results , namely that patients would be less likely to change from tamoxifen to another drug following genotyping . as long as no consensus has been reached on the effect of cyp2d6 genotypes on the efficacy of tamoxifen for preventing breast cancer recurrence , monitoring the serum level of endoxifen , its active metabolite , seems the most appropriate biomarker for adjusting tamoxifen dosages . including this biomarker as a decision making tool in breast cancer therapy seems to be justified at our currently incomplete state of knowledge . moreover , it will remain a valuable biomarker once endoxifen itself , currently in clinical trials , is eventually approved as a drug . the study by lorizio et al . found that 46% ( 6 of 13 ) of the breast cancer patients prescribed tamoxifen and genotyped as poor cyp2d6 metabolizers elected to change their medication to another drug within the following 6 months . this crucial treatment decision , while obviously taken along with their attending physicians , must have been affected by their participation in the informational session held by the researchers before the genetic testing , in which the results of studies examining the effects of cyp2d6 genotypes on breast cancer recurrence were presented . notably , the authors found that about half the patients had previous knowledge about the relevance of cyp2d6 genotypes for tamoxifen therapy , with the source of this knowledge being their nurses or clinicians , the medical literature or the general media ( internet , tv and newspapers ) . yet it seems that performing the genotyping tests and learning about their results in a medical setting affected the decision on switching treatment . this study does not examine the extent to which the decision about changing the medication was driven by the patients or their clinicians . the genotyping results were transferred to patients through their attending physicians , who did not receive specific recommendations along with the laboratory results . it would have been of interest to also interview the clinicians and find out about their role and considerations in taking this decision ; however , this would require a larger study , as in this one only 13 patients of the 235 who were genotyped were found to be cypd6 poor metabolizers . however , this study illustrates that when genotyping relevant to drug response is carried out in a clinical setting along with informing patients about the test implications in advance of the testing , a decision about medication change followed for about half the patients whose test results indicated ( at that time ) that they were unlikely to benefit from tamoxifen . this study conveys important insights for moving personalized medicine forward : offering patients pharmacogenetic testing in the clinical setting along with an educational session on the test relevance for their medication choices is an effective route for taking informed treatment decisions . . the challenge will be to keep medicine participatory and patients fully informed when medicine and personal genomes meet - which may not be as far away as it seemed just a decade ago .

informing patients about risks and benefits of alternative treatment options and choosing between them is becoming a bigger challenge as knowledge about the relationship between the individual 's genetic profile and the efficacy and safety of available medications accumulates . putting personalized medicine into practice requires new modes of information sharing and decision making by patient and physician . this is illustrated by a case study on treatment choices of breast cancer patients following genotyping for cyp2d6 , recently published in genome medicine.see research article : http://genomemedicine.com/content/3/10/64

using dental implants for prosthetic rehabilitation of the edentulous maxilla has become a routine dental procedure . however , low quality of the posterior maxillary bone and a highly pneumatised maxillary sinus can compromise an implant 's survival12 . implant migration into the maxillary sinus can be caused by inexperienced practitioners , unexpected perforations during sinus floor elevation , application of heavy force during implant insertion , and dental implant placement without sinus floor elevation of an excessively pneumatised maxillary sinus3 . if this condition is inadequately treated , the implants can displace to deeper craniofacial cavities , causing infection , tissue necrosis , and adverse reactions to foreign bodies4 . treatment of implant migration into the maxillary sinus includes the classic caldwell - luc operation and the endoscopic approach via the nose5 . in this case report , we will discuss a dental implant that disappeared following its migration into the left maxillary sinus . a 53-year - old male patient was referred to us for retrieval of a displaced dental implant that had migrated into his left maxillary sinus . three months before his referral , the patient underwent a surgical procedure where eight dental implants were inserted to rehabilitate his fully edentulous maxilla . 1 ) using sinus floor elevation , implants placed in the left and right first molar areas were inserted simultaneously . three months postoperatively , the patient 's dentist administered a panoramic radiograph and discovered that one of the implants had migrated into the patient 's left antrum.(fig . 2 ) the patient 's medical and family history was not remarkable , and intraoral examination showed no signs or symptoms of oroantral fistula . after administering computed tomography imaging to localize the implant , we discovered no foreign body in the maxillary sinus.(fig . 3 ) due to the small size of the migrated implant ( 3.4 mm in diameter and 8 mm in length ) and the absence of oroantral fistula , we believe that the implant left the maxillary sinus via the ostium . thoracic and abdominal radiographs were taken for aspiration risk of the implant but no radiopaque objects within the patient 's body were detected . similarly , a six - month follow - up of the patient proved to also be uneventful.(fig . although complications are uncommon , dental implant migration into the maxillary sinus can occur due to inexperienced operators , high pneumatisation of the sinus , and low bone density in the posterior maxilla6 . although implant migration into the maxillary sinus is usually symptomless , it can cause infections that affect the paranasal sinuses and oroantral communications . implants that have migrated into the paranasal sinuses may also cause sinusitis and adverse reactions to foreign bodies7 . migrated dental implants can primarily be diagnosed using three - dimensional computed tomography but can also be observed on panoramic radiographs and lateral or frontal cephalograms . retrieval of migrated implants can be achieved via standard caldwell - luc operations , transoral functional endoscopic sinus surgeries ( fess ) , and transnasal fess4 . some advantages of endoscopic removal of foreign bodies from the atrium include low morbidity , rapid recovery periods , and possible treatment of the affected paranasal cavities4 . for instance , kitamura and zeredo6 describe a case report where a migrated dental implant was removed endoscopically via semilunar hiatus . in their report , access to the maxillary sinus via semilunar hiatus was cited to be undemanding . to our knowledge , this is the first report of a dental implant that migrated into the maxillary sinus and left the sinus cavity without further treatment . because the patient claimed to have experienced no sensation of swallowing the implant , we believe that the dental implant may have left the antrum via the ostium while the patient was sleeping . therefore , this complication may create the risk of foreign body aspiration and life - threatening conditions . in conclusion , foreign bodies in the maxillary sinus should be retrieved as soon as possible to avoid infections , maxillary sinusitis , and the potential risk of foreign body aspiration .

migration of dental implants into the maxillary sinus is uncommon . however , poor bone quality and quantity in the posterior maxilla can increase the potential for this complication to arise during implant placement procedures . the aim of this report is to present a dental implant that migrated into the maxillary sinus and disappeared . a 53-year - old male patient was referred to us by his dentist after a dental implant migrated into his maxillary sinus . the displaced implant was discovered on a panoramic radiograph taken five days before his referral . using computed tomography , we determined that the displaced dental implant was not in the antrum . there was also no sign of oroantral fistula . because of the small size of the displaced implant , we think that the implant may have left the maxillary sinus via the ostium .

in the history of treating night blindness , the ancient egyptian , the babylonians , the greeks , and many other cultures after them , used animal liver as treatment , like what we did in rak . in the egyptian papyrus ebers ( 1500 b.c . ) the recommended cure was : roasted ox liver , pressed , applied ( topical to the eye ) . another ancient egyptian papyrus , kahun 1 ( 1825 b.c . ) , a gynecological treatise , mentions instructions for a woman , can not see , to eat raw liver of an ass . the assyrian medical texts ( 700 b.c . ) they thought it was caused by rays of the moon and cured by application of ass 's liver to the eyes . they did not put the liver itself in the eye but used the extracted oil and probably enjoyed eating the cooked liver . it is very likely that the ancient egyptian ritual treatment also ended with the patient eating the liver . the greeks shifted the recommendation from topical application to frank eating raw beef liver , soaked in honey , to be taken once or twice ( daily ) by mouth . galen ( 130 ad-210 ad ) , recommends : continuous eating of liver of goats . it is clear that the choice of the animal is influenced by its availability in the community . the chinese sun - szu - mo ( 7 century ad ) in his 1000 golden remedies describes a cure by administration of pig 's liver . the juice of lamb liver was applied topically to the eyes of night - blind children . the procedure was exactly as described by the ancient egyptians , except for one small addition : rather than discarding the remaining organ , the ( practitioners ) fed it to the affected child this was never considered part of the therapy itself . similarly , in 1928 aykroyd wrote : i have been told of a custom of steaming the eyes over cooking liver , which is then eaten , as a remedy for night blindness in the canadian newfoundland island . with respect to arab civilization i found that the abbasid physician hunayn ibn ishaq ( 809873 ) recommended in his book seven articles on the eye the same procedure of rubbing the liver oil in the eye , but also added two more suggestions : get the smoke from the cooking liver goes into the eye plus eat the liver . now we know that the cause of night blindness is deficiency in vitamin a and the real therapeutic benefit comes from eating the liver . animal liver , including that of fish , is very rich with vitamin a , because the liver is the store house of vitamin a in the body . some ancient medical practitioners prescribed rubbing the liver oil in the eye , but patients could not resist eating the tasty barbequed liver . hunayn ibn ishaq took the idea from them because he is credited for translating several greek medical books to arabic . in ancient time , they must have realized that there was a nutritional deficiency as the cause of that disease . arabic poetry written by hunayn 's contemporary poet ibn duraid ( 837933 ) who lived in the same city of bagdad , said that night blindness ( asha ) was caused by dinner ( asha ) , that is , lack of dinner : the two words in arabic not only rhyme together but actually sound the same , hence the poetic effect . in the later middle ages , the dutch physician jacob van maerlandt ( 12351299 ) wrote the following poem recommending eating the liver : so , from the above historical review , it was not surprising that such ancient treatment of night blindness was transmitted over centuries to the arabs and reached rak for me to witness . in the late 1920s , the fat - soluble compound in liver was isolated by karrer a swiss scientist and his colleagues , and termed vitamin a. there are two main forms of vitamin a : provitamin a and preformed vitamin a. provitamin a carotenoids are found in plants . there are many forms of provitamin a , but beta - carotene is the only one that is metabolized by mammals into vitamin a. so , after ingestion , the body converts the provitamin into vitamin . preformed vitamin a ( retinol , retinal , retinoic acid , and retinyl esters ) is the most active form of vitamin a ; it is mostly found in animal sources of food , e.g. , liver , and toxicity can occur if excessive quantities are ingested . the liver is rich with vitamin a because about 5085% of the total body retinol is stored in the liver . the top food sources of vitamin a in our area in the gulf are dairy products , liver , and fish . the top sources of provitamin a are carrots , dates , cantaloupe , and squash . dates are very rich with carotenoid . because people in our region consume a lot of dates , night blindness is not very common . in the eye , vitamin a has two major roles : prevention of xerophthalmia ( abnormalities in corneal and conjunctival development ) and photo - transduction ( transforming light into electrical signals for the brain ) . the cone cells are responsible for the absorption of light and color vision in bright light . vitamin a deficiency is rarely seen in the rich communities and industrialized countries . however , it is still the third most common nutritional deficiency in the world . in a large part of the third world ( southern and southeast asia , africa , and south america ) , night blindness , complete blindness , and advanced stages of xerophthalmia occur in many malnourished children and adults . according to the world health organization , 190 million preschool - aged children and 19.1 million pregnant women around the world have a serum retinol concentration below 0.70 mol / l . in our area , the arabian gulf , citizens rarely get night blindness , not only during these days of wealth , but also during the preoil days . both are rich with vitamin a and provitamin a. so , why did my father develop night blindness while the rest of the family , eating the same food , did not ? the answer is that , at that time , he was ill with gallbladder disease . fat soluble vitamins like vitamin a needs to be mixed with bile salts to form small aggregates before it could be absorbed by the gut . people with insufficient bile flow are usually deficient in vitamin a and hence the night blindness . in populations where dietary intake of vitamin a is adequate , there is no evidence that supplementation of vitamin a as an antioxidant is helpful for preventing cardiovascular disease . randomized trials of vitamin a and beta - carotene have shown no benefit for primary or secondary prevention of coronary heart disease . such supplementation may even have harmful effects on cardiovascular mortality , cancer , and bone health . the lesson from the above story of the ancients and how they cured night blindness with food , tells us to avoid unnecessary vitamin supplements and get our nutritional requirement through balanced and healthy food .

the aim of this article is to briefly review the history of night blindness and its treatment from ancient times until the present . the old egyptians , the babylonians , the greeks and the arabs used animal liver for treatment and successfully cured the disease . the author had the opportunity to observe the application of the old remedy to a patient . now we know what the ancients did not know , that night blindness is caused by vitamin a deficiency and the animal liver is the store house for vitamin a.

leiomyosarcoma ( lms ) of the rectum was first described by scott in 1923 . in the former classification of smooth muscle tumor in the gastrointestinal tract , lms was not distinguished from gastrointestinal stromal tumor ( gist ) . since the discovery of the oncogenic role of kit in 1998 , gists have been differentiated from other mesenchymal tumors such as leiomyomas , leiomyoblastomas , and lmss . although 5% of tumors with the clinicopathologic features of gist lack kit expression , it is important to diagnose lms definitively by immunohistochemical profiling of smooth muscle actin ( sma ) , desmin , and cd34 . true lms of the colorectum diagnosed by immunnohistochemical profiling is extremely rare , with a reported frequency of less than 0.1% of all malignancies of the colorectum ; only 13 reports with immunohistochemical definition which was performed properly have been published in reviews of resected gastrointestinal mesenchymal tumors after 1998 , , , , . herein we report an aggressive case of lms in a rectosigmoid lesion with lymph node metastasis . a 76-year - old woman visited our hospital complaining of intermittent anal bleeding that had lasted 5 months . an elevated tumor with central ulceration was found 10 cm from the anal verge by colonoscopy ( fig . the mib-1 labeling index was high ( 80% ) , suggesting a high - grade sarcoma . abdominal ct scan revealed a 10 cm mass in the rectosigmoid that invaded the uterus ( fig . laboratory tests revealed low - grade anemia ( serum hemoglobin 12.8 g / dl ) , but no other abnormalities were noted ; serum carcinoembryonic antigen and carbohydrate antigen 19 - 9 levels were also normal ( table 1 ) . surgical resection with curative intent required rectal anterior resection with appendectomy , total hysterectomy , and bilateral salpingo - oophorectomy due to extensive inflammation involving the appendix , uterus , right ovary , and right fallopian tube . the excised mass was 110 80 60 mm in size . routine hematoxylin and eosin staining showed proliferation of spindle - shaped tumor cells with enlarged nuclei and eosinophilic cytoplasm ( fig . immunohistochemically , the tumor was positive for desmin and sma , whereas c - kit , dog1 , cd34 , and s-100 protein were not expressed ( fig . the tumor was of high cellularity , with up to 10 mitoses per high - power field ( hpf ) and variable areas of necrosis . direct invasion was histologically proven only in the right ovary , but not in the uterus and right fallopian tube . all surgical margins were negative , and one of the 31 lymph nodes retrieved was metastasized by lms . her postoperative course was uneventful . however , 8 months after the surgery , follow - up ct scanning revealed peritoneal and liver metastases . palliative chemotherapy using gemcitabine ( 800 mg / m ) could not control disease progression , and she died 12 months after the surgery . since radiological differentiation between lms and other mesenchymal tumors is difficult , the final diagnosis needs to be confirmed by postoperative pathological examination . the most definitive feature of lms is immunohistochemical positivity for desmin and sma , and negativity for dog 1 , cd34 , s-100 protein , and c - kit . true lms resected completely and with tumor free margins is extremely rare , with only 13 reported cases , , , , . the tumors ranged 20106 mm in size , with 5100 mitoses per 50 hpfs in the previous cases . to the best of our knowledge , lms in the current study is the largest found the rectum , with the highest mitotic index . since regional lymph node involvement has not been reported in colorectal lms , the role of extended lymphadenectomy in lms is controversial . some authors have suggested that residual lymph node dissection should be considered in patients with enlarged nodes discovered during the surgical procedure , . given that lymph nodes can be metastasized as in our case , lymphadenectomy may be recommended for complete removal of giant lmss and/or lmss with prominent mitosis . the accurate identification and pathologic differentiation between gist and lms in the gastrointestinal tract are important because of the excellent response rate of gist to molecular targeted therapy using tyrosine kinase inhibitors , . since the five - year relapse - free survival rate has been reported to be 65% , which is longer than those who did not undergo adjuvant chemotherapy , patients with high - risk gist are recommended to receive adjuvant imatinib after the complete resection of the tumor . additionally , interruption of treatment with imatinib should not be recommended unless patients experience toxic side effects . studies have revealed that the use of imatinib for palliative chemotherapy is also effective because the two - year progression free survival ( pfs ) was 75% with the use of standard imatinib dose in patients with unresectable or metastatic gist , . in contrast , it is difficult to assess the efficacy of palliative treatments of lms due to the paucity of published reports . doxorubicin , ifosfamide , dacarbazine , and gemcitabine are commonly used in first - line chemotherapeutic regimens , although standardized chemotherapy with currently available agents has not yet been established for lms . additionally , gemcitabine and docetaxel are used as second - line therapy in patients with lms ; however , response rates of lms are usually 10%25% in palliative chemotherapy . for these reasons , the two - year pfs rate is still reported to be approximately 45% , which is poorer than the prognosis of patients with gist . furthermore , the overall survival of lms is poor and is reportedly 20%40% at 5 years , . several reports have demonstrated that large tumor size ( > 5 cm ) and high levels of mitotic activity ( > 10/50 hpfs ) are unfavorable prognostic factors in lms , , . in the present case , a large tumor comprising hyperproliferative cells suggested an unpromising outcome at the time of diagnosis ; she experienced early relapse , and only survived an additional 3 months despite chemotherapy with gemcitabine . it is crucial to diagnose lms precisely based on immunohistochemistry , and thereby distinguish it from gist . although lymph node metastasis is rare , lymphadenectomy appears to be important for high - risk lmss to perform r0 resection . written informed consent was obtained from the patient for publication of this case report and accompanying images .

highlightsthe most definitive feature of lms is immunohistochemical positivity for desmin and sma , and negativity for dog 1 , cd34 , s-100 protein , and c-kit.it is crucial to diagnose leioyosarcoma precisely based on immunohistochemistry , and thereby distinguish it from gist.lymphadenectomy appears to be important for high - risk lmss to perform r0 resection .

since first described in 1964 there were several case reports of classical sweet 's syndrome and sweet 's syndrome complicating infections or medications in the medical literature . sweet 's syndrome in human immunedeficiency virus infection ( hiv ) is extremely rare and until date , there are only few case reports . to the best of our knowledge , no case of sweet 's syndrome in hiv infection has been reported from the indian subcontinent till date . we here report a case of sweet 's syndrome in a female with hiv infection . a 67-year - old female patient presented with high - grade fever , conjunctival congestion and painfulskin lesions involving both hands of 1 week duration . skin lesions [ figure 1 ] were multiple erythematous tender papules involving both hands more on the right as well as on the right forearm . some of the lesions had a vesicular appearance as well . there were no pustules or bullae or ulcerations . clinical photograph of pruritic desquamating lesions on the palms hemogram revealed leukocytosis ( 13,400 cells / cmm ) with 90% neutrophils and an elevated erythrocyte sedimentation rate ( 84 mm/1h ) . biochemical parameters including liver function tests , renal function tests , blood sugar and serum electrolytes were within normal limits . skin biopsy was performed , and histopathological analysis revealed dermal edema , dense neutrophilic inflammatory infiltrate in the dermis with a normal overlying epidermis [ figure 2 ] . chest x - ray was normal , and ultrasound of the abdomen demonstrated fatty liver . skin biopsy low power view showing normal epidermis , underneath there is dense neutrophilic infiltrate and edema of dermis ( h and e , 40 ) patient was managed with oral prednisone ( 1 mg / kg / day ) , ciprofloxacin ( po and eye drops ) and other symptomatic measures . prednisone was gradually tapered off over the next 4 weeks , and the patient had no recurrence of lesions after stopping steroids . depending on the clinical setting in which it develops sweet 's syndrome can be classified as classical ( idiopathic ) sweet 's syndrome , malignancy associated sweet 's syndrome and drug associated sweet 's syndrome . classical sweet 's syndrome does have a strong female predilection , but this predominance often lacks with malignancy - related cases . classical sweet 's syndrome occurs commonly between 30 and 60 years although cases have been reported even during the neonatal period . drug - induced sweet 's syndrome although most frequently implicated with granulocyte - colony stimulating factor , has been reported with a wide array of other drugs . the malignancy associated with sweet 's is acute myelogenous leukemia , but other malignancies are also reported to have association with sweet 's syndrome . a list of potential etiological associations of sweet 's syndrome is summarized in table 1 . etiology of sweet 's syndrome the exact pathogenesis of sweet 's syndrome is yet to be elucidated . there have been various postulates on regarding the pathogenesis like septic processes or bacteria linfections , cutaneous hypersensitivity reaction to viral or bacterial or tumor antigens , an autoimmune response , immune - complex deposition disease , effect of cytokines like interleukines , interferons or tumor necrosis factor . however , none of these postulates has been proven unequivocally . the excellent response to steroids supports the role of an inflammatory basis for this rare syndrome . fever is the most common symptom and will present in > 80% patients irrespective of the setting of sweet 's syndrome . fever typically precedes the dermatosis by days to weeks ; however , both can develop simultaneously as well . skin lesions of sweet 's syndrome are typically tender , reddish , papules or nodules and are often distributed asymmetrically . a myriad of ocular manifestations including conjunctivitis , blepharitis , scleritis , uveitis and retinal vasculitis has been described . involvement of liver , spleen , lung , kidneys , and central nervous system has been reported . diagnostic criteria include clinical , laboratory and histological parameters and are summarized in table 2 . in addition to neutrophilic infiltration , histology may reveal dermal edema , fragmented neutrophil nuclei , swollen endothelial cells and dilated small blood vessels . the main stay of treatment of sweet 's syndrome is systemic steroids , but topical or intralesional steroids may be used in case of localized disease . several investigators have also observed similar improvement when using potassiumiodide to treat patients with sweet 's syndrome . other medications found to be useful in the treatment include indomethacin , clofazimine , cyclosporine , and dapsone . sweet 's syndrome lesions may heal spontaneously without any specific therapy in some patients however when untreated lesions may persist for weeks to months . diagnostic criteria for sweet 's syndrome although known to occur with a variety of infections only few reports exist documenting sweet 's syndrome in hiv infected persons . the validity of the association between hiv infection and sweet 's syndrome remains to be established . also , whether the association is directly or indirectly causal , or merely a coincidence needs to be proven with time . with the currently available sparse literature on sweet 's syndrome in hiv infection , the pathogenesis , diagnosis and treatment of this dermatosis remains the same as that of an immunocompetent individual . hopefully , this case serves as a reminder that sweet 's syndrome can be a differential diagnoses for unexplained skin lesionsin hiv - infected .

sweet 's syndrome is an uncommon dermatosis and can be associated with a wide variety of illnesses including infections and malignancies . sweet 's syndrome as a dermatological manifestation in human immunedeficiency virus ( hiv ) infection is rarely reported . furthermore , called acute febrile neutrophilic dermatosis is characterized by fever and skin lesions , which are often erythematous papules and pseudovesicles . diagnosis is based on clinical features and histology . the gold standard for treatment is systemic steroids although many other medications have been tried with variable success . we here report a case of sweet 's syndrome in an hiv - infected patient .

this study is a cross - sectional trial in outpatients with type 2 diabetes using data from the duty registry ( 7 ) end points included levels of modifiable cvd risk factors , i.e. , most recent levels of systolic blood pressure ( sbp ) , ldl cholesterol , and a1c ( intermediate outcomes ) . these outcomes were analyzed as binary variables according to levels considered not in control and therefore requiring more action , as recommended by the american diabetes association ( 8) . a second set of end points was defined to reflect the intensity of medication management strategies of the three outcomes for subjects with risk factor values at or above these cut points . for each cvd risk factor , we calculated the sex - specific proportion of the patients with levels not in control who were currently receiving more intensive medication , presumably reflecting a greater effort to manage the outcome . stratified by the presence or absence of cvd , we used logistic regression models to estimate differences in the levels and treatment of cvd risk factors by sex . we estimated the probability of having cvd risk factors not under control or of receiving more intense medication for those with poorly controlled risk factors and modeled the risk differences between men and women and their confidence intervals ( cis ) . subject characteristics are shown in supplemental table 1 ( available in an online appendix at http://dx.doi.org/10.2337/dc08-0194 ) . approximately 63% of the patients had sbp 140 mmhg , 48% had ldl cholesterol 130 mg / dl , and 24% had a1c levels 8.0% . intensive treatment with antihypertensive agents was done in 39% of the patients , with lipid - lowering drugs in 32% and antihyperglycemic agents in 39% . unadjusted risk differences were larger in women than in men throughout ( online appendix , supplemental table 2 ) . the upper part of table 1 shows the calculated odds ratios for risk factors not under control . after multiple adjustments , among patients with a cvd history , women were significantly more likely than men to have sbp 140 mmhg , ldl cholesterol 130 mg / dl , and a1c levels 8.0% . the largest differences observed between men and women were regarding ldl cholesterol control . among patients without a history of cvd , women were significantly more likely than men to have ldl cholesterol 130 mg / dl , while there was no difference in sbp or a1c . the lower part of table 1 reports the estimated probabilities of the intensity of medication management . in adjusted models , among those with a history of cvd , the medication intensity was similar in men and women with respect to antihypertensive and antihyperglycemic medications , but women in this large german population of patients with type 2 diabetes , women with a history of cvd were more likely to have all three risk factors uncontrolled , with differences in lipid control being the most pronounced . women were also less likely to receive lipid - lowering medications . among patients without a history of cvd , these results are of particular interest , since it has been shown that the stronger effect of type 2 diabetes on the risk of coronary heart disease in women is in part explained by a greater effect of atherogenic dyslipidemia and blood pressure in diabetic women ( 9 ) . our findings are consistent with previous reports ( 5,10 ) , albeit supported by a much larger data source . a cross - sectional analysis in american patients with diabetes found that women were less likely than men to have a1c < 7% , less likely to be treated with lipid - lowering medications , and , when treated , less likely to have ldl cholesterol < 100 mg / dl ( 4 ) . we extend the above data by showing that lack of control is even more pronounced among patients with cvd , a finding with obvious clinical implications . we have recently shown that among patients with diabetes , physicians focus more on antihyperglycemic treatment , although blood pressure and lipid control are more effective in affecting patient - related end points ( 7 ) . the strengths include its large size : > 10 times higher than previously published studies in this field . it has been shown that a target - driven , long - term , intensified intervention aimed at multiple risk factors in patients with type 2 diabetes reduces the risk of cardiovascular events ( 11 ) . the present study shows that diabetic women have poorer control of important modifiable risk factors than diabetic men and receive less intensified lipid - lowering therapy .

objective to assess whether sex differences exist in the effective control and medication treatment intensity of cardiovascular disease ( cvd ) risk factors.research design and methods we performed a cross - sectional analysis including 44,893 patients with type 2 diabetes ( 51% women ) . end points included uncontrolled cvd risk factors ( ldl cholesterol 130 mg / dl , systolic blood pressure [ sbp ] 140 mmhg , and a1c 8% ) and the intensity of medical management in patients with uncontrolled cvd risk factors . multiple - adjusted odds ratios were calculated after stratification for the presence of cvd ( present in 39% of the patients).results women with cvd were less likely to have sbp , ldl cholesterol , and a1c controlled and less likely to receive intensive lipid - lowering treatment . women without cvd were less likely than men to have ldl cholesterol controlled with no differences in sbp or a1c control.conclusionswomen with diabetes and cvd have poorer control of important modifiable risk factors than men and receive less intensified lipid - lowering treatment .

nasopharyngeal angiofibromas are relatively rare tumors that represent 0.5% of all head and neck neoplasms and they predominantly occur in adolescent males ( 1 ) . this type of tumor originates from the region of the sphenopalatine foramen . in most cases , imaging studies will demonstrate a widening of the pterygopalatine fossa and anterior bowing of the posterior wall of the maxillary sinus ( 2 ) . i describe an extra - nasopharyngeal angiofibroma arising from the parapharyngeal space in a middle - aged woman without involvement of the sphenopalatine foramen or the nasopharynx . a 53-year - old woman presented with a diffuse anterior neck swelling of one - month duration . the patient had not undergone any previous surgery . ultrasonography ( us ) showed diffuse goiter with multiple thyroid nodules . a homogeneous and hypoechoic solid mass , adjacent to the upper margin of the right submandibular gland with focal flow signals on color doppler sonography within the mass was detected ( figure 1 ) . a computed tomography ( ct ) scan revealed a circumscribed mass , hypodense to the masseter muscle and hyperdense to the parotid gland in the right parapharyngeal space ( figure 2 a - c ) . after intravenous contrast administration , the mass , 1.7 1.7 1.6 cm in size , demonstrated homogeneous enhancement without infiltration to the adjacent muscle . at that time enlargement of the bilateral thyroid lobes with multiple non - enhancing hypodense and calcified thyroid nodules was also noted . under general anesthesia after surgery , the patient revealed an uneventful postoperative course . at the three - year follow - up examination , angiofibromas are histologically benign , but potentially locally destructive fibro - vascular tumors that account for less than 0.5% of all head and neck neoplasms ( 3 ) . they originate from the region of the sphenopalatine foramen and commonly spread to the sphenoid sinus , the nasal cavity , and the pterygopalatine foramen ( 1 ) . angiofibromas that do not originate from the area around the sphenopalatine region are very rare ( 1 ) . the primary site of extra - nasopharyngeal angiofibromas varies and can include the ethmoid and sphenoid sinuses , the nasal septum , the tonsils , the ears , the trachea , the larynx , and the infratemporal fossa ( 1 ) . the most common site of extra - nasopharyngeal angiofiroma is the maxillary sinus . in the recent literature , there were five more cases of extra - nasopharyngeal angiofibromas reported in the literature since then ( 5 - 9 ) . only one parapharyngeal angiofibroma was reported in a 25-year - old male in the english literature ( 10 ) . they reported homogeneous enhancement on ct and hypervascularity on conventional angiogram in the left parapharyngeal mass . i report the very rare case of an extra - nasopharyngeal angiofibroma arising from the right parapharyngeal space in a middle - aged woman . to the best of our knowledge , this represents the second demonstrated case of an angiofibroma originating in such a location . extra - nasopharyngeal angiofibromas occur more commonly in females and tend to develop at an older age ( 11 ) . the male - to - female ratio is roughly 3:1 and the mean age at presentation is 22 years . clinical manifestations of extra - nasopharyngeal angiofibromas are variable including headache , hoarseness , dysphagia , dyspnea , stridor , and pain according to their location ( 4 , 11 ) . extra - nasopharyngeal angiofibromas may have less specific and confusing clinical symptoms and diagnosis may be delayed ( 4 ) . enlargement of the sphenopalatine foramen with an intensely enhancing mass is the typical pathognomonic radiological finding , especially in young boys ( 1 ) . in the patient with characteristic radiological findings and typical clinical symptoms of nasopharyngeal angiofibromas , preoperative biopsy extra - nasopharyngeal angiofibromas are harder to diagnose radiologically due to their various locations ( 4 ) . unlike nasopharyngeal angiofibromas , lack of hypervascularity on conventional angiograms does not exclude the diagnosis of extra - nasopharyngeal angiofibromas ( 2 ) . an axial ct showed well enhancing mass in the right parapharyngeal space without perilesional infiltration . the mass was thought to be a minor salivary gland origin tumor or a paraganglioma , hemangiopericytoma , solitary fibrous tumor or neurogenic tumor , preoperatively . differential diagnoses of parapharyngeal masses may also include parapharyngeal space metastatic lymph node , lymphoma , inflammatory pseudotumor , tuberculous and fungal infections ( 12 ) . the most acceptable treatment for angiofibroma is endovascular embolization and then surgical excision ( 13 ) . nasopharyngeal angiofibroma seems to have a poorer prognosis than an extra - nasopharyngeal lesion due to inadequate surgical exposure ; consequently , it may result in a higher recurrence rate ( 3 ) . in the present case , complete surgical resection was performed without preoperative embolization . on follow - up after a period of 3 years , there was no tumor recurrence .

nasopharyngeal angiofibroma is a relatively uncommon vascular tumor affecting adolescent males and it characteristically originates in the posterior lateral wall of the nasopharynx . primary extra - nasopharyngeal angiofibroma is very rare . here , i present a case of angiofibroma of the parapharyngeal space in a 53-year - old woman with ct and sonographic findings .

the benefits of ultrasound - guided hysteroscopic metroplasty have been well described in the management of the partial septate uterus . the use of ultrasonography at the time of hysteroscopy provides visualization of the intrauterine instruments within the uterine cavity . this report describes a case where ultrasound - guidance was used to enter a noncommunicating uterine cavity hysteroscopically . the patient underwent a laparoscopy and hysteroscopy to further evaluate the cause of pelvic pain and to treat the mllerian anomaly . under ultrasound - guidance this unique application of ultrasound - guidance in hysteroscopic surgery may aid the surgeon in entering a noncommunicating uterine cavity . mllerian anomalies occur in approximately 1 in 400 women and have been associated with reproductive failure and pelvic pain from endometriosis and hematometra . noncommunicating uterine cavities pose a challenge to the surgeon performing a hysteroscopic metroplasty because the position of the hysteroscope in relation to the cervix and ipsilateral uterine cavity is not known . ultrasound - guidance may aid the surgeon in the treatment of this specific type of anomaly . this report was completed after approval by the institutional review board . a 22-year - old g3 p0030 female with a history of 3 consecutive pregnancy losses presented with secondary dysmenorrhea . the past surgical history was significant for a laparoscopy performed by the patient 's local physician after a mllerian anomaly was suspected at the time of a miscarriage . hysteroscopy performed at the same surgery revealed 2 uterine cavities with 2 tubal ostia separated by a septum . the septal thickness varied from 5.8 mm near the internal cervical os to 8.6 mm at the fundus ( figure 1 ) . a speculum was placed in the vagina , and the 2 cervical ostia were visualized . a uterine manipulator ( kronner medical manufacturing , cooper surgical , shelton , ct ) was placed in the left uterine canal and indigo carmine was instilled . the right cervix was dilated to admit a 22 f operative hysteroscope ( karl storz , endoscopy america inc , culver city , ca ) through which the right uterine cavity was visualized and the right tubal ostia identified . under transabdominal ultrasound - guidance ( atl hdi 5000 ultrasound system , bothell , wa ) , the wall between the 2 uterine cavities was incised at its narrowest point above the internal cervical os with a resectoscope until indigo carmine overflowed into the right - sided cavity ( figure 2 ) . a concomitant laparoscopy revealed a heart - shaped uterus , normal tubes , and ovaries . minimal endometriosis was identified on the right uterosacral ligament and left ovary , which was treated with a laser . a balloon uterine stent ( cook ob / gyn , spencer , in ) was placed inside the uterus , instilled with 2.5 cc of saline , and left in place for 1 week . the image was taken immediately after the ipsilateral uterine cavity was entered in the lower uterine segment . the uterus and cervix are derived embryologically from paired mllerian ducts that fuse medially to form the uterine isthmus and cervix . the fundal portion of the uterus is created by rapid proliferation of cells from the medial walls of the ducts into a septum followed by resorption . anomalies of the uterus result from abnormal fusion of the mllerian ducts or from failure of absorption of the septum . this patient 's anomaly consisted of an externally divided fundus , a well - formed septum , and cervical duplication . thompson et al noted at the time of surgery that in 5 out of 12 cases of double uteri , the uteri divided externally with a well - formed septum internally . thompson et al did not report the incidence of cervical duplication in this series ; however , several case reports have described cervical duplication with or without a uterine or vaginal septum . although patients with double uteri may have normal reproductive outcomes , studies have demonstrated improved outcomes after correction when a history of pregnancy complications exists . since ruge reported a successful pregnancy after removal of a septum in 1884 , many procedures have been described for the treatment of mllerian anomalies . alternative methods to resect the septum have been described by strassmann , tompkins , and jones with numerous modifications . the surgical management of m00fc;llerian anomalies has evolved since the first descriptions , being modified due to evolving techniques . the choice of technique is influenced by vascularity of the incision , trauma to remaining myometrium , and soundness of the uterine scar . the benefits of ultrasound - guided hysteroscopic metroplasty have been well described for a partially sep - tate uterus . the use of ultrasonography at the time of intrauterine surgery provides visualization of the intrauterine instruments as well as the myometrium and septum . in this case , the area initially incised was the narrowest width above the internal os of the cervix . the internal cervical os was identified sonographically at the angle of the cervix with the uterine body . although a small series did not find a high risk of cervical incompetence with resection of the cervical septum , the theoretical risk of cervical incompetence remains . in addition , in this patient , unification of the cervices would have been technically difficult due to the wide separation of the ostia . alternatively , this procedure could be performed blindly with the guidance of a uterine sound or cervical dilator ; however , the direction of the resection and relationship to the cervix is less obvious than with ultrasound . in summary , the use of ultra - sound may aid the surgeon in entering a noncommunicating uterine horn during a hysteroscopic metroplasty .

background : the benefits of ultrasound - guided hysteroscopic metroplasty have been well described in the management of the partial septate uterus . the use of ultrasonography at the time of hysteroscopy provides visualization of the intrauterine instruments within the uterine cavity . this report describes a case where ultrasound - guidance was used to enter a noncommunicating uterine cavity hysteroscopically.methods:a 22-year - old female after her third miscarriage experienced worsening dysmenorrhea . the patient underwent a laparoscopy and hysteroscopy to further evaluate the cause of pelvic pain and to treat the mllerian anomaly . under ultrasound - guidance , the 2 uterine cavities were unified by hysteroscopic metroplasty.results:postoperative sonohysterography demonstrated unification of the 2 cavities.conclusions:this unique application of ultrasound - guidance in hysteroscopic surgery may aid the surgeon in entering a noncommunicating uterine cavity .

from march 2003 though november 2004 , admitting physicians in district and provincial hospitals within 9 provinces of thailand in the north , northeast , central , and southern regions were invited to recruit patients of all ages suspected on clinical grounds to have leptospirosis . clinical features considered were those specifically referred to in the national guidelines ( e.g. , fever , headache , muscle pain , meningism , conjunctival suffusion , and jaundice ) together with hemoptysis , hepatomegaly , diarrhea , hypotension , and reduced urine output . from each patient , a 5-ml serum sample was taken to be cultured for leptospira , another 5-ml serum sample was taken for serologic testing , and a third sample was taken 2 weeks later for serologic testing . . microscopic agglutination test ( mat ) was performed at the world health organization ( who)/united nations food and agriculture organization ( fao)/world animal health organisation ( oie ) collaborating center for reference and research on leptospirosis , brisbane , queensland , australia ( 4 ) . a positive mat was defined as a single titer of > 1:400 or a 4-fold rise in titer between acute and convalescent phase samples . for leptospira culture , 100 l of whole blood , 500 l of plasma , and 500 l of serum were each injected into 3 ml of ellinghausen , mccullough , johnson , and harris ( emjh ) medium and supplemented with 3% rabbit serum and 0.1% agarose , then incubated aerobically at room temperature ( 25c30c ) for 6 months and examined every week for 2 months , every 2 weeks during months 3 and 4 , and once a month during months 5 and 6 . examination was done by placing 1 drop of culture onto a microscopic glass slide and viewing by dark - field microscopy at 200 magnification . positive cultures were referred to the who / fao / oie collaborating center for reference and research on leptospirosis for identification by using the cross - agglutination absorption test ( 4 ) . a total of 700 patients with a clinical diagnosis of leptospirosis were recruited during the study period . all patients had blood samples collected at the hospital for leptospire culture and serologic testing ; convalescent - phase serum samples were obtained during follow - up for 509 ( 73% ) patients . the median age of patients with suspected leptospirosis was 38 years ( range 295 years , interquartile range ( iqr ) 2849 years ) ; 504 ( 72% ) were men . the number of clinically diagnosed leptospirosis cases by month in the north , northeast , central , and southern regions is shown in figure 1 . most cases ( 597 , 85% ) were recorded in 4 provinces in the north or northeast ( table ) . cases were predominantly identified during the rainy season ( june october ) in the north and northeast in 2003 , with a second peak in the northeast , but not the north , during the rainy season of 2004 . cases of clinical leptospirosis by month for each geographic region , thailand , march 2003november 2004 . ci , confidence interval . of the 700 patients who received a clinical diagnosis of leptospirosis , 143 ( 20% ) received a confirmed diagnosis of leptospirosis based on leptospira isolation , mat testing , or both ( table ) . the median age of patients with confirmed leptospirosis was 35 years ( range 1068 years , iqr 2745 years ) ; 121 ( 85% ) were men . the diagnosis was confirmed after isolation of leptospires from 15 ( 11% ) patients ; the geographic distribution is shown in the table . the serovars of cultured leptospira were : l. interrogans serovar ( sv . ) autumnalis ( 7 ) , l. interrogans sv . hebdomadis ( 1 ) , l. interrogans sv grippotyphosa ( 1 ) , and an unidentified serovar ( 1 ) . an additional 128 patients with culture - negative samples had been exposed to leptospira as determined by mat ; results for 96 ( 75% ) were based on a 4-fold rising titer and for 32 ( 25% ) , on a single raised titer of > 1:400 . the geographic distribution of the 143 laboratory - confirmed cases is summarized in the table . most of these patients ( 124 , 87% ) lived in the 4 provinces found in the north and the northeast ( table ) . the month of diagnosis for confirmed cases is shown in figure 2 ; most were during the rainy season . cases of laboratory confirmed leptospirosis and positive predictive accuracy of clinical diagnosis by month , thailand , march 2003november 2004 . the positive predictive accuracy of a clinical diagnosis is defined by the number of laboratory - confirmed cases divided by the number of clinically suspected cases . results for each of the 9 provinces are shown in the table . when only data from centers that reported at least 10 cases were used , positive predictive accuracy ranged from 3% to 29% . diagnosing leptospirosis at the point of care is notoriously difficult in the tropical setting , where several common infectious diseases are often hard to differentiate . positive predictive accuracy for leptospirosis was highest during the rainy season , an observation that is likely related to the higher disease incidence and pretest probability . variability in positive predictive accuracy was seen among the 3 provinces with the highest number of both suspected and true cases . the reason for this is unclear but may relate to perceived risk to the community , local policy , or other factors . the finding that both clinical and confirmed cases of leptospirosis were more common in the north and northeast is consistent with ddc reports . increased incidence in this region may have resulted from > 1 events , such as an increase in the rodent population , a natural reservoir for this pathogen , and a population in which around one third are positive for leptospira in northeast thailand ( 5 ) . alternatively , 1 clone or a small number of bacterial clones may have become adapted for persistence at greater numbers within the natural host or in the environment . it is also possible that 1 clone or a small number of clones have become adapted for enhanced invasion of the human host . the most prevalent serovar isolated was l. interrogans serovar autumnalis ( 7/15 [ 47% ] isolates ) , 6 of which were from cases in the north or northeast . the effect of the low level of accuracy of hospital - based clinical diagnosis of leptospirosis in rural thailand is not known . a common disease in this setting that is easily confused with leptospirosis is scrub typhus ; both diseases would be predicted to respond to doxycycline , an antimicrobial drug often prescribed for undifferentiated fever . further studies are required to define the implications of our findings and determine whether routine laboratory testing for leptospirosis should be implemented in thailand .

we defined the positive predictive accuracy of a hospital - based clinical diagnosis of leptospirosis in 9 provinces across thailand . of 700 suspected cases , 143 ( 20% ) were confirmed by laboratory testing . accuracy of clinical diagnosis varied from 0% to 50% between the provinces and was highest during the rainy season . most confirmed cases occurred in the north and northeast regions of the country .

most commonly , contractures arise where adequate burn care and delivery have not occurred and scar management has not been instigated in a vigorous manner . repair by regeneration can no longer occur when the depth of injury extends beneath the reticular dermis , and healing by secondary intention.1 the resultant wound contraction can lead to contractures over flexor surfaces and its surroundings . for example , limitation of opening jaws may cause difficulties in accessing the dentitions and tissues of the oral cavity , and in using full size of removable denture for the patients . telescopic implant - supported removable partial prosthesis with milled abutments has been used for the patient with burn contracture to provide enough retention and stability for the prosthesis . a 60-year old woman was referred from the plastic surgery department to the department of prosthodontics at yonsei university health system . she had a chief complaint of restoring her oral function and esthetics after series of plastic skin graft procedure done . during the extra oral examination it was noted that the elasticity of muscle and skin tissue around oral angular area was lost due to scars from burns and tissue grafting , which resulted in limited maximum mouth opening of 20 mm . therefore , proper prosthodontic treatment was impossible on the right quadrants of the mouth ( fig . the intraoral examination showed that all of maxillary teeth were missing except tooth number # 25 and splinted gold crowns on # 34 and # 35 , whereas fixed partial denture on # 33 to # 43 were present on the mandible . in order to restore the patient 's masticatory function , prosthodontic treatment plan of using 4 implants on the left quadrants each of two implants were placed on upper and lower posterior left jaws , however no implant on anterior ridge were possible due to severe atrophy of the maxilla . anterior cantilever type removable partial denture was planned to meet patient 's esthetic demand , which consists of a telescopic crown abutment on # 25 and two implant supported abutments on area of # 26 and # 27 in the maxillary area ( fig . 2 ) . on the mandible , implant - supported fixed partial denture on area of # 36 and # 37 was planned . the implants ( strauman basel , switzerland ) ( 4.810 mm ) were placed on predetermined area accompanied with sinus lift ridge augmentation procedure and proper follow up was performed during the 6 month healing period of time . customized abutments were fabricated on implants and designed in the predetermined angle paralleling to the prepared tooth of # 25 ( fig . 3 ) . removable partial denture was inserted to verify phonetics , esthetics and function . key and keyway rigid attachment was used for retention and ledge type rest was used for support ( fig . no complications have occurred in 5 years since the insertion of the prostheses ( fig . removable type of prosthesis was selected as treatment option after considering the factors of oral hygiene maintenance and lip support of patient to improve esthetics . because there was only one natural tooth in the maxilla , we , therefore , had to improve the unfavorable removable partial denture using implants.2 due to the lack of anterior ridge , the implant option on anterior ridge was deemed unavailable , and natural tooth was splinted with implant to support anterior cantilevered prosthesis . combining implants with natural teeth is controversial because of the variations in movement during function.3 a lot of complications regarding implant and tooth - supported prosthesis may arise ; including fracture of the prosthetic components , intrusion of the natural tooth , marginal bone loss , and loss of osseointegration,4 but survival rates of implant and tooth - supported fixed prostheses are comparable to implant - supported fixed prostheses.5 there was no prosthetic complication in implant - and tooth - supported fixed prosthesis in this case . in this case , forces in the cantilevered area were transmitted to the abutments , causing tilting and rotational movements,6 but cantilevered prostheses are preferable when reduced stress is inherent.6,7 the prosthesis in this patient is expected to have a good prognosis since it is designed with hypoocclusion in anterior areas .

the scars and contracture around the oral - facial region may cause difficulty in prosthodontic treatment to restore esthetics and function for the patients , who suffered severe burns . this article presents a technique that uses a fixed partial denture prepared with a conventional milling technique and an attachment to support anterior cantilever removable partial denture , thereby providing a more esthetically acceptable and functional result .

crossed renal ectopia ( re ) is a very rare anomaly with an incidence of 1 in 7500 autopsies or 1 in 14,000 pediatric admissions . solitary crossed re ( scre ) occurs due to a combination of unilateral agenesis and re . we report a case of scre associated with vesicoureteric junction ( vuj ) obstruction in a 7-year - old male child . ultrasonography abdomen revealed hydroureteronephrosis with a solitary left kidney with a nonvisualization of the right kidney . intravenous urogram ( ivu ) findings demonstrated that the ureter from the left kidney was inserted into the bladder on the right side after crossing the midline [ figure 1 ] . renal radionuclide 99m - technetium ( 99mtc ) -scan was again suggestive of absent right kidney and ectopic left kidney with vuj obstruction ( delayed drainage of whole ureter and kidney ) [ figure 2 ] . cystoscopy showed one ureteric orifice on the right side which was normal in caliber and could not be cannulated . upon exploration , diagnosis of vuj obstruction intravenous urogram showing solitary right to left ectopia with grossly dilated ureter crossing the midline renal radionuclide scan showing crossed ectopia with obstruction to drainage at vesicoureteric junction and proximal dilated ureter and pelvis crossed re exists when a kidney is located on the opposite side of the midline from where ureters enter the bladder . scre results due to renal agenesis of one side and contralateral displacement of the ureter to the opposite side . the crossed re is a rare disorder , furthermore the incidence of scre is exceedingly low , and till now only 35 cases have been reported in the literature . scre can be diagnosed by ultrasonography and radionuclide scintigraphy using 99mtc - dimercaptosuccinic acid ( dmsa ) in most of the asymptomatic cases . excretory urography and multi - detector three - dimensional computed tomography urography are excellent for delineating re . in our case , 99mtc - dmsa scintigraphy is useful in the diagnosis of simple re , renal cortical lesions , and function of cortical mass . hydronephrosis is the most significant finding in re , and the associated vur is present in 25 - 70% of cases . dilatation of the ectopic renal pelvis due to the ureteropelvic junction obstruction is present in 37% of patients . other abnormalities associated with re include abnormalities of spinal growth and genital abnormalities such as anorectal malformation , hypospadias , and sacral agenesis . in our case , scre ( right to left ) was associated with vuj obstruction which was the cause of hydroureteronephrosis . to our best knowledge , this is the first case report in the world literature although vuj obstruction with crossed re has been reported in one case which was bilateral . in this case , the ectopic kidney was poorly functioning , while the contra lateral orthotopic kidney was malrotated , with fetal appearance . ectopic kidneys are more disease prone than normal kidney , and they are often hypoplastic or dysplastic . some authors encourage a vcug in boys and cystoultrasonography in girls affected by crossed re for the high risk of associated vur as postnatal us is a poor predictor of vur . crossed renal ectopic kidneys are smaller than simple renal ectopic kidneys and have inherently reduced function . a solitary crossed ectopic renal unit will have further worsening of function if associated with an obstructive or refluxing lesion . furthermore , it is important to distinguish the vur from vuj obstruction as association of vuj obstruction causes a more rapid deterioration of renal function particularly in a solitary renal unit . most patients with scre do not require treatment for this condition , but because of associated vuj obstruction reimplantation was unavoidable in our case . we recommend that the urological evaluation should be sought due to the high proportion of these cases have associated urological anomalies . intravenous urography , magnetic resonance urography , and 99mtc - dmsa are the investigations used for the diagnosis of such unusual cases . an associated vuj obstruction once documented should be treated urgently to prevent deterioration of renal function .

solitary crossed renal ectopia ( scre ) is a very rare anomaly of urinary tract . most cases are diagnosed incidentally . we report a case of scre associated with vesicoureteric junction obstruction in a 7-year - old child which has not been reported in the literature till date .

marginal zone b - cell lymphoma of the mucosa - associated lymphoid tissue ( malt lymphoma ) of the lacrimal sac is a quite rare cause of the lacrimal duct obstruction [ 1 , 2 ] . typically , this entity initially presents with epiphora , followed by dacryocystitis and/or a palpable mass . a lack of specific symptoms may result in difficult diagnosis , which delays decision for biopsy . lacrimal sac tumors are more exposed to inflammatory conditions compared to other ocular adnexal disorders . malt lymphoma may transform into a more aggressive subtype like diffuse large b cell lymphoma . the clinical features of primary malt lymphoma of the lacrimal sac have not been well documented . we report a case of this entity found in a patient who presented merely with epiphora . a 38-year - old woman was referred due to an incidental finding of a mass in her right lacrimal sac on computed tomography ( ct ) for a subcutaneous tumor of the cheek . the ct image showed a mass in her right lacrimal sac without bony erosion ( fig 1a ) . magnetic resonance imaging ( mri ) revealed slight hyperintensity on t1-weighted and t2-weighted images of the extraocular muscles , with homogenous contrast enhancement ( fig 1b e ) . the lumen appeared to be patent with low intensity on t1- but bright on t2-weighted images ( fig 1c e ) . when the sac wall was opened , the lacrimal sac mucosa was markedly thickened with a patent lumen ( fig 2 ) . the lacrimal sac was closed by interrupted sutures using 6 - 0 polyglycolide ( vsorb ; kono seisakusho co. , ltd . , the histopathological examination revealed diffuse infiltration of small round to cleaved lymphocytes , consistent with malt lymphoma ( fig 3a , b ) . on immunohistochemistry , the tumor cells were positive for cd20 , and cd79a , but negative for bcl-2 , cd3 , cd5 , cd10 , cd23 , cyclin - d1 , and bcl-6 ( fig 3c , d ) . her epiphora resolved , with a patent nasolacrimal duct irrigation 1 month after starting rituximab monotherapy . we reported a rare case of a primary malt lymphoma of the lacrimal sac and documented clinical features in detail . as the lacrimal sac contains lacrimal drainage - associated lymphoid tissue which constitutes part of the mucosa - associated lymphoid tissue , malt lymphoma may arise in the lacrimal sac . in fact , it is the commonest subtype occupying one third of all primary lacrimal sac lymphomas . however , the clinical characteristics of this subtype are not fully known because only 8 cases have been reported in the english literature ( table 1 ) [ 2 , 6 , 7 , 8 ] . epiphora with a swollen lacrimal sac wall is a diagnostic sign of a lacrimal sac tumor . however , epiphora precedes tumor palpation for several months in lacrimal sac malt lymphoma [ 2 , 3 , 6 , 7 , 8 ] . diagnosis of a lacrimal sac tumor is difficult without palpation , as epiphora is a nonspecific symptom which is also present in acquired nasolacrimal duct obstruction . therefore , we need to include lacrimal sac tumor in the differential diagnosis in cases of epiphora with successful lacrimal syringing . , it also demonstrated a typical finding of ocular adnexal lymphoma to extraocular muscles as a slight hyperintensity on t1-weighted and t2-weighted images . fluid intensity in the center of the sac represented the patent lumen , which was consistent with intraoperative findings . as radiotherapy , chemotherapy and immunotherapy are effective in the treatment of lacrimal sac malt lymphoma [ 2 , 6 , 7 , 8 ] , surgery should be limited to biopsy in order to avoid surgical complications . intraoperative findings showed a thickened , pinkish gray mucosa that is typical for lacrimal sac lymphoma . the pathological mucosa with a healthy outer wall suggested that the malt lymphoma extended into the sac cavity first and outside of the wall next . this extending manner was consistent with the fact that the mucosa - associated lymphoid tissue localizes in the adenoid layer of the mucosa beneath the epithelial lining . a lacrimal pump system failure resulting in epiphora was likely due to denatured sac mucosa despite of patent lumen macroscopically . chronic dacryocystitis occasionally demonstrates a nodular lymphoid infiltration pattern in subepithelial and intraepithelial areas , mimicking malt lymphoma . increased goblet cell density and hyperplastic mucosal glands in cases with chronic dacryocystitis are also helpful for differential diagnosis . in addition , the presence of round to cleaved centrocyte - like cells and positive immunoglobulin light chain ( kappa or lambda ) supported the diagnosis of malt lymphoma in this case . lacrimal duct obstruction remains persistent in most reported cases despite regression of lymphomas after radiation and/or chemotherapy . this could be due to incomplete obstruction of the lacrimal sac at the time of diagnosis . in addition , incisional biopsy by opening the sac did not result in secondary stenosis . although radiotherapy was used in most reported cases [ 6 , 7 , 8 ] , this treatment often induces lacrimal gland dysfunction , resulting in dry eyes [ 2 , 14 ] . we preferred rituximab immunotherapy to avoid this complication . in summary , we reported a rare case of a lacrimal sac malt lymphoma found on epiphora without palpable mass . mri was helpful in the qualitative diagnosis of this tumor . diagnosis of this case before developing total obstruction resulted in successful preservation of the lacrimal drainage function . this study was approved by the ethics committee of aichi medical university hospital and adhered to the tenets of the 1964 declaration of helsinki .

we report a case of a primary marginal zone b - cell lymphoma of the mucosa - associated lymphoid tissue of the lacrimal sac , which was found in a patient with epiphora without palpable mass . magnetic resonance imaging demonstrated mucosal thickening of the lacrimal sac with a patent lumen , consistent with the intraoperative finding . epiphora resolved , which was confirmed by smooth syringing , 1 month after starting the immunotherapy .

internal limiting membrane ( ilm ) free autografts have been used to repair refractory macular holes instead of using conventional surgical techniques like vitrectomy and peeling off the ilm , with encouraging results according to several authors . furthermore , to treat retinal tears in retinal detachment surgery , laser therapy or cryotherapy are used with excellent results , achieving appropriate sealing of the tears and thus contributing to retinal reattachment . laser therapy is also applied for the transoperative treatment of retinotomies that are occasionally performed to drain the subretinal fluid . not having those means to achieve tear sealing poses a complication that in some hospital sites , mainly in third world countries like mexico , is not entirely uncommon . in this case report we describe the use of an ilm autograft to seal a transoperative retinotomy in a patient who underwent primary vitrectomy for rhegmatogenous retinal detachment and proliferative vitreoretinopathy . a 55-year - old female patient came for consultation with sudden loss of vision and also reporting a rapidly progressive superior altitudinal scotoma of her right eye . on examination , the patient had a visual acuity of hand motion on her right eye , and fundus examination with a goldman 3-mirror lens revealed total retinal detachment with peripheral horseshoe tears in the 1 , 2 , 7 , and 9 o'clock positions , with presence of an inferior and temporal fixed fold . vitrectomy surgery was then performed with membrane peeling under perfluorocarbon fluids , placing a # 287 exoplant with a # 240 band and silicone oil as intraocular tamponade . the tears were all treated with laser therapy . while the air - fluid exchange was being completed , a retinotomy in the raphe was inadvertently made . laser treatment of the retinotomy site was no longer possible at that moment due to malfunction of the laser device . 0.02 ml of brilliant blue g ( fluoron gmbh , ulm , germany ) were injected into the vitreous cavity to stain the ilm . afterwards , a tano scratcher and an ilm forceps were used to peel off a small graft of the ilm adjacent to the retinotomy site . once obtained , it was displaced under a perfluorocarbon fluid bubble and placed inside the retinotomy ( fig 1 , fig 2 ) . finally , a perfluorocarbon fluid - air exchange was performed , and 5 ml of silicone oil were injected into the vitreous cavity . three weeks later the retina was completely reattached and the retinotomy sealed with the ilm graft ( fig 3 ) . it was not possible to take an optical coherence tomography ( oct ) image due to the presence of a lens opacity . peeling of the ilm is nowadays considered one of the main steps that contribute to a higher success rate in macular hole surgery . this has been achieved more efficiently with the use of dyes such as brilliant blue g that provide better visualization of such membrane [ 3 , 4 ] . furthermore , some techniques have been reported using an ilm flap as well as free ilm autografts over and inside the macular hole , with good anatomical and functional results according to several authors [ 1 , 5 , 6 ] . conventionally , to treat rhegmatogenous retinal tears , methods such as diathermy , cryotherapy or laser therapy have been used . in the latter , for example , an inflammation area this effect induces the formation of a chorioretinal scar around the retinal tear that seals it and prevents vitreous fluid from entering into the subretinal space and detaching the retina again . in this case report we describe the use of an ilm autograft to block the retinotomy , since we did not have these conventional methods available anymore at that moment of the surgery . the apparent sealing of the tear at the 3-week postoperative follow - up could be observed clinically , although it was not possible to confirm this finding by an oct image . in different histological preparations , mller cell remains were found in the ilm once it had been removed from the rest of the retina . these cell remains might be involved in the migration and repositioning of photoreceptors as well as in the formation of fibroglial tissue . hence , the ilm autograft might work as a potential source of mller cell remains and as a scaffold where fibroglial tissue can probably grow , contributing to the sealing of the retinal defect [ 1 , 5 , 8 ] . in their study of a modified technique of autologous transplantation of ilm for macular holes , hernndez - da mota and bjar - cornejo reported the presence of oct hyperreflective images where the ilm autograft was placed , which might suggest the presence of this type of tissue filling the macular hole defect . the same phenomenon might occur in rhegmatogenous retinal tears and retinotomies such as the one presented in this case report . animal studies as well as larger case series of patients and eventually comparative studies with conventional techniques are required to determine the real impact of the use of ilm autografts for the treatment of rhegmatogenous retinal detachment and retinal tears . other possible applications of this surgical technique combined with a retinal pigment epithelium autograft might be in diseases such as albinism and degenerative myopia that have little pigmentation of the retinal pigment epithelium .

during a pars plana vitrectomy , an unplanned retinotomy in the raphe was performed in a 55-year - old female patient with rhegmatogenous retinal detachment and proliferative vitreoretinopathy . since diathermy and laser therapy were not available at that moment , it was decided to peel off a small graft of the internal limiting membrane adjacent to the retinotomy site which had been previously stained with brilliant blue g. the graft was displaced under perfluorocarbon fluids and placed inside the retinotomy . three weeks after surgery , the apparent closure of the retinotomy was observed clinically .

diabetic papillopathy ( dp ) is a syndrome characterized by self - limited unilateral or bilateral optic disk swelling associated with minimal or no permanent loss of visual function.13 dp may occur in patients with type 1 diabetes mellitus ( dm)1 and type 2 dm,2,3 with approximately a 0.5% incidence,1 irrespective of metabolic control and severity of diabetic retinopathy . dp is a diagnosis of exclusion , made after other causes of disk swelling have been ruled out . likely ischemic in origin , dp is considered by some authors to be a form of ischemic optic neuropathy.4 the etiology of dp is not well understood , and there is no accepted treatment to alter its natural course . in recent case reports , treatment with a single injection of intravitreal triamcinolone acetate or intravitreal inhibitor of vascular endothelial growth factor ( vegf ) was effective in reducing disk swelling.59 however , only one report was found in a literature search that was performed with intravitreal ranibizumab ( lucentis ; novartis , basel , switzerland ) injection.9 we report a second case of dp that demonstrated resolution of optic disk swelling and rapid visual recovery when intravitreal ranibizumab was administered . a 51-year - old male with a 10-year history of poorly controlled type 2 dm presented with acute painless visual loss in his right eye . his best - corrected visual acuity was 20/320 in the right eye and 20/50 in the left eye . fundus examination of the right eye showed nonproliferative diabetic retinopathy with serous retinal detachment and a swollen optic disk with telangiectatic vessels ( figure 1a ) . fluorescein angiography of the right eye showed optic disk leakage with the presence of microaneurysm ( figure 1b ) . optical coherent tomography imaging of the right eye revealed serous macular detachment and a significant increase in retinal nerve fiber - layer thickness ( figure 1c and d ) . the patient underwent clinical neurologic examination ( including brain magnetic resonance imaging ) , blood pressure measurement , and complete blood count and electrolyte testing , all of which were within normal limits . dp is a diagnosis of exclusion , made after other causes of disk swelling have been ruled out . through full explanation of the expected effects and possible complications of intravitreal ranibizumab injection , informed consent was obtained from the patient before injection , and intravitreal ranibizumab ( 0.5 mg/0.05 ml ) injection was performed using the standard technique . two weeks following injection , there was marked regression of disk swelling and improvement of macular edema , with visual acuity improving to 20/100 ( figure 2a and b ) . three months following injection , there was complete resolution of optic disk swelling , and visual acuity was 20/50 in his right eye ( figure 3a and b ) . many pathological conditions may mimic d p , such as inflammation ( eg , sarcoidosis ) , infection ( eg , lyme disease ) , cancerous infiltration ( eg , leukemia ) , hypertension , papilledema , and disk ischemia.1,3,10 a diagnosis of dp was made due to the following reasons : uncontrolled type 2 dm , painless visual loss , presence of associated macular edema , which is common in dp,1 early disk hyperfluorescence on fluorescein angiography , and good visual outcome posttreatment . recently , intravitreal injection of anti - vegf was reported to be associated with visual improvement and reduced optic disk swelling without adverse events in patients with dp.79 however , the mechanism of action of anti - vegf ( such as bevacizumab or ranibizumab ) in patients with dp is not well understood . the good response to the treatment may suggest that vegf plays an important role in the pathogenesis of dp . in the present case , dp had largely resolved 2 weeks after intravitreal ranibizumab injection , while vision had improved from 20/320 to 20/100 . the spontaneous course of dp tends to be more protracted , with regression of the disk edema occurring over months and visual recovery often being incomplete.11 dp had not reappeared in our patient at the 3-month follow - up , which is three times longer than the effective duration of vegf inhibition after a single ranibizumab injection . a rapid improvement in vision was noted in our patient shortly after intravitreal injection of ranibizumab . therefore , it may be that the intravitreal ranibizumab played a positive role in stabilizing and improving the patient s optic nerve edema . however , the efficacy and safety of this management of dp needs to be proven through further larger clinical studies .

we report a case of diabetic papillopathy that demonstrated a resolution of optic disk swelling and rapid visual recovery when intravitreal ranibizumab was administered . a 51-year - old male presented with acute painless visual loss in his right eye . his vision was 20/320 in the right eye and 20/50 in the left eye . fundus examination of the right eye showed nonproliferative diabetic retinopathy with macular edema and a swollen optic disk . fluorescein angiography showed dye leakage from the right optic disk . optical coherent tomography revealed a significant increase in retinal nerve fiber - layer thickness . magnetic resonance imaging of the brain was normal . the patient received a single intravitreal ranibizumab ( 0.5 mg ) injection . two weeks following injection , there was marked regression of the disk swelling and improvement of macular edema , with vision improving to 20/100 . three months following injection , there was complete resolution of the optic disk swelling . no further treatment was required .

under the influence of some emergencies ( mad cow disease , dioxin in feed ) , there has been a significant development of food laws which led to the enhancement of some normative papers that make up the basis of food security such as the council regulation ( ec ) n. 178 of 28 january 2002 ( european commission , 2002 ) . this last is intended to lay down the general principles and requirements of food law by a series of regulations . more precisely , it has confirmed that food business operators ( fbo ) are the main responsible for the hygienic and healthy production of food and must adopt the most appropriate measures to ensure consumers health and expectations by means of the self - control plan following the principles of hazard analysis and critical control points ( haccp ) . in the present paper , we want to present some critical comments resulting from the activities carried out by the prevention services [ national information system on agriculture ( sian ) and veterinary service ] of the local health service of rome ( asl rm / a ) , italy . we compare them with those acquired from a project of integration concerning the european regulations of the chinese community of prato , carried out by the territorial service of the ausl 4 of prato ( acsa ) . in the period 2007 - 2009 , the veterinary service , in cooperation with the health food and nutrition territorial service of the ausl rm / a , carried out an investigation on the raw fish in japanese restaurants located in the centre of rome ( masotti et al . , 2010 ) . the primary objective was to evaluate the structural , operational and general hygiene of the equipment and the staff , and to assess the freshness of the fish products used and distributed raw . thirty restaurants were inspected : most of them were japanese preparing sushi and sashimi . as for data collection , checklists were arranged individually by the two services , for their investigation , based on the recommendations included in the guidelines of latium region for the implementation of the reg . the activity had several positive effects in terms of the awareness of the issues related to the consumption of raw fish preparations and it resulted in a reduction of non - conformities . still , its most relevant result is the ongoing adoption of the correct procedures for the prevention of anisakis risk by the fbos involved . particularly , the first checks showed a wide use of fresh product not subject to any remediation by freezing . on the contrary , as a result of the prescriptive acts , a greater spread of freezing of the raw material performed on site using correct procedures was detected the following years . as an alternative , the purchase of the product already subject to necessary remediation was registered . therefore , the project can be considered as a successful example of the interaction between official control and fbos , especially given that it has been able to act not only on the mere application of regulatory provisions , but also on the cultural approach in preparing of traditional ethnic foods . in the period 2009 - 2010 , in the province of the ausl 4 in prato , a project was launched , aiming at improving the quality level of the retail outlets managed by the chinese community operators . the project involved all the professionals of the competent authority for food safety of the ausl 4 in prato ( acsa ) ( armani et al . , 2011 ; guidi et al . , the activity involved 24 shops , including 9 supermarkets which sell meat and fish products , 6 supermarkets which sell meat and fish products and other food products , 4 restaurants , 2 stores , 1 street vendor . similarly to the services of asl rm / a , the investigation was carried out by adopting the method used to perform audits , i.e. by customising the checklist for the assessment of health standards in the production , preparation , administration and sale of foodstuffs of plant origin and drink and the haccp checklist official controls . on the whole , the 24 controlled stores showed the following faults : hygienic - sanitary ( 100% of the controlled stores ) ; traceability / labelling of products ( 100% ) ; training of personnel ( 100% ) ; prescriptions not acquitted ( 25% ) . in particular , it was found that the plan for self control is formally present in all the stores , but in 90% of them it is not understood and is disregarded , although the documents have been translated into chinese . during the inspection activities , 91 proceedings were performed , of which : 33 administrative sanctions , 32 prescriptions , 3 administrative seizures , 6 reports of crime , 4 destructions , 3 seizures and 2 temporary closures . at the end of the activity , anyway , a substantial decrease in serious non - conformities and the permanence of not serious non - conformities was recorded . in addition , a marked improvement of the use of labelling in italian language on products was found even if accompanied by a poor traceability of the food . furthermore , the staff and the management training on the european legislation on food safety showed no improvement . the results of these two experiments allow you to make some considerations that can be extended to all kinds of food stores in the area . it has been shown that very few fbos have a thorough knowledge of the regulations on food safety , especially on food production sanitation . when comparing official control and fbos , the difficulties one may come across with and have to take into consideration are to face cultural and linguistic differences . as highlighted , in line with the content of the 2008 report of the ministry of health , the diversification of food production , in relation to the different sectors and the size of the fbo , causes a non - uniform awareness of fbo in the way the application of self - control systems is concerned , in order to ensure the product food safety of . in the evaluation of the inspection activities carried out by the competent authorities , in fact , a high prevalence of non - conformities in the framework of general hygiene prerequisites is confirmed , in the application of the haccp system and in the staff training . therefore , it is vital to provide a general training program , involving the operators in charge of the control and fbo , in order to allow the effective and definitive resolution of the non - compliances , through the proper use of the audit , an instrument introduced by the regulations of the hygiene package and not yet properly used throughout the national country ( italian ministry of health , 2008 ) . indeed , the type of non - conformities detected has highlighted the inadequacy of the role played by the consultants , with regards to the application of the haccp . in an ethnic community , this makes an expert advice highly desirable , which may interact with fbos in a better way from a linguistic viewpoint , and , above all , which is to be a real reference point for the different critical aspects for the fbo . the inadequate application of the self - control systems ( prerequisites of haccp ) is also reflected in the recurrence of non - conformities in the quality control activity of finished food products . as a matter of fact , it was observed that , the obligation for the fbo to have a self - control plan has been envisaged for 16 years . an intervention by the central authority would be desirable , in order to rule the activities of the self - control consultants , considering the possibility to establish an official list of people in charge of the self - control , who are to be certified and trained , as well as the ability to designate people who are not suitable for that activity . the initiatives to provide the consumer with correct information should be encouraged and could be developed directly at the level of food marketing and service , through periodic campaigns reporting on the major food risks .

the purpose of the present paper is to highlight some critical situations emerged during the implementation of long - term projects locally managed by prevention services , to control some manufacturing companies in rome and prato , central italy . in particular , some critical issues on the application of self - control in marketing and catering held by chinese operators are underlined . the study showed serious flaws in preparing and controlling of manuals for good hygiene practice , participating of the consultants among food business operators ( fbos ) to the control of the procedures . only after regular actions by the prevention services , there have been satisfying results . this confirms the need to have qualified and expert partners able to promptly act among fbos and to give adequate support to authorities in charge in order to guarantee food safety .

included in our report were all cases meeting the national surveillance case definition for ld ( 5 ) reported to the maryland department of health and mental hygiene ( dhmh ) with a known date of onset from 1993 through 1998 and a residential zip code mailing address . demographic data from the 1990 census are publicly available for the zip code level ( 6 ) . in 1990 , population figures for maryland zip codes ranged from 39 to 56,594 ( median 3,042 ) . to obtain larger units of population for more stable estimates of incidence , small zip codes were combined with the next smallest neighboring zip codes until the aggregated zip code area ( azca ) reached the size of > 600 residents or more . annual average incidence per zip code or azca was calculated as the average number of cases from 1993 through 1998 per 100,000 population . one hundred six small - population zip codes were combined with others to give 50 azcas , ranging in size from 616 to 51,683 ( median 1,791 ) . most azcas were located in western maryland ( non disease - endemic area ) and the eastern shore ( highly endemic area ) . the 1990 census did not contain population data for 15 zip codes , so their incidence could not be calculated . analysis and data management were performed with epi - info version 6.04 and microsoft excel version 7.0 ; maps were created with arcinfo ( esri , redlands , ca ) . a total of 2,399 cases reported to the dhmh with a known date of onset from 1993 through 1998 met the national surveillance case definition for ld . this report includes the 2,371 ( 99% ) patients for whom mailing addresses were available . only 6 of the 33 zip codes from western maryland ( garrett , allegheny , washington , and frederick counties ) reported cases of ld during the study period . two areas of high incidence are evident : the upper eastern shore ( cecil , kent , queen anne s , caroline , and talbot counties ) , and focal areas north and east of baltimore city in baltimore and harford counties ( figure ) . this latter area is part of an arc of increased incidence , from montgomery county in the south , extending northeast through howard , southeastern carroll , baltimore , and harford counties into cecil county . this arc parallels the fall line , the topographic boundary where the coastal plains meet the piedmont and land elevations begin to rise towards the appalachian mountains . average annual incidence of lyme disease by zip code , maryland , 19931998 . in baltimore county , the area with the highest incidence extends along the vegetational corridors bordering the gunpowder falls river system and associated reservoirs . in harford county , a similar but less confined linear pattern follows the runs of broad creek and deer creek . on the upper eastern shore high incidence the scattered zip codes on the lower eastern and lower western shores that show higher incidence than surrounding areas are all azcas with rather small populations and resultant wide confidence intervals for their incidence estimates . the detailed mapping of ld in maryland identifies an area of high ld incidence north of baltimore city that is not be apparent when mapping on the county level ( 3 ) . when analyzed by counties , focal high incidence along gunpowder river and deer creek is diluted by adjacent areas of lower incidence , especially the northern inner suburbs of baltimore city with their comparatively urban environment . these foci are aligned along the larger rivers and creeks in an environment that is ideal for transmission of the disease . within the floodplain and on valley slopes of the rivers descending from the piedmont lie corridors of forest and brush , cutting through rural and suburban areas . farms , estates , individual houses , and housing developments lie within and adjoin these ideal tick habitats . the extent of the high - risk area in baltimore county is congruent with glass s detailed gis results ( 4 ) . in contrast , maryland s upper eastern shore , a rural area situated entirely in the coastal plain with an ideal tick habitat , has uniformly high ld incidence . however , southern maryland ( north of the potomac river ) and the lower eastern shore have a low incidence of ld . this correlates with limited ecologic data showing much lower b. burgdorferi infection rates in i. scapularis in southern maryland and the lower eastern shore than in the upper eastern shore ( 7,8 ) . almost no ld was reported in western maryland from frederick county westward ; this virtual absence is consistent with the low prevalence of i. scapularis and low b. burgdorferi infection rates in this tick species , despite an abundance of rodents and deer in this mountainous region ( 7,8 ) . spot - mapping of ld cases is useful for tracking ld transmission but can be misleading about incidence because population density is not taken into consideration . on a spot map , based on the absolute number of cases , a sparsely populated area with high ld incidence may be indistinguishable from another area with high population density and low incidence . incidence figures for counties containing both highly urban and highly rural areas are likely not representative of the rural areas because of the concentration of population in the urban part . health - care providers must appreciate the fact that , for instance , more cases of ld are reported from baltimore county ( population ~1 million ) than from cecil county ( population 20,000 ) , even though the county - level incidence of ld is much higher in cecil county . characterization of ld incidence on the zip code level is feasible using data collected routinely by local health departments . zip code level data provide more detailed information than county level data and require less data and effort than gis risk modeling based on vegetation parameters and tick distribution ( 4,911 ) . although ld risk - mapping based on prevalence of infection in ticks would be the most accurate method ( 12 ) , tick data are often unavailable , out - of - date , costly , and difficult to collect . a potential limitation of our report is that incidence has been referenced to residential addresses , whereas patients may have been infected elsewhere . however , residence in an ld - endemic area is a well - recognized risk factor for infection ( 12 ) . many studies have reported that patients with ld usually believe they were infected at their homes , places of work , or some nearby recreational site ( 1317 ) . a calculated entomologic risk index showed a strong positive relation with the geographic ld case rate in rhode island ( 18 ) . most ( 58% ) of our study participants who remembered a tick bite believed it occurred at or near their place of residence ; an additional 21% were bitten during recreation and 9% at work . patients in high incidence areas were more likely to report a tick bite near their home than were those living in more urban areas or in western maryland . referencing ld cases to their residence is a useful proxy for the actual place patients acquired a tick bite . knowledge of focal ld risk distilled from mapping on the zip code level is of value to the general public . it can focus efforts to reduce tick exposure and increase motivation to use appropriate preventive measures when tick exposure is unavoidable . such mapping can also aid health - care providers in assessing the likelihood of a particular patient s having ld ( 19 ) . meltzer et al . estimated how much the cost - effectiveness of ld vaccination depends on individual risk ( 20 ) . mapping ld incidence in detail complements the centers for disease control and prevention s ( cdc s ) recommendation that the ld vaccine be administered based on residential , occupational , and recreational risk assessment ( 21 ) . the cdc report recognizes the need to develop maps of geographic distribution of ld with improved accuracy and predictive power beyond the county - based national ld risk map .

to support diagnostic and preventive decision making , we analyzed incidence of lyme disease in maryland on the zip code level . areas of high incidence were identified on the upper eastern shore of the chesapeake bay and in counties north and east of baltimore city . these latter foci , especially , are not visible when mapping lyme disease on the county level .

they arise either from trapped pouches of ectoderm near normal folds , or from the failure of surface ectoderm to separate from the neural tube . aural cholesteatomas can be defined as keratinizing squamous epithelium growing in the middle ear cleft . this abnormal skin growth is characterized by a destructive process involving an accumulation of desquamated keratin arising from the squamous epithelium and pathologically affecting the middle ear or the mastoid process [ 1 , 2 ] . a 3-year - old boy had previously been treated for otitis media with effusion with bilateral grommets nine months ago . he then had excision of a 0.75 cm in diameter of cystic swelling in the left postaural region at a district general hospital . he was then referred to our tertiary hospital , four weeks after his second surgery with a persistent discharging granulation at the site of the left post - aural wound . he was evaluated with pure tone audiometry showing 10db conductive hearing loss in left ear and normal tympanometry . computerised tomography ( ct ) of the temporal bones ( figure 1 ) was performed and showed destruction of ossicles of the left ear with a soft tissue shadow filling the left middle ear and the left mastoid cavity with a sinus extending through a defect in the mastoid bone to the subcutaneous tissues in left postaural region . a left modified radical mastoidectomy was performed through a postaural approach with an elliptical incision around the postaural granulation . the granulation was found to be in continuity with a tract that extended into a defect in the mastoid bone . the mastoid cavity was exposed to reveal that the tract was in continuity with a cholesteatoma sac within the mastoid ( figure 2 ) . the facial canal and the labyrinth were normal . the long process of incus was eroded and the cholesteatoma sac was bridging to preserve the sound conductive mechanism . the thin arrow indicates cholesteatoma sac and the thick arrow shows the tract communicating from the sac to the postaural skin . histopathology of the tissue removed from the mastoid cavity showed features consistent with inflammatory polyp and cholesteatoma . as the tympanic membrane appeared intact at the time of surgery , the cholesteatoma was considered to be congenital . cholesteatoma is an abnormal accumulation of keratin - producing squamous epithelium in the middle ear , epitympanum , mastoid or petrous apex . it has been further defined as a threedimensional epidermoid structure exhibiting independent growth , replacing middle ear mucosa , and resorbing underlying bone . although it is not a neoplastic lesion , it can be insidious and potentially dangerous to the patient . the perimatrix or lamina propria is the peripheral part of the cholesteatoma consisting of granulation tissue , which may contain cholesterol crystals . bone resorption is stimulated by a variety of factors , including inflammation , local pressure , keratin and specific cytokines , such as interleukins . in addition to destruction of the ossicles , bony erosion can lead to fistula of the lateral semi - circular canal , facial palsy , total sensorineural hearing loss , sinus thrombosis and intracranial invasion . in acute mastoiditis it is common for the pus to erode the lateral wall of the mastoid bone leading to sub - periosteal and then subcutaneous spread of infection . epidermoid cysts and cholesteatomas are characterized by similar tissue layers , namely basal membrane , basal , intermediate and keratin layers . these two types of lesion have very similar histological features and are thus difficult to identify on the basis of microscopic examination in the absence of information on their origins . this is the first reported case of congenital cholesteatoma presenting with a post aural subcutaneous cystlike swelling in a child . a case of subcutaneous cholesteatoma has been described in a 71 yearold man who had previously had a radical mastoidectomy and also another case of congenital cholesteatoma presenting as a preaural swelling with facial palsy has been reported in the literature . in our patient the bony destruction was entirely due to the congenital cholesteatoma , as he had not previously had any mastoid surgery . the aerated nature of paediatric mastoid air cells system also allows easy extension of such lesion . what was initially thought to be an isolated benign cyst in the postaural region was in fact subcutaneous extension of an underlying cholesteatoma . this case demonstrates the value of pre - operative radiological investigation and the need for a high index of suspicion when excising lesions from unusual head and neck sites .

the authors report a case of a three - year old boy , who presented with discharge from the site of a previously excised postaural lesion reported to have been an epidermoid cyst . it was found at operation that he had a fistulous connection between the site of the excised lesion and a congenital cholesteatoma sac in the mastoid bone . this case demonstrates the rare occurrence of congenital cholesteatoma eroding through the mastoid bone to the subcutaneous tissues and mimicking an epidermoid cyst . such a presentation in a child has not previously been reported in the literature . the clinical , radiological , surgical and histological features of this case are discussed .

growth hormone - secreting pituitary adenoma ( ghpa ) is a rare , chronic , systemic disease that is associated with premature death and significant morbidity.1 it is related to high levels of growth hormone ( gh ) and insulin - like growth factor - i ( igf - i ) . the standardised mortality index ( the ratio of observed mortality in the acromegalic population to expected mortality in the general population ) ranged from 1.2 to 3.3 . if left untreated , patients with acromegaly can die approximately10 years earlier than the healthy subjects . according to prior studies , approximately 60 , 25 and 15% of the patients die from cardiovascular disease , respiratory complications and cancer , respectively.23 high gh / igf - i levels and heart disease are the main factors related to poor outcome in these patients.4 some studies have shown that cerebrovascular disorders are a frequent cause of death , particularly among women , but they also involve patients who have been treated differently many years ago ( craniotomy , radiotherapy);thus , a deleterious effect of these treatments ( especially radiotherapy ) can not be ruled out.1 although the quality of life is affected in patients with ghpa , it can be partially improved by effective treatment.5 treatment options for acromegaly include surgery , medical therapy and radiotherapy . the cost of treatment including medications and the possibility of major side effects represent important limitations of the medical therapy.26 in our region , a patient with acromegaly will need an estimated usd15,000 annually for medical management using octreotide . surgical treatment provides rapid control of gh / igf - i levels and is the first line of treatment for gh - secreting adenomas according to different neuroendocrinology societies and pituitary centres.67 classically , transsphenoidal microsurgery has been considered the best surgical approach for most gh - secreting adenomas . however , in the past decade , the endoscopic approach to the treatment of sellar lesions has become an important option for the resection of pituitary adenomas.678 some of the advantages of this approach include improved visualisation , less nasal trauma , increased patient comfort and , potentially , better results with respect to total tumour resection.8910 in this case series , we aim to describe the results of surgery for the treatment of this rare condition in our centre . we also analyse the current literature related to the treatment of acromegaly and highlight the challenges encountered during the management . the study included three patients ( two males and one female ) with age range of 19 - 32 years [ table 1 ] . the female patient had no light perception in the left eye and was also significantly worried about her amenorrhoea . all patients had frontal bossing , skin thickening , painful joints , prognathism , jaw malocclusion , teeth separation and tongue and acral enlargement summary of the the patients demographics and results automated perimetry of patient 3 radiological characteristics of the adenomas the first patient initially declined surgery and opted for octreotide [ figure 2 ] . he was treated with short - acting octreotide ( the only form available , 50 mg b.i.d . ) and showed significant reduced joint pain . however , he was unable to continue the drug after only1 week of medication because of its high cost ; he subsequently opted for surgery . ( a and b ) preoperative and postoperative contrast cranial computerised tomography of the first patient ( a and b ) preoperative magnetic resonance imaging ( mri ) and postoperative healed scar of the second patient two of the patients underwent pterional craniotomy ab initio , and one abandoned the proposed endoscopic transsphenoidal approach and opted for craniotomy because of intraoperative instrumentation issues . postoperative igf - i was normal 3 months after surgery in all patients [ table 1 ] . the most common cause of excessive gh secretion is a monoclonal benign pituitary tumour ( adenoma ) in more than 90% of the cases . this uncommon disorder is characterised by an acquired progressive somatic disfigurement , involving mainly the face and extremities , but other organs as well . the disease affects both men and women equally,111213 with a prevalence of 40 - 70 cases per million inhabitants and an annual incidence of 3 to 4 new cases per million inhabitants.3 we treated only 3 patients over a 2-year period . a recent study from belgium suggests the prevalence of acromegaly to be approximately100 - 130 cases per million inhabitants.14 in germany , where screening of ghpa was performed by systematic igf - i measurement , the prevalence of biochemical acromegaly was even higher ( 1,043 per million).15 owing to its insidious onset , ghpa is often diagnosed late ( 4 to > 10 years after onset ) , at an average age of approximately 40 years . the reasons for the rare occurrence of this condition in our centre may be due to lack of diagnosis , as many patients attribute their illness to spiritual forces and thus do not present to the hospital . symptoms and signs of ghpa are classical , but pre- and postoperative biochemical assays are not readily available in financially challenged regions . in addition , the cost of the test is quite prohibitive to most of the patients . the specific goals of treatment are to reduce the volume of the pituitary tumour , avoid tumour relapse , normalise disease markers ( gh and igf-1 ) , slow or reverse the clinical signs and symptoms , preserve normal pituitary function and restore life expectancy to the general population . radiosurgery is not available in the country , and the use of medical treatment is not sustainable by most of our patients . the cost of medical treatments , which may be required indefinitely , must be weighed up against not only the risks of radiotherapy but also against the cost in our region . on the other hand , surgery is relatively more affordable and is probably , pragmatically , the best treatment of choice in low - resource practice . globally , the use of preoperative treatment with somatostatin analogues is debatable.51617 surgery is recommended as the primary modality of therapy because it treats the local population with a good probability of a cure . although one of the cases done in our study of a transsphenoidal approach had to be abandoned intraoperatively because of instrumentation issues ( this is not an uncommon event though ) , surgery was successful in all cases with no morbidity .

the management of acromegaly caused by an uncommon growth hormone - secreting pituitary adenoma can be challenging in low - resource african subregion . we conducted a study over a 2-year period to describe the results and challenges following surgical treatment of this rare condition in our centre . the clinical outcome was defined as successful based on the surgeon 's intraoperative observation , postoperative neuroimaging findings and neuroendocrinological results . a total of three patients ( two males and one female ) aged 19 - 32 years were included . visual impairment was the main presenting symptom in all the three patients . the postoperative period was uneventful . acromegaly is an uncommon disorder in our region . surgery is the treatment of choice in low - resource practice .

cholera case - patients were identified from updated lists from juba county s 5 cholera treatment centers ( ctcs ) , 2 of which were located in camps for internally displaced persons ( idps ; i.e. , persons who have left their homes but stayed within their country s borders ) . preventive oral cholera vaccination was conducted in the 2 idp camps before the outbreak began in juba . a case - patient was defined as a juba county resident > 2 years of age who 1 ) had an acute illness characterized by > 3 loose , watery stools within 24 hours or 2 ) was confirmed to be positive for v. cholerae infection by rapid diagnostic testing or culture during the cholera outbreak that began in juba county on april 23 , 2014 . for cholera case - patients enrolled in the study , a control matched by neighborhood , sex , and age was identified and invited to participate in the study . the study team traveled to the case - patient s village and worked with the local village leader or a social mobilization volunteer to identify a matching control from a household within a 100-m radius of the case - patient . a control was a juba county resident > 2 years of age with no history of clinical illness or no laboratory evidence of v. cholerae infection during this cholera outbreak . to match case - patients and controls by age , age groups of 35 years ( e.g. , 24 , 59 , and 6569 years of age ) were used . a team of 19 trained research assistants administered a pretested , semistructured questionnaire and conducted environmental assessments to evaluate the use of safe drinking water , improved sanitation facilities , personal and food hygiene , and oral cholera vaccination . using epi info ( centers for disease control and prevention , atlanta , ga , usa ) , we calculated matched unadjusted and adjusted odds ratios by using bivariate and multivariate models , respectively , to identify risk factors for cholera . a total of 134 matched pairs of case - patients and controls were enrolled in the study during june 26july 29 in 2014 ( figure ) . of the 134 case - patients enrolled , 9 were confirmed by culture and 104 by a cholera rapid diagnostic test ( onsite rapid test ; ctk biotech , san diego , ca , usa ) ; the remaining 21 were identified by epidemiologic linkage ( i.e. , a resident of juba > 2 years of age with > 3 loose stools in 24 hours after the beginning of the cholera outbreak ) . mean delay between admission to the ctc and interview after enrollment in the study was 21 days ( range 055 days ) ; most case - patients who were interviewed were admitted during the peak transmission phase of the outbreak ( figure ) . the distribution of age , sex , residence of origin , education level , and occupation were comparable among case - patients and controls ( table 1 ) . most ( 118 [ 88% ] ) case - patients enrolled in the study visited the ctc within 1 day of onset of cholera symptoms . all 134 patients had diarrhea ; 112 ( 84% ) had vomiting ; 45 ( 34% ) had abdominal cramps ; 37 ( 28% ) had some dehydration ( i.e. , any 2 signs of dehydration , including 1 major sign ) ; and 31 ( 23% ) had severe dehydration ( 23% ) . timeline showing number of cholera cases ( total cases = 2,260 ) , deaths from cholera ( total deaths = 43 ; case fatality rate 2.0% ) , and dates of study for cholera outbreak in juba county , south sudan , epidemiologic weeks 1743 ( april 23october 20 ) , 2014 . bivariate and multivariate analyses showed that persons who ate food outside their home before illness onset and those who traveled outside their home village ( even within the county ) before illness onset were significantly more likely to develop cholera ( table 2 ) . conversely , treating drinking water at home and receiving > 2 doses of oral cholera vaccine ( self - reported ) were protective against cholera ( table 2 ) . eating outside the home as a risk factor in this cholera outbreak popular eating places in juba county included roadside food vendors and restaurants in markets that did not meet minimum food hygiene standards yet remained open during the outbreak because public health inspection of eating establishments and a ban on roadside food vending were not uniformly enforced . our study identified recent travel to cholera outbreak areas as a risk factor , also a finding consistently associated with cholera spread to new locations during previous cholera outbreaks ( 10 ) . * the first 2 factors increased risk for cholera , whereas the other 2 factors decreased risk . or , odds ratio . travel from home to any area affected by cholera during the 2014 outbreak in south sudan . self - reported . also , as reported in previous cholera outbreaks in south sudan , uganda , haiti , and zimbabwe , household chlorination of drinking water was associated with significantly lower risk for developing cholera in our study ( 7,8,9,11 ) . in our study , water samples from case - patient households that did not chlorinate their drinking water showed evidence of contamination with fecal coliforms ( > 10 counts/100 ml ) . similarly , water samples from water storage vessels in 2 case - patient households that did not treat their drinking water were contaminated with fecal coliforms ( > 10 counts/100 ml ) . we found that oral cholera vaccination was associated with a significantly reduced risk of cholera infection and a vaccine effectiveness of 90% ( table 2 ) . these limitations include underreporting of high - risk behaviors , recall bias , potential misclassification of asymptomatic case - patients , narrow age ranges that caused difficulty in identifying matching controls , shared environmental risk factors ( e.g. , shared water source ) for case - patients and controls , unmeasured variables ( i.e. , factors not measured in this study , such as being an idp ) , and loss of oral cholera vaccination cards ( i.e. , vaccinations were self reported ) . for this cholera outbreak in south sudan , we found that travel and eating outside the home were risk factors for becoming ill and that treating drinking water at home and getting oral cholera vaccination provided protection against illness . for cholera prevention and control in humanitarian crises , we recommend that global oral cholera vaccine stockpiles be enhanced so that preventive oral cholera vaccination can be used to augment traditional interventions , such as improved access to safe drinking water and public education about risk factors .

we conducted a case control study to identify risk factors for the 2014 cholera outbreak in juba county , south sudan . illness was associated with traveling or eating away from home ; treating drinking water and receiving oral cholera vaccination were protective . oral cholera vaccination should be used to complement cholera prevention efforts .

extradural hematoma ( edh ) occurs in approximately 2% of all patients with head injuries and 515% of patients with fatal head injuries . bilateral edhs accounts for 210% of all acute edhs in adults but are exceedingly rare in children . posterior fossa edhs occurs in 5% of all cases of edhs . in cases of edh of nonarterial origin , the bleeding is from rupture of arachnoid granulations , diploic emissary veins , and dural sinuses . acute infratentorial edh with supratentorial extension is an infrequent lesion , and only a few articles are published in the literature . the presence of infratentorial edh leads to sudden death and quick deterioration , and its complication is more significant than supratentorial edh as it masks the features of supratentorial edh . we reported a rare case of bilateral occipital edh with supratentorial extension in a child . a 12-year - old male child presented to our emergency department with a history of road traffic accident . at the time of admission the child was conscious , his vitals were stable and routine investigations were within normal limits . noncontrast computed tomography ( ct ) scan showed two well - defined , biconvex , extra - axial hyperdense blood attenuating ( ct attenuation value + 62 hu ) collections noted in occipital region , one on each side ( maximum thickness on left side is 2.2 cm and on right side 1.4 cm ) with few internal hyperdense areas s / o acute extradural hemorrhage with active bleed ( swirl sign ) [ figure 1 ] . evidence of an undisplaced fracture was noted in occipital bone extending superiorly up to lambda with posterior sagittal sutural diastasis [ figure 2 ] . noncontrast computed tomography head showing two extra - axial hyperdense blood attenuating collections in occipital region with swirl sign ( axial , sagittal and coronal view ) noncontrast computed tomography head showing undisplaced fracture of occipital bone with posterior sagittal sutural diastasis within hours , the child became irritable with respiratory distress , and there was also visual agnosia in the left eye . left paramedian suboccipital craniectomy was done in an emergency using high speed 8 mm burr and kerrison punch forceps . the edh was then gently removed with the help of cup forceps and suction [ figure 3 ] . the clot on the right side was also removed with suction via same craniectomy site . postoperative recovery was uneventful , and the child was discharged on the eighth postoperative day . intraoperative photograph showing hematoma during posterior fossa decompression intraoperative photograph showing hemostasis with placement of dural hitch sutures there have been sporadic reports of bilateral edhs with an incidence ranging from 0.5% to 2% of all edhs . in clinical practice , edhs in children are more frequently venous ( from tears of a dural sinus or diploic veins ) than arterial and consequently have a better prognosis than edhs in adults . in a present case report , infratentorial edh occurred because of damage to the diploic veins , and supratentorial component was due to stripping of the dural sinuses . pediatric edh is more frequently seen in the posterior fossa or simultaneously above and below the tentorium . edh does not cross - cranial sutures because the periosteal layer of the dura is tightly adherent to the sutures . huisman and tschirch studied 57 children with proven edh and showed that 11% of edhs ( seven patients ) extended across cranial sutures . associated fractures are somewhat less frequent . however , in this case , a fracture in occipital bone had extended superiorly with posterior sagittal sutural diastasis . low - density areas within the hyperdense collection ( swirl sign ) are thought to represent active bleed within the hematoma . in this case once bilateral infratentorial edh is diagnosed , urgent surgical treatment should be considered . with the widespread use of ct scan . acute infratentorial edh with the supratentorial extension should be strongly suspected in the presence of fracture of occipital bone with sagital suture diastasis . to conclude , early surgical intervention in the bilateral occipital ( infratentorial ) edh confers better prognosis and lesser mortality .

extradural hematoma ( edh ) occurs in approximately 2% of all patients with head injuries . bilateral edhs account for 210% of all acute edhs in adults but are exceedingly rare in children . posterior fossa edhs occurs in 5% of all cases of edhs . edhs in children are more frequently venous ( from tears of a dural sinus or diploic veins ) and consequently have a better prognosis than edhs in adults . once the diagnosis of beh is confirmed , urgent surgical treatment should be considered . we are reporting such rare form of injury as bilateral occipital edh with supratentorial extension in 12 years child following road traffic accident .

foreign body ingestion in adults passes through the gastrointestinal ( gi ) tract uneventfully within a week in 80% of cases , and < 1% of patients develop complications including visceral wall perforation [ 1 , 2 , 3 ] . long , narrow , or pointed foreign bodies can be associated with a higher risk of impaction , as well as complications such as perforation . intra - abdominal foreign body perforations of the gi tract have been reported in all segments ; most frequently , in regions of acute angulation such as the rectosigmoid or the ileocecum , or proximally to a site of pathologic narrowing or obstruction [ 1 , 5 ] . foreign body perforation of the gi tract has a wide spectrum of clinical presentations , which can be either acute or chronic . in the former , gi perforation secondary to foreign body ingestion may present as odynophagia or abdominal pain . however , perforations of the stomach , duodenum and large bowel , due to their thicker mucosal walls , may present in a more insidious manner , including hemorrhage , appendicitis , bowel obstruction and even ureteric colic [ 7 , 8 , 9 , 10 ] . with these varied and nonspecific clinical presentations the types of accidentally ingested foreign bodies directly depend on dietary habits and vary according to the country , age , and cognitive status of the patient . gastric perforation secondary to the ingestion of fish or chicken bones and shell fragments have been generally reported , but only a few cases caused by rabbit bone fragments are known [ 11 , 12 ] . we report the case of a gastric perforation caused by the ingestion of a rabbit bone fragment to raise awareness of a rare but potential cause of gi lesion . a 59-year - old woman was admitted to the emergency department with a 1-month history of nausea and vague , mild epigastric pain . on physical examination , she was conscious with mild pyrexia and an area of tenderness in the epigastric region . a previous abdominal ultrasonography ( us ) was unremarkable with the exception of gallbladder gallstones . the patient was treated by her general practitioner with a 10-day course of broad - spectrum antibiotics , with mild benefits . laboratory investigations revealed raised inflammatory markers ( esr 59 mm / h , wbc 16.09 10 l ) with no other main abnormalities . an abdominal us confirmed the presence of gallbladder gallstones , however , without signs of acute cholecystitis ( fig . 1 ) . the us also revealed the presence of an ill - defined hypoechogenic lesion , next to the gastric antrum ( fig . precontrast and contrast - enhanced computed tomography ( ct ) scans showed a 4-cm hyperdense linear image surrounded by an ill - defined heterogeneous fluid collection with peripheral enhancement , suggestive of a perigastric abscess with an enclosed foreign body ( fig . explorative laparotomy revealed a bone fragment embedded within a purulent fluid collection between the anterior gastric margin and the abdominal wall . the specimen was removed and sent for histological analysis . following the drainage of thick liquid pus material , a gastric discontinuity was noted , and a partial gastrectomy was completed along the greater curvature of the stomach . a cholecystectomy was also performed and a drainage was placed in the sub - liver space . the patient was treated with intravenous antibiotics and discharged in good clinical conditions on the 9th postoperative day , with normal blood biochemical examination ( wbc 9,300 10 l , hgb 11.8 g / dl ) . histopathological examination of the omental - gastric specimens revealed the presence of an animal bone fragment surrounded by severely inflamed tissue . during a careful reconstruction of the case history , the patient recalled in the adult population , perforation of the gi tract by ingested foreign bodies is usually secondary to involuntary accidental ingestion and is frequently caused by foreign bodies included in meals . the main predisposing factor for their ingestion is the use of dentures that could reduce the sensory feedback of the palate . other factors include very young and old age , cognitive impairment , history of psychosis , being fast eaters , and alcohol or drug abuse [ 1 , 2 ] . a foreign body that perforates the wall of the gi tract may take several different possible courses , including lying in the visceral lumen at the site of perforation , or passing through the wall and migrating to a distal organ . for this reason , too , the preoperative diagnosis of stomach perforation caused by ingested foreign bodies tends to be insidious . in fact , the clinical presentation can be elusive because bone fragments can cause omental reactions mimicking tumors , and most patients do not remember the ingestion of specific foreign bodies [ 5 , 7 , 8 ] . the use of plain radiography for the detection of nonmetallic ingested foreign bodies is unreliable because large soft - tissue masses and fluid can obscure the minimal calcium content of the bone , particularly in altered or obese patients . in addition , because the foreign body is impacted gradually and the perforation is covered with fibrin , it is difficult to find free pneumoperitoneum . therefore , the use of a ct scan should be preferred and will usually demonstrate a linear calcified lesion surrounded by an area of inflammation . nevertheless , the accuracy of ct is limited by the lack of observer awareness , and a high index of suspicion must be maintained to reach a correct diagnosis . the types of accidentally ingested foreign bodies vary from country to country and directly depend on dietary habits . gastric perforation secondary to ingestion of shell fragments and fish or chicken bones have been widely reported , but only a few cases caused by rabbit bone fragments are known [ 11 , 12 ] . this low frequency is probably related to the lower worldwide consumption of this type of meat . the ingestion of rabbit bone fragments , even if rarely reported , should not be overlooked as a possible cause of gastric perforation and abscess formation in patients with unexplained abdominal pain , especially in countries , such as italy , that are reported to have one of the highest consumption rate of rabbit meat per inhabitant . the authors declare that there are no conflicts of interest regarding the publication of this article .

the majority of accidentally ingested foreign bodies is excreted from the gastrointestinal ( gi ) tract without any complications . sometimes sharp foreign bodies like chicken and fish bones can lead to intestinal perforation and may present insidiously with a wide range of symptoms and , consequently , different diagnoses . we report the case of a 59-year - old woman presenting with fever and a 1-month history of vague abdominal pain . computed tomography ( ct ) showed the presence of a hyperdense linear image close to the gastric antrum surrounded by a fluid collection and free peritoneal air . at laparotomy , a 4-cm rabbit bone fragment covered in inflamed tissue was detected next to a gastric wall perforation . rabbit bone fragment ingestion , even if rarely reported , should not be underestimated as a possible cause of gi tract perforation .

the morbidity of animal bites is directly related to the severity of the initial wound . thus , urologists are not usually familiar with management and principles for treating this condition . the authors report the case of a 38-year - old male with a severe mule bite injury to the genitalia causing complete penile and anterior urethra amputation and scrotal wound with no involvement of its contents . to our knowledge a 38-year - old countryman , married and father of a young girl , with no past medical history was referred to our department for evaluation after an attack by his mule . at presentation , a complete amputation of the penis with a wide haematocele was noted ( figures 1 and 2 ) . the patient was in good hemodynamic condition despite the important bleeding reported by his family before admission . the scrotal lesion was classified as grade ii according to the american association for the surgery of trauma organ injury scale , when penile trauma was classified as grade v following the same injury scale . after brief debridement and copious irrigation of the wound with saline and povidone - iodine solution , the patient had an ultrasound that showed the testes and epididyms of normal appearance . in the operating room and under a general anaesthesia , a wide right haematocele was present , and careful examination of the testis and epididymes and spermatic cord showed no anomalies . thereafter , we found a complete pullout of all erectile bodies : the corpora spongiosum , cavernosa at their bony insertions , and whole anterior urethra . profuse bleeding occurred after removal of the huge hematoma at the insertion of the corpora cavernosa for a full exploration . therefore , we decided to proceed to catheterization of the urethra at its membranous part ( the remaining distal end of the urethra ) by a foley catheter . a phalloplasty was performed from the remaining penile shaft with a scrotal flap ( figure 3 ) . however , in a second step , a final phalloplasty will be performed with a groin flap . a penile prosthesis the penrose drain was removed on the second postoperative day , and the patient was discharged on the seventh postoperative day , when no signs of infection were noted . psychological support was provided to the patient by psychiatric consultations first in our urology division , then , after being discharged . a perineal urethrostomy was planned , with the consent of the patient , after complete resorption of the hematoma . genital trauma due to animal bite is rare . wolf et al . presented 4 new cases of dog bite and reviewed 4 previously reported cases . however , in the largest series in the literature , gomes et al . reported 10 new cases . of the 2 men and 8 boys 8 were attacked by dogs , 1 by a horse , and 1 by a donkey , respectively . but no patient of this series has been severely bitten by a mule . dog bites are a common form of trauma in the united states with an incidence of 12.9/10,000 individuals . kyriakidis et al . described partial amputation of the penis due to a dog bite , while piza - katzer and latal reported a case of penile skin loss . donovan and kaplan treated amputation of the cord and amputation of the glans in 1 case each . the injuries reported by wolf et al . included testicular loss in 2 of their 4 cases . gomes et al . had 5 patients who presented with minimal or no skin loss , including 2 with urethral lacerations . there was moderate - to - extensive tissue loss in 5 patients , including degloving penile injury in 2 , traumatic spermatic cord amputation in 1 , complete penile and scrotal avulsion in a 5-month - old infant , and partial penectomy in 1 . nevertheless , no complete penile and anterior urethra amputation has been reported in adults . management of such injuries comprises debridement of devitalized tissue and copious wound irrigation with saline and antiseptic solutions . when there is no infection , immediate primary closure along with prophylactic broad - spectrum antibiotics is performed . in our patients , the remaining penile shaft closed the wound decreasing the important bleeding flow immediately after the animal bite . but in our patient , this part of the surgical management was the most difficult . the deepness of the operating field and the important bleeding flow ( figures 1 and 2 ) made stitches very difficult to make . thereafter , the foley catheter ( figure 2 ) facilitates the insertion of stitches around the remaining end of the urethra , not far from prostatic apex . thus , the foley catheter once inserted helped prevent haemostasis stitches to close urethra lumen . if the wound remains uninfected , reconstruction should follow later , but after removing possible necrotic tissue . particularly in cases of partial penile amputation , or in the rare event of unilateral or bilateral testicular loss , primary reconstruction should be attempted after removing any existing necrosis . moreover , in any penile amputation injury , possible urethral involvement must be considered , and this requires an adequate diagnosis . the long - term followup of urethral injuries from animal bites shows the worst results , with urethrocutaneous and urethroscrotal fistula or recurrent urethral strictures . therefore , some authors postulate that injuries to the urethra should be treated later , when it can safely be assumed that there is no inflammation in the sensitive urethral tissue . one of the main problems in bite wounds is the risk of infection , which often occurs in the first 48 h after the injury . this risk is up to 30% in uncomplicated wounds . after removing dead tissue and antiseptically cleaning the wound , a broad - spectrum antibiotic is obligatory , even if there is no firm evidence of pathogenic agents . in addition , the vaccination status should be verified ; if such protection is insufficient , then vaccination against tetanus ( or rabies ) should be immediate . the most frequent bacterial species are staphylococcus , streptococcus , escherichia coli , and anaerobes . although rare , genital trauma caused by animal bite is a potentially severe condition with distinctive characteristics . irrigation and debridement of dead tissue is the cornerstone of treatment , and they should always be performed . primary closure is possible in most cases and usually achieves good functional and cosmetic results . systemic diseases potentially transmitted by animals must be considered , and prophylaxis should be administered accordingly . thereafter , the extraordinary emotional situation of the patient must be considered , and a psychiatric supports should be provided .

animal bite is rare with few cases reported in the literature . the morbidity of animal bites is directly related to the severity of the initial wound . most victims are boys , and dog bites are the most common injury . infectious complications are unusual , since treatment is sought early ( wein 2007 ) . thus , urologists are not usually familiar with management and principles for treating this condition . the authors report the case of a 38-year - old male with a severe mule bite injury to the genitalia causing complete penile and anterior urethra amputation and scrotal wound with no involvement of its contents . to our knowledge , no such case had ever been reported in the medical literature . this kind of emergencies is challenging for urologists .

a previously healthy 33-year - old male was referred to our retina service for sudden onset of intermittent floaters and flashing lights in his right eye which developed 3 weeks following inactivated hepatitis a , yellow fever , and typhoid vaccinations . these vaccines were given prophylactically in preparation for a 2-week trip to panama at the ohio state university wexner medical center travel and immunization clinic . the patient had previously received one dose of the hepatitis a vaccine 12 years prior ( he did not complete the series ) and had never before been exposed to typhoid or yellow fever immunizations . on examination , pupil responses , visual fields to confrontation , and intraocular pressures ( 14 mmhg ) were normal bilaterally . fluorescein angiography of the right eye demonstrated scattered peripheral hyperfluorescent lesions correlating with those seen in the fundus , and there was no evidence of disc leakage ( figure 1 ) . a comprehensive review of systems was negative for fever , rash , oral ulcers , arthralgias , headache , or vertigo . his ocular examination revealed continued low grade anterior vitreous inflammation and the peripheral retinal lesions began to demonstrate early hyperpigmentation ( figure 2 ) . because of the nonprogressive examination findings and given the excellent visual acuity , it was decided to continue close observation . at 8 weeks , the patient s symptoms had resolved , the vitreous was clear of cellular inflammation , and the fundus revealed punched out areas of retinal pigment epithelium atrophy . vaccines potentiate both humoral and cellular immune responses by exposing lymphocytes to pathogen - specific antigens which triggers an inflammatory t cell - mediated reaction and initiates the steps of adaptive immunity and development of cellular memory . autoimmune phenomena have been infrequently observed following vaccination , and possible mechanisms proposed to explain some of these occurrences include the roles of excessive lymphocyte activation , cytokine expression , and molecular mimicry.1 cross - reactivity of an activated immune response against uveal antigens may occur when peptide fragments presented to t cells show close conformational resemblance to uveal self - peptides . melanin - associated molecules present in choroid have been shown experimentally to be capable of inducing ocular inflammation.2 another possible method of immune system activation following vaccination relates to the use of vaccine adjuvants . adjuvants are routinely combined with vaccines to potentiate their immunogenic activity and these have been used in animal models to create uveitis.2 aluminum - containing adjuvants are used widely in vaccine formulations licensed for use in the us , including hepatitis a. crucial to the adjuvant activity of aluminum is the influx of inflammatory factors , including dendritic cells , to the site of injection . these cells can recognize and process foreign antigens , as well as provide the costimulatory molecules necessary for activating plasma cell production of target antibodies.3 rarely , erythema , subcutaneous nodules , contact hypersensitivity , and granulomatous inflammation have been observed with the use of aluminum adjuvants.4 the currently used preparation of typhoid and yellow fever vaccines are free of adjuvants . there are a few case reports in the literature describing posterior uveitis syndromes occurring shortly after vaccination . specifically , multiple evanescent white dot syndrome was reported 10 days following simultaneous hepatitis a and yellow fever vaccines , and acute posterior multifocal placoid pigment epitheliopathy was described in association with hepatitis b and varicella vaccinations.57 a review of 32 cases of uveitis following hepatitis b vaccination included a few reports of positive rechallenge response in patients who developed recurrent uveitis following separate doses of the hepatitis b vaccine.8 most of the reported cases of uveitis demonstrated self - resolution , although recently a case of ampiginous choroiditis was described following human papilloma virus vaccination which required treatment with oral prednisone and resulted in macular scarring.9 multifocal choroiditis is an inflammatory disease characterized by multiple , small , yellow fundus lesions , and vitreous inflammation . presenting symptoms include blurry vision , floaters , and photopsias . although the etiology of multifocal choroiditis remains unknown , there have been suggestions of autoimmune and viral associations . the choriocapillaris and choroid are richly invested with certain potential antigen - presenting cells and the retinal pigment epithelium can be induced to express major histocompatibility complex class ii molecules , suggesting it may be able to interact with t lymphocytes . there is experimental evidence that high densities of t lymphocytes , b lymphocytes , and macrophages can infiltrate the choroid and stimulate synthesis of cytokines capable of altering the subsequent immune response.10 during the late stage of the disease , the spots adopt a rim of hyperpigmentation and often assume the classic punched - out appearance of retinal pigment epithelium atrophy with bands of subretinal fibrosis at their margins . to our knowledge , this is the first reported case of multifocal choroiditis following vaccination . although this case does not prove causation and the relationship remains presumptive , it does suggest that some vaccinations can act as a trigger for immunologic initiation that could subsequently lead to the development of noninfectious uveitis .

the paper describes the first reported case of multifocal choroiditis following simultaneous hepatitis - a , typhoid , and yellow fever vaccinations . a 33-year - old male developed sudden onset of flashing lights and floaters in his right eye 3 weeks following hepatitis a , typhoid , and yellow fever vaccinations . fundus examination and angiography confirmed the presence of multiple peripheral chorioretinal lesions . these lesions demonstrated characteristic morphologic changes over a period of 8 weeks which were consistent with a diagnosis of self - resolving multifocal choroiditis . vaccine - induced intraocular inflammation has been described infrequently . we demonstrate the first case of self - resolving multifocal choroiditis following simultaneous administration of hepatitis a , yellow fever , and typhoid immunizations .

a 36-year - old man with an itching and tingling sensation in the left l2 dermatome area for 3 months was referred to our clinic for pain management . the patient did not have significant past medical history , and before coming to the clinic , he had been treated with gabapentin 600 mg 3 times a day by a dermatologist but the symptoms were not improved . there were no unusual finding in the biopsy other than subcorneal pustules , hyperkeratosis and parakeratosis ; therefore , he was referred to our department for pain control . he was also complaining of allodynia and paresthesia , and his visual analogue scale ( vas ) was 2 out of 10 ( 0 = no pain , 10 = worst possible pain ) . his motor function was intact and there was no sensory loss . his knee jerk reflex was increased ( 3+/3 + ) thus a thoraco - lumbar spine magnetic resonance imaging ( t - l mri ) was performed due to suspicion of an upper motor neuron ( umn ) lesion . in the mri , an intramedullary lesion 1.2 cm in size was found in the dorsal subpial surface of the t11 - 12 level , and this was diagnosed as type ii avm in the t11/12 level of the spinal cord ( fig . a spinal angiography was performed on the patient and the results revealed that the lesion was a spinal cord avm supplied by the anterior spinal artery from the left t9 intercostal artery . it was also contrasted from the left l1 lumbar artery ( artery of adamkiewicz ) ( fig . the patient was transferred to another hospital for removal of avm with cyber knife , and after the operation , the symptoms were improved . laboratory tests are the tzank smear , a serological test such as the indirect fluorescent antibody method , and detection through molecular biology or immunology such as tissue cultures . a dermatomal rash is not present in zsh so it is difficult to suspect hz , and hence , serological , immunological , or molecular biological tests are not performed during the acute phase . only when the pain continues chronically are many cases diagnosed by the characteristics of pain . however , when a patient complains of continuing dermatomal neuropathic pain , a detailed history taking and accurate physical examination should be done to differentiate zsh from sciatica , collapsed intervertebral disc , spinal stenosis , tumor , angina , renal colic , cholecystitis , costochondritis , intercostal neuralgia , entrapment syndrome , pleurodynia , myofascial pain syndrome , and other neuropathic pain before diagnosis . in this case , the patient complained of tingling pain with a vas score of 2 and accompanying itching , paresthesia , and allodynia in the left l2 dermatome for 3 months . during the physical examination , motor and sensory function was intact but the knee jerk reflex was increased to 3 + on both sides . hence , an umn lesion above the l2 level was suspected , an avm in the t11/12 level was discovered in the t - l mri . the umn is the pathway from the brain and brainstem to the anterior horn of the spinal cord . the lower motor neuron ( lmn ) is the pathway from the anterior horn of the spinal cord to the skeletal muscles . in this case , only the knee jerk reflex was hyperactive with l2 dermatomal pain without any other general symptoms or symptoms in the upper extremity . therefore , a spinal cord lesion in a level higher than the l2 level was anticipated , and a t - l mri was performed to diagnose the avm . other symptoms that suggest an umn lesion are weakness , paralysis , spasticity , and muscle atrophy , but these symptoms were not observed in our patient . only the hyperactivity of the knee jerk reflex was observed , but if the babinski response ( the most sensitive indicator of the umn ) had been checked , it would have been more helpful in making the diagnosis . avm is the abnormal development of blood vessels which occurs during the 4th to 8th week of embryonic development when blood vessels are differentiated into arteries , veins , and capillaries . normally arteries and veins are connected by capillaries , but there is no true capillary bed in avm ; thus over time , a tangle is formed in the blood vessel connecting the artery and vein . therefore , oxygenated arterial blood is shunted and supplied directly into the venous system which can cause temporary or permanent ischemia in the surrounding tissue . avm can develop anywhere in the body and there can be various manifestations of symptoms according to the location and blood flow . usually , it develops in the limbs , neck , and face , and it can cause pain , congestive heart failure , and hypotension from vascular congestion and so on . cerebral avm can cause seizure or severe headaches , but mostly there are no symptoms . spinal avm has a prevalence of approximately 10% for cerebral avm and less than 5% for an intraspinal mass . as in this case , it usually occurs in middle - aged men in the lower thoracic or lumbar region . typical early symptoms are dermatomal lancinating pain , weakness / paralysis , numbness , and paresthesia [ 9 - 11 ] . spinal avm glomus - type ( type 2 ) with a tightly compacted group of arterial and venous vessels inside a short segment of the spinal cord , and usually , it is supplied by one artery . spinal avm is very rare with a prevalence of less than 1% , shows various clinical symptoms , and is difficult to differentiate from other diseases such as tumors , herniated nucleus pulposus , multiple sclerosis , meningovascular syphilis , tuberculosis , and transverse myelitis . however subarachnoid hemorrhage or spinal cord hemorrhage due to avm rupture and the subsequent acute neurologic deficit are increasing by 2 - 3% every year , and the mortality at first hemorrhage reaches 10 - 30% ; therefore , an accurate differential diagnosis is very important . additionally , in this case , the avm was discovered in the mri , but there are many cases which can not be detected by mri thus an angiography must be performed for diagnosis . in this case , spinal avm was accurately diagnosed through a detailed neurologic examination , and the appropriate treatment was given . therefore , when a patient complains of dermatomal neuropathic pain without a rash , a detailed and accurate physical examination should be performed before diagnosing zsh , and laboratory tests should be performed for the acute phase to differentiate it from other diseases that cause neuropathic pain .

zoster sine herpete ( zsh ) is difficult to diagnosis during an acute period due to the absence of the characteristic zosteriform dermatomal rash ; therefore , progression to postherpetic neuralgia is more common than typical zoster . in addition , misdiagnosis of other neuropathic pain as zsh is common in clinical situations . here , we report a case of spinal arteriovenous malformation that mimics zsh . this is a rare condition ; therefore , high clinical suspicion for a correct diagnosis and proper examination are not easy . however , early diagnosis and definitive treatment are essential to prevent neurologic deficit and mortality .

although common as an agent for suicidal or homicidal poisoning , these drugs are remarkably safe . deep coma is a rarity for benzodiazepine poisoning , with severity of central nervous system ( cns ) depression depending on the dose , the age of the patient and his or her clinical status . treatment of a certain case of acute benzodiazepine poisoning is with supportive measures and the administration of flumazenil . if a proper drug history is available , the diagnosis can be made with confidence . on the other hand , in the absence of a proper history , diagnosis depends on high index of a clinical suspicion and reliance of laboratory parameters , which are most commonly nonchalant . we present a case of acute benzodiazepine poisoning without any guiding history of drug intake , which was successfully diagnosed and treated . magnetic resonance imaging ( mri ) of the brain revealed peculiar symmetrical isolated bilateral globus pallidus t2 hyperintensity ( bgpth ) without any other focal lesion or altered signal anywhere else . we believe this to be the first report of such a peculiar mri finding in the setting of acute benzodiazepine poisoning from india . in july 2010 , a 52-year - old male was admitted in an unarousable state . his relatives found him in this condition the morning before , when he was apparently sleeping past his normal waking time . there was no history of any drug intake in the recent or distant past . on immediate evaluation , a quick neurological examination revealed an unconscious state with absent vestibule - ocular reflexes , unresponsive plantar reflexes with generalised hypotonia and depressed deep tendon reflexes . systemic examination was entirely nonchalant except for the fact that the patient appeared to snore prominently . arterial blood gas showed a normal ph ( 7.38 ) with , pao2 of 96 mmhg , paco2 of 41 mmhg with normal bicarbonate ( 23 mmol / l ) and anion gap ( 12 meq / l ) . while collecting the gastric lavage samples for toxicological purposes , a plain computed tomography ( ct ) scan of the brain was done , considering the patient was in neurological shock following a cerebrovascular accident , which came to be absolutely normal . the complete hemogram , liver and renal function tests and electrolytes were normal ( hb 11.6 gm / dl , total leucocyte count 7600/l [ neutrophil 73% ] , creatinine 0.9 mg / dl ) . awaiting the reports of the gastric lavage samples and without any improvement ( or deterioration of the patient ) , an mri brain with contrast was done , which revealed bgpth [ figure 1 ] . among the common causes of bgpth ( discussed below ) , carbon monoxide poisoning and the encephalopathies were excluded by clinical setting and cyanide and h2s poisonings were excluded by the absence of lactic acidosis ( blood lactate 0.6 magnetic resonance imaging of the brain of the patient showing bilateral t2-weighted globus pallidus hyperintensities ( indicated by arrows ) therefore , we were facing an unconscious patient who was snoring with borderline carbon dioxide retention and had a bgpth inexplicable by common causes . keeping in mind that benzodiazepines sometimes cause coma , snoring and obstructive apnea , we screened the patient 's urine sample . the patient or his relatives could not give any reference regarding the source of benzodiazepine he was not used to taking benzodiazepine as sedative or hypnotic . presence of benzodiazepine in urine and absence of similar past history ruled against endozepine4 stupor syndrome . the patient was given a trial dose of flumazenil ( 500 g iv ) , to which he dramatically responded with almost full recovery of consciousness level . this was followed by a therapeutic dosage of flumazenil ( 500 g iv up to 2 mg ) . the patient recovered uneventfully by the second day of therapy and fifth day of admission , and was discharged the next day . the patient had been on regular follow - up for the past 1 year and did not show any neurological symptoms . the severity of the cns depression is influenced by the dose , the age of the patient and his or her clinical status prior to the ingestion . a large retrospective study including 702 benzodiazepine poisoning cases found that 56% had severe cns depression , with 47% requiring orotracheal intubation and 18% requiring artificial ventilation . are carbon monoxide , cyanide , hydrogen sulfide , methanol , disulfiram , osmotic myelinolysis , uremic or hepatic encephalopathies , radiation therapy or cerebrovascular accidents . there is only a single report of a 62-year - old patient with suicidal olanzapine poisoning with choreoathetosis and mri of brain showing bilateral small hyperintense foci in globus pallidi . in this regard , it is important to remember that bgpth may occur as part of the normal ageing process , especially after the age of 60 years , and it is rare in younger people . in the absence of a definite history of drug intake , an unconscious elderly man becomes a diagnostic nightmare . three clinical pointers helped us crack our dilemma snoring , carbon dioxide retention and bgpth . we would like to emphasize that poisoning is an important differential in similar clinical scenarios , even without any definite drug history . a high index of clinical suspicion and urine screening tests become of paramount importance for diagnosis . this report emphasizes the importance of a high index of clinical suspicion for proper diagnosis . we believe this to be the first report of such a peculiar mri finding in the setting of acute benzodiazepine poisoning from india .

benzodiazepine is a commonly encountered agent of poisoning , which is readily diagnosed by a pertinent history of drug ingestion and the clinical scenario . in the absence of a proper drug history , the diagnosis becomes challenging . proper clinical assessment , urine assays and imaging play a very important role in reaching the diagnosis . we present a case of acute benzodiazepine poisoning without a history of drug intake . the key pointers toward diagnosis were an unarousable state with obstructive apnea . magnetic resonance imaging ( mri ) of the brain revealed peculiar symmetrical isolated globus pallidus t2 hyperintensity . we believe this to be the first report of isolated bilateral basal ganglia t2-weighted hyperintensity in mri in the setting of acute benzodiazepine poisoning from india .

perilunate fracture dislocations display a continuum of severity , but trans - scaphoid , trans - styloid , trans - triquetral perilunate fracture dislocations are rarely reported in the literature . a 19-year - old student presented with pain and swelling of the wrist , 4 h after crashing his bicycle . radiographs revealed fractures of the radial and ulnar styloids , the scaphoid waist and triquetrum , along with a perilunate dislocation ( figure 1 ) . he continued to complain of pain and numbness in the median nerve distribution and so underwent emergent carpal tunnel release . he returned to the or for definitive fixation 1 week later , when the swelling had settled . note the dorsal dislocation of the capitate and the volar rotation of the lunate on the lateral radiograph . note the absence of joint space around the lunate as well as superimposition of the capitate on the pa radiograph . the distal radius fracture was fixed with a synthes 2.4 mm variable angle two - column plate through an extended fcr approach . a standard volar scaphoid approach then confirmed significant comminution of the scaphoid at the fracture site and a cartilage defect on the capitate head . good compression was achieved with this screw , within the limitations of the bone loss . the wrist was screened with the image intensifier in movement , and the scaphoid was seen to be moving as 1 unit . at this point , the overall stability of the wrist was reassessed , and it was found that there was still a significant degree of mid - carpal instability resultant from the triquetral fracture . crossed 1.25 mm k - wires were , therefore , inserted through the triquetrum into the lunate and through the triquetrum , hamate and into the capitate to stabilize the ulnar side of the mid - carpal joint . following insertion of these wires , the pronator quadratus was repaired with interrupted 4 - 0 monocryl , and then the skin incision was closed with subcuticular 4 - 0 monocryl . the wound was dressed with steri - strips , telfa , gauze , webril , and a volar plaster thumb spica slab . the patient was again seen at 8-weeks postop with follow - up x - rays that showed a united scaphoid fracture ( figure 2 ) . after the postoperative cast was removed , the patient completed five visits of physical therapy with a certified hand therapist . the patient progressed to weighted range of motion exercises using free weights and resistance bands . perilunate fracture dislocations usually occur from a fall on an outstretched hand leading to hyperextension and supination of the carpus in relation to a pronated forearm . failure begins with the radial aspect of the wrist , including the radial styloid , scaphoid waist , proximal pole of the scaphoid , or scapholunate joint . as the energy moves distal and ulnar , the capitate body may fracture or disruption of the capitolunate joint can occur . next , injury to the base of the hamate , the triquetrum , or the lunotriquetral joint takes place . finally , the energy exits ulnarly and fracture of the ulnar styloid may be present . the energy propagated through the scaphoid and continued through the greater arc injuring the radial collateral ligament . it continued by creating an avulsion fracture of the triquetrum via the palmar ulnotriquetral ligament and finally exited through the ulnar styloid . rather than identifying ligamentous structures that were injured through soft tissue dissection , perilunate fracture dislocations frequently go undiagnosed ( up to 25% ) in the emergency department which leads to delayed treatment . a reason for under diagnosis is poor radiographic images and lack of scrutiny on the part of the reader . the arcs of gilula should be noted on the pa view , while alignment on the lateral view should be determined . these injuries are difficult to manage with only 50% of patients achieving excellent mayo scores after fixation . the study by capo et al . found the average flexion extension arc was 82 , forearm rotation was 155 , and grip strength averaged 59% of the uninjured hand using a combined volar and dorsal approach . showed that percutaneous screw and k - wire fixation in acute transcaphoid perilunate dislocations demonstrated impaired flexion and supination when compared to the contralateral , normal wrist . at an average of 58-weeks postop , massed revealed a 52% flexion extension arc in comparison to uninjured controls in chronic perilunate injuries ( mean of 29 weeks from injury ) . operative transcaphoid perilunate dislocations had better outcomes with regard to mayo wrist scores and range of motion compared to nonoperative management . common problems such as loss of motion and decreased grip strength can persist despite appropriate operative fixation . however , forli et al . showed that the presence of radiological arthritis and static carpal instability did not reduce the function at a minimum of 10 years of follow - up . osseous and ligamentous injury severity in fracture dislocations about the wrist has led several authors to recommend open reduction and internal fixation . we had excellent results by 4-month follow - up , achieving a painless wrist with good rom and strength ( figure 3 ) . our patient s injury differed from the majority of other reports due to the transstyloid radius and ulna components . reported a good result after a similar injury that required external fixation and carpal pinning . concluded that reduction of the scaphoid and mid - carpal joint , as well as restoring the articular surface of the lunate led to full , pain - free motion . obtained a pain - free outcome that lacked normal range of motion at 6 months . in the series by herzberg , they demonstrated fixation of the scaphoid alone without addressing the ligamentous injury led to further dissociation , translation , and other carpal collapse patterns . additionally , raab et al . showed good results in professional athletes with purely ligamentous perilunate injuries fixed with k - wires at 5-year follow - up . we have shown that an excellent clinical outcome can be achieved with prompt orif of osseous structures and stabilization of the wrist ligamentous injury in this fracture dislocation pattern .

abstracttrans - scaphoid , trans - radial styloid , trans - triquetral perilunate fracture dislocations are rare . we describe a 19-year - old male who suffered this injury after crashing his bicycle . he underwent open reduction internal fixation and percutaneous pinning . scaphoid union was achieved at 8 weeks . near complete range of painless motion was achieved by 4 months .

potassium - titanyl phosphate ( ktp ) laser vaporization of the prostate for treating symptomatic benign prostatic hypertrophy has recently proven to be efficacious with minimal patient morbidity . ktp laser vaporization can create an immediate cavity almost bloodlessly , with the added benefit of little - to - no learning curve and the prospect of successful , same - day , catheter - free discharge . however , longer follow - up studies or reports about possible complications are lacking . dystrophic calcification results from the deposition of calcium in soft tissues despite that there is no generalized disturbance in the calcium or phosphorus metabolism ; this malady is often seen at sites of previous abnormalities or damage . the entity of dystrophic calcification is well described in the rheumatological and dermatological literature , and especially in the setting of dermatomyositis and scleroderma ( 1 ) . we report an unusual case of dystrophic calcification of the bladder neck after performing ktp laser vaporization of the prostate . a 78-yr - old man was presented with 1-yr history of dysuria , frequency and urgency . he had received ktp laser vaporization of the prostate at another hospital 16 months ago . according to past medical history postoperative care had lasted 7 days , which was longer than usual , and then he had been discharged . the laboratory results showed a plasma calcium level of 9.3 mg / dl and a plasma phosphorus level of 4.7 mg / dl , which were both within the normal ranges . the urinary sediment contained 10 - 29 white blood cells / high power field with 5 - 9 red blood cells / high power field , and bacterial culture of the urine was sterile . transrectal ultrasonography of the prostate showed prostate volume of 9 ml and there was diffuse calcification around the internal urethral sphincter ( fig . the cystoscopic findings revealed stones located in the bladder neck , and these were lodged into the previous surgical site . the entire bladder neck was encircled by stones and even in the mucosa of the bladder neck . the stones were so tightly lodged into the mucosa of the bladder neck that they could n't be disintegrated by performing lithotripsy in situ . therefore , transurethral resection was performed on the mucosal - lodged stones and the calcified tissues ( fig . hematoxylin and eosin staining of the surgical specimen showed extensive basophilic staining that was suggestive of calcification within the fibrotic tissues ( fig . foley catheter was removed on the postoperative 4th day and uroflowmetry showed the peak / mean velocity to be 15/11 ml / sec and the self / residual urine volume was 210/30 ml . the bladder irritative symptoms completely disappeared by the postoperative 4 months follow - up . it often occurs in conjunction with such collagen vascular diseases as dermatomyositis , scleroderma and systemic lupus erythematosis ( 1 ) . there have been reported cases of calcium deposition secondary to burn wounds , repeated trauma , needle sticks in the heels of neonates , thumb sucking by children , radiation and surgery ( 2 - 4 ) . although rarely seen in the genitourinary tract , it has been associated with schistosomiasis ( 5 ) , renal and ureteral tumors ( 6 ) , renal parenchymal disease ( 7 ) , churg - strauss vasculitis ( 8) , and polyarteritis nodosa ( 9 ) . however , there have been no reports of dystrophic calcification and stones formation on the bladder neck associated with previous ktp laser vaporization of the prostate . all mammalian extracellular fluids are supersaturated with calcium phosphate , but inhibitors such as matrix gla protein , oseoprotegrin and osteopotin prevent crystal deposition under normal physiological conditions ( 10 , 11 ) . damaged cell membranes leak calcium ions into cells , and these ions are subsequently concentrated by mitochondria to levels that are high enough to form crystals ( 2 , 4 ) . it has also been suggested that necrosis creates an acidic environment that lacks calcification inhibitors ( 12 ) . hydroxyapatite crystals are formed first within the protective microenvironment of the membrane microspace . in rare cases , dystrophic medical therapy , including administering etidronate disodium , sodium warfarin , diltiazem , aluminium hydroxide and intralesional corticosteroids , is the first - line treatment ( 1 ) . the indications for surgery are the presence of painful masses , recurrent infection , ulceration , local functional impairment and cosmetic motivation ( 13 ) . in the present case , ktp laser vaporization of the prostate seems to combine the tissue - debulking properties of transurethral resection of prostate ( turp ) with the well - known hemostatic properties of other laser treatments . it is a safe and relatively bloodless procedure that results in a shorter duration of catheterization , immediate symptomatic improvement and less severe postoperative irritative symptoms . however , concerning the efficacy and safety of this system , the majority of the studies about this deal with the short term results , which were excellent , but longer follow - up studies or reports about complications have been lacking . bachman et al . conducted animal studies using ex - vivo , blood - perfused porcine kidneys , and they compared the hemostatic efficiency of ktp laser vaporization with turp - like tissue resection . the authors showed the zone of coagulation necrosis was larger in the ktp group compared with the conventional turp group ( 0.9 vs. 0.6 mm , respectively , p<0.01 ) and ablation of 16 cm of tissue was accomplished much faster in the turp group ( 20 vs. 100 sec , respectively , p<0.0001 ) ( 14 ) . in this case , the patient had no systemic imbalances of calcium and phosphate metabolism and had n't received any procedures or surgeries that used calcium solution . so , the development of the bladder neck calcification was likely to be due to dystrophic calcification that was caused by the previous operation . additionally , the larger coagulation zone and longer operation time , compared to turp , might have caused relatively more severe injury of the bladder neck and thus promoting dystrophic calcifications .

dystrophic calcification can be defined as a calcification that occurs in degenerated or necrotic tissue . it is associated with multiple clinical conditions , such as collagen vascular diseases . it involves the deposition of calcium in soft tissues despite no generalized disturbance in the calcium or phosphorus metabolism , and this is often seen at sites of previous inflammation or damage . potassium - titanyl phosphate ( ktp ) laser vaporization of the prostate is safe and relatively bloodless procedure that results in a shorter catheterization , immediate symptomatic improvement , and less severe postoperative irritative symptoms . however , longer follow - up studies or reports about complications are lacking . here in we report a case of dystrophic calcification and stone formation on the entire bladder neck after performing ktp laser vaporization of benign prostate hyperplasia . that was treated by lithotripsy and transurethral resection .

we report involvement of unusual site in ilio - acetabular region with breach in articular cartilage . intra - operatively breach in acetabular roof was seen which was missed in the scan . this case report emphasizes about the rarity of the lesion in this location and the rare chances of breach in articular cartilage of the joint . bone desmoid tumor was an unknown entity until when jaffe hl reported a case in 1958 . it is a locally aggressive benign tumor reported commonly in mandible and meta - diaphyseal region of long bones . we report a case of desmoid tumor of ilio - acetabular region in a 40 year old female . a 40 year old female came with complaint of pain in the left hip region which aggravated on squatting and climbing stairs . radiological evaluation showed a lytic lesion in the left ilio - acetabular region , surrounded by a sclerotic margin superiorly . mri scan was done which showed a well defined homogenous lytic lesion with no break in the cortex and no soft tissue involvement ( fig . plain radiograph of pelvis with both the hips which shows a well defined lytic lesion over the left ilio - acetabular region . there appears no obvious breach in the cortex or the articular margin a ct scan of the pelvis was taken to rule out any breach in the cortex . there appears no obvious discontinuity through an ilio - inguinal incision the lesion was approached through inner table of ilium . a defect on the roof of the acetabulum was found which was missed by the scan ( fig . thorough curettage was done and the roof defect was reconstructed with a cortical graft harvested from the inner table of the ilium . there appears a hollow curetted lesion within which a breach in the roof of the acetabulum is seen histopathological finding showed multiple spindle shaped fibroblast cells with small and elongated nuclei in the background of dense collagen fibres . histopathology specimen picture showing spindle shaped fibroblast cells with small and elongated nuclei over a background of collagen fibers . there appears no nuclear atypia or mitotic activity patient was kept non weight bearing for 3 months with gradual return to full weight bearing walking . at follow up of 20 months patient is symptom free and xray showing no evidence of lytic lesion ( fig 5 ) . however , on ct scan there appears a persistent lytic area which we suspect to be a recurrence ( fig 6 ) . hence patient is kept on regular follow - up . a 20 month post operative x ray picture showing well maintained articular margin and the joint space . the lytic lesion seems filled up ct scan of the hip taken at 20 month post op , however shows lytic lesion anteriorly which seems to be a recurrence few cases have been reported since then and common occurrence being in mandible and meta - diaphyseal region of the long bone . recognition of desmoplastic fibroma is important because on radiology and histology , the lesion may be mistaken for an indolent , benign fibrous lesion or more aggressive spindle - cell sarcomas . it commonly occurs in the age group between the adolescent to 40 years with no specific sex predominance . patients commonly come with complaint of pain ; however there are few reported cases of pathological fracture especially those involving tibia and femur . the lesions are well defined with no sclerosis or periosteal reaction except if there is associated pathological fracture . if a soft tissue lesion is invading onto the bone , they are eccentric in position with sclerosis due to endosteal bone formation . significant t2 shortening of a non - sclerotic fibro - osseous lesion should place desmoplastic fibroma high among the diagnostic considerations . radiologically they have to be differentiated from giant cell tumor , fibrous dysplasia ( long bone lesions ) , fibrosarcoma , brown tumors , and chondromyxoid fibroma and hence a tissue diagnoses is must . the differentiation from a low grade fibrosarcoma is difficult though . as these are rare tumors , there is no standard protocol for treatment of these lesions . all kinds of procedures like intralesional , marginal or wide resection are reported as a treatment . but if wide resection would result a major functional deficit , an attempt of intralesional curettage with bone grafting seems warranted . desmoid fibroma of ilio - acetabular region is very rare hence a great sense of suspicion is required for the appropriate management . this case report emphasizes about the rarity of the lesion in this location and the chances of breach in articular cartilage of the joint . desmoid tumor , though rare , should be kept as one of the important differentials for the lytic lesion around pelvis . one should also define whether there is any associated cortical or articular margin breach ( as seen in our case report ) and then decide further management regarding reconstruction .

introduction : desmoid tumor of bone is a rare benign tumor . it is reported commonly in mandibular and meta - diaphyseal region of long bones . we report involvement of unusual site in ilio - acetabular region with breach in articular cartilage.case report : a 40 year old female presented with pain in the left hip . radiologically , a lytic lesion at ilio - acetabular region was seen . intra - operatively breach in acetabular roof was seen which was missed in the scan . curettage and defect reconstruction was done . histopathology reported as desmoid tumor . 20 months post - operatively patient was symptom free.conclusion:desmoid tumor is a rare bone tumor . this case report emphasizes about the rarity of the lesion in this location and the rare chances of breach in articular cartilage of the joint . the chances of recurrences are high with intralesional curettage .

the pemphigus diseases , which include some of the most severe bullous autoimmune skin reactions , are seen predominantly in middle - aged and elderly individuals . all nonendemic pemphigus diseases , including paraneoplastic pemphigus , have been reported to occur in adolescents and even very rarely in children younger than 10 years . pemphigus herpetiformis ( ph ) is considered a variant of pemphigus displaying clinical features similar to dermatitis herpetiformis and a diverse histopathologic pattern with intraepidermal and subcorneal microabscesses , eosinophilic spongiosis , or superficial bullae with usually scant acantholytic cells [ 1 , 2 ] . the diagnosis is based on detection of ig g antikeratinocyte cell surface antibodies , both bound in vivo and in circulation . in the literature , few cases of pemphigus are reported in children . this rarity in childhood may be only apparent as a result of its difficult diagnosis . an 12-year - old boy was seen at our department in march , 2007 , with erosive plaques , vesicles , bulls , and crusted lesions associated with severe itching persisting for six months . the boy 's family history and personal history were unremarkable , and his growth and development had proceeded normally . on examination , we observed vesicular , bullous lesions , some having clear contents and some being purulent with erosive arciform plaques and crusted lesions ( figures 1(a ) and 1(b ) ) . they were seen especially over the back , buttocks , chest , abdomen legs , and arms . histologic examination of one of the lesions showed an intraepidermal bulla containing rare acantholytic epidermal cells , eosinophil and neurophil cells . the superticia1 and reticular dermis had an inflammatory infiltrate of eosinophile and neutrophile around some of the blood vessels ( figures 2(a ) and 2(b ) ) . iga intercellular deposits on epidermis were not seen ( figures 3(a ) and 3(b ) ) . a diagnosis of ph was made , and treatment with dapsone 2 mg / kg per day resulted in total clinical remission . however , two months later , new vesicles reappeared and treatment was begun with prednisone at a dose of 2 mg / kg daily . there was a very good response ; lesions regressed slowly , until they completely disappeared after 4 weeks of treatment . the daily dose of the prednisone was lowered gradually according to the clinical improvement , and , at the time of this report , one year after onset of the disease the skin involvement was being kept under control on a low maintenance dose of 10 mg of prednisone daily . autoimmune blistering diseases are extremely rare in children . a review from a paediatric dermatology referral center for a population of 4 million people identified only 23 cases of immunobullous disease in patients less than 18 years of age over a 16-year period . a recent review of pemphigus vulgaris ( pv ) in children found only 46 cases reported in the literature . pemphigus herpetiformis ( ph ) was first introduced by jablonska and colleagues as a variant of pemphigus . various terms have been used to describe this condition , such as acantholytic herpetiform dermatitis , pemphigus controlled by sulfapyridine , and mixed bullous disease . clinical manifestations consist of erythematous , urticarial plaques , and vesicles that present in herpetiform arrangement . histological findings of hp are variable and include eosinophilic spongiosis and subcorneal pustules with minimal or no apparent acantholysis [ 9 , 10 ] . immunofluorescence findings show that most patients with ph have igg antibodies against keratinocyte cell surfaces . dapsone is the first choice of drug in the ph treatment given as monotherapy or combined with systemic corticosteroids . in most reported cases , the use of systemic corticosteroids was necessary to achieve clinical remission . in some cases , however , the use of immunosuppressive drugs such as azathioprine and cyclophosphamide becomes necessary to induce clinical remission or to help in reducing steroid dose so that its collateral effects are minimized . based on its clinical features , some researchers reported the disease as a distinct entity and consider it to be different from classic pemphigus because of its clinical peculiarity and benign course . however , others described it as a variant of pemphigus foliaceus ( pf ) or pv . we believe the boy presents a pemphigus herpetiformis variant because of the clinical features of dermatitis herpetiformis ( vesicles , bulls , and itching ) and the immunologic ( igg and c3 deposit on intercellular epidermal ) and histological features ( intraepidermal bulls and acantholysis ) of pemphigus with an eosinophilic spongiosis . various types of pemphigus including vulgaris , foliaceus , erythematosus , vegetans , and paraneoplastic pemphigus can be seen in childhood . the disease must not be forgotten as a possible cause of erosive mucocutaneous disease in children . it is important that antibody titers , management plans , and outcomes for this disease be reported so that greater knowledge of the effects of various treatment regimens can be obtained .

pemphigus herpetiformis ( ph ) is one of the less common forms of pemphigus . ph in children is unreported . we describe a case of a child who developed ph . observation . a 12-year - old boy was seen at our department with erosive plaques , vesicles , and crusted cutaneous lesions associated with severe itching persisting for six months . histologic examination showed an intraepidermal bulla containing rare acantholytic epidermal cells with eosinophilic spongiosis . direct immunofluorescence demonstrated intercellular ig g and c3 deposit . the serum titer of antibodies against intercellular epidermal was 1/200 ui / l . diagnosis of ph was made , and treatment with dapsone 2 mg / kg per day resulted in total clinical remission . however , two months later , new vesicles reappeared and treatment was begun with prednisone at a dose of 2 mg / kg daily . there was a very good response . discussion . childhood pemphigus herpetiformis is a rare disease , often initially misdiagnosed . it must not be forgotten that the disease is a possible cause of erosive mucocutaneous disease in children .

radiopaque objects such as teeth , metals , and gravels are easily detectable in the radiograph sometimes , advanced imaging modalities such as computed tomography ( ct ) , and magnetic resonance imaging ( mri ) are required to correctly detect and locate the position of foreign bodies . in many instances , ct and mri may not be available in the dental office and hospitals for the detection of foreign bodies . in such situations , it is important to use various available conventional radiographic techniques for the localization of foreign bodies . plain film radiography is the initial screening modality used for the detection of suspected foreign body . in the case of a wooden foreign body , only 15% are well - visualized on plain radiographs and are therefore often missed or misdiagnosed . hence , different radiographic technique should be used to localize the object instead of relying on a single radiographic image . this unique case illustrates the importance of using different radiographic modalities to detect the presence of retained foreign bodies using conventional radiographic technique . a 35-year - old male patient , a carpenter by profession reported to our department with swelling and pain on the left mandible since 3 weeks [ figure 1 ] . he met an accident 1 month before while polishing the furnished wood in the workplace and lost his consciousness due to hit of the wooden piece on the face during the incident . he had taken to the primary health centre by the coworkers and received the emergency care . after 1 week swelling formed on the left mandible with difficulty in opening mouth and associated pain . extra orally , a vertically placed wound scar with suture marks of approximately 6 cm in length is seen extending from a line joining the ala - tragus line toward the lower border of mandible [ figure 2 ] . swelling over a digital panoramic radiography was taken , which showed multiple faint mixed radiopaque radiolucent appearances in the periapical region of mandibular left first permanent molar and second molar with a radiolucent line in between giving the radiographic feature of osteomyelitis and fracture of the jaw [ figure 3 ] . on careful examination , tiny multiple radiopaque spots are seen on the radiograph within the area of interest having a glittering appearance . to assess whether any breach in the lower cortex and also to ascertain what exactly the glittering thing was , a mandibular occlusal radiograph was taken . in the occlusal radiograph , well - defined multiple linear radio opaque structure of approximately 4 cm long and 5 mm wide not contacting the bone was detected [ figure 4 ] . with the patient under general anesthesia , the involved area was explored intraorally and multiple pieces of the fractured polishing disc were retrieved [ figure 5 ] . the patient had an uneventful recovery in the postoperative period with improvement in mouth opening and reduction of swelling and pain in the left mandible [ figures 68 ] . a 35-year - old male patient presented with swelling on the left mandible wound scar and suturing mark seen on the left part of face digital panoramic radiography showing mixed radiopaque radiolucent appearance on the root apex of mandibular left first molar and second molar mandibular occlusal radiography showing well defined linear radiopacity not contacting the bone surgical removal of retained foreign body under general anesthesia retrieved pieces of wooden polishing disc retrieved pieces of polishing disc postoperative digital panoramic radiography after the removal of foreign body this unique case is an example illustrating the variations seen in the appearance of retained foreign bodies in different radiographic images . the retained wooden foreign body was initially missed in the digital panoramic radiograph and its radiographic appearance was misinterpreted . since wood is an organic material with low density only 15% of wooden foreign bodies plain radiographs are almost unable to detect their presence in soft tissues unless they are associated with a radiopaque substance . retained foreign body retrieved from the patient was a polishing disc made of wood with small particles of lead incorporated in it . this might be penetrated deep into the vestibule extra orally during the accident and the left sutured in the place from the primary health centre . the detection of wood is , especially important because it can serve as an unrecognized medium for infection . wood , with its porous consistency is an excellent medium for microorganisms , and the retained wooden foreign matter may result in cellulitis , abscess , or fistula formation . the wooden foreign matter may also result in synovitis or osteomyelitis if joint or adjacent osseous structures become involved . history and clinical presentation of the patient gave the impression of mandibular fracture , and the digital panoramic radiograph was justifying the diagnosis with radiographic appearance mimicking osteomyelitis . incorporated lead in the disc giving the glittering appearance and the possibility of fracture made us to think about exposing the region with occlusal radiography [ figure 9 ] . our case also highlights the limitations of panoramic radiography in such situations due to magnification and low resolution when compared with intraoral radiographs . in this case , occlusal radiography provided the resolution needed to characterize the borders and internal structure of the wooden particle accurately . radiograph of wooden polishing disc showing glittering appearance due to incorporated lead particles a radiologist must be aware of various radiographic appearances of foreign bodies [ figure 10 ] . plain film radiograph is a two - dimensional representation of three - dimensional object , a radiologist can derive three - dimensional information about the object using various radiographic methods . sometimes , simple conventional radiographic technique will be quite effective in the localization of such foreign bodies . it should be mandatory to screen the patients with suspected injuries using different radiographic techniques to rule out the presence of retained foreign bodies . posterior - anterior radiograph of retained foreign body in the left mandible region foreign body entrapment may be common , but the uniqueness lies in the nerve wrecking task of incorporating the wide spectrum of unimaginable materials that could cause the entrapment . the nature of the materials could be challenging even for a seasoned radiologist to apply the right permutations and combination of radiographic imaging modalities to overcome the clinical and radiological diagnostic dilemma . sometimes simple conventional radiographic technique will be quite effective in the localization of such foreign bodies . it should be mandatory to screen the patients with suspected injuries using different radiographic techniques available to rule out the presence of retained foreign bodies . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . a radiologist must be aware of various radiographic appearances of foreign bodies . sometimes simple conventional radiographic technique will be quite effective in the localization of such foreign bodies . it should be mandatory to screen the patients with suspected injuries using different radiographic techniques available to rule out the presence of retained foreign bodies . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .

exact localization of foreign body is important for planning the treatment required for its retrieval without much tissue damage . plain film radiography is the initial screening modality used for the detection of suspected foreign body . however , about one - third of all the foreign bodies are missed in the initial radiographic examination . in case of a wooden foreign body , only 15% are well - visualized on plain radiographs and are , therefore , often missed or misdiagnosed . hence , the different radiographic technique should be used to localize the object instead of relying on a single radiographic image . here , we present a unique case of foreign body entrapped in the soft tissue appeared initially as osteomyelitis and fracture of the mandible in the digital panoramic radiograph , and finally detected as a foreign body in mandibular occlusal radiography .

cocaine is a potent stimulant of the central nervous system which causes vasoconstriction and can damage all organ systems . it blocks presynaptic sympathetic reuptake of norepinephrine ( ne ) , causing accumulation in synaptic clefts . ne induces alpha - adrenergic stimulation and vasoconstriction which is the underlying culprit in organ damage . we herein present a case of cocaine - induced splenic rupture with a review of the literature . a 39-year - old white male with history of cocaine and heroin abuse , presented to the emergency department with chief complaint of left flank pain . physical exam revealed the patient to be stable , alert and oriented , however , diaphoretic . laboratory data showed leukocytosis of 15 000 and a hemoglobin and hematocrit of 12.6 g / dl and 38.5% . contrast enhanced ct of the abdomen and pelvis showed blood extravasating from the superior aspect of the spleen with a large hemoperitoneum ( fig . the patient then underwent ct angiography with coil embolization of the proximal splenic artery ( fig . 2 ) . the repeat hemoglobin revealed a drop to 6.0 g / dl , but responded to transfusion . . figure 2:splenic artery angiogram after placement of embolization coil showing patency of flow , no active extravasation . splenic artery angiogram after placement of embolization coil showing patency of flow , no active extravasation . repeat ct showed a collection in the left upper quadrant with partially necrotic areas within the spleen ( fig . figure 3:the islands of preserved spleen lit up with iv contrast surrounded by a large collection of old blood and devitalized tissue . the islands of preserved spleen lit up with iv contrast surrounded by a large collection of old blood and devitalized tissue . the patient then underwent ct guided drainage of the collection which was positive for methicillin - sensitive staphylococcus aureus ( fig . splenic rupture is a rare but serious complication from cocaine abuse . given the ubiquitous prevalence of abuse and the potential for death from intraperitoneal bleeding , the prompt diagnosis and treatment of cocaine - induced disease including splenic rupture is essential . cocaine attains high concentrations within the spleen and the associated vasoconstriction has been shown to transiently reduce splenic volume on average by 20% . splenic hemorrhage may occur from arteriolar rupture from accelerated hypertension or from infarction and subsequent hemorrhage following resolution of vasospasm [ 1 , 3 ] . the current management for the majority of splenic injury is a non - operative approach with adjunctive transcatheter splenic artery embolization ( tsae ) which is effective for non - operative management in 90% of blunt splenic trauma two general methods for tsae of splenic injuries have been advocated . one option is coil embolization of the proximal splenic artery , which decreases arterial pressure in the spleen but allows continued perfusion through collateral arteries . the other method is superselective embolization of the bleeding arterial branch . to arrest the hemorrhage from splenic injury and to minimize infarct due to embolization , it is important to select appropriate method of tsae on the basis of the features of arterial injuries [ 46 ] . our patient was managed with coil embolization of the proximal splenic artery and subsequently maintained partial perfusion of splenic tissue . a study conducted over an 11-year period reports that minor complications of fever , left - sided pleural effusions , and coil migration occur in 34% of patients while major complications of splenic abscess , infarction , cyst , and contrast induced renal insufficiency occur in 14% of patients who undergo tsae . of the patients in the study , of patients with major complications had undergone distal embolization , defined as distal to the main splenic artery trunk . during the post - procedure course , our patient was noted to have minor complications of fever and left - sided pleural effusion as well as a major complication of intra - abdominal abscess . it is generally recommended that patients who undergo emergent splenectomy receive pneumococcal , meningococcal and h. influenzae ( hib ) vaccinations 2 weeks postoperatively , however , the guidelines are unclear regarding post- tsae patients . 99.2% of active trauma surgeons immunize their postsplenectomy patients , whereas only 15.4% of surgeons immunize those who undergo splenorrhaphy and 8.4% immunize patients who are managed nonoperatively . it has been proposed that the decision to vaccinate be based on splenic function post - tsae . based on ct volumetric analysis , coil embolization of the main splenic artery resulted in only a mild degree of splenic volume loss over the long term , with a mean of 15% decrease in volume . patients that underwent distal tsae demonstrated a 21% volume reduction while patients with proximal tsae demonstrated a 4% reduction . our patient was vaccinated given that post - procedure ct showed the spleen to be partially necrotic with only two islands of well - perfused , preserved spleen depicting > 15% decrease in splenic volume .

abstractsplenic rupture is a rare but serious complication from cocaine abuse . given the ubiquitous prevalence of abuse and the potential for death from intraperitoneal bleeding , the prompt diagnosis and treatment of cocaine - induced disease including splenic rupture is essential . the management for splenic rupture from traumatic and atraumatic etiology has shifted from emergent laparotomy and splenectomy to non - operative approach with transcatheter splenic artery embolization . we report a 39-year - old male with a significant substance abuse history who presented with atraumatic splenic rupture . he was managed nonoperatively with adjunctive transcatheter splenic artery embolization . his post - procedure course was complicated by an intra - abdominal abscess requiring drainage via interventional radiology guided pigtail catheter placement and intravenous antibiotics . this case report is intended to raise awareness of the potentiating effects of cocaine use in this patient population and highlight questions raised during this patient 's management .

gastrointestinal stromal tumors ( gists ) , which arise primarily in the gut wall , are most commonly mesenchymal neoplasms closely related to the interstitial cells of cajal . gists typically carry gain - of - function mutations in genes encoding the kit receptor tyrosine kinase ( cd117 ) or platelet - derived growth factor receptor a , both of which are involved in cell survival , development and proliferation . although gists can arise at any location in the gastrointestinal tract , they are found most often in the stomach ( 60 - 70% ) , followed by the small intestine ( 20 - 30% ) , colon and rectum ( 5% ) , and esophagus ( < 5% ) . in most cases in the stomach , gists show the appearance of a submucosal tumor , with a broad base and smooth surface within normal gastric mucosa , and sometimes have central delle or depression . in larger cases , central ulceration or necrosis presenting with gastrointestinal bleeding the gist of the stomach described in this report was a rare case , with narrow stalk - like based , uneven protruding mass presenting with severe acute anemia despite small size . a 56-year - old woman was admitted to fujita health university with epigastric pain and nausea during the previous one month . laboratory evaluation on admission showed severe anemia ( red blood cell count 138 10/l , hemoglobin level 4.3 g / dl , hematocrit 13.7% ) ( table 1 ) . esophagogastroduodenoscopy revealed a narrow stalk - like based , hemorrhagic and uneven protruding lesion in the lesser curvature of the gastric upper corpus ( fig . 1 ) . endoscopic ultrasonography showed a low echoic mass , measuring 2.0 cm in diameter , within the fourth layer , suggesting that the tumor originated from the intrinsic muscle layer , and showed growth to the mucosal side ( fig . the labeling index ( li ) for mib-1 , determined by counting positively stained nuclei among 1,000 tumor cells , was 1% . although the histological findings suggested that the tumor was probably a benign gist , laparoscopy - assisted local resection was performed after 2 weeks because the patient had continuous severe anemia and epigastric pain . the resected tumor was 1.8 1.5 1.0 cm in size within the locally resected surgical speciemen . the tumor was elastic but hard in consistently , and its surface was uneven and irregular ( fig . the tumor cells originated from the intrinsic muscle layer and showed growth to the mucosal side , cropping out to the surface in most areas of the protruding lesion . histological assessment revealed that the tumor was composed of elongated spindle - like cells , containing rounded or oval , relatively uniform nuclei without apparent atypia ( fig . the li for mib-1 , determined by counting positively stained nuclei among 1,000 tumor cells , was about 1% ( fig . immunohistochemical staining of the tumor by the avidin - biotin peroxidase complex method showed that most of the tumor cells demonstrated immunoreactivity for kit ( fig . the tumor described in this report showed immunoreactivity for kit and cd34 , but not for asma , s100 and desmin , implying that the tumor was pathologically a gist with a lack of differentiation toward smooth muscle , and neural elements . most gists in the stomach generally show the appearance of submucosal tumors , with a broad base and smooth surface within normal gastric mucosa , and sometimes have small central delle or depression . in larger cases , central ulceration or necrosis presenting with gastrointestinal bleeding however , the gist of the stomach described in this report showed narrow stalk - like based , uneven protruding mass presenting with severe acute anemia despite the small size of less than 2 cm , and local resection was needed due to continuous severe anemia and epigastric pain . although the association between the patient 's gastric symptoms and this small submucosal tumor is not fully understood , the considerable amount of blood in the stomach due to continuous bleeding may have led to the gastric symptoms such as epigastric pain and nausea . histological assessment showed that the tumor cells showed growth to the mucosal side , cropping out to the surface in most areas of the protruding lesion , and only a small part of the tumor was within nontumoral gastric mucosa , despite the tumor cells originating from the intrinsic muscle layer . thus the case of our report may be a rare case for its morphological appearance as well as clinical course . metastasis and/or recurrence after surgery may be observed for several gists , despite an initial diagnosis of a benign tumor . therefore , it is recommended to apply risk classification based on tumor size and mitoic count under a hpf . the mib-1(ki-67 ) li or the presence or absence of tumor necrosis is also reported to reflect proliferation activities and malignant outcome . the gist described in our report was less than 2 cm in diameter without necrosis , and had low mitotic activity ( 0/50 hpf ) and li for mib-1 ( about 1% ) . in this context , our gist may have a very low malignant potential and thus metastasis and/or recurrence may not be observed in the future . actually , the patient in this report has been disease - free 18 months after surgery .

we report the case of a 56-year - old woman who had a gastrointestinal stromal tumor ( gist ) of the stomach . she was admitted to our hospital for epigastric pain , nausea , and severe acute anemia ( hemoglobin level 4.3 g / dl ) . esophagogastroduodenoscopy revealed a narrow stalk - like based , hemorrhagic and uneven protruding lesion in the lesser curvature of the gastric upper corpus . although the tumor was less than 2 cm in diameter and was probably a benign gist according to histology , laparoscopy - assisted local resection was needed because the patient had continuous severe anemia and epigastric pain . histological assessment showed that the elongated spindle - like tumor cells originated from the intrinsic muscle layer , and was shown with growth to the mucosal side , cropping out to the surface in most areas of the protruding lesion . only a small part of the tumor was within nontumoral gastric mucosa . most of the tumor cells demonstrated immunoreactivity for kit and cd34 in the cytoplasm but not for sma , s100 , and desmin . mitotic activity ( 0/50 high power field ) and the labeling index for mib-1 ( about 1% ) were low . the gist of the stomach described in this report was a rare case with a narrow stalk - like based , uneven protruding mass presenting with severe acute anemia despite small size .

blunt traumatic rupture of the diaphragm is a serious injury that is often difficult to diagnose . ( 1 ) it is an uncommon injury whose incidence is rising because of the increasing number of road traffic accidents in the last few years , together with better pre - hospital and hospital resuscitation of severely injured patients and improved diagnostic facilities . ( 2 ) in developing countries where initial care of severely injured patients and diagnostic facilities are less than optimal , blunt traumatic diaphragmatic rupture ( btdr ) may go undiagnosed . high index of clinical suspicion is required because of the missed diagnosis and potential for delayed presentation . we report a case of delayed presentation of post traumatic diaphragmatic hernia and reviewed the literature to highlight challenges in diagnosis and management of such cases . a 32 year old male was admitted to the emergency department with a history of nausea , vomiting , pain in the chest and upper abdomen as well as breathlessness of 3 days duration . he had a history of blunt trauma to the abdomen following a fall from a tree 4 years previously . he was then treated conservatively and no diaphragmatic hernia was detected . on physical examination the patient appeared pale , dehydrated and had dyspnea . examination of the left chest showed decreased movement on respiration , absent breath sounds , bowel sounds were present and dullness on percussion . apart from a mild anaemia ( 9gm / dl ) , all blood tests were within normal limits . the chest x - ray showed a large air - fluid level in the left thoracic cavity with a nasogastric tube within it , collapse of left lung field , obliteration of cardio - phrenic angle and shifting of the mediastinum toward the right side . ( figure 1 ) the presence of the nasogastric tube was confirmed with the presence of gurgling sound on auscultation after administration of air through it . barium meal was carried out to localize the stomach and showed the presence of the stomach in the thoracic cavity confirming diagnosis of post - traumatic diaphragmatic hernia . ( figure 2 ) the patient was counseled and prepared for surgery through a left sub costal incision . the findings at surgery were that two - thirds of the stomach , omentum and spleen had herniated through a 10 cm x 7 cm tear in left hemidiaphragm into the chest . riolfi performed the first successful repair in 1886 . not until 1951 , when carter et al . ( 3 ) approximately 0.8%-1.6% of patients with blunt trauma shows a rupture in the diaphragm . ( 4 ) blunt trauma accounts for 75% of ruptures , and penetrating trauma accounts for the rest . approximately 69% of hernias are left - sided , 24% are right - sided , and 15% are bilateral . ( 5,6 ) patients with delayed diaphragmatic herniation frequently present months to years after the initial injury with manifestations of visceral herniation , incarceration , obstruction , ischemia from strangulation , or perforation . in our case symbas et al . , observed a delay in diagnosis in 8% of cases of diaphragmatic injury from 18 h to 15 years after injury . ( 7 ) chest radiography is the standard in the advanced trauma life support ( atls ) protocol for a trauma workup . approximately 23 - 73% of traumatic diaphragmatic ruptures will be detected by initial chest radiograph , with an additional 25% found with subsequent films . chest radiographic findings that indicate traumatic rupture include the following ( 8) : abdominal contents in the thorax , with or without signs of focal constriction ( collar sign)nasogastric tube seen in the thorax , elevated hemidiaphragm ( > 4 cm higher on left vs right ) anddistortion of diaphragmatic margin . abdominal contents in the thorax , with or without signs of focal constriction ( collar sign ) nasogastric tube seen in the thorax , elevated hemidiaphragm ( > 4 cm higher on left vs right ) and distortion of diaphragmatic margin . conventional ct scan has been reported to have a sensitivity of 14%-82% , with a specificity of 87% . helical ct has increased sensitivity 71 - 100% , with higher sensitivity for left vs right . ct findings indicating rupture include the following ( 8) : direct visualization of injurysegmental diaphragm non - visualizationintrathoracic herniation of visceracollar signperidiaphragmatic active contrast extravasation direct visualization of injury segmental diaphragm non - visualization intrathoracic herniation of viscera peridiaphragmatic active contrast extravasation ultrasonography ( focused assessment with sonography for trauma [ fast ] scan ) has been reported to detect diaphragmatic hernias . ( 9 ) our patient was diagnosed by abdomino - thoracic x - ray and barium meal study . the difference is based on the degree of adhesion present in the thoracic cavity and the state of the herniated organs . after reduction of the abdominal contents , the diaphragm is usually repaired simply with monofilament nonabsorbable suture as in our case . delay in presentation of a diaphragmatic hernia in this case could be explained by various hypotheses . delayed rupture of a devitalized diaphragmatic muscle may occur several days after the initial injury . however the more likely explanation is a possible delayed detection assuming that the diaphragmatic defect occurring with injury manifests only when herniation occurs . in conclusion , a history of trauma must be investigated to diagnose patients with a delayed post traumatic diaphragmatic hernia . a high index of suspicion and the use of relevant radiological investigation is essential for early diagnosis . all cases whether diagnosed pre - operatively or intraoperatively must be repaired surgically either by laparotomy , thoracotomy , a thoraco - abdominal approach or by minimal access surgery .

blunt traumatic diaphragmatic rupture is an uncommon but severe problem that is usually seen in polytraumatized patients including blunt abdomino - thoracic trauma due to road traffic accidents , fall from height and penetrating injuries . regardless of the mechanism , diagnosis is often missed and high index of suspicion is vital . late presentations are associated with increased morbidity . we report an interesting case of blunt traumatic diaphragmatic hernia in a 32-year - old man presenting 4 years after the initial abdomino - thoracic injury due to fall from height . he had herniation of the spleen and stomach . through a left sub costal incision , the herniated organs were reduced and diaphragmatic defect closed with interrupted prolene suture .

severe dysmenorrhea is a major problem for unmarried females , with endometriosis as a common suspect , frequently debilitating and psychologically taxing . depending on the access and expertise of the gynecologist various medicines are prescribed and surgeries performed . a 22-year - old unmarried female was treated for a year by a gynecologist , for progressive dysmenorrhea . she had severe cyclical pain in the lower abdomen for seven to eight days , including two to three days prior to menses . although she had experienced severe dysmenorrhea since puberty , it had increased over the last one year . an initial abdominal ultrasound revealed a right - sided endometrioma 9.5 8 7 centimeters and a left - sided endometrioma of 4 to 5 centimeters . a laparotomy for bilateral endometrioma was performed in july 2007 . a right ovarian mass with multiple cysts , with septae , was found , and subsequently the chocolate cyst was drained and the adhesions separated . the left ovary also had a small chocolate cyst , which was drained , after which the uterus regained its normal size and oval shape . this patient had consulted another gynecologist who had given her leuprolide injection , 3.75 mg depot , for two months . in march 2008 , she came as a tertiary reference , tired , with no relief . on examination the uterus was of normal size , however , the right adnexa appeared bilobed with internal echoes , right endometrioma was 5 centimeters , and ca 125 was 77.60 u / ml . a conservative treatment with dydrogesterone tablets , 10 mg , from day 525 , for three months , was given . the patient had some relief , but returned in september 2008 , with complaints of rectal heaviness and severe dysmenorrhea . ca 125 decreased to 37.70 u / ml , abdominal ultrasound revealed multiple cysts in the right ovary ranging from 3.6 to 5 centimeters . she wanted to avoid surgery , so we started her on low - dose oral contraceptive ( oc ) pills for two months . in december 2008 , she had unbearable dysmenorrhea with tenderness in the pelvic region . an abdominal ultrasound revealed the uterus with right endometrioma of 4.3 centimeters and also a thick wall , a 6.9-centimeter granular echogenic area on the right side , ca 125 was raised to 236 u / ml . a computed tomography ( ct ) scan showed congenital absent right kidney with a compensatory enlargement of the left kidney , right endometrioma 4.5 centimeters , left endometrioma 6.1 centimeters , and a pelvic mass extending from the right endometrioma beneath the psoas muscle . the left - sided endometrioma of 4 to 5 centimeters , adherent to the uterus [ figure 1 ] , was separated from the uterus using bipolar electrocautery and a harmonic scalpel . left endometrioma adherent to the uterus the right - sided mass was attached to the uterus with a fibrotic band [ figure 2 ] . we suspected the mass to be a noncommunicating rudimentary horn , as the right tube and round ligament were lateral [ figure 3 ] . a band connecting the right rudimentary horn with the uterus uterus and the rudimentary horn also the right ovary was adherent to the horn . the right - sided ureter course was identified well beneath the rudimentary horn [ figure 4 ] . with blunt dissection the round ligament was attached to the rudimentary horn , which was separated using a harmonic scalpel [ figure 5 ] . ureteric course identified coagulating round ligament attached to the rudimentary horn the rudimentary horn was freed from all the sides using bipolar and harmonic scalpel , along with the fibrotic band that attached the horn to the uterus [ figure 6 ] . band coagulated using harmonic scalpel the left ovary and tube , the uterus , and the right ovary were preserved [ figure 8 ] . the left ovary & tube , uterus and the right ovary were preserved the total operating time was 114 minutes . approximately 90% of these unicornuate uteri with rudimentary horns are noncommunicating , however , fine anatomical variations may be encountered , particularly in the attachment of the rudimentary horn to the unicornuate uterus . a series of obstructive mllerian anomalies describe the unicornuate uterus with rudimentary uterine horn , which can be either fixed or separated . when no fusion occurs with the contralateral duct , a fibrous or fibrous muscular band usually connects the two horns . in this case also a fibrous band had connected the rudimentary horn with the uterus . however , when the horn is lined by a functional endometrium , the resulting obstructed menstrual flow can lead to severe cyclical pain of the most common and clinically significant type . it is also susceptible to many gynecological and obstetric complications that may be avoided by the removal of the rudimentary horn and its tube . nevertheless , this subgroup may encounter fine anatomical variations , particularly in the attachment of the rudimentary horn , which may influence the surgical treatment . in order to facilitate the surgical procedure , recent literature has suggested that magnetic resonance imaging ( mri ) provides a considerably improved and accurate means of diagnosing and identifying mllerian anomalies . of late , three - dimensional sonography has been introduced into clinical practice and it offers advantages over two - dimensional scanning , as it provides fine anatomical details , useful for preoperative planning . renal agenesis was also seen in patients with uterine agenesis ( 2/5 cases ) and unicornuate uterus ( 2/7 cases ) . all 11 cases of obstructed uterus didelphys were associated with renal agenesis ipsilateral to the side of the obstructing transverse hemivaginal septum . renal agenesis in patients with uterus didelphys is often ipsilateral to an obstructing , transverse , hemivaginal septum . this case report identifies the possibility of a rudimentary horn with functioning endometrium as a prime cause of pain , rather than endometriosis , in the unmarried girl who also had renal agenesis . the renal genesis was on the same side ( right ) as the rudimentary horn . an attentive mind with good clinical and sonographic evaluation , backed by operative laparoscopy , makes a big difference in the management , relief , and prognosis of such cases . thus we conclude that in unmarried young patients with suspected endometriomas , suspicion of unusual pathologies such as functioning rudimentary horn can be the root cause of their pain and debility . laparoscopy is of gold standard for diagnosis and treatment in such patients a great relief .

an unmarried girl with severe dysmenorrhea had laparotomy for bilateral endometriomas , later treated with danazol and gnrha depo injection for more than three years . on laparoscopy she had a large , noncommunicating , functioning rudimentary horn with ipsilateral renal agenesis . laparoscopic excision of the rudimentary horn gave major relief from the suspected endometrioma , which was not the cause of her severe pain .

radiology is a fundamental method for diagnosis and planning in endodontics , since one of the basic concepts of endodontic treatment is working length determination for establishing the correct final distance between the end of obturation and the tooth apex . investigations on this issue have primarily addressed the reduction in the radiation dose to the patient and improvement in definition of the radiographic image . a less costly option to increase the resources of radiographic image would be the indirect digitization of the conventional radiograph4 . digitized images can be modified by resources available on softwares for image handling in order to make adjustments , such as brightness , contrast , densitometric quantification , relief image and pseudo - colorizations1,4,6,7 . the digital resources might optimize the quality control of radiographic examination by revealing alterations not observed on the radiographs during examination on a film viewer2,3,15,16 . the digital systems further offer the possibility of quantifying the distance between two points in an image , which would be one of the major advantages of digital systems in endodontics2,9,13 . this study evaluated comparatively the quality of conventional radiographic imaging and indirect digitization of radiographs obtained during endodontic procedures . fifty sets of 4 radiographs take during endodontic treatment of single- and multi - rooted teeth at the undergraduate endodontics clinic of the dental school of bauru , usp were selected . the radiographs were obtained with size 2 , type e radiographic films ( ektaspeed ; kodak company - rochester , ny . usa ) in x - ray units at 10ma and 60kvp ( espectro ii ; dabi atlante , ribeiro preto , sp , brazil ) , following the bisecting angle technique . each radiograph of the set of four corresponded to one stage of endodontic treatment ( canal length determination , gutta - percha point selection , lateral condensation and final obturation ) . radiographic processing was performed with developer and fixing solutions for radiographic films ( kodak company - rochester ) by the time - temperature method . at the first stage of the study , the radiographs were mounted on cards with 4 frames , identified by numbers and evaluated on a film viewer , surrounded by a dark card to reduce the excess light , in a dark environment . each radiograph was measured and analyzed , separately , by 3 calibrated examiners , two of them were undergraduate dental students and a maxillofacial radiologist , using magnifying glass ( x4 ) and a millimeter ruler . measurements were related to the distance between the material in the root canal and the tooth apex ( file / apex , main gutta - percha point / apex and obturation / apex ) . the quality of radiographic images was also evaluated , using the following scale : -poor : radiographs , yet allowing observation of the apex and periapical region of the tooth .- acceptable : radiographs allowing good observation of the apex and periapical region of the tooth .- poor : radiographs , yet allowing observation of the apex and periapical region of the tooth . acceptable : radiographs allowing good observation of the apex and periapical region of the tooth . radiographs were digitized on a scanner with transparency adapter of 8 bits , model hp scanjet 4c / t ( hewlett packard , usa ) at 300 dpi in a tiff format , transferred to a pc computer with pentium processor , windows system and imported to the digora for windows software version 1.51 ( orion corporation soredex , helsinki , finland ) . free brightness / contrast adjustment was performed on digora ( figure 1 ) and the images were once again evaluated as to their quality , following the " poor , acceptable and good " scale . after image enhancement , the distance between the tooth apex and the end of the root filling material the arithmetic mean of the measurements achieved by the three examiners was calculated and used for statistical analysis by two - way analysis of variance ( p < 0.01 ) . the measurements of the first and second stage were compared by two - way analysis of variance with repeated measurements ( table 2 ) . statistically significant difference ( p<0.01 ) there was statistically significant difference between the measurements obtained with the conventional method and the digora software . on average , digora measurements were 0.117 mm larger than those recorded on the conventional radiographs ( table 1 ) . with regard to comparison of image quality , in the conventional method 64.5% of the images were scored as good , 31.5% as acceptable and only 4% as poor ; whereas in the digora system , there was 100% of good images , as they had been improved by adjustment of brightness and contrast . the sensitivity of the conventional radiographic method is not the problem , but rather the ability of clinicians to interpret the images . in this context , the digital method has several advantages , due to its versatility and possibility of image manipulation1,10,12 . the digitized images may enhance the conditions for diagnosis , treatment planning and follow - up compared to conventional radiographs , due to the technological possibilities available through digital softwares3,11,13 . the examiners scored 64.5% of images as good in conventional radiographs and 100% in indirectly digitized images . the factor that most contributed to the improvement in image quality was the adjustment of brightness and contrast , performed by the examiners according to their own judgment . the difference between the filling material and apex measurements was 0.117 mm larger when the measurements recorded with the millimeter ruler on the film viewer were compared to those obtained on digora . despite the statistically significant difference , the clinical significance of a measurement of one tenth of millimeter in endodontic treatment is not relevant . the observation of larger measurements by digora may be related to the highest measuring accuracy of the software and increased image size when analyzed on the computer screen . the accuracy of measurements achieved on indirect digital radiographs by the digora system was tested and did not reveal statistically significant difference between the actual and digital measurements , yet the low sample size may lead to loss of diagnostic information . comparison between intraoral digital sensors and conventional radiographic film for root canal length determination and measurement of endodontic files of different sizes has been previously performed2,5,9,13 . these studies revealed the superiority of conventional radiographic film for detection of smaller files , whereas there was no statistically significant difference for size 15 files13 . however , the authors recommend the use of the digital system because of the possibility of reducing the patient 's exposure to ionizing radiation and time reduction in obtaining and processing digital images . the quality of images obtained on the digora system was higher because brightness and contrast could be adjusted . this is an advantage of this software over conventional radiographs because the adjustment of images of lower quality avoids repetitions and consequently reduces the patient exposure to radiation . on the basis of these results it may be concluded that the quality of indirectly digitized images was superior to that of conventional radiographs . the images of the filling material on the digitized images were 0.117 mm larger than on the conventional image .

the aims of this study were to evaluate the quality of indirect digitized radiographic images taken during endodontic procedures and to compare the measurements recorded with this technique to those obtained from conventional radiographs . two - hundred conventional periapical radiographs taken at the undergraduate endodontics clinic of the dental school of bauru were digitized . the conventional and indirect digitized images were compared by three examiners as to the quality and accuracy of the measurements recorded during endodontic treatment , in canal length determination , gutta - percha adaptation , lateral condensation and final obturation . the conventional radiographs were observed on a film viewer , surrounded by a dark card , and measured with magnifying glass and a millimeter ruler ; the indirect digitized images were evaluated on the digora for windows software , with free utilization of the bright / contrast tool . unlike the conventional radiographic images , all indirect digitized images were considered as having a high quality . the distance between the filling material and the root apex was 0.117 mm larger , on average , for the digora system ( p<0.01 ) . the measurements achieved by the investigated radiographic methods were clinically similar and they are thus equivalent . changes in brightness and contrast of the images using digora software improved the diagnosis .

currently , more than 150 000 partial or complete hiv genome sequences are available in the central hiv database at los alamos national laboratory ( 1 ) ; these data are crucial for the development of drugs against aids . analysis of hiv sequence data is challenging , however , since hiv is among the most genetically variable organisms known and recombinations of different hiv subtypes are very common ( 2 ) . hiv-1 is divided into three major phylogenetic groups , one of which the m group is responsible for the aids pandemic ( 3,4 ) . this group is classified into ten subtypes , some of which are further divided into sub - subtypes . accurate classification of hiv-1 subtypes and recombinants is of crucial importance for epidemiological monitoring and drug development . therefore , a number of software tools have been developed to classify hiv genome sequences and to identify phylogenetic breakpoints and subtypes in recombinant strains ( 5,6 ) . we recently developed a hmm - based method to compare nucleic acid sequences to a given multiple alignment a of a sequence family s for which a classification into subclasses is available ( 7 ) . we called this method jumping profile hidden markov model ( jphmm ) since our approach is a probabilistic generalization of the jumping - alignment ( jali ) algorithm proposed by spang et al . , a query sequence s is aligned to a multiple alignment a of a sequence family s = { s1 , , sn}but s is not aligned to the alignment a as a whole , but different parts of s can be aligned to different individual sequences si from a. within an alignment of the query s to the sequence family s , jumps are allowed between different sequences from s depending on where the strongest degree of similarity is found . for a jump between two sequences si and sj , a penalty is imposed , similar to the familiar gap penalty used in standard sequence alignment . this approach is particularly useful if the query sequence s is a result of phylogenetic recombinations such that different parts of s are related to different sequences from the family s. jali has been shown to perform well if an alignment a is to be searched against a sequence database ( 9 ) . in our jphmm approach , we assume that a partition of the sequences from the family s into subclasses is given . each subclass is modeled as a profile hidden markov model ( 10 ) . within a subclass , the usual transitions between match , insert and delete states are possible , as in standard profile hmm theory but in addition , our model allows transitions between profile hmms corresponding to different subclasses , so a path through our model can switch back and forth between different subclasses . in ( 7 ) , we found that jphmm is a useful tool to predict phylogenetic breakpoints and subtypes in recombinant hiv and hepatitis c sequences ( 11 ) . for hiv subtyping , we start with a pre - calculated multiple alignment of hiv-1 genome sequences consisting of all major subtypes and sub - subtypes ; these ( sub-)subtypes are modeled as profile hmms in our jphmm approach . phylogenetic breakpoints and ( sub-)subtypes to which a query sequence s is aligned , reliably indicate the real ( sub-)subtypes in recombinant hiv sequences . to evaluate our tool and to compare its prediction accuracy to competing methods such as simplot ( 12 ) and rdp ( 13 ) , we used a large set of real and simulated data from hiv-1 and hepatitis c. these test runs demonstrated that jphmm is far more accurate than existing tools for phylogenetic breakpoint detection . details of this program evaluation are described in ( 7 ) . to make jphmm available to the hiv research community , we set up an easy - to - use www interface at gttingen bioinformatics compute server ( gobics ) : at our server , the user can paste or upload up to 5 full - length hiv-1 genome sequences that is to be searched for phylogenetic breakpoints and subtypes . our server uses a pre - calculated multiple alignment of 309 hiv sequences from the major hiv ( sub-)subtypes obtained from the hiv database at . these sequences include nine subtypes a d , f , g , h , j , k , and a persumed recombinant 01_ae . subtype a has two sub - subtypes , a1 and a2 ; similarly f has two sub - subtypes , f1 and f2 . b and d could be regarded as sub - subtypes because their relative distance and relation are similar to a1 and a2 , f1 and f2 , respectively . but we still consider b and d as subtypes , not sub - subtypes because of historical reasons ( 14 ) . 01_ae , though being called recombinant , contains the only information of subtype e. thus we include 01_ae in the alignment . the alignment of these sequences has been carried out using hmmer ( 15 ) and subsequent manual improvement . a hyperlink to the results of the program run is returned to the user by e - mail . the result file contains a list of fragments of the input sequence that are assigned to different subtypes and sub - subtypes , including predicted breakpoints between these fragments . in addition , the output file contains a graphical representation of the predicted recombinant fragments within the hiv-1 genome . one is based on the original sequence position , and the other is based on hxb2 numbering . hxb2 ( genbank accession number k03455 ) is the most commonly used reference strain for many different kinds of hiv-1 functional studies . the hxb2 numbering provided for the output breakpoints is especially useful to facilitate the identification of the precise location of interest in hiv sequences . it should be mentioned that our tool is sometimes not sensitive to detect hiv-1 subtypes h , j , k , as only few full - length genome sequences of these subtypes are available to train our model . for these subtypes , we recommend to compare the results of jphmm with those of other hiv-1 subtyping tools , for example , rip ( ) . as shown in ( 7 ) , the overall prediction accuracy of our method is high compared with alternative approaches . nevertheless , it would be useful for the user to assess the relative reliability of individual predicted breakpoints . in principle , this is possible by using posterior probabilities that can be calculated using the forward and backward algorithms as explained in ( 16 ) . we are currently implementing these algorithms to estimate the ( local ) reliability of our predictions . for predicted recombinants , users of our software may want to know putative parental sequences . our method can not provide this information directly , since jphmm compares input sequences to a model derived from a pre - calculated alignment of representative sequences . it is possible , however , to search predicted recombinant segments of input sequences against the hiv-1 database to retrieve potential parent sequences . the output file contains a list of fragments from the input hiv-1 sequences that are assigned to different hiv subtypes , including predicted breakpoints . at the bottom of the file , a graphical representation of the input sequence

detecting recombinations in the genome sequence of human immunodeficiency virus ( hiv-1 ) is crucial for epidemiological studies and for vaccine development . herein , we present a web server for subtyping and localization of phylogenetic breakpoints in hiv-1 . our software is based on a jumping profile hidden markov model ( jphmm ) , a probabilistic generalization of the jumping - alignment approach proposed by spang et al . the input data for our server is a partial or complete genome sequence from hiv-1 ; our tool assigns regions of the input sequence to known subtypes of hiv-1 and predicts phylogenetic breakpoints . jphmm is available online at .

the risk of developing coronary heart disease ( chd ) depends on several factors that are related to both lifestyle and genetics . recent genome - wide association studies have identified around 50 chromosomal loci that are robustly associated with chd . mega et al studied whether the developing of genetic risk score ( 27 genetic variants associated with chd ) will help in risk stratifying patients receiving statin therapy in both primary and secondary prevention . the investigators analyzed data from the following studies: the primary prevention population : the malmo diet and cancer study ( mdcs ) , jupiter , and ascot studies , where genetic samples were available from 27817 , 8749 and 6978 people consecutively. the secondary prevention population : care , and prove it - timi 22 , where genetic samples were available in 2878 and 1999 individuals consecutively.the genetic risk score was derived on the basis of 27 snps that were significantly associated with coronary artery disease at genome - wide level in previous analyses . each individual participant received a score equal to the sum of the numbers of risk alleles for each snp weighted by the log of the odds ratio reported with the snp in the original report . the primary prevention population : the malmo diet and cancer study ( mdcs ) , jupiter , and ascot studies , where genetic samples were available from 27817 , 8749 and 6978 people consecutively . the secondary prevention population : care , and prove it - timi 22 , where genetic samples were available in 2878 and 1999 individuals consecutively . the investigators used the cox proportional hazard models to assess the risk of coronary heart disease for each quintile of genetic risk , in which they used the first quintile as a reference group . additionally , the risk categories low [ quintile 1 ] , intermediate [ quintile 2 - 4 ] , and high [ quintile 5 ] and per 1 sd were calculated . these analyses were done on the participants in mdcs , and in the placebo or low - intensity statin treatment groups of the applicable trials . higher genetic risk scores were associated with a raised risk of coronary heart disease , independent of established clinical predictors . specifically , when evaluating participants in low , intermediate , and high genetic risk categories , a gradient of risk coronary heart disease was evident in the studies ( see table 1 ) . baseline ldl cholesterol and hdl cholesterol levels were similar across genetic risk score categories within each trial , as were the absolute and the percentage changes with statin therapy . analyses were done to investigate the clinical benefit of statin therapy across the genetic risk score . the relative risk reductions were 34% in low , 32% in the intermediate , and 50% in high genetic risk score categories in the primary prevention trials , and 3% in low , 28% in intermediate , and 47% in high genetic risk score categories in the secondary prevention trials . when the data were combined , the gradient of relative risk reductions with statin therapy across low , intermediate , and high genetic risk score categories were 13% , 29% , and 48% , respectively ( p value for trend = 0.02777 ) . similarly , in terms of the absolute risk reductions , a graded increase in the benefit of statin therapy across the genetic risk score categories was evident in both the primary and secondary prevention trials . correspondingly , the number needed to treat to reduce coronary heart disease events in 10 years with statin therapy in primary prevention differed depending on genetic risk score ; 66 for low genetic risk score , 42 for intermediate risk and 25 for those individuals with high risk score . the current study demonstrated that combining the 27- genetic variants that were individually associated with the risk of coronary heart disease into a risk score could identify people at increased risk of chd events , including incident chd in primary prevention populations and recurrent chd events in secondary prevention populations . furthermore , observation from the four statin trials suggests that individuals with a high genetic risk score have both a greater absolute and relative benefit from statins and the benefit is larger among high when compared to the intermediate risk group . the investigators outlined the limitations of the study including , first data from several studies were used in the analysis , and each data has its own criteria , treatment allocation , and duration of follow - up . second , the numbers needed to treat were calculated by extrapolation of the effect of statin therapy during a 10-year period , and treatment effect could vary overtime . third , these analyses were done within completed clinical trials , and the genetic risk score was not used specifically as an enrollment criterion , moreover the analyses were conducted in statin trials that yielded positive outcomes only . fourth , although the investigators focused on genetic variants that were associated with the risk of chd . finally , the gradient of the relative risk reduction across genetic risk score categories in the study was unexpected . in an accompanying editorial by schunkert and samani , they commented that the study by mega et al illustrates the expanding clinical use of genetic discoveries in chd , from identifying new therapeutic targets to prioritizing ( or de - prioritizing ) existing targets for medical intervention to now potentially providing a valuable algorithm for improved precision in prediction of event rates and responses to treatment . they did suggest that this genetic risk score will need further careful evaluation , including testing the genetic score in the context of scores that are presently recommended to see how this score modulates risk calibration and discrimination provided by those established scores . additionally , the cost - effectiveness of incorporating this genetic risk score assessment will need to be established . the genetic risk score identified individuals at increased risk of chd across primary and secondary prevention populations . furthermore , people with high - risk scores had the largest relative and absolute risk reductions with statin therapy .

genetic variants have been associated with the risk of coronary heart disease ( chd ) . mega et al studied the association of a genetic risk score based on 27 genetic variants with incidents of recurrent chd , adjusting for traditional risk factors using data from a community based study and 4 randomized controlled trials of both primary and secondary prevention with statin therapy . when individuals were divided into low , intermediate and high genetic risk categories , a significant gradient in risk of incident and recurrent chd was shown .

sample size was calculated to measure a difference of greater than 2 mm of hg between the two instruments with an estimated standard deviation of 4 mm , for 80% power and a type 1 error of 5% . we needed 34 patients to measure this difference and recruited 40 consecutive adult subjects attending a glaucoma prevalence study for the trial . all subjects who could undergo visual acuity measurement , refraction , slit - lamp examination and applanation tension measurement were eligible . corneal pathology including astigmatism of 2 diopter ( d ) or greater , inability to measure iop or history of allergy to proparacaine or fluorescein were reasons for exclusion . intraocular pressure was measured by one of two examiners , using the zeiss at 030 ( gatz ) ( carl zeiss , jena , germany ) and the inamil-5110 ( gati ) ( inami and co. , tokyo , japan ) goldmann type applanation tonometers . at the start of the day the instruments were calibrated as per the manufacturers instructions and were used for examination only if they were accurately calibrated . to minimize the influence of any iop - lowering effect induced by applanation tonometry on the results , the sequence of measurements was randomized . applanation tonometry was performed first on one randomly selected instrument followed immediately after by measurement on the second instrument . the iop was measured after anesthetizing the cornea with sterile 0.5% proparacain eye drops ( paracain , sunways , mumbai , india ) and staining the tear film with fluorescein strips . the tonometer was set to the zero mark prior to the start of the examination . measurements on both instruments for a subject were performed by a single observer who was blinded to the actual readings , which were read and recorded by the second examiner who then reset the applanation tonometer to the zero mark . the iop was measured two consecutive times for each eye on each instrument , and the mean of the two readings was used for analysis ; if there was a difference of greater than 2 mm of hg between the readings , a third measurement was taken and the median of three readings was taken . by convention , the right eye was examined first for every patient . the iop was measured on the second instrument , placed in the same examination room , almost immediately after completing recording on the first instrument . intraocular pressure was compared using the paired t test and agreement was assessed by the altman and bland plot.6 forty eyes ( 40 subjects , mean age : 53.3 sd 7.9 years , 22 males , 18 females ) were included . mean iop ( sd ) on gatz was 15.32 ( 6.80 ) mm hg ( range:9 mm hg - 36 mm hg ) and on gati 13.52 ( 5.65 ) mm hg ( range:7 mm hg - 30 mm hg ) ( p<0.001 , 95% ci of the difference : -2.48 to -1.11 ) . the bland and altman plot [ figure 1 ] revealed a tendency for higher iop recordings on the gatz ( 95% limits of agreement:-2.47 to 6.16 mm hg ) . the absolute level of iop may not be as important to glaucoma diagnosis as was once thought , however , the trend of iop measurements is relevant in the management of glaucoma patients . we attempted to minimize their influence on the study results . in order to avoid an observer bias , two observers were used for the study - however , the same observer made iop measurements on both instruments for a single patient . to minimize the effect of a possible transient lowering of iop following applanation tonometry , on the results , we randomly allocated the order of iop measurement by either machine . the iops on the second instrument were measured within a few minutes of the measurement on the first machine to minimize any temporal variation in iop . lower iop readings were consistently recorded on the inami at . in 70% of subjects iop differences were within the clinically acceptable range of 2 mm . with both instruments calibrated accurately as per the manufacturers recommendations better agreement between the instruments would be expected . since manometric measurements were not made in any of our subjects it is difficult to comment on which instrument was giving erroneous results . the clinical implications of this variation on disease diagnosis are considerable since both instruments are widely used in asia . it could result in potential misdiagnosis of normal tension glaucoma ( ntg ) or primary open angle glaucoma ( poag ) . follow - up may not be affected as significantly as long as all measurements are made on a single instrument by a single observer keeping other factors such as calibration and the time of measurement constant . however , with increasing cross - referrals and the use of multiple instruments in various clinics the possibility of lack of agreement between devices has to be kept in mind . with the large number of similar applanation tonometers available , agreement between other commercially available devices needs to be assessed . in this study if this was indeed the case there should not have been a significant difference between devices and certainly not a difference of the magnitude that was found . perhaps , the manufacturers need to reassess the calibration procedures for these devices . in conclusion , we demonstrate yet another variable in iop measurements , the effect of which could be minimized by performing baseline and follow - up iop measurements on a single instrument . additionally , therapeutic decisions should be made keeping in mind the possibility of an inter - instrument variability .

the aim of the study was to assess agreement between two commercially available applanation tonometers for the measurement of intraocular pressure ( iop ) . forty subjects underwent iop measurement on two accurately calibrated goldmann type applanation tonometers ( zeiss at 030 ( gatz ) and inami l-5110(gati ) ) . the order of examination was randomized and observers were masked to the iop recorded . the mean of two consecutive readings , from a randomly selected eye for each subject , was used for analysis . agreement was assessed using the altman and bland plot . the mean ( sd ) iop readings on gatz was 15.32 ( 6.80 ) mm hg and on gati was 13.52 ( 5.65 ) mm hg ( p<0.001 , 95% ci of the difference : -2.48 to -1.11 ) . the 95% limits of agreement on the altman and bland plot were:-2.47 to 6.16 mm hg ) . there was significant inter - instrument variability between the two accurately calibrated goldmann type applanation tonometers studied .

prognosis of advanced or metastatic pancreatic adenocarcinoma is poor , with a median overall survival between 6.8 and 11 months for all histological types . herein we report a case of a patient treated by the combination of gemcitabine plus oxaliplatin ( gemox regimen ) with a complete radiological response . a 50-year - old male complained of epigastric and right hypochondriac region pain in april 2001 . a ct scan showed multiple cystic tumors in the liver and spleen , a pseudocystic tumor on the pancreatic isthmus and an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion . the octreoscan and tumor marker ca 19.9 concentration were normal . in may 2001 , chemotherapy with gemcitabine 1,000 patient experienced grade 1 urticaria at cycle 4 and grade 2 thrombopenia , justifying a 75% dose reduction at cycle 7 . evaluation after 4 cycles showed partial response with a 50% reduction of the liver and spleen tumors . after 9 cycles , a complete tumor response was obtained . since 2002 , physical examination and biology have remained normal . the initial biopsy was reanalyzed , and the initial diagnosis was confirmed . at the age of 61 years , a localized prostatic adenocarcinoma was diagnosed , treated by radical prostatectomy . in 2013 , a personal and family history of breast cancers ( sister at 34 years , mother at 60 years and the grandmother ) and a bladder cancer ( brother ) led to a genetic consultation with brca1 and brca2 genetic testing . the patient as well as a cousin and his two sons were found to carry a brca2 mutation . in january 2015 , the patient had no clinical symptoms , a sequelary segmentary portal hypertension and well - epithelialized varicose vein on gastroscopy and he was relapse free . the mean age at diagnostic is 60 years , with a male preponderance ( 65% ) . the most common diagnosis is due to mass effect such as abdominal pain , jaundice or weight loss . this tumor is often associated with pancreatic panniculitis ( weber - christian disease ) , perhaps due to high serum lipase concentrations . data on chemosensitivity are only available from case reports . different regimens of chemotherapy ( gemcitabine , lv5fu2 , oxaliplatin , cisplatin , irinotecan , docetaxel and paclitaxel ) were tested alone or in combination . nevertheless , the combination of gemcitabine plus oxaliplatin was not investigated . objective response rates were described with folfirinox , folfox , lv5fu2-gemcitabine , lv5fu2 , weekly paclitaxel and lv5fu2-cisplatin . however , no complete response was observed . acc is associated with a better prognosis than ductal adenocarcinoma at all stages of disease . in metastatic disease , schmidt et al . described a 5-year overall survival rate of 17.2% versus 2.8% for ductal carcinoma . in unresected patients , wisnoski et al . reported an overall survival of 25 versus 3 months . the longest survival observed in the literature was 11 years compared to 14 years in our patient . brca2 mutations are known to be associated with pancreatic cancer , representing 2% of the total pancreatic cancer cases . nowadays , there are ongoing trials testing parp inhibitors that show promising results with regard to platinum - based chemotherapy regimens . we reported the first case of metastatic acc treated with the gemox regimen . despite the lack of study in these patients , a chemotherapy regimen with oxaliplatin seems to be the most effective combination with long survival . in these patients

pancreatic acinar cell carcinoma ( acc ) is a rare entity . herein we present the case of a 50-year - old male patient with an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion and multiple metastases in the liver and spleen . a liver biopsy showed a pancreatic acc . the patient received 9 cycles of gemcitabine plus oxaliplatin ( gemox regimen ) , which had to be stopped because of a persistent grade 2 neuropathy . a ct scan showed complete response after 14 years . at the age of 61 years , a localized prostatic cancer was diagnosed , treated by prostatectomy . the patient carried a brca2 mutation . none of the precedent case reports describe a chemosensibility to the gemox regimen . in spite of the lack of study in these patients , chemotherapy with oxaliplatin seems to be the most effective . long survival can be expected .

a 69-year - old man without underlying disease developed a productive cough 5 days before visiting chungbuk national university hospital . he was prescribed cold medicine in a local clinic . the day before visiting our hospital , he had fever , chills , breathlessness , and pain in the right chest wall , and thus , he visited our emergency room . on arrival in the emergency room , he was alert , with a blood pressure of 90/60 mmhg , a pulse rate of 84/min , a body temperature of 37.1 , and an oxygen saturation of 79% on room air . arterial blood gas analysis revealed a ph of 7.434 , a carbon dioxide partial pressure of 34.9 mmhg , an oxygen partial pressure of 41.0 mmhg , bicarbonate of 23.6 mmol / l , and oxygen saturation of 78.3% . his white blood cell count was 1,710/l , hemoglobin 14.6 g / dl , hematocrit 45.1% , and platelet count 122,000/l . blood chemistry showed ; urea nitrogen 27 mg / dl , creatinine 1.4 mg / dl , total protein 5.8 g / dl , albumin 3.5 g / dl , aspartate aminotransferase 28 iu / l , alanine aminotransferase 20 iu / l , and c - reactive protein 11.40 mg / dl . prothrombin time and activated partial thromboplastin time were normal at 11.9 and 34.7 seconds , respectively . an initial chest x - ray showed haziness in both lower lung fields ( fig . 1a ) . after admission , the patient experienced respiratory distress , and thus endotracheal intubation was performed . he was then transferred to the intensive care unit ( icu ) and received mechanical ventilator support , and was administered ciprofloxacin and piperacillin / tazobactam . after 2 days in icu , the patient was hypoxic despite a fio2 of 1.0 , and showed septic shock despite the administrations of inotropic agents . subsequently , his pneumonia improved progressively until on the 7th day based on his oxygen demand , blood examination , and radiographic findings ( fig . abdominal computed tomography was performed and showed fluid and reduced contrast enhancement indicating edema in the proximal large intestine ( fig . , a large fluid collection was observed in the abdominal cavity with necrosis of the colon from the ileocecal valve to the upper rectum , but without bowel perforation . with the exception of some inflammatory change , he was thought to have developed hypoxic damage of the bowel during initial hospitalization , but a tissue examination during the active treatment of septicemia and acute renal failure , revealed colonic infarction due to mucormycosis ( fig . , he was given amphotericin b beginning at 7 days postoperatively , but unfortunately , his condition deteriorated and he died at 34 days postoperatively . the fungi occur in air , soil , and food , and are filamentous organisms consisting of broad irregular hyphae . infection is commonly due to rhizopus ( 47% ) , rhizomucor , absidia , and mucor species , in the family mucoraceae . sporangiospores are typical infective forms , whereas angio - invasive hyphal forms are responsible for tissue invasion and dissemination . the invasion of blood vessels by hyphae leads to arterial thrombosis , tissue infarction , and necrosis , whereas venous invasion causes hemorrhages . mucormycosis is classified into rhinocerebral , pulmonary , cutaneous , gastrointestinal , central nervous system , and disseminated / miscellaneous types based on its clinical presentation and the body site involved . of these , the gastrointestinal type constitutes only 7% of cases , but its reported mortality is around 85% , and its incidence is increasing . initial manifestations of gastrointestinal mucormycosis are abdominal pain and distension , fever , and hematochezia , and the most frequent presentations are perforation , bleeding , and epigastric distention . serologic tests currently have no role in the diagnosis of mucormycosis , and the gold standard for diagnosis is the detection of tissue invasion by hyphae in a specimen . treatment involves the elimination of any risk factors , surgical debridement or resection , and the use of antifungal agents . mucormycosis usually occurs in immunocompromised patients with a severe systemic disease , such as , diabetes , renal failure , malnutrition , a malignant tumor , tuberculosis , syphilis , leukemia , or lymphoma , and in those taking steroids or on immunosuppressive therapy after transplantation . few cases of gastrointestinal mucormycosis in immunocompetent patients have been reported [ 8 - 10 ] . two cases have been reported in purely immunocompetent patients , five cases related to trauma or clinical conditions ( table 1 ) . physicians experience difficulties detecting and diagnosing intraabdominal problems in critically ill patients on mechanical ventilator support with an endotracheal tube while sedatives are administered . in this described case , a patient with normal immunity developed colonic infarction while being treated in the icu for pneumonia and septic shock . we initially considered that the ischemic damage was due to his shocked state , but the cause was eventually revealed to be the angioinvasive intestinal mucormycosis . although a patient with no underlying disease , previously or with no evidence of immunocompromised state , develops unexplained abdominal distension or pain , aggressive efforts should be made to diagnose the cause , and unusual causes , like mucormycosis , should be considered in the differential diagnosis .

mucormycosis is a fatal opportunistic fungal infection that typically occurs in immunocompromised patients . the classical manifestation of mucormycosis is a rhinocerebral infection , and although primary gastrointestinal infection is uncommon , it has an extremely high mortality rate in immunocompromised patients . furthermore , cases of gastrointestinal mucormycosis in an immunocompetent host are rarely reported . here , we describe our experience of a male patient , with no underlying disease , who succumbed to a bowel infarction caused by intestinal mucormycosis during mechanical ventilatory care for severe pneumonia and septic shock .

ectopic kidney is a relatively rare renal anomaly however , an endourologist does encounter stone disease in an ectopic kidney occasionally . factors such as anomalous blood vessels and tortuous ureter with high insertion can lead to poor drainage and predisposition to the formation of renal calculi in these patients . the common management option for such stones is laparoscopy or ultrasound guided percutaneous nephrolithotomy ( pcnl ) . we report our experience with the use the recently described micro - pcnl or microperc for two such cases . two male patients , aged 57 and 60 years respectively , presented to us with calculi in ectopic pelvic kidneys . the first patient had undergone a flexible ureterorenoscopy elsewhere during which the stone could not be reached due to difficult angulation and inflamed tissue leading to poor vision . computed tomography urogram revealed an ectopic malrotated left kidney lying over the sacrum . there was a 13 mm 11 mm sized calculus in the renal pelvis [ figure 1a and b ] . in the second patient , computed tomography urogram revealed a normal right kidney and an ectopic malrotated left kidney lying over the sacrum . there was an 18 mm 17 mm sized calculus ( 1232 hu ) in the pelvis and a 5 mm 4 mm sized calculus ( 423 hu ) in the lower calyx of the ectopic kidney [ figure 1c and d ] . ( a ) x - ray kub showing renal calculus in pelvis of pelvic ectopic kidney with double j stent in situ , ( b ) computed tomography urogram showing the pelvicalyceal system anatomy , ( c ) x - ray kub showing renal calculus ( yellow arrow ) in ectopic kidney , ( d ) computed tomography urogram showing the pelvicalyceal system anatomy ( location of pelvic stone - yellow arrow ; location of lower calyceal stone - red arrow ) both procedures were performed under general anesthesia . in lithotomy position , ureteric catheterization was carried out under cystoscopic guidance using 7 fr ureteric catheters over a 0.035 inch glidewire ( terumo , tokyo , japan ) . the position was changed to supine - oblique with a sandbag under the ipsilateral hemipelvis to move the overlying bowel away from the kidney . an ultrasound probe was pressed against the anterior abdominal wall to displace the bowel away from the line of access . further , colour doppler was used to rule out any significant blood vessel in the path of needle puncture . three - way connector was attached to the needle , allowing saline irrigation , passage of a 0.9 mm flexible microperc telescope , and a 272 m holmium : yttrium aluminum garnet ( ho : yag ) laser fiber [ figure 2 ] . the stones were completely fragmented to dust with the laser . an x - ray and ultrasound was obtained on the first post - operative day to document stone clearance and to rule out any fluid collection . the urethral catheter was removed on the first post - operative day and the patient was discharged . ( a ) surface view of patient position and ultrasound guided percutaneous renal access , ( b ) intraoperative ultrasonography picture showing entry of puncture needle ( yellow arrows ) into lower calyx containing calculus ( red arrow ) , ( c ) confirmation of access into lower calyx by antegrade contrast study to delineate pelvicalyceal system , ( d ) intraoperative surface view showing microperc instruments , ( e ) puncture site at the end of the procedure ( yellow arrow ) , ( f ) post - operative x - ray kub showing complete clearance operating times were 30 and 35 min respectively . in the first patient , ureteric catheter was left in situ for 1 day . in the second patient , ureteric catheter was replaced by a double - j stent at the end of the procedure . visual analog pain scores ( 1 - 10 scale ) on first post - operative day were two and three respectively . first post - operative day and 1 month follow - up x - ray and ultrasound kub revealed complete stone clearance and no evidence of fluid collection in the abdomen . options for managing small renal calculi in pelvic ectopic kidney are shock wave lithotripsy ( swl ) , retrograde intrarenal surgery ( rirs ) , ultrasound or laparoscopy guided pcnl . since these kidneys are surrounded by bowel and bone , moreover , the clearance of fragmented stones is also impaired due to high insertion of ureter and impaired pyeloureteral motility due to surrounding fibrous bands . rirs can be technically demanding due to abnormal and tortuous course of the ureter of a pelvic kidney . this was exemplified by our first patient in whom the stone could not be reached with flexible ureteroscope and the procedure had to be abandoned . the potential hazards in percutaneous access in an ectopic kidney are : ( 1 ) risk of injury to surrounding bowel , particularly if track dilatation is carried out through the bowel , ( 2 ) abnormal vasculature resulting in bleeding from tract dilatation and ( 3 ) spillage of fluid into peritoneal cavity . microperc is a minimally invasive form of pcnl in which percutaneous renal access and stone fragmentation are achieved in a single - step using a 16 g needle . since dilatation is not performed , potential hazards associated with it are avoided . fluid collection is less likely during puncture and at the end of the procedure since the needle puncture site closes quickly . both our patients had rapid post - operative recovery , probably due to lack of fluid spillage and hence no paralytic ileus . though laparoscopic guidance has been advocated for percutaneous access of an ectopic kidney , in experienced hands , ultrasound guidance can provide a safe entry into the appropriate calyx . appropriate patient positioning to move bowel away from the kidney , placing a sand bag to push the kidney towards the anterior abdominal wall and compression with the ultrasound transducer allow safe access into the kidney . further , the use of colour doppler rules out any significant blood vessel along the path of needle puncture . the hemoglobin drop in both of our patients was minimal , supporting the safety of this procedure . in laparoscopy guided pcnl , an abdominal drain may need to be left in situ for a prolonged period because of persistent urinary leakage . the average hospital stay in a series of 15 patients who underwent laparoscopy guided pcnl was 4.8 days ( 4 - 11 days ) . the patients in that series had sequential removal of nephrostomy tube , urethral catheter and abdominal drain , which lead to the prolonged post - operative stay . thus , ultrasound guided microperc is a safe and efficient technique in the management of small renal calculi in ectopic kidneys and it is a minimally invasive procedure with short hospital stay .

management of stone disease in an ectopic kidney is challenging . laparoscopy or ultrasound guided percutaneous nephrolithotomy and retrograde intra - renal surgery are the preferred techniques for these stones . we performed ultrasound guided microperc using a 16 g needle for the management of renal calculi in pelvic ectopic kidneys in two patients . there was no intraoperative or post - operative complication . both patients had complete stone clearance and were discharged on the first post - operative day . ultrasound guided microperc is a safe and effective option for the management of small renal calculi in pelvic ectopic kidneys .

congenital epulis ( also known as congenital gingival granular cell tumour ) is a rare benign congenital growth of the newborn . it was first described in 1871 by neumann , hence the alternative name is newmanns ' tumour . it usually presents at birth with an obvious mass arising from the gingival mucosa of the maxilla or mandible . the size of the mass varies from a few millimetres to 9 cm in diameter . the recommended treatment is surgical excision under local or general anaesthetic , although spontaneous regression has been reported . there are no reports of recurrence , even if incomplete margins are excised , malignant change , or future disruption to teeth or gums . an otherwise healthy 1-day - old girl was referred to a large teaching hospital in cardiff for diagnosis and treatment of two large masses protruding from her mouth . the baby had normal antenatal scans at weeks 12 and 20 , and pregnancy had been unremarkable , other than mother being group b streptococcus positive from a high vaginal swab . mother was fit and well gravida 2 para 1 , with no drug history or family history of note . baby was born at term plus eight days weighing 3.85 kg , pink and breathing spontaneously ( apgar : 9 - 10 ) . on examination there were two fleshy , pedunculated masses arising from the upper and lower alveolar ridges measuring 4 3 3 cm just to the right of the midline . she was booked for excision of these masses under general anaesthesia ( figure 1 ) . both masses were removed with an eliptical insion to the peduncles ( figure 2 ) . the child was breastfeeding the day after surgery , and discharged home the following day . the two masses were fixed and examined histologically ( figures 3 and 4 ) . they showed sheets and clusters of cells containing abundant granular eosinophlic cytoplasm and small uninform nuclei , along with some myxoid areas and areas of haemorrhage and ulceration , confirming the diagnosis of congenital epulis . it commonly presents in the neonate , although prenatal diagnosis with ultrasound has been reported as early as 26 weeks gestation . the lesion usually arises over the incisor - canine region of the maxilla ( maxillary / mandibular ratio 3:1 ) . simultaneous involvement of both maxillary and mandibular alveolar ridges occurs in approximately 10% of reported cases . the diagnosis is usually made on clinical grounds alone , although difficulties may arise when the size of the lesion is small , or the index of suspicion is low . mri is useful for diagnosis , and superior to ultrasound , showing the gingival origin of congenital epulis without local extension . epulis is a greek term literally meaning of the gums and is used to describe a wide variety of gum lesions , regardless of their pathological origin . histologically , congenial epulis shows remarkable similarity with the more common granular cell tumours ( gcts ) [ 2 , 11 ] . there are , however , many distinguishing features , such as occurrence solely in the neonate , typical location , plexiform arrangement of capillaries , and lack of pseudoepitheliomatous hyperplasia . gcts are ubiquitous neoplasms occurring in all age groups , very rarely affecting the gingiva , and can occasionally show malignant change . immunohistochemical studies have revealed further differences , demonstrating the reactivity of gcts to s-100 protein and laminin , and their absence in congenital epulis . vered et al . have also recently expanded the immunophenotypic distinction between the two , showing gcts stain positive for ngfr / p75 and inhibin- , whereas congenital epulis does not . cgts are considered to arise from schwann cells , and hence show strong reactivity to s-100 protein . various theories of the origin of congenital epulis include myoblastic , neurogenic , odontogenic , fibroblastic , and histocytic . it has been suggested that the occurrence of congenital epulis solely in neonates , and more commonly in females , implies a hormonal mechanism of development . however , numerous reports have shown no evidence of either oestrogen or progesterone receptors , and as such suggest an alternative histogenesis [ 11 , 14 ] . in a review of 33 lesions , conclude that the immunohistochemical profile does not imply any specific cell types for the histogenetic origin of congenital epulis . no estimation of incidence of congenital epulis has been made to date , to the best of our knowledge . one centre in the usa saw only two cases over the period of 21 years . in university hospital of wales , a tertiary referral centre for otolaryngology and neonatology , this is the only recorded case of congenital epulis since 1980 , a total of 28 years . using incidence of live births ( 157,454 ) within that time period , we calculate an incidence of 0.0006% ( upper 95% confidence interval : 0.0035% , the inverse of the cumulative beta distribution ) . although most likely an underestimate , this calculation will serve as an approximation of incidence before a more thorough estimation can be undertaken .

congenital epulis , also known as neumann 's tumour , is a rare congenital growth affecting the gingival mucosa of neonates . it is benign condition , seen more frequently in females , with multiple epuli occurring in only 10% of cases . the cause and origin of congenital epulis remains unclear . in this article we present a case report of an otherwise healthy female neonate with two congenital epuli arising from the upper and lower gingival margin , which were successfully treated with surgical excision . we also present a review of the literature and an estimation of the incidence of congenital epulis based on our institutions figures , of 0.0006% ( upper 95% confidence interval : 0.0035% ) .

lymphoid neoplasms of the conjunctiva may appear as an isolated tumor or as part of systemic disease.15 generally , conjunctival lymphoma presents as a mass lesion on the superficial ocular surface . these lymphomas are classically described as salmon - colored patches , and may present bilaterally in up to 20% of patients.4,5 lesions have a fleshy appearance and may develop rapidly . b cell lymphoma is the most common type of conjunctival lymphoma , whereas the t cell form is very rare.25 in this report , we document the clinical and histopathological findings in a patient with conjunctival lymphoma that was the first presentation of systemic malignant lymphomatous t cell proliferation . on march 15 , 2006 , a 67-year - old male patient came to our attention complaining of pain and hyperemia in his left eye . anterior segment slit - lamp biomicroscopy of the left eye showed a pink - yellow colored mass infiltrating the bulbar conjunctiva in the lower fornix of the eyelid and a corneal ulcer at the 9 oclock position with perilimbal congestion and chemosis ( figure 1a and b ) . the left eye was treated with topical steroid / antibiotic medication with an ocular bandage for one week . a control visit showed disappearance of corneal ulcer , and a diagnostic punch biopsy of the conjunctival mass was taken for immunohistochemical examination . the conjunctiva was infiltrated by a diffuse proliferation of small- to medium - sized lymphoid cells which were partly necrotic . focal effacement of the serous glands of the conjunctiva was observed ( figure 2 ) . the neoplastic cells were homogeneously cd3 + , cd5 , cd20 , and cd43(mt1)+/. a high proliferation rate reaching 80% of the neoplastic population was observed , as detected by ki67/mib1 immunoreactivity ( figure 3a and b ) . over the next two weeks slit - lamp biomicroscopy revealed was followed carefully by the oncology service for staging that was classified as ivb . he was receiving a regimen of chemotherapy comprising cyclophosphamide 750 mg / mq , epidoxorubicin 50 mg / mq , vincristine 1.4 mg / mq , and prednisone 50 mg / mq on days 1 and 5 . simultaneously , the patient underwent locoregional radiotherapy with a total of 20 gy to the retro - orbital region . ocular involvement of t cell lymphoma may include orbital , conjunctival , eyelid , uveal , and vitreous localization.1 malignant ocular t cell lymphoma can be associated with systemic involvement.25 despite a typical appearance , the differential diagnosis includes scleritis and several other benign tumors of the ocular surface , including squamous papilloma , pyogenic granuloma , and lymphangiectasis.4 there are two important aspects concerning this case report . firstly , the initial manifestation of this tumor was at the lower fornix of the left conjunctiva that was easy accessible to diagnostic punch biopsy , which enabled early diagnosis of t cell lymphoma . secondly , general examination showed systemic involvement with ethmoidal , maxillary and sphenoidal sinus masses , and pancreatic infiltration . lymphoid tumors of the conjunctiva are associated with systemic lymphoma in about 31% of patients.4 they may not be symptomatic for many months or years.15 in this case , we could not identify the primary site of the lymphoid tumor because ocular involvement was found to be associated with extraocular systemic lymphomatous proliferation . t cell lymphoma of the conjunctiva as a primary manifestation of systemic cancer is an extremely rare entity . punch conjunctival biopsy may be the first diagnostic pathway useful to initiate a search for systemic involvement of a malignant lymphoid tumor of the t cell variety .

this report documents a case of t cell lymphoma manifesting only with a conjunctival mass . a 67-year - old man underwent a diagnostic punch biopsy , histopathological examination , and immunohistochemical study for a pink - yellow colored mass infiltrating the bulbar conjunctiva in the lower fornix of the eyelid . a biopsy specimen of the conjunctival mass was found histopathologically to be a malignant t cell lymphoma . systemic involvement was diagnosed within four weeks after the initial diagnosis by computed tomography , showing evidence of extension at the level of the ethmoidal cells , optic nerve , periorbital tissue , and pancreas . t cell lymphoma of the conjunctiva as a primary manifestation of systemic cancer is an uncommon entity . punch biopsy may be the first diagnostic pathway useful to initiate a search for systemic involvement of a malignant lymphoid tumor of t cell lineage .

kaposi 's varicelliform eruption ( kve ) was first described in 1887 by moritz kaposi . it is a widespread cutaneous infection with a virus which normally causes localized or mild vesicular eruptions in patients with pre - existing skin diseases . a great majority is caused by infection with herpes simplex virus ( hsv ) type i and is usually called eczema herpeticum ( eh ) . localized forms are not uncommon and if complicated by secondary bacterial infection , may cause diagnostic difficulty . a rare subtype is psoriasis herpeticum which is the occurrence of kve in psoriasis . till date a 15-year old girl who was undergoing treatment for psoriatic erythroderma developed relatively asymptomatic vesicular lesions over the forehead which rapidly became pustular and spread to involve the whole face . she gave past history of recurrent episodes of erythroderma of five years duration and skin biopsy had proved it to be secondary to psoriasis . she also gave past history of recurrent episodes of painful vesicles over the lips that healed spontaneously within a week . the present episode of erythroderma was being treated conservatively with oral antihistamines and topical emollients at the time of eruption . constitutional symptoms were absent . physical examination revealed an eruption of numerous vesicles and pustules , localized to the face [ figure 1 ] . diagnosis of psoriasis herpeticum was made based on classic clinical features along with tzanck smear finding and positive hsv serology . patient improved with oral acyclovir 400 mg thrice daily along with oral cloxacillin 500 mg four times daily and skin lesions cleared completely by the tenth day [ figure 3 ] . vesiculopustular lesions localized to the face multinucleated giant cells ( leishman stain , 100 ) healed lesions after antiviral therapy kaposi 's varicelliform eruption is a distinct cutaneous eruption caused by hsv type 1 and 2 and rarely vaccinia virus or coxsackie a16 virus , superimposed on a pre - existing dermatosis . numerous nomenclatures have been used to describe the types of kve depending on either the causative virus ( eczema herpeticum , eczema vaccinatum , etc . ) or the pre - existing dermatosis ( psoriasis herpeticum ) . though kve is commonly a widespread eruption , localized forms may be seen when pre - existing dermatosis is confined to limited areas . the most common predisposing condition for kve is atopic dermatitis but it has been described in various other diseases like pemphigus foliaceus , congenital ichthyosiform erythroderma , darier 's disease , grover 's disease , familial benign chronic pemphigus , allergic contact dermatitis , irritant contact dermatitis , pityriasis rubra pilaris , rosacea , ichthyosis vulgaris , staphylococcal scalded skin syndrome , drug eruptions , cutaneous t - cell lymphoma , and lupus vulgaris . it may also follow second - degree burns , topical application of tacrolimus for atopic dermatitis , laser therapy , dermabrasion , and vaccination with bcg . kve most commonly occurs in second and third decades of life . after an incubation period of about 10 days ( range 519 days ) constitutional symptoms develop 23 days after the onset of eruption , with associated regional lymphadenopathy . till date only four cases of kve developing on pre - existing psoriatic erythroderma have been reported . the first report was by saraswat et al , in 2002 in a patient with erythrodermic psoriasis , while on treatment with methotrexate . rosenberger et al , in 2005 reported three cases of kve in psoriasis and coined the term psoriasis herpeticum . two of these patients were on oral methotrexate and the third was on acitretin , goeckerman regimen and whirlpool therapy . it is speculated that an impaired barrier function of the epidermis and defective host immune response are factors responsible for in increased susceptibility to kve . one recent study by howell et al , concluded that cathelicidin peptide ll -37 might be deficient in patients with atopic dermatitis , explaining the increased susceptibility to kve . cathelicidin family of inducible antimicrobial peptides is an integral component of innate immune response that has activity against bacteria , fungal , and viral pathogens . wollenberg et al , reported reduced levels of plasmocytoid dendritic cells , which play a role in host viral defense , in skin samples of patients with atopic dermatitis when compared to psoriasis . even though previously psoriatic skin was thought to be immune to kve , a study by goodyear et al , proved it otherwise . they found that hsv replicated quickly to a higher titre in explants of both atopic dermatitis and psoriatic skin when compared to normal skin . eczema herpeticum ( eh ) is a self - limiting disorder but carries a minor risk for severe and bilateral herpetic ocular disease . eh can be successfully treated with oral acyclovir in a dose of 200 mg five times a day or 400 mg three times a day . in children with eh , 4080 mg / kg / day is given in 34 divided doses ( maximum of 1 g / day ) .

kaposi 's varicelliform eruption ( kve ) is a widespread cutaneous eruption caused by viruses , especially herpes simplex virus in patients with pre - existing dermatoses . psoriasis herpeticum refers to the rare occurrence of kve in patients with psoriasis . we report a case of kve localized to the face in a patient with exfoliative dermatitis secondary to psoriasis . this case is being reported to make the treating clinician aware of the possibility of kve in patients with psoriatic erythroderma .

a 72-year - old woman who was diagnosed with graves disease and mild bilateral ophthalmopathy 1 year ago and treated with propylthiouracil was admitted to our hospital because of grittiness , eye discomfort , proptosis , and swelling of both eyes ; exophthalmos , eye pain , visual disturbance , conjunctive congestion and chemosis in the right eye [ fig . 2 ] . the patient had undergone corticosteroid treatment with 16 mg / day methylprednisolone for 1 month and than 30 mg / day deflazacort for 2 months for the opthalmopathy . her laboratory results were as follows ft4 : 1.19 ng / dl ( 0.7 - 1.7 ) , tsh : 2.24 mu / l ( 0.4 - 4 ) , tsh receptor antibodies and thyroid peroxidase antibodies were positive . the orbital mri images revealed exophtalmos in the right eye , an enlargement in the right extraocular muscle and in the right retro - orbital fatty tissue . the patient 's ophthalmopathy was diagnosed as asymmetrical thyroid ophthalmopathy but also , carotico - cavernous fistula was considered in differential diagnosis . a cerebral angiography was performed and a dural carotid cavernous fistula was observed on the right side [ fig . this dural ccf was draining through the right superior opthalmic vein as well as the left superior opthalmic vein via posterior intercavernous sinus connection . endovascular treatment of the right - sided superior opthalmic vein drainage was accomplished by closing the right cavernous sinus with platinum coils . however , although the small left - sided drainage was persisted since then , there was no left - sided superior opthalmic vein drainage and therefore right - sided drainage was decided to be left untreated . after 2 months , the symptoms regressed , a full vision of right eye was obtained and radiological improvement was noted [ fig . 2 ] . exophthalmos , proptosis , periorbital swelling , conjunctive congestion and chemosis in the right eye , ocular statement 2 months after embolization treatment cerebral angiography shows a dural carotid cavernous fistula on the right side draining through the right superior opthalmic vein as well as the left superior opthalmic vein via posterior intercavernous sinus connection ( arterial and venous phase ) graves disease occurs between 20 and 50 years of age and in 2 % of women . proptosis , ophthalmoplegia , orbital congestion , periorbital edema , conjunctival chemosis , ocular motility change , eyelid retraction , and uncommonly compression of the optic nerve are the most common manifestations of go . patients with severe ophthalmopathy have high titers of thyrotropin - receptor antibodies . up to half of all graves patients have ophtalmopathy ; more than 70% of the remaining half can be demonstrated to have ophthalmopathy on magnetic resonance imaging ( mri ) examination of the orbits . whenever go is a possible diagnosis , particularly in a patient with unilateral exophthalmos , orbital imaging is always indicated . villadolid et al . reported that 71% of untreated graves cases with no clinical opthalmopathy showed extraocular muscle enlargement on mri . according to the consensus statement , go can be treated by systemic gcs , surgery , or both . gcs as the first - line treatment ; if the response is poor after 12 weeks , they should be submitted to urgent surgical decompression . gcs ( with or without orbital radiotherapy ) if the orbitopathy is active ; surgery should be considered if the orbitopathy is inactive . although clinically unilateral go occurs occasionally , orbital imaging generally confirms the presence of asymmetric bilateral disease . idiopathic orbital inflammation , orbital cellulitis , primary or metastatic tumors of the orbit , orbital hemorrhage and carotid cavernous fistulas should be considered in differential diagnosis of an asymmetric or unilateral eye involvement . carotid cavernous fistulas develop between the cavernous sinus and carotid arteries as a result of abnormal connections . pulsatile proptosis , chemosis , periorbital edema , conjunctival injection , head , eye , or facial pain , double vision , glaucoma , and loss of vision are the clinical symptoms . ct and mri may show enlargement of the extraocular muscle or the superior ophthalmic vein . twenty to fifty percent of all dural carotid cavernous fistulas can be closed spontaneously ; most of them can also be closed by the endovascular embolization treatment . to the best of our knowledge of the medical literature , there is only one case report in which the carotid cavernous fistula and go occur concurrently . after radioiodine and methimazole treatment she was admitted to the hospital upon worsening of the clinical signs of ophthalmopathy . ct image findings revealed an enlargement of the right extraocular muscles and a marked prominence of the ipsilateral superior ophthalmic vein . cerebral angiography showed a dural ccf and clinical improvement was obtained following endovascular treatment . in our case , the patient who was diagnosed with graves disease with mild bilateral ophthalmopathy 1 year ago received propylthiouracil treatment since then . asymmetrical thyroid ophthalmopathy was considered as the initial diagnosis , but the mri findings and the symptoms of the right eye lead us to exclude ccf in differential diagnosis . in cerebral angiography a dural carotid cavernous fistula was observed , and after endovascular treatment full vision was obtained at the right eye . the diagnosis of go was based on the presence of symptoms and signs of both eyes and on the radiological findings . these two unusual conditions could be simultaneously occur in the same eye . increased vascularity , ocular and intracranial pressure changes , pressure on draining veins are all potential pathophysiological etiologies in patients with graves disease that could create a situation conducive to fistula formation . in conclusion , ccf 's should be considered in the differential diagnosis of the go who are not responding to the standard treatment and when there is a unilateral or asymmetrical eye involvement .

graves ophthalmopathy ( go ) is one of the frequent manifestations of the disorder which is an inflammatory process due to fibroblast infiltration , fibroblast proliferation and accumulation of glycosaminoglycans . eye irritation , dryness , excessive tearing , visual blurring , diplopia , pain , visual loss , retroorbital discomfort are the symptoms and they can mimic carotid cavernous fistulas . carotid cavernous fistulas are abnormal communications between the carotid arterial system and the cavernous sinus . the clinical manifestations of go can mimic the signs of carotid cavernous fistulas . carotid cavernous fistulas should be considered in the differential diagnosis of the go patients especially who are not responding to the standard treatment and when there is a unilateral or asymmetric eye involvement . here we report the second case report with concurrent occurrence of go and carotid cavernous fistula in the literature .

vitamin d deficiency is common in children , with an estimated prevalence of 9% in american children aged 1 - 21 years ( serum vitamin d less than 15 ng / ml ) . the association between vitamin d , antiepileptic drugs , and poor bone health in individuals with epilepsy was first recognized in 1979 and is thought to be particularly relevant in childhood as this is the time of maximum bone mineralization . children and adolescents treated with antiepileptic drugs are known to have problems with bone metabolism , bone mineral density loss , and 23 times the fracture risk of healthy controls . it has been postulated that this might be because of the effects of antiepileptic drugs and the ketogenic diet , and confounding factors such as mobility , nutrition , and obesity . the mechanism of bone problems may be related to increased liver vitamin d breakdown secondary to induction of cytochrome p450 and stimulation of osteoclastic activity , direct effects on bone cells , resistance to parathyroid hormone , inhibition of calcitonin secretion , and impaired calcium absorption . significant disparity exists among clinicians , with less than half performing any sort of screening for vitamin d status and bone health in patients with epilepsy . the international society for clinical densitometry has not specified epilepsy in its recent position development statement as an indication for bone mineral density measurements in children . a cochrane review in 2009 , which did not include a recent randomized controlled trial , found no reliable evidence to support the routine use of vitamins in patients with epilepsy . the american academy of pediatrics has recommended that all children receive 400 iu of vitamin d supplements as infants and continue through adolescence , but does not recommend vitamin d doses specifically for children on antiseizure drugs . however , a recent systematic review and meta - analysis of randomized controlled trials concluded that vitamin d supplementation is not effective in improving bone mineral density in healthy children and adolescents , but suggested that supplementation of deficient children may be clinically useful . table 1 lists 26 articles which are studies of markers of bone health in children with epilepsy . there are 14 cohort studies comparing bone mineral density and/or bone biochemistry in children treated with antiepileptic drugs and healthy controls . of these , seven studies found a significant difference , four found no significance , and three studies did not comment on the statistical significance of any difference between the groups . there are 12 cohort studies observing bone mineral density and/or bone biochemistry in children treated with antiepileptic drugs . these found a prevalence of 25-oh vitamin d levels less than 20 of between 25% and 75% in children treated with antiepileptic drugs ( with varying degrees of ambulation , duration of epilepsy , and number of antiepileptic drugs ) . there is great variation between studies in terms of controlling for confounding factors ( e.g. mobility and diet ) . table 2 shows six studies of vitamin d therapy in children treated with antiepileptic drugs ( aeds ) . the only randomized controlled trial was limited in terms of duration of therapy , lack of controlling for diet / exercise , and lack of study of compliance despite not attaining target vitamin d levels . it demonstrated no significant difference between high- and low - dose vitamin d , and no change in bone mineral density compared to healthy controls after 1 year of treatment . this may be a better outcome than in unsupplemented children with epilepsy whose bone mineral density has been shown to decrease with time in previous studies . two cohort studies showed a significant increase in bone mineral density ( one large and one small ) , two cohort studies showed a significant improvement in bone biochemistry , and one cohort study showed a significant improvement in bone biochemistry and healing of rickets with vitamin d therapy . three of the six studies used bone biochemistry as an indirect marker of bone health , rather than bone mineral density . it is difficult to compare the bone mineral density improvements as the studies use different units rather than a standardized z - score . as there is little description of comorbidities ( the rct is the only study to give a detailed description of comorbidities ) , there is potential for confounding with regard to comorbidities , such as mal - absorptive disorders , treatment with drugs other than aeds which may affect bone health , nutrition , and immobility in those studies not pre - selecting an immobile group . most of the studies of bone mineral density markers in children with epilepsy [ table 1 ] have found little significant difference in bone mineral density markers in children with epilepsy . however , these studies may be biased as most included a small sample , and did not include large enough numbers to enable comparison between specific antiepileptic drugs or therapies , or between different epileptic syndromes . of those studies in which this comparison was attempted , a significant association between sodium valproate and markers of poor bone health was found ; but as this was only in small numbers , it might represent a spurious association that has not been excluded . in addition , many of the studies did not correct for confounding factors such as mobility , nutrition , and obesity . one small study that considered the effect of ambulatory status did however , find a significant difference in bone health between ambulant and non - ambulant children . studies of vitamin d therapy in children with epilepsy have been similarly limited by lack of stratification with regard to factors that influence bone health such as comorbidities , nutrition , obesity , and mobility . hence although there is little evidence for an effect , there is limited evidence for no effect . the only randomized controlled trial was limited in terms of duration of therapy , lack of controlling for diet / exercise , and lack of study of compliance despite not attaining target vitamin d levels . studies to date are inconsistent and of limited quality . in particular , there is a need for larger studies , using clinically significant outcomes such as fractures , including at risk populations such as symptomatic generalised epilepsy , impaired mobility , and polytherapy . at the present time in the absence of good evidence to the contrary , there remains concern that children with epilepsy are at risk of poor bone health and that vitamin d therapy may be beneficial . as low - dose vitamin d supplementation ( 400 iu per day ) is now recommended for healthy children and it is biologically feasible that children with epilepsy may be at higher risk of clinically significant deficiency , it is important that neurologists ensure that low - dose vitamin d supplementation should be prescribed and compliance followed up in children with epilepsy .

children and adolescents treated with antiepileptic drugs are known to have problems with bone metabolism , bone mineral density loss , and 23 times the fracture risk of healthy controls . we reviewed the literature regarding bone mineral density in children with epilepsy and vitamin d therapy in children treated with anti - epileptic drugs . studies of bone mineral density markers in children with epilepsy have mostly found little significant difference in bone mineral density markers in children with epilepsy . they have been limited by small sample size and many of the studies have not corrected for confounding factors such as comorbidities , mobility , nutrition , and obesity . studies of vitamin d therapy in children with epilepsy have shown little evidence of effect and have been similarly limited by lack of stratification with regard to confounding factors . there is a need for larger studies , using clinically significant outcomes such as fractures , including at risk populations such as symptomatic generalised epilepsy , impaired mobility , and polytherapy . at the present time in the absence of good evidence to the contrary , there remains concern that children with epilepsy are at risk of poor bone health and that vitamin d therapy may be beneficial . as low - dose vitamin d supplementation ( 400 iu per day ) is now recommended for healthy children and it is biologically feasible that children with epilepsy may be at higher risk of clinically significant deficiency , it is important that neurologists ensure that low - dose vitamin d supplementation should be prescribed and compliance followed up in children with epilepsy .

ludwig 's angina is a potentially life - threatening infection of the sublingual and submandibular space which involves the neck and floor of the mouth . it is one of the rare medical emergencies in a critical care setting , which could turn out to be devastating if unidentified at the earliest . if angioneurotic edema is coincidental with features of ludwig 's angina , it becomes more challenging . this case report adds more information in managing ludwig 's angina along with antibiotic adverse reactions . a 52-year - old obese gentleman initially presented to a local hospital with complaints of fever , shortness of breath for 10 days , and wheezing for the last 3 days . he was treated for acute exacerbation of asthma and referred to our hospital . in the ward , he was treated for asthma exacerbation with oxygen by facemask , nebulization ( budesonide , ipratropium , and levosalbutamol ) , and intravenous hydrocortisone ( 100 mg for every 8 h ) . the patient 's condition improved and he was planned to be discharged on the third day from hospital admission . on the day of discharge , he developed fever , trismus , dysphagia , submandibular pain , and swelling in the neck causing airway obstruction . ultrasonography ( usg ) neck was done which reported enlarged parotid and submandibular glands with cervical lymphadenopathy . the patient became drowsy and was immediately shifted to the intensive care unit ( icu ) . suspecting ludwig 's angina , piperacillin / tazobactam ( 4.5 g for every every six hours ) was prescribed . on icu admission , the patient was normotensive , tachycardic ( 120 beats / min ) , tachypneic ( 26 breaths / min ) , and had a high - grade temperature ( 101.3f ) . his arterial blood gas revealed respiratory acidosis ( ph 7.19 , pco2 83.2 mmhg , and hco3 32.1 meq / l ) . he had leukocytosis with white blood cell count of 20,300 cells / mm ( normal range 450012,500 cells / mm ) . a follow - up fiber - optic laryngoscopy examination confirmed edema of the nasopharynx and oropharynx causing obstruction to the airway with normal glottis . in view of increasing neck swelling and persistent airway obstruction , a difficult intubation was anticipated and preceded with surgical tracheostomy . on the third dose of piperacillin / tazobactam , the patient developed facial edema , redness , and erythematous papules all over the body , and the patient was treated with clindamycin ( 600 mg for every six hours ) and metronidazole ( 500 mg for every eight hours ) . computed tomography ( ct ) neck was done once the patient was stabilized which revealed extensive subcutaneous and mediastinal emphysema , edematous changes in the subcutaneous and intermuscular planes of neck , mucosa of pharynx , and had cervical lymphadenopathy [ figures 1 and 2 ] . he was weaned off the ventilator and transferred to the ward after five days of icu stay . the swelling completely subsided ; tracheal tube was decannulated and he was subsequently discharged after 12 days of hospitalization . he was advised to take inhalers ( tiotropium , levosalbutamol , and budesonide ) and continue clindamycin ( 600 mg for every six hours ) and metronidazole ( 500 mg for every eight hours ) to complete a course of 10 days . the patient was explained about the adverse drug reactions he had during hospitalization . he was also advised to be cautious when he takes any medicines and to reveal the history of drug allergies whenever he visited a doctor . the patient had proper follow - up with the pulmonology outpatient department and remained stable with no further episodes of exacerbation . ludwig 's angina is an uncommon , aggressive , rapidly progressing , and fatal infection with high mortality rate if unidentified and not treated appropriately . more than 70% of the cases have odontogenic etiology , while the other causes include foreign body , tonsillitis , epiglottitis , pharyngitis , oral laceration , mandible fracture , endotracheal intubation , traumatic bronchoscopy , malignancy , or surgery . this patient did not have any typical dental history , hence strongly indicates the other causes of ludwig 's angina . the most common causative organisms involved are the oral flora : streptococcus species , staphylococcus species , and anaerobes . clinical manifestations include fever , tachypnea , tachycardia , swelling and pain in the oral cavity and anterior of neck , dysphagia , stridor , hoarseness , and respiratory distress . it can present as a mild infection and evolves quickly to brawny bilateral neck swelling , tongue edema , fever , and dysphagia . ensuring the airway is most important in the treatment of ludwig 's angina . considering that this case had continuous airway obstruction and difficult intubation , he was electively taken for surgical tracheostomy . piperacillin / tazobactam or benzyl penicillin with metronidazole are the preferred first - choice antibiotics in perimandibular infections . for patients allergic to penicillins , alternative treatment includes clindamycin or vancomycin . it becomes graver and challenging when the patient develops hypersensitivity reactions to most of the treatment options . there is high chance of cross - reactivity among the beta - lactam group of antibiotics including penicillins , cephalosporins , and carbapenems , unless reviewed separately for skin testing , graded challenge test , and desensitization . neck radiography and usg are useful to identify the soft - tissue involvement . after assuring the airway , ct and magnetic resonance imaging ( mri ) are useful modalities to define the extent of soft - tissue infection . in this case , ct imaging was done after securing airway ; it defined the space infections with great clarity . early identification , securing the airway , and antibiotic administration are the keystone to better survival . the establishment of airway is done by fiber - optic intubation , tracheostomy , or cricothyroidotomy . ct and mri are useful modalities to define the extent of soft - tissue infection , considered only after assuring the airway .

a 52-year - old obese gentleman presented to the hospital with complaints of fever and shortness of breath for 10 days . he was admitted in the ward and treated for acute exacerbation of asthma . he was shifted to the intensive care unit ( icu ) for persistent fever , neck swelling , airway obstruction and desaturation . ludwig 's angina was suspected and computed tomography of neck confirmed it . a difficult airway was anticipated and preceded with surgical tracheostomy . the patient had hypersensitivity reactions to piperacillin / tazobactam ; hence , he was treated with clindamycin and metronidazole . the patient improved and was discharged after five days of icu stay and 12 days of hospitalization . this case summarizes the rare incidence of ludwig 's angina with antibiotic adverse reactions . if angioneurotic edema is coincidental with features of ludwig 's angina , it becomes more challenging . early identification , securing the airway , and antibiotic administration are the keystone to better survival .

daptomycin is licensed in the usa and europe for the treatment of complicated skin and soft tissue infections caused by gram - positive organisms , including both susceptible and resistant strains of staphylococcus aureus , at a dose of 4 mg / kg daily . additionally , it had been licensed for the treatment of various infections due to susceptible organisms , including serious and life - threatening gram - positive infections , vancomycin - resistant enterococcal ( vre ) infections and endocarditis with associated bacteraemia at 6 mg / kg once daily . several studies also established dosing recommendations in patients undergoing various modes of renal replacement therapy [ 15 ] . daptomycin is not only primarily ( 78% ) excreted unchanged in the urine but also shows a favourable in vitro activity against gram - positive uropathogens , making it an ideal candidate to treat severe urinary tract infection ( uti ) . despite the rising prevalence of vancomycin - resistant enterococci in patients with complicated uti , there is a paucity of clinical data on daptomycin in this clinical situation . here , we now report the first case of successful uti treatment caused by vre using daptomycin in a renal transplant recipient with a complex medical history . a 68-year old caucasian female was admitted to the emergency room of our tertiary care hospital with rapidly progressing fever and hypotension . upon arrival the malnourished patient ( height 158 cm ; weight , 46 kg ; body mass index 18.4 kg / m ) had a blood pressure of 88/56 mmhg and a heart rate of 110/min . she presented with nausea and dizziness , but was conscious ( glasgow coma scale 13 ) . her past medical history was significant for kidney transplantation about 6 months prior to the admission due to underlying hypertensive kidney disease . she suffered from leucoencephalopathy as well as liver function impairment due to cirrhosis after longstanding alcohol abuse until 6 years prior to admission . laboratory analysis was remarkable for increased inflammatory parameters [ white blood count 22 000/l , c reactive protein ( crp ) 356 mg / l ] . mol / l when compared with the last available outpatient creatinine of 99 mol / l 7 days before . liver function test showed an increase in aspartate aminotransferase ( 143 u / l , upper limit 31 u / l ) and alanine aminotransferase ( 124 urinary sediment was positive for leucocytes ( > 20/view field ) and erythrocytes ( 1120/view field ) without the presence of any casts . after transfer to our unit the initial antibiotic therapy was switched from meropenem to daptomycin ( first dose 10 mg / kg body weight followed by 7.7 mg / kg doses ) . this was deemed to be prudent as the patient 's last uti , 4 months prior to this admission , was caused by a vancomycin - resistant enterococcus faecium , tested to be susceptible only to gentamycin and linezolid . although we wanted to avoid the former one due to its nephrotoxicity , especially in the state of acute kidney injury , the latter one was deemed inappropriate due to the impaired liver function , as the liver plays an important role in the elimination of the drug . in another culture , persistence of a vancomycin - resistant e. faecium was confirmed ( minimum inhibitory concentration : vancomycin 8.0 mg / l ; linezolid 0.5 mg / l ) . unfortunately , daptomycin was not tested in this resistogram as , at that time , it was not part of the routine test panel . the peak crp declined rapidly and function of the renal graft improved over the 5-day therapy ( figure 1 ) . 1.serum creatinine and crp levels of a renal transplant patient at last outpatient visit as well as during the hospital course / treatment of severe uti with daptomycin . serum creatinine and crp levels of a renal transplant patient at last outpatient visit as well as during the hospital course / treatment of severe uti with daptomycin . uti is the most common infection following renal transplantation , accounting for about half of all infectious complications in this patient population . in the first month after transplantation resistance to multiple antibiotics , including vancomycin , has increased in prevalence , particularly in infections involving e. faecium , where the rate of vancomycin - resistance can be as high 10% in north america and 7% in europe , varying in european countries . especially in patients with impaired renal function and concomitant hepatic dysfunction the management of utis caused by enterococcus spp . has become challenging because of the limited therapeutic options available that almost exclusively consist of nephrotoxic or hepatically eliminated drugs . our case as well as previous anecdotal reports suggest that daptomycin might be an effective alternative for uti caused by vancomycin - resistant enterococci as it is neither harmful for the kidney nor cleared by the liver . j.t.k . has received funds for speaking at symposia organized on behalf of novartis germany and has also received funds for research from novartis germany .

there is an increasing prevalence of urinary tract infection ( uti ) caused by multiresistant gram - negative enteric bacilli such as extended - spectrum beta - lactamase as well as gram - positive enterococci whose vancomycin - resistance can be as high as 25% . we report on a 68-year - old caucasian female with a uti caused by a vancomycin - resistant enterococcus faecium , only tested to be susceptible to gentamycin , linezolid and daptomycin . within a day after administration of the bactericidal daptomycin clinical and laboratory signs of infection regressed and graft function recovered . our case suggests that daptomycin might be an effective alternative for uti caused by vancomycin - resistant enterococci .

the peak incidence is in the third and fourth decades , between 25 and 35 years of age . the second peak of prevalence occurs in the 71 - 90-year - old age group , with metastasis and lymphoma most common . the third smaller peak occurs in children where yolk sac tumors and teratoma occur . of testicular tumors , germ cell tumors ( gcts ) non - seminomatous gcts include embryonal cell carcinoma ( 20 - 25% ) , teratoma ( 5 - 10% ) , choriocarcinoma ( 1 - 3% ) , and mixed tumors ( 20 - 40% ; a mixture of virtually all histological types).1 the term burned - out testicular tumor refers to a regressed testicular tumor which presents with its metastases . since it was first described by prim in 1927 , around 70 cases were reported in the literature , mostly in literature related to pathology and urology a 22-year - old male complained of hemoptysis , weight loss , and abdominal pain for the past 2 weeks . chest x - ray showed bilateral , multiple , variable size opacities suggestive of metastasizes ( figure 1 ) . at king khalid university hospital , workup to assess for the primary source was performed which include scrotal ultrasonography that revealed bizarre - shaped macrocalcification ( focal clump of calcification ) is present in the central aspect of the right testis ( figure 2 ) . computed tomography ( ct ) scan of abdomen and pelvis showed large para - aortic complex soft tissue lesions with central necrosis representing enlarged lymph nodes as a result of secondary deposits ( figure 3 ) . the patient s serum -human chorionic gonadotropin ( -hcg ) level was elevated , i.e. , 9020 iu / l . other laboratory investigations including -fetoprotein chest x - ray showing bilateral , multiple , variable size , pulmonary nodules ultrasound of right testis showing a focal clump of calcification present in central aspect of testis . a hypoechoic irregular halo was noted abdominal computed tomography scan showing a large retroperitoneal mass on the left side of the aorta with central necrotic component trucut biopsy was obtained from one of the lung nodules , which showed microscopic features of hemorrhagic necrotic tissue with scattered mono- and multinucleated malignant cells . a panel of immunohistochemical stains was performed and shows that the tumor cells are positive for cytokeratin pan , epithelial membrane antigen , and -hcg . four months later , the patient present with slurred speech and decrease attention , for which he undergo magnetic resonance imaging brain showed hemorrhagic left temporoparietal space occupying lesion with adjacent vasogenic edema highly suggestive of hemorrhagic metastasis . the term burned - out testicular tumor refers to a regressed testicular tumor which presents with its metastases.2 the pathogenesis of this phenomenon may be that the high metabolic rate of the tumor causes it to rapidly outgrow its blood supply . the patients may present with widespread metastases , but no primary tumor except for an area of calcification within the testis.3 they often demonstrate little or no remaining viable tumor , with mostly scarring and fibrosis found at histologic analysis after orchiectomy . the syncytiotrophoblasts involved in these lesions with choriocarcinoma produce -hcg which is raised in these tumors . with early widespread metastasis , patients may present with symptoms referable to their metastases rather than a palpable testicular mass . the primary tumor and metastases are often hemorrhagic.4 patients with mixed gcts with choriocarcinoma fair better than those with pure choriocarcinoma tumors , but a very high level of -hcg ( 50,000 iu / l ) has a poor prognosis with a 5-year survival rate of 48%.4 high - resolution sonography of the scrotum with linear high - frequency transducers allows the detection of small , highly echogenic foci , hypoechoic zones , microlithiasis , or microcalcifications . at scrotal us , they appear as a hypoechoic or ill - defined , intratesticular calcified lesion . during ct scan of the abdomen , when large retroperitoneal tumors or lymph nodes are detected in young men , ultrasound examination of bilateral testis is recommended , even if a mass is not palpable in the scrotum.5 scrotal sonography is very important for the detection of intratesticular lesions , especially in patients with extragonadal metastatic involvement and normal palpation for the testis . a burned - out testicular tumor should be considered when punctuate echogenic foci are seen without any evidence of hypoechoic mass lesions . metastatic disease secondary to burned - out lesion has the same prognosis as a primary testicular malignancy .

burned - out testicular tumor is a very rare clinical entity . there is no clinical finding in the testicle because it regresses spontaneously without any treatment and generally presents with metastases . clinical examination of the testis and scrotal sonography are pivotal in the initial diagnosis of such neoplasms . we present a case of a 22-year - old male with hemoptysis , weight loss , and abdominal pain for the past 2 weeks and no palpable lesion on testicular examination . no relevant past medical history .

abnormal fusion of mullerian ducts or insufficient absorption of the uterine septum results from anatomical changes in the female genital system . the least frequent form of these changes is the unicornuate uterus with a rudimentary horn . the frequency of congenital uterine anomalies in a fertile female population is about 1/200 to 1/600 , whereas the frequency of rudimentary horn is 1/100 000 . this anatomical abnormality may lead to many obstetrical and gynecologic complications . besides gynecologic complications , such as endometriosis , primary infertility , and hematometra , anomalies of the urinary system and obstetrical problems , such as malpresentation , habitual abortus , and premature birth , can occur . the patient is a 27-year - old , nulligravid , woman admitted to the emergency service of the inonu university medical faculty with acute abdomen and hospitalized in the obstetrics and gynecology clinic . her age at menarche was 13 , and she had severe dysmenorrhea since age 14 . her menstrual cycle was 28 days , and her menstrual period was about 5 to 7 days with a normal amount of bleeding . since marriage , 3 years earlier , she and her husband had been using the coitus interruptus method of contraception . she had an approximately 3x4-cm palpable mobile , tender , hard mass in the right adnexal region . blood pressure was 90/60 mm hg , with a heart rate of 108 per minute . laboratory values were as follows : hemoglobin 10.5 g / dl , hematocrit 37% , white blood cell count 12 000 per mm , platelet count 105 000 per mm . ultrasonographic evaluation revealed a 36x39-mm smooth contoured , homogenous solid mass in her right adnexal region . exploratory findings during laparoscopy were a left uterine deviation with normal anatomical localization and normal size of the left tube . the left round ligament arose from the left uterine cornual region ; however , the right round ligament arose from the rudimentary horn . the noncommunicating rudimentary horn , approximately 45 mm in size , suspended by a fibrous band to the uterus was seen on the right side ( figure 1 ) . the right uterine tube arose from the superior portion of the rudimentary horn and had a normal anatomic shape and size . because a morcellator was unavailable , the excised rudimentary horn was put into a glove and removed through a 3-cm incision on a suprapubic trocar insertion site . evaluation of the urinary tract with radiographic methods was planned postoperatively , but the patient conceived 2 months after the operation , and urinary tract evaluation was postponed . noncommunicating rudimentary horn , 45 mm , suspended by a fibrous band to the uterus visible on the right side . unilateral hypoplasia of the mullerian duct is a congenital anomaly resulting from a rudimentary horn . the pathology is classified into 4 groups by the american society of reproductive medicine ( asrm ) as unicornuate uterus with communicating rudimentary horn , unicornuate uterus with noncommunicating rudimentary horn , isolated unicornuate uterus , and noncavitated unicornuate uterus with noncommunicating rudimentary horn . generally , such abnormalities result in an ectopic pregnancy ( 22% ) and spontaneous abortion ( 16% ) . diagnosis of this pathology can be made incidentally during gynecologic examination or during surgical intervention because of acute abdomen as seen in our case . possible causes of abdominal pain in these patients are the distention of the uterus because of blood accumulation in the noncommunicating cavity of the rudimentary horn , hematometra , pyometra , and torsion . another problem with the noncommunicating rudimentary horn is rudimentary horn pregnancy . since myometrial tissue is thin in a rudimentary uterus , uterine rupture the presence of gestation in a noncommunicating rudimentary uterus can be explained by transperitoneal migration of sperm . the pain of endometriosis in these cases is usually serious and can cause severe dysmenorrhea , chronic pelvic pain , and dyspareunia . surgical removal of the noncommunicating horn is commonly performed if it is thought to contain functional endometrium , to prevent endometriosis and pregnancy complications . in our case , removal of the horn could have resulted in relief of dysmenorrhea complaints , but we are unable to observe it because the patient conceived after the operation . a review of the literature shows that operative laparoscopy can be used for removal of a rudimentary horn successfully and minimally invasively . especially for the younger women in the fertile period as in our case , the rudimentary horn must be excised because the intervention will prevent possible endometriosis development . thus , besides complications , such as torsion , distention , and acute abdomen , possible infertility is avoided .

unicornuate uterus with a rudimentary horn is the rarest congenital anatomic anomaly of the female genital system , causing many obstetrical and gynecologic complications . the frequency of this pathology is approximately 1/100 000 . a rudimentary horn usually develops following insufficient development of mullerian ducts . these patients present with dysmenorrhea , dyspareunia , and chronic pelvic pain because of endometriosis and rarely with acute abdominal symptoms following distention and torsion of the noncommunicating rudimentary horn . the case of a patient referred for acute abdomen after distention of a noncommunicating rudimentary horn is presented herein .

genetic epilepsy with febrile seizures plus ( gefs ) is characterized by childhood - onset epilepsy syndrome with febrile seizures ( fs ) and a variety of afebrile epileptic seizures . its inheritance is autosomal dominant and its spectrum is comprised of a range of mild to severe phenotypes varying from classical febrile seizures to dravet syndrome . gefs patients manifest a mutation encoding voltage - gated sodium channel subunits ( scn1a , scn1b , scn2a ) and gaba receptor subunits ( gabrg2 , gabd ) . typically , the mutations are missense mutations , and about 80% of affected individuals present with some form of seizure disorder . some families include individuals with focal epilepsy , particularly temporal lobe epilepsy ( tle ) of varying severity . while surgical resection is one treatment option for the drug - resistant focal symptoms of gefs , due to the genetic defect , children are unlikely to benefit from cortical resection . we treated a girl with an scn1a mutation and generalized tonic - clonic- and complex partial seizures ( gtcs , cps ) by vagus nerve stimulation ( vns ) . this girl had the first fs at the age of 6 months ; in the next 8 months she had 6 more fss . multiple antiepileptics , i.e. clobazam , valproate , phenobarbital , carbamazepine ( cbz ) , lamotrigine , and levetiracetam failed to inhibit her seizures and she was admitted to our hospital at the age of 5 years . long - term video - eeg could detect 4 cpss off - drug state . during 3 cpss , we observed conjugate deviation to left , extension of the left limbs , flexion of the right limbs , and body - axis rotation to left after motion arrest . eeg showed diffuse polyspikes in the right hemisphere followed by high - voltage 12 hz waves at t6 and o2 ( fig . 1 ) . during a cps observed in the later part of the monitoring period spike waves began at o1 and t5 with oral automatism , conjugate deviation to right , and right body - axis rotation . no abnormalities were detected on mri- , iomazenil- and imp - spect- , and fdg - pet studies . meg showed a broad dipole cluster in the right posterior temporal- , parietal- , and occipital lobe ( fig . there were no gtcss in the afebrile state during the monitoring period , but explanation by her parents indicated she had primary gtcs without secondary generalization in afebrile state and often coincided with fever . her father and brother had a history of fs ; her sister suffered afebrile seizures . gene examination after vns implantation detected the mutation ; scn1a encoded the -subunit of a sodium - gated channel . vns in the first year decreased the frequency and severity of both gtcss and cpss ( fig . 3 ) . the seizures were remained , and cbz was deduced to avoid the possibility of seizure induction by sodium channel blockers in scn1a - related seizure disorders . her intelligence quotient was in the normal range and she attends a regular elementary school . gefs families are grouped into 4 broad subphenotypes , i.e. classical gefs , borderline gefs , unclassified epilepsy , and an alternative syndromal diagnosis . borderline and classical gefs share many characteristics ; early - onset fss with focal epilepsies including cpss is a phenotype they have in common . barba et al . suggested that scn1a gene mutations and malformations during cortical development may reciprocally affect each other in determining the mechanisms that underlie seizure generation . . performed neocortical resection in 6 scn1a mutation - positive children with treatment - resistant epilepsy . they concluded that cortical resection was unlikely to be beneficial due to the genetic defect and unexpected mild diffuse cortical malformations . a meta - analysis revealed a genome - wide significant association between mesial tle with hippocampal sclerosis and febrile seizures at the sodium - channel gene cluster on chromosome 2q24.3 of scn1a . no patient who underwent resective surgery for tle with an scn1a mutation however , 2 patients with gefs with an scn1b mutation were successfully treated by temporal lobectomy . both had the c121w mutation , a characteristic of the scn1b mutation ; one patient also presented with hippocampal sclerosis . terra et al . reported a post - vns implantation seizure reduction of approximately 50% in 36.8% of patients at year 1 , in 43.2% at year 2 , and in 42.7% at year 3 . vns is thought to modulate electrical stimuli to the nucleus tractus solitarius and the brainstem reticular formation , and to interrupt the characteristic synchronous activity of seizures . also in this patient , gtcs and cps in the first year after vns were decreased at the same drug condition before operation . generally , sodium channel blocker can induce or increase seizures in scn1a - related seizure disorders . but sodium channel blocker would effective in this patient and coordination of antiepileptics could decrease the seizures during follow up periods ( table 1 ) . cerebrospinal fluid studies showed a significant increase in gaba after 34 months of vns , but no significant decrease in glutamate , aspartate , or 5-hiaa after 3 to 9 months of vns . in a mouse model , the scn1a mutation predominantly impaired sodium - channel activity in gabaergic interneurons and led to decreased inhibition without affecting excitatory cortical pyramidal neurons . this pathogenesis suggests that vns is a suitable treatment for pharmacoresistant gefs with the scn1a mutation as it exerted favorable effects on dravet syndrome with the scn1a mutation . focal epilepsies including complex partial seizures are considerably less common in gefs + spectrum . vns would become good treatment option to pharmacoresistant gefs + with both refractory generalized tonic - clonic seizures and partial seizures .

genetic epilepsy with febrile seizures plus ( gefs+ ) is characterized by childhood - onset epilepsy syndrome . it involves febrile seizures and a variety of afebrile epileptic seizure types within the same pedigree with autosomal - dominant inheritance . approximately 10% of individuals with gefs+ harbor scn1a , a gene mutation in one of the voltage - gated sodium channel subunits . considerably less common are focal epilepsies including complex partial seizures . we report vagus nerve stimulation ( vns ) in a 6-year - old girl with gefs+ who exhibited refractory generalized tonic - clonic seizures and complex partial seizures .

postoperative portomesenteric venous thrombosis ( pmvt ) is a potentially devastating morbidity , occurring after gastrointestinal and laparoscopic surgeries . the procoagulant obese state , laparoscopy , and manipulation of the portomesenteric venous system ( pmvs ) probably contribute to pmvt . often nonspecific in presentation , diagnosis is made through a high index of suspicion and imaging . we report a case of superior mesenteric venous thrombosis , which was detected after laparoscopic sleeve gastrectomy ( lsg ) and was successfully managed . an lsg was performed for a 50-year - old morbidly obese ( body mass index- 52 kg / m ) , hypertensive , and sleep apnoeic patient . clexane ( enoxaparin ) 60 mg ( sanofi - aventis , surrey , uk ) subcutaneously 12 hours before surgery . a thigh - length graduated compression elastic stocking was applied to the lower limbs before shifting to the operation theater , supplemented by thigh - length intermittent pneumatic compression ( ipc ) device ( kendall scd express , covidien , ma , usa ) applied prior to induction of anaesthesia till ambulation the next morning . a four - port technique was employed along with liver retraction provided by a nathanson liver retractor . gastrolysis was performed by ultrasonic shears ( harmonic ace , ethicon endo - surgery , puerto rico , usa ) . alesser curve gastric sleeve was created using the echelon flex 60 stapler ( ethicon endo - surgery , cincinnati , ohio , usa ) with six - row cartridges ( endoscopic linear cutter reloads , ethicon endo - surgery ) over an endoluminal bougie ( 12.7 mm [ 38 fr ] gastric calibration tube , ethicon endo - surgery ) . the suture was imbricated with continuous 2 - 0 polydiaxonone sodium ( pds ) suture ( ethicon endo - clip suture , ethicon ) . intraoperative endoscopy was performed to confirm gastric sleeve size , hemostasis and integrity . a jackson pratt drain ( biovac , biometrix , the netherlands ) was then placed along the staple line . the procedure lasted 135 min and was performed under a 15 mmhg carboperitoneum . the portomesenteric circulation was not visualized or manipulated . the patient was ambulated and introduced to clear liquids day 1 postoperatively after an upper gastrointestinal gastrograffin contrast study demonstrated the integrity of the gastric sleeve . he was discharged on postoperative day 3 after removal of the surgical drain and was on daily subcutaneous low molecular weight heparin ( inj . the patient presented with progressive central abdominal pain and nausea , 15 days after lsg . on examination , he was found to be afebrile and having normal vital parameters . was performed to screen the very obese abdomen , which revealed thrombosis of the superior mesenteric vein ( smv ) extending till the portal confluence with dilatation of proximal small bowel [ figures 1 and 2 ] . abdominal noncontrast computed tomogram demonstrating superior mesenteric vein pathology abdominal contrast - enhanced computed tomogram demonstrating superior mesenteric vein thrombosis there was no history of other risk factors for thrombosis . he was started on subcutaneous low molecular weight heparin ( inj.fraxiparine 5700 units subcutaneously twice daily ) . with continued anticoagulation , the patient remained asymptomatic and completed a 3-month course of oral anticoagulation in consultation with a vascular physician . a follow - up scan at 2 months revealed no thrombus in the smv and a hematological workup for thrombophilia revealed no thrombophilic disposition . more than 30 cases of pmvt have been reported after an lsg accounting for 0.3%-1% of post - lsg morbidity . the clinical presentation is variable and ranges from subtle , nonspecific abdominal discomfort to severe pain with most cases presenting about 2 weeks postsurgery and being symptomatic about 2 days before presentation . the best initial imaging modality to confirm the diagnosis currently is an abdominal doppler ultrasound or cect . the management of pmvt post - bariatric surgery , is contingent on the length and viability of the affected segment of the bowel . the duration of post - thrombotic anticoagulation is contingent on underlying thrombophilic states and reports in literature range from 6 months to a year for those without , to life - long prophylaxis for those with pre - existing thrombophilia . we advocate a close follow - up with noninvasive imaging modalities to confirm dissolution of thrombus and prevent recurrence . anticoagulation was discontinued at 3 months as the patient remained asymptomatic , became pmvt - free , and to mitigate the risk of bleeding due to continued anticoagulation . a prothrombotic state secondary to pre - existing thrombophilia , morbid obesity , medications , laparoscopy , and possible operative manipulation or interruption of segments of the pmvs , probably contributes to postbariatric pmvt . considering the not uncommon incidence of this serious morbidity and the procoagulant state in bariatric surgery , certain authors have prompted the listing of pmvt as a formal complication of bariatric surgery and its inclusion in the clinical differential for abdominal pain after bariatric surgery . we suggest that a high index of suspicion be maintained postbariatric surgery to detect and promptly treat pmvt . all bariatric surgical patients complaining of nonresolving abdominal discomfort or pain in the postoperative period should be evaluated for pmvt by noninvasive imaging ! the current experience is nascent , but growing evidence could lead to the routine surveillance of pmvt in the postbariatric surgical patient . as bariatric surgery grows in volume , pmvt may increasingly be prophylaxed for , screened for , found , and treated .

postoperative portomesenteric venous thrombosis ( pmvt ) is being increasingly reported after bariatric surgery . it is variable and often a nonspecific presentation along with its potential for life - threatening and life - altering outcomes makes it imperative that it is prevented , detected early and treated optimally . we report the case of a 50-year - old morbidly obese man undergoing a laparoscopic sleeve gastrectomy who developed symptomatic pmvt two weeks postsurgery , which was successfully treated by anticoagulant therapy . we provide postulates to the etiopathological mechanism for this thrombotic entity . the growing recognition that obesity and bariatric surgery create a procoagulant state regionally and systemically provides impetus for designing the ideal protocol for pmvt prophylaxis , which could be more common than currently believed . we support the early screening for pmvt in the postbariatric surgical patient with unexplainable or intractable abdominal symptoms . the role of routine surveillance and the ideal duration of post - pmvt anticoagulation is yet to be elucidated .

a 58-year - old man who has had lower urinary tract symptoms for a year , developed acute urinary retention , and was subsequently catheterised . on examination , trial of void was attempted with alpha - blocker medication ( alfuzosin ) but failed . he was then planned for transurethral resection of the prostate ( turp ) but it was postponed a few times due to active urinary tract infection . while waiting for his surgery , he had a 2-weekly change of his catheter . during the last change of catheter , it was found to be stuck and the balloon of the catheter could not be deflated . an ultrasound was done but the balloon was not seen and the tip of the catheter was seen to be calcified . there was also surrounding haematoma and clots . an attempt to puncture the balloon with a spinal needle under ultrasound guidance the tip of the catheter with the balloon was stuck at the base of the bladder neck . a suprapubic puncture was made under ultrasound guidance and a size 16 french ( f ) urinary bladder catheter was inserted . after 2 weeks , a guide wire was inserted through the suprapubic catheter into the bladder . the tract was then dilated with dilators from the percutaneous lithotomy set , till it reached size 24 f. a size 22 f rigid cystoscope was then introduced into the bladder with the guide wire in place ( fig . the patient made an uneventful recovery and was discharged the following day with a date given for turp . a guide wire is inserted through the suprapubic catheter tract into the bladder followed by a 22 f rigid cystoscope . complications associated with the use of urinary catheters include infection , bladder spasms , catheter encrustations , and retained catheters ( 1 ) . the main cause of catheter encrustation is infection by urease - producing organisms , particularly proteus mirabilis ( 2 , 3 ) . these organisms colonise the catheter , forming a bio film ( 4 , 5 ) . the bacterial urease generates ammonia from the urea , and the urine becomes alkaline . under these conditions , crystals of calcium and magnesium phosphate are formed and a crystalline bio film develops , which eventually blocks the flow of urine from the bladder ( 68 ) . the main route of infection of the catheterised urinary tract is through the urethra along the outer surface of the catheter . infections , however , can occur from contaminants in the urine drainage bag ascending through the catheter lumen into the bladder ( 9 ) . patients with a retained encrusted foley catheter suffer from pain in the genitalia , bladder irritability causing spasms and urinary leakage , besides the great discomfort caused by urinary retention . the removal of an encrusted catheter is not only painful , but can cause trauma to the urethra . encrustation can lead to significant damage to the delicate urethral wall while the catheter is being removed resulting in bleeding , scarring , urethral strictures , and infection ( 10 ) . the management of a retained foley catheter with encrustation and stone formation around the bulb of the catheter is a complicated problem due to the resistance offered by the overlying calculus encrustation . in such circumstances since any technique used to remove the catheter must not disturb the patient or create any additional morbidity , minimally invasive techniques have been preferred over these procedures . ( 11 ) described using intraluminal pneumatic lithotripsy ( swiss lithoclast ) for the removal of encrusted urinary catheters . in each case a pneumatic lithotripsy probe was inserted into the lumen of the catheter and advanced in a jackhammer - like fashion . this technique resulted in disruption of the intraluminal encrustations and straightening of the tubes so that they were removed in an atraumatic manner . even though they recommended its use as a first - line treatment of removing encrusted urinary catheters , its efficacy is not known in cases of extraluminal encrusted urethral catheters . extracorporeal shock wave lithotripsy ( eswl ) has been utilised in the treatment of the bladder stones . borrowing this principle , kunzman et al . ( 12 ) has even used eswl to fragment the stone - like encrustation over the retained foley catheter balloon . after removal of the encrustation , the balloon can be deflated manually or , if it is stuck , a spinal needle can be used to puncture the balloon with ultrasound guidance . in this patient , we did not attempt eswl because the catheter could not be pushed into the bladder further . the balloon and the encrustation therefore , it was shielded by the pelvic bones and the shockwaves would not be able to break - up the encrustation . the lithoclast , on the other hand , could not be introduced via the urethra because it was difficult to negotiate the lithoclast through the urethral lumen with the urinary catheter in place , surrounding the haematoma and enlarged prostate . since we could not use the eswl or lithoclast , we resorted to first inserting a suprapubic catheter . after the suprapubic track had matured ( about 2 weeks ) , we introduced a cystoscope after dilatation of the track , done under spinal anaesthesia . a stone - crushing forceps was then used to fragment the encrustations . after removing the encrustations , the balloon was easily punctured with a spinal needle under ultrasound guidance . with this , the catheter could be easily removed . the options for removal of an encrusted and stuck urinary catheter include using the eswl or introducing the lithoclast via the urethra to fragment the encrustations . this would , however , need a catheter that is not stuck at the bladder neck . the authors have not received any funding or benefits from industry or elsewhere to conduct this study .

urinary bladder catheter encrustations are known complications of long - term urinary catheterisation , which is commonly seen in clinical practice . these encrustations can impede deflation of the balloon and therefore cause problems in the removal of the catheter . the options in managing an encrusted and incarcerated urinary bladder catheter include extracorporeal shock wave lithotripsy and lithoclast . we describe here another technique of dealing with a stuck and encrustated catheter , via direct crushing of the encrustations with a rigid cystoscope inserted through a suprapubic cystostomy tract .

a 19-year - old girl with rett syndrome was scheduled for elective phacoemulsification with intraocular lens insertion for immature cataracts . we did not know about her family history and other medical history because she was an orphan and she could not communicate with our medical team . but there was some description on the consultation paper from her orphanage . according to the paper , her physical disability had steadily progressed although her developmental milestones had been normal until the age of ten months , and she was known to have diffuse brain atrophy with mental retardation , including typical autistic disorder and convulsions . she also had untreated scoliosis and she had been bedridden for 5 years . on examination , her weight was 23 kg ( below the 5th percentile ) , her body temperature and blood pressure were normal and she was not distressed . the thyromental distance was 3.5 cm and the airway was normal except for trismus and excessive secretions . the preoperative hemoglobin level was 11.0 ( g / dl ) and the other lab tests were normal . on chest x - ray , the heart and lungs were unremarkable , although severe scoliosis of the t / l - spine and diffuse osteoporosis were observed . a head ct scan revealed diffuse brain atrophy , and the background was slow on the eeg . the patient was quiet on arrival to the operating room without premedication . in the operating room , her pulse oximetry was 99% on room air and her blood pressure was 110/75 mmhg . after loss of consciousness , mask ventilation was started with 2 vol% of sevoflurane . because we expected her trismus would be improved after injection of rocuronium , direct laryngoscopy was attempted . so , the fiberoptic - guided nasotracheal technique was initially chosen considering the extremely limited mouth opening , but this was unsuccessful because her nostrils were too small to intubate with a tube with an internal diameter of 5.0 mm . before an attempt was made with a 4.5 mm tube , we realized that the small space due to the malocclusion was large enough to handle a 480-sized oral airway ( diameter : 0.48 mm ) . fiberoptic - guided orotracheal intubation through this space was then successfully performed with suction ( fig . the trachea was intubated with a 5.0 mm oral tracheal tube and it was fixed at 18 cm . anesthesia was maintained with n2o 2 l / min , o2 2 l / min , sevoflurane 1.0 - 2.0 vol% , a tidal volume of 200 ml and a rr of 18/min . after the completion of surgery , which lasted 1 hour and there were no complications , the nitrous oxide and sevoflurane were discontinued , and the muscle relaxant was reversed with glycopyrrolate 0.2 mg and pyridostigmine 10 mg . we tried to wean her from the ventilator , but her recovery was slow and complicated by episodes of shaking . the patient was then transferred to the pacu after her respiration was found to be adequate . she remained drowsy in the pacu and it took a full day for her to regain full consciousness . rett syndrome is characterized by a devastating deterioration of the central nervous system in young females and it is accompanied by neurological problems that are associated with failure to thrive . there is a lack of sufficient data to make reliable estimates about the life expectancy beyond the age of 40 , although the average life expectancy of a girl with rett syndrome may be in the mid-40s . microcephaly due to growth retardation , diminished muscle tone , decreased eye contact and inattentive behavior develop during this period . immobility , respiratory dysrhythmia ( hyperventilation , periodic apnea ) , gastrointestinal dysfunctions ( peristalsis , esophageal atony , obstipation , etc . ) , spasticity , joint contraction and arrhythmias due to cardiac transduction defects also appear with disease progression . so , there are several points to consider for the anesthetic management of rett syndrome patients . the presence of scoliosis and muscular tonicity and the breathing abnormalities that range from centrally mediated hyperventilation to apnea make it necessary to evaluate the respiratory status using oxygen saturation to predict the outcomes before surgery . there is also a great risk of aspiration pneumonia during anesthetic induction or emergence due to esophageal atony . there are no reports about anesthetic experiences in patients with rett syndrome and who have limited mouth opening . but it can cause difficulties to correctly position the patient and if it involves the tm joint like our case , the anesthesiologist can be confronted with problems during orotracheal intubation or airway management . antegrade fiberoptic - guided orotracheal or nasotracheal intubation , retrograde guidewire - assisted fiberoptic intubation and blind nasal intubation can be considered . fortunately for us , antegrade fiberoptic - guided orotracheal intubation was possible to perform by an experienced anesthesiologist . yet we also prepared a tracheostomy set to secure the airway in the event of severe difficulty when securing the airway . we should check the breathing patterns , the preoperative arterial blood gases , the presence of gastrointestinal reflux and autonomic disturbances , the seizure history , the ecg and the musculoskeletal system . we should carefully interview the patient and the guardians for obtain proper information about the patient . however they thought that patients with rett syndrome may be unduly sensitive to sedative drugs and they exhibit slow recovery from anesthesia . so , the anesthesiologist should have great concern about the choice of anesthetics and maintaining the proper depth of anesthesia . in conclusion , we experienced great difficulty securing the airway in an adult female patient with rett syndrome during minor surgery , and this is the first report about the anesthetic management of a patient with rett syndrome . the patient with rett syndrome has many difficulties and points to be considered for anesthetic management , as described above . anesthesiologists should have proper knowledge about the disease and the patients to avoid the complications and problems that can be encountered during the perioperative and postoperative periods .

rett syndrome is a neurological disease that occurs only in females and it manifests with mental retardation , seizures , movement disorders , autistic behavior and abnormal breathing . a 19-year - old female with rett syndrome underwent ophthalmologic surgery under general anesthesia at our institution . airway control was difficult due to her limited mouth opening . we recommend that anesthesiologists should have proper knowledge about this disease and the patients to avoid the complications and problems that can be encountered during the perioperative period .

sodium valproate ( sv ) has a simple chemical structure , and it offers anticonvulsant and mood stabilizing properties . we present the first case , to our knowledge , of a pediatric bipolar patient who developed diurnal and nocturnal enuresis during treatment with sv . a 14-year - old girl , was referred to our child psychiatry clinic because she was experiencing a mania episode . both a magnetic resonance imaging scan and an electroencephalography revealed no hint of an organic cause of her manic symptoms . since her history also revealed a depressive episode , she was diagnosed as having bipolar disorder type 1 using the kiddie - sads - present and lifetime version.1 then , the young mania rating scale2 was used to measure the severity of her manic symptoms ; her score was 45 . we also administered risperidone 1 mg / day to resolve the ideas of reference symptom throughout the course of treatment . at the 15th day , the dose of sv was titrated to 750 mg / day ( 250 mg at noon and 500 mg at night ) . about 4 weeks later , when the dose of sv was titrated up to 500 mg twice a day ( a blood level of 75g / ml ) , z developed diurnal and nocturnal enuresis every day at the fifth week , she even had urinary incontinence in the classroom at school . z had not taken sv before , and her personal history revealed that she achieved urinary bladder control at 4 years old ; since then , she had had no urinary incontinence . the family history was negative for enuresis , and urological and neurological evaluations were normal . because of the enuresis , the sv dose was reduced to 500 mg / day ( a blood level of 38.3 g / ml ) , and her bedwetting disappeared completely 2 days later . at her follow - up visit half a year later , she appeared to be well adjusted to the treatment , which consisted of sv at 500 mg / night and risperidone 1 mg / day . previous studies have claimed that risperidone may induce enuresis.3,4 however , the girl had taken risperidone 1 mg / day throughout the course of her treatment in our case . furthermore , enuresis appeared with the increased dosage of sv and disappeared with the decreased dosage of sv . therefore sv , but not risperidone , was related to bedwetting in the present study . our literature search identified 14 case reports that described the induction of enuresis in children with epilepsy who were treated with sv , but there was an absence of literature examining nonepileptic patients.49 moreover , none of the patients presented with diurnal enuresis . in line with these case reports , z s symptoms disappeared after dose reduction , indicating a dose - related side effect.59 an explanation for the exacerbation of enuresis during sv treatment remains speculative . according to choonara,7 two of the most likely explanations include that enuresis is a consequence of increased depth of sleep,7,8 or that enuresis is secondary to a central effect that occurs on the thirst center , resulting in polydipsia.7 increased thirst has been demonstrated in several studies with sv.10,11 in addition , sv may induce changes in renal tubules.8 however , the hypothesis of increased depth of sleep as a cause does not seem to explain the daytime enuresis symptom experienced by z in the present study . in any case , clinicians may wish to be vigilant about this adverse effect when treating pediatric patients with sv , as the side effect can be detrimental to treatment adherence .

sodium valproate ( sv ) is effective and well tolerated for the treatment of epilepsy and mood disorders . there are several cases of sv - induced enuresis noted in epileptic patients . no alternative medication has been suggested to manage this problem . we present the first case of a pediatric bipolar patient who developed diurnal and nocturnal enuresis during sv therapy . clinicians should be aware of this side effect , as it has not been reported in nonepileptic patients , and it may be detrimental to treatment adherence .

oncogenes are mutated forms of cellular genes that are responsible for driving rampant cellular proliferation . however , the expression of an activated oncogene in normal human cells does not lead to their transformation , but to a permanent cell cycle arrest known as cellular senescence . hence , oncogene - induced senescence ( ois ) serves as a tumor suppressor mechanism that restricts the progression of pre - neoplastic lesions . the role of oncogene - induced reactive oxygen species ( ros ) production in cancer development has been a controversial subject . ros is a collective term describing a number of highly reactive molecules and free radicals that are formed upon incomplete reduction of oxygen . ros can either directly damage or modulate redox - sensitive signaling pathways , depending on the molecular concentrations at which they are present . in particular , the role of oncogenic ras - regulated ros production in cellular transformation has been the subject of several studies . nonetheless , it remains unclear whether oncogenic ros have a direct role in genome instability and , if so , which molecular mechanisms are involved . we approached this issue by taking a closer look at the mode of action of oncogene - induced ros in the model system of h - ras oncogenic activation in normal human fibroblasts . we observed that removal of ros by the broad specificity scavenger n - acetyl cysteine ( nac ) inhibits oncogene - induced hyperproliferation , prevents activation of the dna damage response ( ddr ) , and reduces the establishment of senescence . this experiment led us to propose the novel concept that oncogene - induced ros are modulators of cell hyperproliferation that engage the ddr by exposing cells to dna replication stress . we then focused on the origins of the oncogene - induced ros , which are ubiquitously generated through diverse enzymatic pathways . in particular , we turned our attention to the nox family of oxidases because initial studies suggested them as a source of ros in cancer . our experiments identified the ras - rac1-nox4 axis as the pathway responsible for ros generation by oncogenic ras in human cells . therefore , we propose that ras - induced ros are mitogenic signaling molecules for the initial hyperproliferative phase , which is causally associated with the altered dna replication that precedes and prompts ddr activation and ultimately leads to senescence . cell transformation is associated with bypass of ois and ongoing rampant proliferation . since oncogene activation hijacks the ros - modulated proliferative cues for the initial hyperproliferative phase , we investigated the effect of ros scavengers and nox4 inhibitors on proliferating cells . these cells either harbor mutations to bypass ois or are fully transformed , and either express oncogenic ras or do not . we observed that the expression of oncogenic ras in a cell is sufficient to confer sensitivity to ros manipulations . we also treated cells derived from human pancreatic cancer with gemcitabine , the standard of care chemotherapy treatment for pancreatic cancer , and nox4 inhibitors . although we observed a modest effect of nox4 inhibitors alone , we discovered an exciting synergic effect of nox4 inhibitors and gemcitabine that reduced the half - maximal inhibitory concentration ( ic50 ) of this nucleoside analog by up to 6-fold . deregulation of survival pathways by nox - derived ros through jak , stat , akt , and nfb pathways has been previously implicated ; however , the mechanism by which combined use of nox4 inhibitors and gemcitabine enhances apoptosis in cancer cells remains unclear . using a well - established mouse model of ras - induced pancreatic cancer , we reported increased induction of nox4 during progression from the earliest neoplastic lesions to more invasive ones . in addition , we observed robust nox4 expression in a set of human pancreatic tumors , with signal intensity correlating with neoplastic stage . importantly , and concordant with our results in cultured cells , nox4 levels in these human lesions correlated with markers of ddr activation . cancer cells produce elevated levels of ros and exhibit altered metabolic pathways and regulatory mechanisms in order to maintain redox balance . therefore , high levels of ros production are counterbalanced by an equally high antioxidant activity . studies of the same murine model showed that an antioxidant system mediated by nrf2 and nqo1 is activated upon oncogenic ras activation . however , on the basis of our experimental results it appears that this compensatory response to ras - induced ros production is not sufficient to fully buffer ras - mediated ros accumulation during the process of tumorigenesis . another example of altered redox homeostasis resulting from an impaired nox4-nrf2 balance has recently been reported in the context of lung fibrosis . a recent article proposed a novel axis involving activation of p38 through nox1 for ros generation and consequent malignant cellular transformation upon oncogenic k - ras activation in rodent fibroblasts . another study reported that oncogenic h - ras cooperates with nox1 in rodent fibroblasts or with nox4 in human fibroblasts for establishment of ois . therefore , it is worth exploring whether the oncogenes that transform both human and murine cells toward tumorigenicity exhibit different preferences for ros source between the two species . noxzymes are differentially expressed not only among distinct tissues , but also among several different types of cancer ( see fig . one member of the family is activated as the primary source , whereas the enzymes collaborate in other cancers , for example upregulation of both nox1 and nox4 in colorectal cancer . understanding which oncogenic stimulus activates each nox enzyme as an initial source for transformation and later proliferation , and how this is influenced by the in vivo microenvironment during tumorigenesis , will undoubtedly help the design of better therapeutic strategies based on ros manipulations . i am indebted to fabrizio dadda di fagagna for his support throughout the entire project , which is now complemented by a recent publication . i also thank fabrizio dadda di fagagna , michael bremang , and hans - peter wollscheid for critically reading this manuscript .

work carried out primarily in the laboratory of fabrizio dadda di fagagna unveils the mitogenic properties of ras - induced reactive oxygen species ( ros ) and their relationship with the dna damage response . combined data from studies of cultured cells , zebrafish models , and clinical material consistently support a role of the ras - rac1-nox4 axis in ros induction , hyperproliferation , and senescence .

carney syndrome ( cs ) is an autosomal dominant multiple neoplasia syndrome that includes cardiac , endocrine , cutaneous , and neural tumors . although the age at presentation may vary , cs is usually diagnosed in young patients , predominantly in female gender . cardiac myxomas seen in the course of cs are most often encountered in the left atrium . extra - cardiac manifestations include pigmented skin lesions , cutaneous myxomas , adrenal cortical disease , myxoid mammary fibroadenoma , and testes tumors in male patients . pituitary adenoma , melanotic schwannomas , and thyroid disease may be encountered in lower percentages . in this case report , we describe a middle - aged female patient who underwent surgery with the diagnosis of a large left atrial myxoma associated with cs . a 46-year - old female patient was admitted to our clinic with palpitation , cough , and exertional dyspnea . physical examination revealed pigmented lesions , especially scattered in the neck , chest , and extremities . the thyroid gland was mildly enlarged on palpation , but thyroid ultrasonography did not reveal any nodular or diffuse enlargement . she had been followed up with fibroadenomas located at the upper quadrant of the right breast . echocardiography revealed a left atrial mass , 6 4 cm in size , originating from the interatrial septum that was protruding into the left ventricle through the mitral valve during diastole ( figure 1 ) . peak pulmonary artery pressure was measured to be 65 mmhg , which was accompanied by mild to moderate tricuspid regurgitation . biochemical and hormonal parameters as well as thyroid hormone levels were within normal limits , except for mildly elevated hepatic enzymes ( serum glutamic oxaloacetic transaminase [ sgot ] = 55 iu / l , serum cortisol = 10 g / dl , insulin like growth factor [ igf]-1 = 202 ng / dl , growth hormone [ gh ] = 5 ng / dl , and oral glucose tolerance test with 100 gr glucose : fasting blood glucose level = 110 mg / dl ; 1 hour blood glucose level = 170 mg / dl ; 2 hour blood glucose level = 153 mg / dl and 3 hour blood glucose level = 143 mg / dl ) . left atrial myxoma ( white arrow ) seen on echocardiographic evaluation four chamber view genetic analysis of the patient ( especially the prkar1a gene ) could not be accomplished because of the lacking laboratory facilities in a newly established medical center . the aorta was cross - clamped and the heart was arrested with cold blood cardioplegia . left atriotomy was performed.a large encapsulated soft and lobulated left atrial myxoma , 6 4 cm in size , was attached to the interatrial septum with a broad base . the tumor was excised from the interatrial septum with its pedicle ( figure 2 ) . postoperative echocardiography revealed mild tricuspid regurgitation with a peak pulmonary artery pressure of 30 mmhg . the postoperative course was uneventful and the patient was discharged at the 5th postoperative day without any complication . we described a middle - aged patient with left atrial myxoma associated with concomitant clinical manifestations , which are components of cs : pigmented skin lesions and fibroadenomas of the breast . the overall lifespan of patients with cs is decreased , and cardiac causes of death are the most common among other etiologies . our patient s age was higher , which may reflect the late onset of the disease as well as late admission to a medical center . since more than half of the cases are familial , application of rigorous screening protocols are suggested for the first - degree relatives of the affected patients . our patient s first - degree relatives were screened for the cardiac and other manifestations of cs . the recurrence rate of the myxomas encountered in the course of cs is higher than that of the sporadic cases and this is an important issue since it may require multiple surgical interventions . postoperative short and long - term follow - up controls are crucial in these patients . cs is a complex genetic multiple endocrine neoplasia syndrome with various components relating to different organ systems . different clinical properties are well known and diagnostic criteria are defined concerning the large variety of these manifestations . cardiac myxomas are a corner stone of this disease since cardiac manifestations and related complications account for the majority of the mortality seen in the course of cs .

abstractcarney syndrome ( cs ) is an autosomal dominant multiple neoplasia syndrome that includes cardiac , endocrine , cutaneous , and neural tumors . cardiac myxomas can be seen in the course of cs . a 46-year - old female patient was admitted to our clinic with palpitation , cough , and exertional dyspnea . physical examination revealed pigmented lesions , especially scattered in the neck , chest , and extremities . echocardiography revealed a left atrial mass , 6 4 cm in size , originating from the interatrial septum that was protruding into the left ventricle through the mitral valve during diastole . the tumor was excised from the interatrial septum with its pedicle . the patient was discharged at the fifth postoperative day without any complication . cardiac myxomas are a cornerstone of this disease since cardiac manifestations and related complications account for the majority of mortality seen in the course of cs .

the article from wu and colleagues investigates the question of using immunostimulation as a new approach in treating sepsis . the incidence of sepsis , and particularly severe sepsis , is expected to markedly increase in the next decade due to the aging population and more widespread use of therapies that compromise host immunity in cancer and autoimmune diseases . advances in sepsis therapy have occurred and the surviving sepsis campaign recommendations have led to a reduction in sepsis mortality from roughly 50% to 30 to 35% . the remaining high rate of death and the failure of the most recent high - profile clinical trials in sepsis still argue for an innovative adjuvant approach to this highly lethal disease . beyond the design of these trials , incertitude on some key pathophysiologic mechanisms should be clarified to allow change in the paradigms of sepsis syndrome and patient care . among the newly accepted concepts , the occurrence of immunodepression soon after the initial phase of sepsis has gained credibility . to be clinically relevant , such a concept has : to be confirmed in a large - size population ; to be diagnosed and quantified by standardized methods ; to be observed not only on circulating immune cells , but also within organs that fail in sepsis ; to be based on molecular mechanisms ; and to be reversible , even partially , by clinical - based therapy able to improve outcome . almost all of these criteria have been validated in clinical conditions , except the proven benefit for outcome . the article from wu and colleagues addressed this question using thymosin alpha-1 ( t1 ) , a molecule with known immunostimulating properties . the concept of sepsis - induced immunodepression has been extensively described ex vivo , especially in septic monocytes , with impaired responses to additional immune challenges compared with healthy cells . importantly , such immunodepression was recently demonstrated to occur in immune cells infiltrating into organs in patients dying from severe sepsis . remarkable to note is that most immune effector cells are involved , resulting in defects in both innate and adaptive immunity . moreover , the magnitude and duration of immunosuppression are now well documented to be associated with worse outcome and increased risk for nosocomial infections . the underlying mechanisms are complex : the predominance of anti - inflammatory cytokines ; the alteration of t - lymphocyte populations in number and function ; the fractional increase in t - regulatory inhibitory lymphocytes ; metabolic failure of immune cells ; and epigenetic modifications induced by the cell microenvironment . interestingly , this immunodepression has been shown to be reversible using different immunostimulatory therapies including ifn , granulocyte - macrophage colony - stimulating factor , and , recently , il-7 . such treatments may boost host immunity , thereby leading to more effective eradication of the primary infection , prevention of secondary infection , decreasing latent virus reactivation and , hopefully , improving outcome . the present randomized control trial of t1 is in line with this new direction . despite a lack of understanding of some of its mechanism(s ) of action , immunomodulatory activity of t1 on effector cells of the innate immunity has been well described . t1 can induce t - cell and dendritic cell maturation as well as increasing il-12 expression . this randomized controlled trial tested the early administration of t1 on day-7 and day-28 mortality and on severity of organ failure and mhla - dr expression . the main result was a reduction in 28-day mortality in the t1 group ( 26% ) versus the control group ( 36% ) ( p < 0.06 ) with an associated increase in mhla - dr and no change in severity of organ failure . first , the trial is designed to reduce crude mortality , which includes both sepsis - attributable mortality and mortality related to underlying disease . an adjunctive immune therapy would only impact septic - induced organ failure and death and would require a larger study population . second , the drug or the placebo was given to all patients having the entry criteria that were not based on immune competence . the results might have been different if enrollment of the patients had been based on immune monitoring and restricted to those patients with documented immunosuppression . despite its significant limitations and undefined mechanism of action , this randomized control trial is one of the first such trials using a known immunostimulating agent to reduce 28-day mortality . despite the largely un known mechanism of action of the drug and nonselection based on assessed immunodepression , the observed marginal positive p value in favor of t1 confirms the interest to perform other carefully conducted immunotherapeutic trials based upon markers of immune suppression .

a recent randomized controlled clinical trial of the immunostimulatory agent thymosin alpha-1 was conducted and showed a trend toward improved survival in patients receiving the drug ( p = 0.06 ) . although this was a relatively small study and the exact mechanism of action of thymosin alpha-1 is not known , the present results further support the evolving concept that , as sepsis persists , a hypoinflammatory and immunosuppressive condition ensues and therapy that augments host immunity may be advantageous . other immunomodulatory agents including granulocyte - macrophage colony - stimulating factor have shown promise in small trials in sepsis . in addition , there are a number of new immunoadjuvant agents such as il-7 and anti - programmed cell death-1 that are showing remarkable abilities to enhance host immunity and improve outcomes in a variety of clinical disorders , including cancer and chronic viral infections . animal studies show that these new immunoadjuvant agents improve survival in several clinically relevant models of sepsis . given the relative safety of thymosin alpha-1 and these other new immunomodulatory agents as well as the persisting high mortality of sepsis , a strong case can be made for larger well - designed trials using immunoadjuvant therapy in patients who have documented immune suppression . immunotherapy offers new hope in the treatment of sepsis and may dramatically change the face of the disease .

a 56-year - old male presented with chronic on and off pain in the left eye his systemic examination revealed multiple subcutaneous tumors in distal upper limbs and more than 6 caf - au - lait macules ; but all were < 15 mm in diameter . his vision was 20/30 in right eye and counting fingers at 3 feet in left eye . applanation tonometry readings were 13 mm hg and 50 mm hg in right and left eye , respectively . dark - room gonioscopy showed appositional closure of the drainage angle with multiple peripheral iris convexities in the right eye and 360 synechial angle closure in the left eye . ubm presented a ring - shaped solitary iridociliary cyst spanning 360 in the right and 270 , sparing nasal quadrant , in the left eye . the cysts had hyper - reflectile walls and sonolucent interiors , suggestive of primary neuroepithelial cysts [ fig ultrasound biomicroscopy showing iridociliary cyst in right ( a ) and left ( b ) eye we did nd : yag laser iridotomy , peripheral to the collarette of the right iris at 8 oclock hour where the diameter of the cyst was narrowest on ubm to avoid cyst rupture [ fig . 2 ] . post iridotomy , the angle recess was narrow , but the scleral spur was visible all around on dark - room gonioscopy in the right eye . the right optic disc was healthy , and the left had advanced glaucomatous cupping [ fig . 3 ] . magnetic resonance imaging of brain and orbits was unremarkable , except for a diffuse t2 hyperintense signal in the left optic nerve . note its atypical location at 8 oclock hour optic disc photographs of right ( a ) and left ( b ) eye ultrasound biomicroscopy of right ( a ) and left ( b ) eye . note narrow but open angle recess in a and absence of iridociliary cyst in b the left eye was painful despite maximum antiglaucoma medications . this eye underwent g - probe assisted inferior 180 contact transscleral diode cyclophotocoagulation ( 810 nm laser ; iridex corporation , ca , usa ) . post procedure , severe inflammation of the anterior chamber and neovascularization of the iris occurred , which resolved gradually with steroid and cycloplegic eye drops . the iop in the left eye remained between 40 and 48 mm hg though the patient was pain - free . on repeat ubm , the inferior ciliary processes were shrunken , but the cyst was no longer imaged [ fig . primary iridociliary cysts , in the majority , are stationary lesions located focally and rarely cause visual complications . angle closure , secondary to iris and ciliary body cysts , is related to the forward bulk of the iris cyst and occlusion of the posterior chamber and is scarcely reported.[13 ] a postulated mobility of the ring - shaped iridociliary cyst during movements of the pupil might make an additional contribution to the angle closure . absolute glaucoma , as in the left eye of our patient , is not attributed to iris or ciliary body pigment epithelial cyst(s ) in the past . except for a single report of 7 members of a family with familial aortic dissection and having pupillary margin iris pigment epithelial cysts , no relation of primary cysts of iris or ciliary body epithelium to any systemic disease has been reported . the clinical features of our patient were insufficient to meet the diagnostic criteria of neurofibromatosis ( nf)- 1 . nevertheless , iris pigment epithelium cyst is formed by separation of the 2 layers of epithelium and can not be explained by the pathogenesis of nf . laser iridocystotomy when the cyst could not be visualized in mydriasis by gonioscopy is also reported . this treatment results in pigment dispersion , and carries the potential risk of inflammation and cyst recurrence . however , laser iridotomy did suffice to relieve oppositional angle closure in the right eye of our patient [ fig . the treatment resulted in inadvertent iridociliary cyst rupture with no troublesome sequel over the follow - up . thus , cyst rupture is a potential complication of diode cyclophotocoagulation in eyes with large iridociliary cyst(s ) .

primary iridociliary cysts can induce plateau iris configuration and angle closure glaucoma . we report a patient with bilateral , primary , ring - shaped , solitary iridociliary cysts . the right eye displayed normal intraocular pressure , oppositional iridocorneal angle closure , and healthy optic nerve head . the left eye had advanced chronic angle closure glaucoma . the management strategy varied between eyes and is discussed . this , to the best of our knowledge , is the first report of transscleral diode laser application in an eye with a large iridociliary cyst .

according to the eau guidelines nephron - sparing surgery ( nss ) has a similar oncological outcome to that of radical surgery . however , nss is not suitable in some patients with localized renal cell carcinoma ( rcc ) because : 1 ) of locally advanced tumor growth , 2 ) partial resection is not technically feasible because the tumor is in an unfavorable location , 3 ) and/or significant deterioration of the patient 's general health . in these situations the gold standard curative therapy remains radical nephrectomy , which includes radical removal of the tumor - bearing kidney . acute pancreatitis has not yet been described in the published literature as a complication of radical nephrectomy . a 56-year - old patient was referred to the department of urology at the medical university of graz for right renal tumor ( ct scan verified a 12 cm renal mass ) to perform a radical nephrectomy . the preoperative anesthetic screening of our patient showed asa ii according to the american association of anesthesiology and well - controlled hypertension with normal laboratory blood tests . there were no co - existing infections the histological examination of the right kidney revealed a renal cell carcinoma ( rcc ) . the operation was uneventful . however , one day later the patient complained of gradually increasing , serious abdominal and back pain , including jaundice and fever . on physical examination , the patient had neither a history of biliary lithiasis nor chronic alcohol consumption or infections . laboratory data ( blood ) demonstrated urea 61 mg / dl ( n : 10 - 45 ) , uric acid 8.5 mg / dl ( n : 3.4 - 7 ) , potassium 4.3 mmol/ ( n : 3.5 - 5 ) , calcium 1.99 mmol / l ( n : 2.202.65 ) , aspartate aminotransferase 216 iu / l ( n : 0 - 35 ) , alanine aminotransferase 134 iu / l ( n:0 - 45 ) , lactate dehydrogenase 404 iu / l ( n:120 - 240 ) and amylase 989 iu / l ( n : 13 - 53 ) , lipase 2154 ( 0 - 60 ) , and procalcitonin 3.45 ( n:0 - 0.5 ) . white blood cell count was also raised ( 13000 ) , as well as crp 268 mg / l ( n:0 - 8 ) , bilirubin 1.43 ( n:0.1 - 1.2 mg / dl ) , and creatinine 2.78 mg / dl ( 0.6 - 1.3 ) . an abdominal ct scan was performed and demonstrated parenchymal edema of the pancreas ( figs . 1 and 2 ) . the patient was referred to the intensive care unit and treated conservatively for 15 days his medical condition improved and he was referred to the department of gastroenterology , where mr cholangiopancreatography showed no biliary stones , and a normal liver . the international symposium on acute pancreatitis in 1992 defined pancreatic necrosis as the presence of one or more diffuse or focal areas of non - viable pancreatic parenchyma . so , based on the patient 's clinical presentation and ct scan findings , the diagnosis of acute pancreatitis as a complication of radical nephrectomy was considered . the patient was referred to the intensive care unit ( icu ) and oral intake was prohibited . a nasogastric tube was inserted for decompression and parenteral nutrition was started from a central vein . ten days later , serum pancreatic enzyme levels and white blood cell count returned to normal ranges bowel movements became normal , and the patient passed stool . on the same day , the nasogastric tube was removed and restricted oral intake was permitted . on day 16 transperitoneal radical open nephrectomy is a generally accepted treatment for a renal mass greater than 10 cm in size . in the published literature according to burkey retroperitoneal dissection and ischemia could be risk factors for postoperative pancreatitis . however , this was not the case in our patient and trauma to the head of pancreas was avoided . according to milian et al the mortality rate of pancreatitis was 8.3% for conservative medical treatment versus 26.5% for surgical treatment . physicians should consider pancreatitis as a potentially life - threatening adverse event associated with propofol [ 6 , 7 ] , which may be due to failure of free fatty acid metabolism secondary to inhibition of free fatty acid entry into the mitochondria . we hypothesize that propofol could have played a role in the pathophysiology in this condition , because no biliary stone was demonstrated on preoperative or postoperative radiologic studies in the presented case . additionally , no other predisposing factors ( e.g. alcohol , infection , addiction or hypercalcemia as well as history of chronic pancreatitis ) for pancreatitis were present in our patient .

radical open nephrectomy is considered the standard treatment for kidney tumors or masses greater than 10 cm . we present a rare case of acute pancreatitis that occurred after right radical transperitoneal nephrectomy , which was treated by nonsurgical conservative interventions.the incidence of acute pancreatitis after renal surgery is not known in the literature.a 56-year - old man developed acute pancreatitis postoperatively after radical transperitoneal nephrectomy . an initial ct scan showed an enlarged pancreas with hypodense , heterogeneous consistency and with peripancreatic , perihepatic , mesenteric , and pelvic fluid collections . this complication was managed conservatively .

since the introduction of serial transverse enteroplasty ( step ) in 2003 ( 1 ) , this procedure has become popular in the surgical management of short bowel syndrome because of its easy and simple operative technique ( 2 , 3 ) . however , in cases of extreme short bowel , the lengthening of the bowel from one step is not enough to escape from short bowel syndrome . recent studies have reported that step could be performed again as a second procedure after primary step ( 4 - 7 ) . herein , we present a case of extreme short bowel syndrome that was successfully treated by the second step procedure . a 2.63-kg , 36-week gestation female neonate was born by elective cesarean delivery . at the third day of life the proximal bowel was only 10 cm in length from the treitz ligament with dilatation , and the distal bowel was 5 cm in length from the ileocecal valve . step was performed using 6 endo gia staplers on the proximal small bowel that resulted in an increase in intestinal length from 10 cm to 15 cm . the entire small bowel length was 20 cm after primary anastomosis with the distal small bowel ( fig . postoperatively , parenteral nutrition was discontinued because of cholestatic jaundice , but she was able to gain weight very slowly by enteral feeding , even though she needed intravenous electrolyte solution to prevent dehydration . by 6 months of age , the patient 's body weight had increased to 3.71 kg , but she still required intravenous electrolyte solution and her small bowel was dilated again . as a result , we decided to perform the second step . the patient had relatively even dilatation of the small bowel with 4 - 5 cm in width . the previous proximal small bowel had gained length , from 15 cm to 30 cm , and the distal small bowel had grown from 5 cm to 12 cm . the second step was performed using 5 endo gia staplers on the previous proximal small bowel that resulted in an increase in intestinal length from 30 cm to 45 cm . , the patient was tolerable to enteral feeding with body weight gain . at 8 months of age ( 2 months after the second step ) , her body weight was 4.41 kg after weaning the intravenous fluid , and she was discharged from the hospital . at the age of 2 yr , she has shown good growth and her body weight has increased to 9.8 kg , although her body weight is still in the low percentile of the growth chart ( 4th percentile ) and she has developed vitamin d deficiency . in the management of short bowel syndrome , the length of the small intestine is one of the important treatment factors ( 8) . although few reports have shown successful results in the short segment of the intestine , favorable outcomes are usually seen in cases of relatively long segments of intestine ( 9 ) . for this reason , various types of intestinal lengthening procedures have been developed ( 10 - 12 ) . step , the most recently developed method , is favored for its simple technical feasibility ( 3 ) . originally this procedure was designed for longstanding short bowel syndrome patients with a dilated small bowel , and it could easily be adapted to newborn patients who have proximal jejunal atresia with short dilated residual small bowel ( 6 , 13 ) , such as our case . however , in cases of extreme short bowel , bowel lengthening by step has not been enough to escape from short bowel syndrome , and subsequent tpn and its complication would still bes a major risk factor of survival . in such cases , after animal studies of second step ( 7 ) , several reports revealed and supported its advantages ( 4 - 6 ) . this procedure offers not only the original advantages of technical ease and simplicity , but it also apparently has the benefit of additional intestinal length . the intestinal elongation produced by second step could be a critical length of intestine in treating short bowel syndrome . our case also supports the utility of second step . in our study , the patient was able to survive without tpn after the first step , but her bowel length was not enough to escape from short bowel syndrome . although natural growth of the small bowel would be helpful in managing short bowel syndrome , elongation of the small bowel length by the second step would be critical lengthening to rescue the patient from short bowel syndrome . while our second step was performed without major difficulties , several points of caution must be noted . deformed mesenteric blood supply and uneven dilatation of the bowel , which had happened after the first step in our case , are problematic to staple firing . the surgeon has to ensure that the blood supply between each staple line is not injured by the staples . second , there are few options for placing the staple lines to make a uniform diameter of bowel and reduce the blind ends ( 4 - 7 ) . we adopted a simple addition of staple line between previous staple lines without further modification . because of limitations of previous studies , it is difficult to recommend the best method of making staple lines , but it should be tailored to individual cases . in addition to these concerns , unquestionably , abdominal exploration should be performed very carefully to avoid unexpected injury to the bowel . our case was successful in weaning the patient off the tpn and freeing her from intravenous fluid by the second step . however , the patient is still in a very low percentile in body weight and height . she also has vitamin d deficiency , which probably originated from the short length of her bowel . this indicates that nutritional evaluation and support should be an essential part of management , even when the second step is successful .

the serial transverse enteroplasty ( step ) procedure is a novel technique to lengthen and taper the bowel in patients with short bowel syndrome . the advantages of step include not only technical ease and simplicity , but also the ability to repeat the procedure . herein , we report a case of extreme short bowel syndrome that was successfully treated by the second step procedure . a 3-day old newborn girl underwent step because of jejunal atresia with the small bowel length of 15 cm , but her bowel elongation was not enough to escape from short bowel syndrome . at the age of 6 months , she underwent a second step procedure . the bowel lengthening by the second step made her tolerable to enteral feeding with body weight gain and rescued her from short bowel syndrome . this case showed that second step is very helpful in treatment of extreme short bowel syndrome .

a 26-year - old male from the shush ( a county in khuzestan province in iran ) , referred to alzahra hospital , isfahan , iran , with fever and two deep purulent lesions for past one month . the lesions ( about 8 4 cm in diameter ) were surrounded by erythematous and edematous borders and central necrosis . lesions were evident on the distal part of the left leg , fixed - size at the beginning and accompanied by foul - smelling yellow discharge . 1 ) . considering research ethics , the past history and the present clinical data were recorded in a data sheet . on his recent hospitalization , he was diagnosed as suffering from myelodysplastic syndrome ( mds ) by a bone marrow aspirate ( bma ) and bone marrow biopsy ( bmb ) , following a graduate pancytopenia . the patient was treated with cefixime , itraconazol and ciprofloxacin , without any improvement in clinical conditions . deep purulent lesions on distal part of left leg in addition , he was on treatment with phenytoin compound due to the epilepsy from childhood . blood culture , antinuclear antibody ( ana ) , antineutrophil cytoplasmic antibody ( anca ) , coombs test , chest x - ray ( cxr ) and echocardiography ( ecg ) did not show any abnormality . doppler ultrasound of lower limbs was normal and no sign of deep venous thrombosis ( dvt ) was seen . massive soft tissue swelling was seen by the lower limb sonography , and soft tissue computed tomography scan impressed cellulites and myositis . on hematological consultation , bma and bmb were recommended for the patient but no abnormal finding was detected . he received granulocyte colony - stimulating factor ( g - csf ) but no improvement in blood count was seen . treatment was started with meropenem ( 1 g q8h ) , vancomycin ( 1 g q12h ) , cefepime ( 2 g q8h ) , metronidazol ( 500 mg q6h ) , cotrimoxazole ( 2 tablets q8h ) , rifampin ( 300 mg q12h ) and amphotericin b ( 50 mg / day ) . amphotericin b was discontinued due to the severe side effects such as fever , chills , headache , nausea , vomiting and phlebitis at the site of the infusion . laboratory evaluations for the patient because of the gross appearance of the lesions which was similar to cl , skin biopsy was taken from lesions which was negative for leishman - donovan bodies in three times recheck . in addition , indirect fluorescent antibody ( ifa ) screening for leishmaniasis was negative . for definite diagnosis , diagnostic splenectomy was performed , which pathological exploration showed a bulk of leishmania protozoa in patient 's spleen . this is a case report of vl with cutaneous lesions in iran which was negative for leishman - donovan bodies and immunological test . leishmania can affect the skin , viscera , or mucocutaneous areas , varying according to the geographic region and the host response.5 leishmaniasis directly affects hundreds of thousands people per year , with millions of individuals at risk worldwide.7 leishmaniasis can manifest with various coetaneous manifestations , based on the genetic differences within the host or the parasites.8 the diagnosis of cl is made on the basis of a typical lesion , a history of exposure and demonstration of the parasite on the lesion.9 this infective skin disease manifests as localized , often self - healing single lesions , intermediate forms which frequently produce mucosal lesions.10 many of infected patients often experience a delay in diagnosis and inappropriate treatment.11 our case was an interesting one with some cutaneous lesions , grossly similar to cl , without any leishman body inside . in addition , it seems strange that an enlarged spleen full of leishmania protozoa but without positive ifa ( indicator of vl ) was found . in this new case , we can not rule in / out cl , due to dermatological presentation and absence of leishman - donovan bodies . reported a case of human immunodeficiency virus / visceral leishmaniasis ( hiv / vl ) co - infection associated with cutaneous lesions in a patient from jahrom , in iran . they demonstrated that it is better to evaluate the diagnosis of visceral leishmaniasis in patients who present with cutaneous leishmaniasis and hiv infection.2 in our case , regardless of the spleen filled with leishmania protozoa , vl could be ruled out due to the negative ifa . one interpretation for this case may be the accompanying mds . since serological abnormalities like hyper- or hypogammaglobulinemia is reported to be about 18 - 65% in the cases afflicted by mds,12 it may mask the proper immunological response to the leishmania parasites . level of anti - leishmania antibody in immunocompromised patients is 50 times lower than those with normal immune system.13 the inconsistent finding was the absence of leishman - donovan bodies in the wound smears , that is not interpretable with mds . the more reasonable suggestion that we can make is that the patient may be afflicted with vl after the cl . but the spared bone marrow in a patient with a spleen full of leishman - donovan bodies is interesting , if the diagnosis is vl . leishmaniasis is an endemic disease of iran that unfortunately , there is no definite treatment for this disease.14 the epidemiological trends of concomitant cl and vl cases have changed today , which needs further attempt for better diagnostic / therapeutic approaches . our case demonstrated that negative smears for leishman - donovan bodies in cutaneous lesions similar to cl , and negative ifa in suspected vl should not draw us away from the diagnosis of leishmaniasis in the suspected cases .

we report a case of visceral leishmaniasis ( vl ) in a patient from shush in iran with cutaneous lesions , negative for leishman - donovan bodies , enlarged spleen filled by leishmania protozoa and negative immunological test . the patient was a 26-year - old male , who attended hospital with fever and two deep purulent lesions on the distal part of his left leg . on physical examination , the patient had splenomegaly . laboratory results were as follows : pancytopenia , positive c - reactive protein ( crp ) , elevated erythrocyte sedimentation rate ( esr ) and lactate dehydrogenase ( ldh ) levels .the necessary treatment was administrated to the patient . biopsy of lesion for leishman - donovan body was negative . in addition , indirect fluorescent antibody ( ifa ) screening for leishmaniasis was negative . diagnostic splenectomy was performed which pathological exploration showed a bulk of leishmania protozoa in patient 's spleen . twenty days later , this patient expired .

a 69-year - old lady presented with diminution of vision in the left eye since a month . on examination , best corrected visual acuity ( bcva ) in the right eye was 20/30 and in the left eye was 20/60 . fundus examination of both eyes revealed non - central geographic atrophy and soft drusens at the macula . temporal periphery of the left eye revealed subretinal exudation with altered subretinal hemorrhage and subretinal pigment epithelium ( rpe ) hemorrhage [ fig . 1 ] . ffa revealed window defects in the macula corresponding to rpe atrophy , in both eyes . blocked fluorescence was noted at the temporal periphery in left eye due to subretinal and sub - rpe hemorrhage . a provisional diagnosis of pehcr was arrived at . however , icga revealed the presence of multiple hyperfluorescent polypoidal lesions in the temporal periphery at the choroidal level , suggestive of pcv [ fig . 1 ] . after obtaining an informed consent and explaining about its off - label use , the patient underwent intravitreal bevacizumab ( avastin , genentech labs , san diego , ca , usa ) ( 1.25 mg in 0.1 ml ) injection in her left eye . fundus examination revealed a significant reduction of subretinal hemorrhage and exudates in the left eye . icga was repeated and showed persistent leakage from peripheral choroidal polyps in the left eye . when reviewed 2 months later , her clinical condition showed significant improvement while icga showed persistent leakage from the peripheral polyps in the left eye [ fig . 2 ] . icga - guided laser photocoagulation was done to the peripheral polyps with a slit - lamp laser delivery system . two months later , her clinical condition further improved and icga showed no evidence of any active polyp in the left eye [ fig . 3 ] . she was then followed up quarterly ; fundus examination was stable and bcva maintained . at the last review , 27 months after laser treatment , her bcva was 20/30 in both eyes [ fig . 4 ] . at presentation . ( a ) color fundus photograph of the left eye reveals subretinal altered hemorrhages and mounds of sub - rpe hemorrhage ( arrowheads ) in the temporal periphery . ( b ) indocyanine green angiography reveals multiple , peripheral , discrete , hyperfluorescent , polypoidal lesions ( arrows ) at the choroidal level , suggestive of idiopathic polypoidal choroidal vasculopathy six weeks after second bevacizumab injection . color fundus montage ( a ) shows consolidation of hard exudates and reduction in the sub - rpe mounds of hemorrhages in the temporal periphery ( arrow ) . indocyanine green angiography ( b ) reveals persistent polyps ( arrows ) two months after laser photocoagulation . ( a ) color fundus photograph of the left eye reveals significantly reduced subretinal altered hemorrhages ( arrowheads ) . ( b ) indocyanine green angiography shows no leak from the area of treated polyps at 27 months follow - up , color fundus photograph of the left eye reveals water - mark area of rpe atrophy ( right pointing arrows ) and a subretinal scar ( left pointing arrows ) choroidal vascular lesions of pcv are preferentially found at the posterior pole of the retina , although the lesions could also be found in the peripheral retina in up to 63% eyes . idiopathic pcv is a peculiar vascular abnormality of the inner choroid , composed of network of branching vessels terminating in aneurysm - like enlargements with episodic serosanguineous detachments of the retinal pigment epithelium and neurosensory retina . when pcv lesions accompany ped , the lesions are usually located at the margins of ped . the network of vessels usually emanates from the peripapillary area or less commonly as an isolated macular lesion . pehcr is a characteristic peripheral degenerative disorder where chorioretinal lesions lead to small or large lesions of poorly defined accumulations of subretinal or sub - rpe hemorrhage , exudation , or both . lesions are seen in temporal quadrant twice as commonly as in nasal quadrant . frequently , it has a benign outcome , although it can be vision threatening because of hemorrhage or exudation . anatomical differences may be responsible for the contrasting appearance of lesions in the macula and in the retinal periphery . pehcr can be associated with disciform macular degeneration , which is a common macular disorder seen in elderly ; rarely it can occur in the periphery . there is a paucity of such reports in the literature , although its exudative and hemorrhagic presentations account for 8% and 5% , respectively , of pseudo - melanomas of the posterior uvea . bevacizumab has anti - angiogenic and anti - permeability effects that help in resolution of subretinal exudation . laser photocoagulation of the choroidal polyps leads to thrombosis and occlusion , with cessation of leakage . rationale of sequential anti - vascular endothelial growth factor ( anti - vegf ) treatment followed by more definitive laser photocoagulation is that the anti - vegf agent aids in resolution of subretinal fluid , thus making the polyp more amenable to focal laser photocoagulation . moreover , laser photocoagulation helps in vaso - occlusion of the polyps and regression of vascular network associated with pcv without the inherent limitations of intravitreal anti - vegf agents . however , cryopexy carries the disadvantage of inducing marked inflammation . to conclude , sequential intravitreal bevacizumab and icga - guided laser treatment of peripheral polyps seems to stabilize and improve the treatment outcomes in eyes with peripheral pcv as it stabilizes the choroidal vasculature and prevents further leakage .

a 69-year - old lady presented with complaints of decreased vision in left eye since one month . best corrected visual acuity ( bcva ) was 6/18 in that eye . fundus examination revealed non - central geographic atrophy and soft drusens at macula in both eyes . temporal periphery of left eye revealed subretinal exudates with altered sub - rpe hemorrhage mimicking peripheral exudative hemorrhagic chorioretinopathy ( pehcr ) . fundus fluorescein angiogram showed window defects at macula and blocked fluorescence at temporal periphery in left eye . however , indocyanine green angiography ( icga ) revealed active peripheral choroidal polyps . the patient was successfully treated with intravitreal bevacizumab and icga - guided laser photocoagulation . 27 months after laser treatment , bcva improved to 6/9 . rationale of consecutive anti - vascular endothelial growth factor ( vegf ) treatment followed by more definitive laser photocoagulation is that anti - vegf aids in resolution of subretinal fluid , thus making the polyp more amenable to focal laser photocoagulation which stabilizes the choroidal vasculature and prevents further leakage .

lactic acidosis ( la ) is known to occur in patients of leukemia and lymphoma as a terminal event . it was first described in patients with acute leukemia by field et al . in 1963 . diagnostic criteria of la are ph less than 7.35 along with plasma lactate concentration greater than 5 mmol / l . however , la as the initial presentation of non - hodgkins lymphoma ( nhl ) is extremely rare . a 35-year - old male presented with intermittent fever , loss of appetite and fatigue of 5 months duration . he was afebrile with a pulse of 140/min , and a respiratory rate of 36/minute . pallor , pedal edema along with right cervical lymphadenopathy was present . on systemic examination a firm mass of 10 cm hemoglobin was 5.1 gm / dl , wbc 9200/cu mm , platelet count 2,72,000/cu mm ; renal and liver functions were normal with serum ( sr ) urea 37 mg / dl , sr creatnine 0.7 mg / dl , sr bilirubin 0.5 mg / dl , sr sgot 52 units / l , sr sgpt his sr ldh was raised with a value of 876 iu / l and sr albumin was markedly low 1.8 gm / dl . blood gas analysis showed ph 7.17 , pco2 23 mmhg , hco3 8.4mmol / l , serum lactate 12.7 mmol / l , suggestive of metabolic acidosis [ table 1 ] . computerized tomography ( ct ) scan showed dilated small bowel loops with mural thickening along with the presence of homogenous mesenteric and retroperitoneal masses measuring about 7 cm along with mediastinal lymph nodes . a biopsy of the abdominal lymph node revealed nhl ( subtype dlbcl ) , immunohistochemistry of tumor was positive for cd20 and negative for cd3 and cd10 . bone marrow was not involved by abnormal lymphoid cells . as blood sugar , urea and creatinine were normal , blood cultures were sterile , no history of intake of metformin or other medication causing la and other common causes of metabolic acidosis were ruled out . the patient was started on soda bicarbonate , broad spectrum antibiotics , although no focus of infection was detected . simultaneously , he was given cyclophospamide 500 mg on day 1 and day 2 , dexamethasone 8 mg three times daily day 1 to day 4 and doxorubicin 30 mg on day 3 . patient 's vitals improved gradually and after 1 week , his lactate level came down along with improvement in ph and bicarbonate levels . he subsequently received six courses of r - chop ( rituximab , cyclophospamide , doxorubicin , vincristine , prednisolone)-based chemotherapy . he had a partial response that was short lasting and developed progression of lymphoma within 2 months of sixth course of r - chop . showing following parameters [ arterial blood gas , blood sugar , vitals ] before and after adding chemotherapy lactic acid is a degradation product of glucose in anaerobic conditions . in anaerobic conditions , there are two types of la , hypoxic ( type a ) and non - hypoxic ( type b ) . malignancy is often associated with type b. causes of la in malignancy are either due to overproduction of la or under utilization of la or both . tumor cell have a different metabolic milieu as compared to non - malignant cells . rapidly dividing tumors this leads to anaerobic glycolysis with activation of ldh and production of lactate . however , hypoxia is not the only mechanism of high lactate level in malignancy . otto warburg in 1930 demonstrated that cancer cells rely on glycolysis even in the presence of oxygen , a phenomenon known as aerobic glycolysis warburg effect . apart from hypoxic environment , elevated lactate level may also occur due to liver metastasis , over expression of type ii hexokinase , regulatory effect of insulin - like growth factor . in malignant cells even under aerobic conditions there is increased activation of mitochondrial - bound type 2 hexokinase , a rate - limiting enzyme of glycolysis . this occurs due to aberrant production of insulin - like growth factors along with its binding proteins and tumor necrosis factor ( tnf- ) by tumor tissue . in our patient , the poor prognostic impact of la in malignancy can be judged from fact that out of the total 29 published cases of lymphoma with la , 25 eventually died ( pubmed search database ) . hence , la in lymphoma portends a very poor prognosis . the molecular basis for this may be due to a functional impairment of cytotoxic t cells . activated t lymphocytes are also dependent on glycolysis during the period of proliferation and cytokine production and thus produce lactate . this transport requires proton - linked monocarboxylate transporters , which co - transport protons and lactate anions following a concentration gradient . but lactate produced by the tumors disrupts the lactate gradient between intracellular and extracellular compartment of cytotoxic t cells present within the tumor bed . role of buffering agents like soda bicarbonate to treat la is only supportive and temporary . anemia can rarely be a cause of la in this case as la persisted even after blood transfusion . in this case though there were other confounding factors like low albumin , b - symptoms and bulky disease that are causes of poor prognosis so we can say that la has an additive effect for poor prognosis in high - risk cases of lymphoma . the literature review of the cases of la in lymphoma also suggests that these cases were having one or other high - risk features along with la . la is rare in patients with lymphoma . it is associated with high tumor burden along with aggressive biological and clinical behavior and

lactic acidosis ( la ) has been reported to be associated with high grade lymphoma as a terminal event . its causes are multi - factorial . it can either occur due to overproduction of lactic acid by rapidly dividing tumor or due to its underutilization due to involvement of liver by lymphomatous deposits . the prognosis of lymphoma associated with la is dismal . we present a patient of non - hodgkins lymphoma ( nhl ) who presented with la , after an initial response succumbed .

emphysema of orbit is usually considered a self - limiting , transient phenomenon . however , mass of air in the orbital can cause occlusion of central retinal artery leading to potential for severe visual loss , so the rapid diagnosis and management is essential to preserve vision . it can present with proptosis , orbital pain , loss of vision , raised intraocular pressure , and central retinal artery occlusion causing ischemic optic neuropathy , rarely orbital compartment syndrome . it can be caused by preceding head injury , infection , barotrauma , complications of surgery , injury from compressed - air hoses , and following forceful injection of air into the orbital soft tissue spaces . a 7-year - old girl presented to our trauma hospital with a complaint of proptosis of left eye , pain in the orbital region associated with swelling and discoloration of both eyes lids following a fall from height . examination showed glasgow coma score of 15/15 , presence of bilateral raccoon 's eye with proptosis of left eyeball , visual acuity of 6/6 on both eyes with restricted medial gaze movement of left eyeball , rest of the neurological evaluation were within normal limit . an urgent computerized tomography ( ct ) scan head and facial bones including the orbits was performed and revealed fracture of medial orbital wall of left orbit , [ figure 1 ] . bone window revealed fracture of orbit roof with small pneumocephalus over left basifrontal region [ figures 2 and 3 ] . she was kept in hospital and managed conservatively , over 4 days her vision remain stable with the subsidence of pain and reduction of periorbital swelling . at the last follow - up , noncontrast computerized tomography orbit of a 7-year - old girl , bone window view showing collection of air in multiple pockets adjoining to medial rectus in left orbit noncontrast computerized tomography head , bone window view showing basifrontal pneumocephalus noncontrast computerized tomography orbit , bone window view showing fracture of orbital roof with frontal bone fracture noncontrast computerized tomography head demonstrating pneumocephalus orbital emphysema is clinically characterized by presence of air pocket in the orbit acting as a mass effect . it can be caused by orbital wall fracture , include forceful nose blowing , postsurgical and pressure changes during air travel . fractures of orbital wall typically occur at the thinnest sector including the medial wall permitting entry of air from the paranasal sinus . thickness of the medial wall of orbit is thinner , about 0.25 mm , while isolated medial wall fractures occur in approximately 1030% of cases of orbital trauma . in pediatric age , orbit bones are elastic so , function as a trap door which results in a higher incidence of muscle entrapment following orbital wall fractures . trapped air pocket in periorbital spaces due to ball valve action of orbital soft tissue causes displacement of fracture fragment leading to herniation into the sinus cavity . depending on rapidity of air entrapment , total volume of entrapped air , presence of ball valve mechanism , patient can present with a range of clinical symptoms including proptosis , loss of vision , increased intraocular pressure , and central retinal artery occlusion , or orbital compartment syndrome . clinically , patient presents with orbital pain , hypoesthesia , and restriction of ocular movement , diplopia or proptosis , and vision loss . computerized tomography scan aids in identifying the presence of orbital emphysema and anatomical location of air pocket underlying adjoining fracture of medial or floor or roof of orbit . our case had fracture of medial wall and roof of orbit with associated basifrontal pneumocephalus . the aim of management of orbital emphysema associated with orbital fracture is to put a constant vigilance to look for raised intraorbital pressure effect like restricted ocular motility , sluggish pupillary reaction , disc edema or decreased visual acuity . drainage of trapped air in the subcutaneous tissue , early detection and prompt remedial measures are necessary to preserve good vision . hence , when orbital emphysema shows signs of pressure effect - orbital compartment syndrome should be suspected and emergency decompression is necessary . it can be simply carried out by inserting a 24-gauge needle connected with underwater seal for regulated drainage of collected air causing mass effect . other methods are typically performed by either needle aspiration of trapped air or canthotomy which causes rapid decompression and complete resolution of raised intraorbital pressure symptoms .

orbital emphysema is usually considered as self - limiting condition , rarely carries a risk of vision loss as catastrophic consequences due to occlusion of the central retinal artery . it can occur as a consequence of head trauma associated with fracture of the orbit , previous surgery , infection or exposure to high - pressure air jet . authors report an interesting 7-year - old girl who presented with proptosis of left eye , associated with swelling of eyelids of both eyes . computerized tomography scan head and orbit revealed orbital emphysema in the left eye with fracture of medial wall of left orbit and fracture of orbit roof with small pneumocephalus over left basifrontal region . fortunately , she responded well to conservative therapy . current article reminds uncommon condition to prevent potential visual loss , describing briefly management options .

merkel cell carcinoma ( mcc ) is an uncommon cutaneous malignancy with an estimated 1,500 cases diagnosed annually in the us . it is most commonly seen in men in their 70s and on sun - exposed skin . moreover , when it is found in immunosuppressed patients , the clinical course can be more fulminant . the cell of origin remains elusive and the non - descript nature of the histology has led to various descriptions of the tumor , which include small cell cancer of the skin , trabecular cutaneous tumor , or anaplastic skin cancer . although the regional lymph nodal basin may be occultly involved in as many as 24% of cases at presentation , and hematogenous spread is well documented and is importantly linked to overall survival , simultaneous or synchronous presentation of the cutaneous lesion is unusual . even more odd than what may be explained as a regional field effect causing adjacent simultaneous lesions is the process of metachronous cutaneous lesions , particularly when the presentation is disparate in location . we present the case of a male patient who first presented to medical attention in september 2005 , when he was 67 years of age . he complained of a pea - sized , non - erythematous , non - pruritic lesion overlying the medial compartment of the left infrapatellar leg . he was seen by one of the authors ( e.p.p . ) who re - excised the lesion and performed a sentinel node staging simultaneously . the sentinel node procedure revealed 2 nodes involving the superficial groin , both of which contained metastatic disease . the patient underwent staging studies , including a contrast - enhanced ct scan of his chest , abdomen , and pelvis , which showed no evidence of metastatic disease . given the regional nodal involvement and the neuroendocrine nature of the tumor , the patient was recommended adjuvant cisplatin and etoposide chemotherapy , and he completed 4 cycles . he then received complete 5,600 cgy of external electron beam teletherapy to the tumor bed plus a 2-cm margin . he had been followed closely on a quarterly basis with clinical examinations and annual chest radiography when , in november 2008 , he developed a cystic and erythematous lesion overlying the right olecranon process ( fig . he visited his plastic surgeon , and an excisional biopsy demonstrated a 1-cm focus of mcc with a close deep margin . he underwent a re - excision and sentinel node mapping , revealing no residual local disease and negative regional axillary nodes . a staging ct - pet fusion failed to reveal metastatic disease . treatment with 5,000-cgy electron beam teletherapy to the tumor bed was continued , completing that course uneventfully . the patient 's medical history was notable for osteoarthritis , osteoporosis , hyperlipidemia , hypertension , cardiac dysrhthymia , and vascular disease . medications included celebrex , fosomax , kcl , diovan , toprol , vytorin , coumadin , norvasc , and tramadol . his family history was notable for a brother who died of a primary brain tumor and a daughter who had a rhabdomyosarcoma . he was a retired carpenter who consumed alcohol socially and did not use tobacco products . this patient example of a metachronous mcc represents a distinct entity . to our knowledge , only one previous case report of a metachronous mcc has been reported in the literature and those authors concluded that the second occurrence represented a field defect given the proximity of the two tumors as well as a sophisticated dna comparison linking the lesions to the same cellular precursor . when metachronous lesions of a rare malignancy occur , the clinician is obligated to pursue an underlying cause . there are no known risk factors associated with the development of mcc , and this malignancy is not associated with any known genetic family of cancers . a family history of a brain tumor and sarcoma suggests a potential li - fraumeni syndrome , though this rare mutation in the p53 gene has not been associated with mcc . immunosuppression has been shown to increase the likelihood of unifocal mcc ; however , it has not been reported to increase the risk of metachronous lesions , nor does this patient have any history of conventional forms of immunosuppression . several studies have suggested a role for polyomavirus in the etiology of mcc , and virus - specific assays assess the involvement of the infectious agent . the patient presented in this report had no clinical manifestations of this virus , but a specific assay was not performed , leading us to query whether his metachronous presentation of mcc was virus - induced . indeed , addressing the specific clonal rearrangement of the two separate lesions may have added to the evidence that the two tumors were related ( perhaps in their viral association ) or truly separate . although it is tempting to identify a potential viral pathogen that may be associated with mcc , the development of an anti - viral therapy or immunization remains of scientific import only for the present and perhaps , because of the rarity of mcc , will never become a practical consideration . because of the potential increase in risk that this patient may develop future disparate mcc lesions , regardless of the etiology , he is being followed closely with clinical dermatologic surveys . other surveillance studies , such as laboratory or radiographic surveys , appear unwarranted in the absence of signs or symptoms of disease .

merkel cell carcinoma ( mcc ) typically presents as an isolated cutaneous lesion with a measurable risk of regional lymph node involvement . uncommonly , synchronous or metachronous lesions have been described to be attributed to a field effect . this case report describes a patient presenting with metachronous lesions , separated by several years , which can not be attributed to a field effect given the tumor distribution .

colonoscopy is considered as one of the most commonly performed medical procedures and deemed to be safe . however , the complication rates are increasing as its use is growing due in part to the successful promotion of colorectal cancer screening and prevention guidelines . post - colonoscopy , the incidence of colonic perforation ( cp ) , is rare with an estimation of 0.19 - 0.21% . though intraperitoneal perforation is common , extra - peritoneal perforation with pneumoperitoneum , pneumomediastinum , and subcutaneous emphysema is exceedingly rare . a 64-year - old african american female with past medical history significant for hypertension and cerebral aneurysm status after repair presented with sudden onset of diffuse chest and abdominal pain . she described the pain as persistent , dull in nature , non - radiating and aggravated by movement . she also complained of shortness of breath and two episodes of non - bloody , non - bilious vomiting . before colonoscopy , she was in her usual state of health . for the current procedure , she had adequate bowel preparation and the findings were positive for wide mouthed diverticulosis of the sigmoid colon without other associated findings . no biopsy was performed . clinical examination revealed normal vital signs with subcutaneous emphysema and air crepitus at the base of her neck bilaterally and on the anterior chest wall . computed tomography ( ct ) scan of the chest , abdomen and pelvis revealed massive free retroperitoneal and intraperitoneal air and massive pneumomediastinum with extensive gas dissecting throughout the neck ( fig . ( b ) ct scan of the abdomen and pelvis with massive free retroperitoneal and intraperitoneal air . she was admitted to the intensive care unit for close monitoring and was managed conservatively with bowel rest , intravenous fluids and broad spectrum antibiotics . the patient condition improved without the need for surgical intervention , her diet was gradually advanced and a repeat ct scan of the chest , abdomen and pelvis after 4 days showed interval improvement of the pneumomediastinum and pneumoperitoneum . she was discharged in a stable condition and advised to follow up with outpatient medical clinic . the incidence of colorectal perforation following colonoscopy has been reported to range between 0.16% in diagnostic colonoscopies and 0.44% in therapeutic colonoscopies . risk factors for perforation include advanced age , inflammatory bowel disease , diverticulitis , malignant masses and performance of therapeutic procedures such as polypectomy [ 2 - 4 ] . literature suggests sigmoid colon as the most commonly involved site for perforation followed by the cecum , which could be explained by anatomic variations , frequent location of diverticula , polyps and subsequent therapeutic interventions predisposing to thermal or mechanical injuries . three different mechanisms describe the cause of perforation : pneumatic perforation , mechanical perforation , and perforation associated with therapeutic colonoscopy . over distension by insufflated air can cause rupture of colon wall leading to pneumatic perforation while shaft or tip of the endoscope which excessively pressured the intestinal lumen can cause mechanical perforation . therapeutic colonoscopy associated perforations likely result after colon polypectomy , pneumatic dilation , endoscopic mucosal resection and electroceutical injury caused by snares or hot biopsy forceps use . with polypectomy , this risk rises to 0.3 - 1% , and with hydrostatic balloon dilatation of colonic strictures , higher rate ( 4.6% ) may be expected [ 7 , 8 ] . rarely , after colonoscopy , air can accumulate in certain extra - peritoneal body cavities such as the mediastinum , scrotum , subcutaneous tissues , or pleura . the subcutaneous tissue offers the least resistance to expansion and thus , subcutaneous emphysema is usually the first to manifest . the soft tissues in the neck are connected via a continuum of facial planes with the mediastinal cavity , which creates the pneumoperitoneum . furthermore , rupture of the mediastinal pleura due to a high pressure of insufflated air may lead to pneumothorax . patients with cp can present with symptoms and signs of peritonitis ( mainly abdominal pain and tenderness ) within several hours after the completion of colonoscopy . as the patients with therapeutic colonoscopies tend to have a smaller size of the perforation as compared to diagnostic , these have a delay in presentation compared [ 12 - 14 ] . the suspicion of cp should be kept in mind , if a patient has fever , abdominal pain or distention following the colonoscopic examination , even several days after the procedure . these patients can be diagnosed and treated for cp on the basis of generalized peritonitis without the radiologic evidence of perforation . in clinically in doubt , a plain x - ray of the abdomen should be taken to rule out intraperitoneal air . ct scanning and magnetic resonance imaging are also of great help to identify the free gas . water - soluble contrast enema can be used to confirm a concealed perforation . the management of cp has been a controversial issue , though it is effectively managed by both conservative and non - conservative ( operative ) strategies [ 16 , 17 ] . these patients should be closely monitored for clinical improvement which is usually seen within 24 - 48 h. if there is no improvement or worsening of condition , surgical interventions can be opted . recently , another valid approach has been reported in patients with small lesions and without signs of peritonitis , where endoscopic clipping is followed by conservative treatment . surgical treatment is indicated when there is evidence of peritonitis signs , the presence of the distal obstruction to the perforation site and the worsening or no improvement after conservative treatment [ 8 , 19 ] . in conclusion , during a colonoscopy , physicians should be aware of this rare complication as failure to recognize and treat such complications can be unsurprisingly fatal . in conclusion , during a colonoscopy , physicians should be aware of this rare complication as failure to recognize and treat such complications can be unsurprisingly fatal .

colonoscopy is performed for both diagnostic and therapeutic indications . although rare , associated complications can be quite serious . the frequency of these complications depends mainly on the skills of the physicians doing the procedure , and the diagnostic or therapeutic indications . major complications include adverse anesthetic related events , aspiration pneumonia , bleeding , and colonic perforation . we present a rare case of a post - colonoscopy perforation presenting with subcutaneous emphysema and free mediastinal , and intra - peritoneal air . the patient was successfully managed conservatively with complete resolution of symptoms .

we report the case of a 58-year - old woman with intracranial hemorrhage associated with stress - induced cardiomyopathy . left ventricular dysfunction was confined to midventricular segments , and manifested with transient anterolateral wall aneurysm . although wall motion was severely impaired in the mid - segments , an almost preserved global midventricular radial strain forecasted rapid improvement of ventricular function . this case highlights how deformation imaging can help in clinical practice to interpret the subtle signs of recovery from left ventricular dysfunction . a 58-year - old woman was admitted one year ago to the neurointensive care unit of our hospital for sudden onset of intracerebral and subarachnoid hemorrhage of the right frontal lobe . she had a history of tobacco use and moderate alcohol abuse , hypertension and chronic obstructive pulmonary disease . her surgical history included left lumpectomy for breast cancer , amputation of the third finger on the right hand and jaw surgery a few years ago . at admission her vital signs were within the normal range : blood pressure 140/87 mmhg , heart rate 71 beats per minute ( bpm ) , respiratory rate 20 breaths per minute and oxygen saturation on ventilator 100% . the electrocardiogram ( ecg ) at admission showed normal sinus rhythm ( 60 bpm ) and possible septal necrosis . right craniotomy for evacuation of the hemorrhage and clipping of right middle cerebral artery aneurysm were performed . troponin i levels showed a slight increase from 0.33 to 3.55 ng / ml during the first two days , and then decreased to 1.96 ng / ml on the third day postsurgery , and similar variations were seen for the levels of creatine kinase - mb fraction ( from 4.0 to 11.5 to 4.3 ecg on the second day showed deep negative t waves in almost all leads with prolonged qtc interval [ figure 1 ] . due to abnormal myocardial necrosis markers and ecg , a two - dimensional ( 2d ) transthoracic echocardiogram was ordered , and revealed a normal left ventricular cavity size with hyperdynamic basal motion , akinesis of midventricular segments and nearly normal apical motion [ figure 2 ] . left ventricular global systolic function was moderately decreased with left ventricular ejection fraction at 35% ( normal values > 55% ) . electrocardiogram on second day post - right craniotomy for evacuation of hemorrhage and clipping of right middle cerebral artery aneurysm showing deep negative t waves in almost all leads with prolonged qtc interval ( 580 ms ) two - dimensional transthoracic echocardiography in the apical four - chamber view showing the left ventricle a ) at end diastole and b ) mid - systole . basal and apical segments contract well in contrast to midventricular segments the next day , the patient underwent coronary angiography , which ruled out any coronary artery disease . however , the ventriculogram surprisingly revealed an aneurysmatic anterior wall [ figure 3 ] , which then was characterized by another transthoracic echocardiogram as an anteroseptal wall aneurysm with near - normal motion of the other segments [ figure 4 ] . global radial strain was calculated and , despite seriously compromised midventricular anteroseptal motion , midventricular - averaged radial strain was at the lower end of normal range ( i.e. 16% ) . left ventriculogram showing systolic anterior wall aneurysm two - dimensional transthoracic echocardiography in the apical long - axis view showing anteroseptal wall aneurysm with nearly normal motion of the other segments in a ) end diastole and b ) mid - systole as predicted by the global radial strain in the previous examination , another transthoracic echocardiogram performed three weeks later documented the complete resolution of wall motion abnormalities [ figure 5a and b ] , with completely normal radial strain values [ figure 5c ] . two - dimensional ( 2d ) transthoracic echocardiogram in the apical long - axis view showing complete resolution of wall motion abnormalities at a ) end diastole and b ) mid - systole . on the left , a short - axis view of the mid - left ventricle is shown , and a 2d speckle tracking technique is used to obtain the curves representing radial deformation of each wall segment ( right ) . the averaged global strain is 57% ( represented by white curve ) isolated left ventricular midventricular dyskinesia is a rare variant of stress - induced cardiomyopathy , although it has been described in association with cerebral injuries . left ventricular mechanics in stress - induced cardiomyopathy have not been well defined , although some data about the longitudinal and radial strain are available . our case highlights the importance of global radial strain as a predictor of myocardial wall motion improvement , even when 2d transthoracic echocardiogram did not forecast the improvement .

introduction : we report the case of a 58-year - old woman with intracranial hemorrhage associated with stress - induced cardiomyopathy.results:left ventricular dysfunction was confined to midventricular segments , and manifested with transient anterolateral wall aneurysm . although wall motion was severely impaired in the mid - segments , an almost preserved global midventricular radial strain forecasted rapid improvement of ventricular function.conclusions:this case highlights how deformation imaging can help in clinical practice to interpret the subtle signs of recovery from left ventricular dysfunction .

chorea - acanthocytosis ( chac ) is a rare autosomal recessive neurodegenerative disorder characterized by generalized chorea , orofaciolingual dyskinesia with dysphagia and dysarthria , muscle wasting , hyporeflexia , and behavioral disturbance6 ) . chac can be diagnosed by western blot identification of chorein or sequencing of the vps13 gene4,11 ) . medical treatment is mostly ineffective and deep brain stimulation ( dbs ) has recently been attempted for management of chac . we report here on a genetically confirmed case of chac in a patient who showed significant improvement after bilateral dbs of the globus pallidus interna ( gpi ) . we also provide a review of the literature regarding the treatment outcome of this rare condition . a 36-year - old man presented with exacerbation of slurred speech , orofaciolingual dyskinesia , tongue and lip biting , choreiform movements of the head and neck , and gait disturbance during the course of three years . there was no family history of similar neurological disorders . on examination , we observed rapid bending of the neck and trunk , alternating lateral flexion of the trunk when walking , resulting in frequent falls . muscle power and muscle bulk , and sensory function were intact . the patient did not show any psychiatric disorder during the psychiatric interview . mini - mental status examination ( mmse ) laboratory tests , including serum creatine kinase , iron , ferritin , lactate , lipids , and lipid electrophoresis were unremarkable . brain mr imaging ( 3.0-t mr system , verio , siemens , ag , erlangen , germany ) showed bilateral atrophic putamina and head of caudate nuclei on a t2-weighted image ( fig . finally , genetic testing confirmed a homozygous nonsense mutation in exon 37 ( c.4411c > t ; p.arg1471 ) in the vps13 gene . his symptoms did not respond to medications , including diazepam , baclofen , trihexyphenidyl , haloperidol , and tetrabenazine . during the two - year follow - up period , his choreic movement and gait disturbance showed gradual worsening and he was no longer able to walk independently . we recommended dbs for the patient for control of symptoms and for improvement of functional capacity . after written consent was obtained , he underwent bilateral implantation of a quadripolar electrode ( model 3387 ; medtronic , minneapolis , mn , usa ) into the gpi under generalized anesthesia so that the lowest contact terminated at the bottom of gpi ( fig . we performed ct scan in order to confirm adequacy of the electrode locations and absence of hemorrhage followed by placement of an internal pulse generator ( soletra ; medtronic ) . in the six - month postoperative evaluation , motor section of unified huntington 's disease rating scale ( uhdrs ) score was 59 in the preoperative evaluation , and 36 after two months . one year after dbs implantation , the score had decreased to 34 and mmse scores showed no change . the final dbs settings were as follows : right , 2.3 v , 60 s , 130 hz ; left , 2.9 v , 60 s , 130 hz . gpi - dbs provided a rapid benefit , which lasted for two years during the follow - up period . this result supports the usefulness of dbs for symptomatic treatment in cases of chac , however , there are still few data regarding long term benefit ( table 2 ) . dbs has been used in treatment of hyperkinetic movement disorders , including dystonia and l - dopa induced dyskinesia in parkinson 's disease . dbs has recently been employed for relief of symptoms of chorea in patients with huntington 's disease and chac . in patients with chac , a paucity of case reports of dbs have demonstrated improvements in chorea and activities of daily living , although with some variability in treatment outcome . one published case of a patient with chac reported improvement in choreic movements following dbs of the ventralis oralis posterior nucleus of the thalamus3 ) . however , gpi is a more preferable target for treatment of primary dystonia or neuroacanthocytosis and almost all therapeutic effects were obtained by gpi - dbs . high - frequency stimulation has been reported to be effective not only in generalized dystonia , but also in several cases of huntington 's disease1,10 ) , senile chorea15 ) , and cerebral palsy2 ) . in agreement with a few reports7,12,13 ) , our patient showed improvement of his symptoms with high - frequency stimulation ( 130 hz ) . however , the opposite has been reported in some cases of chac , where improvement was only achieved with low - frequency stimulation ( 40 hz ) with worsening at high - frequency stimulation ( 130 hz)5,8 ) . the exact reason for these contrasting effects is unclear and conduct of further studies will be needed . few studies have reported on the long - term effect of dbs in treatment of chac . miquel et al.9 ) recently reported a significant long - term improvement of motor symptom severity ( improvement 20% in uhdrs - motor score ) in 61.5% of patients at a mean follow - up period of 2.5 years . they found that gait improvement , orofacial movements and tics , head drops , and trunk spasms were dbs - sensitive symptoms , while dysarthria and feeding problems were less responsive . in our case , during long - term follow - up , the patient showed improvement of 42.3% within one year and 39.0% within two years after surgery . among his various symptoms , abnormal truncal flexion , tongue and lip biting showed marked improvement within several months after surgery , while dysarthria did not show significant improvement . this has been attributed to the adverse effect of dbs9 ) or progression of the disease . chac is a rare autosomal recessive disorder characterized by generalized chorea and morphological abnormalities in red blood cells . medical treatment is usually ineffective and dbs has been tried in management of chac . in recently reported cases , including the one reported here , dbs of the gpi resulted in significant improvement of symptoms in chac . although dbs can not cure all symptoms of chac , it can improve the quality of life for patients . therefore , dbs is a valuable treatment option and should be considered as a treatment option for chac patients who are refractory to medical therapy .

chorea - acanthocytosis ( chac ) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis . pharmacotherapy for control of involuntary movements has generally been of limited benefit . deep brain stimulation ( dbs ) has recently been used for treatment of some refractory cases of chac . we report here on the effect of bilateral high - frequency dbs of globus pallidus interna in a patient with chac .

a 53-year - old male patient visited our hospital due to a huge mass in the anterior area of the left lower leg as the chief complaint . forty four years ago , the patient was bitten by a snake and the area below the left knee joint was tied with a string and maintained for 3 months to prevent the spread of snake venom . a mass developed with time while the left lower leg was healing gradually . over a period of several years , the mass gradually increased in size . no special findings were detected in his past medical history , except for diabetes . at the time of admission , the physical examination revealed a huge mass in the muscle layer along the anterior tibial muscle in the left lower leg that was palpated . however , no redness , no heating sensation or no pyrogenic reaction was detected in the vicinity . the ankle joint was in an almost ankylosed state at 5 of plantar flexion , there was no abnormal sensation below the lower leg , and the blood circulation was good . the radiograph showed well marginated radioopaque density in the parosteal area of the midshaft of the tibia ( fig . the size of the mass was approximately 10 3 cm and there was no evidence of tibial erosion or cortical thickening . the mri showed a dense bone forming tumor in the parosteal area of the midshaft of the tibia ( fig . it showed homogenous low signal intensity on the t1 , t2 weighted images and there was no evidence of medullary cavity communication or a connection stalk . it was 10 3 4 cm in size and covered with a hard fibrotic sheath . there was no connection between the mass and the adjacent bones , and the proximal and distal areas of the mass were connected by a fibrotic cord . the histological examination showed an extensive amorphous pink substance with calcific material due to necrosis of the skeletal muscle and fibrin ( fig . 4 ) . the simple radiographs taken after surgery showed no residual mass , and the wound healed without necrosis or infection . it is primarily the sequelae of compartment syndrome that developed after trauma , and tends to progress slowly over a period of several years.4,5 ) it was reported to develop primarily in the lower legs but some cases in the arm have been reported.6 ) among the reported cases , trauma preceded its development in most cases , and the mass developed over a long time after the injury . according to several reports , it can develop over a period ranging from 10 to 64 years.1 - 6 ) in our case , it developed gradually over 40 years after the injury . et al.1 ) suggested that an initial compartment syndrome decreases the circulation within a limited space resulting in necrosis and fibrosis . with time , the mass enlarges due to the repeated intralesional hemorrhage.7 ) radiologically , the calcified mass grows along the muscles , and it shows a pattern of invasion to the entire muscles or the entire compartment . the incidence of such lesions is rare and the radiological findings reveal an aggressive pattern . sometimes , they are misdiagnosed as soft tissue sarcomas.3,6,7 ) therefore , it is important to differentiate these lesions from soft tissue sarcomas that show a calcified pattern . the treatment of calcific myonecrosis involves excision of the mass . however , several authors reported complications , such as postoperative infections in 30% of cases treated viau et al.10 ) and malisano and hunter9 ) performed incision and drainage in two patients with anterior compartment lesion in the lower leg and left the wound open . nevertheless , a secondary infection appeared due to the incomplete resection of the lesion , and one patient underwent a below - knee amputation . since such a method could induce a secondary infection , chronic fistula formation , etc . , it is better to treat the condition by the repeated aspiration of the mass to avoid secondary infection . o'keefe et al.1 ) performed marginal debridement , and in one patient , the surgical wound was left open after resecting the lesion . early et al.8 ) reported satisfactory results in 2 patients , in whom the remaining empty space generated after complete resection of the calcified mass was filled with tibial muscles . in our case , after removing the mass , complete wound closure was performed without filling with soft tissues . compression dressing was administered for two weeks after surgery , which prevented the generation of dead space . there were no complications , such as infections in the surgical area and fistula formation during the 3 year follow - up after surgery . cacific myonecrosis is a rare disease that develops as a sequelae of trauma , and is difficult to diagnose . it can be observed radiologically in a single muscle or throughout a compartment , and occurs frequently in the anterior compartment . we report our experience of a patient , who developed calcific myonecrosis over a 44 year period after trauma and was treated with a marginal excision , along with a review of the relevant literature . this condition should be suspected in cases with a soft tissue mass in the same area as compartment syndrome after trauma . moreover , the development of calcific myonecrosis can be prevented by detecting compartment syndrome early and administering the appropriate treatment .

calcific myonecrosis is a rare late post - traumatic condition , in which a single muscle is replaced by a fusiform mass with central liquefaction and peripheral calcification . compartment syndrome is suggested to be the underlying cause . the resulting mass may expand with time due to recurrent intralesional hemorrhage into the chronic calcified mass . a diagnosis may be difficult due to the long time between the original trauma and the symptoms of calcific myonecrosis . we encountered a 53-year - old male patient diagnosed with calcific myonecrosis in the lower leg . we report the case with a review of the relevant literature .

the united states scoliosis research society defines idiopathic scoliosis as a cobb s angle > 10 , which increases the displacement and curvature of spine in the left and right sagittal plane1,2,3 . patients with idiopathic scoliosis have a three - dimensional deformation with lateral curvature and rotation of the vertebral body . the concave surface of the spinal curvature produces forward protrusion and the convex surface of the curvature produces a rearward protrusion in the ribs of the connecting vertebral body . consequently , if the spinal red muscles becomes weak , the white muscle becomes overused and the red muscle becomes weaker because the deep spine muscles are mostly red slow - twitch muscle fibers5 . therefore , stability exercises with isotonic contractions are emphasized rather than simple mobility exercises to correct posture and regain control of white and red muscles in patients with idiopathic scoliosis6 . these exercises provide sensorimotor and kinesthetic stimulation , thereby correcting the abnormal breathing patterns present in patients with scoliosis7 . the us scoliosis research society recommends evaluating cobb s angle in patients with idiopathic scoliosis , which is measured from a standing posture radiograph8 . a detailed intervention with emphasis on active holding to regain stability is needed to improve idiopathic scoliosis with a broad range of deformation9 . therefore , this case study was performed to analyze the effect of the schroth method with emphasis on active holding to regain stability , manage pain , and correct the cobb s angle in patients with idiopathic scoliosis . the subjects were three patients with idiopathic scoliosis and cobb s angle 10. it applied the schroth method individually to subjects during 60 min sessions , three times per week , for 15 weeks . intensity is based on the subject s curve type and the level of passive support . this study excluded subjects who had experienced treatments for idiopathic scoliosis , smokers , patients with asthma , lung disease , central nervous system disease , or a history of spine surgery . consent was obtained from all subjects , and this study was approved by the university of sahmyook university ethics board . it evaluated idiopathic scoliosis by measuring cobb s angle ( intersecting angle between the perpendicular to lines drawn parallel to the superior and inferior endplates of the most tilted vertebrae ) at the coronal plane of the entire spine by x - ray ( ed150l / r-500 - 150 ; shimadzu inc . , , erlangen , germany ) and a computer - aided design program ( cad impact , seoul , korea)8 . a 21-year - old and male with idiopathic scoliosis had low back pain and iliocostal syndrome ( visual analogue scale [ vas ] score : 4 ) , cobb s angle of 20 , and left deviation of the lumbar and right deviation of the hip and pelvis . it applied the schroth method to stabilize the center area between the iliac crest and the twelfth thoracic vertebra . the apex of the lumbar curvature was mobilized in a muscle cylinder position of the right lateral . after applying the schroth method , the vas score decreased to 0 and cobb s angle was 10.8 ( fig . change in cobb s angle in case 1 subject was a 29-year - old female with idiopathic scoliosis had neck pain , headache ( vas score : 8) , cobb s angle of 20.3 and a right deviation of the thoracic and a left deviation of the hip and pelvis . it applied the schroth method with reverse force on the shoulder to stabilize the hip and pelvis . after applying the schroth method , the vas score decreased to 0 and cobb s angle was 4 ( fig . change in cobb s angle in case 2 a 25-year - old female with idiopathic scoliosis had the neck pain and psoas syndrome ( vas score : 8) , cobb s angle of 30.6 , and a right deviation of the lumbar and a left deviation of the thoracic . it applied the schroth method to the center area between the iliac crest and the twelfth thoracic vertebra for stabilization . then , the apex of the lumbar curvature was mobilized on left side by lying in the muscle cylinder position . after applying the schroth method , the vas score decreased to 3 , and cobb s angle was 18.5 ( fig . 3fig . the schroth method was developed based on biomechanical and neurophysiological concepts without compensating for the curve in the spine , which undergoes axial elongation , deflexion , derotation , rotational breathing , and stabilization7 . in addition , a therapist educated in the memory of corrected posture recognizes abnormal during activities of daily living8 , 9 , 12 , 13 . patients with idiopathic scoliosis must consider the of changed appearance after treatment and adjustment of cobb s angle8 . therefore , a detailed intervention is required with emphasis on active holding to gain mobility and stability9 . this case study was performed to analyze the effect of the schroth method on decreasing pain and cobb s angle in patients with idiopathic scoliosis . this case study showed a decrease in pain and cobb s angle in all subjects after applying the schroth method with emphasis on active holding . previous studies have also reported significant decreases in pain and cobb s angle and increased quality of life8,9,10,11 , 13 . thus , the schroth method allows patients to regain normal posture of elongation , de - rotation , and de - flexion of the spine by continuous feedback of visual information8,9,10,11 , 13 . therefore , the schroth method decreases pain and cobb s angle by inhibiting compensation and restoring appearance . therefore , further research may be needed with more subjects to verify the effect of applying the schroth method to treat patients with idiopathic scoliosis .

[ purpose ] the purpose of this study was to examine the effect of the schroth method ( three - dimensional convergence exercise ) of emphasis of active holding on pain and cobb s angle in patients with scoliosis . [ subjects and methods ] it applied the schroth method program of emphasis of active holding individually to three subjects three time per week for 15 weeks . all subject were measured cobb s angle and pain . [ results ] after 15 weeks , pain and cobb s angle decreased compared to values before . [ conclusion ] these results demonstrate the benefit of the schroth exercise program of emphasis of active holding on decreasing pain and cobb s angle in patients with idiopathic scoliosis .

acantholytic dyskeratosis ( ad ) is a histologic pattern characterized by hypergranulosis , acantholysis , suprabasilar clefting , and formation of corps ronds and grains . this can be seen in darier 's disease ( dd ) or dyskeratosis follicularis , transient acantholytic dyskeratosis ( tad ) or grover disease ( gd ) , as well as in isolated lesions , such as warty dyskeratoma ( wd ) , acantholytic dyskeratotic acanthoma , and as an incidental histopathologic finding of isolated ad . dd and tad occur most commonly in seborrheic areas and wd most commonly in the head , neck , trunk , and extremities . we present a case of acantholytic dyskeratosis that is unique based on its genital location and cystic architecture . a 53-year - old woman presented with an otherwise asymptomatic cyst on her left vulva of uncertain duration . there was no known history of generalized skin conditions with similar histology and there were no associated lesions of the vulva or the cervix . the lesion was surgically removed and submitted for routine histologic processing and staining with hemotoxylin and eosin . on microscopic examination , there were fragments of cystic epithelium with areas of hypergranulosis , acantholysis , suprabasilar clefting , and corps ronds , and grains formation [ figures 13 ] . these features were felt to represent isolated dyskeratosis follicularis arising in the context of an infundibular cyst . hematoxylin and eosin 20 medium power view of cystic lesion demonstrating hypergranulosis , acantholysis , and corps ronds formation . h and e 100 medium power view of cyst demonstrating corps ronds , grains , and suprabasal acantholysis . changes of dyskeratosis follicularis are unusual in the genitalia but there have been cases of multiple papular lesions on the genital area reported under various terminology , including papular acantholytic dyskeratosis and acantholytic dermatosis of the vulvocrural fold . in fact , in a review of 28 cases of isolated dyskeratotic acanthoma , none occurred in the genital area . we were able to identify three reports of solitary genital lesions of isolated ad [ table 1 ] . one of the six vulvocrural cases described in the series by cooper exhibited cystic invaginations with multiple follicular connections and histologic features of lora et al . 's report of wd on the mons pubis also histologically showed a similar cup - shaped cystic invaginations involving multiple indundibulae . and of the three vulvar cases of wd reported by duray et al . , at least one appears to show a cystic invagination adjacent to or involving a pilosebaceous unit . however , none of these genital cases of ad demonstrated changes with a truly cystic architecture , as seen in our patient . cases of warty dyskeratoma located on the genital area our patient 's lesion could be considered to represent an unusual presentation of wd . wd commonly presents as a solitary , keratotic papule or nodule on the sun - exposed areas in middle - aged males . histologically , wd displays broad , well - circumscribed invagination of a hyperplastic epidermis with hypergranulosis and papillomatosis , suprabasilar acantholysis , villi formation of the dermal papillae , and presence of dyskeratotic squamous cells ( corps ronds and grains ) . histologic overlap of cystic , nodular , and cup - shaped patterns have been noted in a large clinicopathologic review of wd . the cystic lesions are described as well - circumscribed nodules composed of one or several large infundibular cystic structures filled with masses of keratin material located in the papillary and upper reticular dermis . the cystic structures have a stratified squamous epithelial lining with foci of hypergranulosis and papillomatosis . a focal connection to the epidermis is sometimes observed . although it lacked the typical hyperplastic villous - like proliferations at the periphery seen in other cases of wd , this lesion presented herein otherwise exhibited hypergranulosis , dyskeratosis resulting in iris and target morphologic features of corps ronds , and suprabasilar acanthoysis , all of which are typical of wd . the cystic architecture described by kaddu et al . in other locations is reminiscent of our lesion , suggesting our lesion may lie on a spectrum of infundibular cyst and wd . contiguity with cystic infundibular structures described in the genital cases reported by cooper and lora et al . show a clear - cut histologic connection of these lesions to rudimentary pilosebaceous units or at least were located adjacent to a pilosebaceous unit . in another retrospective study , ck1 and ck10 were expressed in the suprabasal levels of the wd cases , whereas ck5 and ck17 were appreciated in the basal layer . this pattern of expression of cytokeratin markers further supports wd as an adnexal neoplasm with follicular differentiation . the term follicular dyskeratoma has been proposed to acknowledge the follicular infundibular origin of wd . in keeping with this theory , we feel that the infundibular cystic features seen in our patient 's lesion represent a spectrum of changes that can be seen in follicular dyskeratoma . vulvar wd with cystic configuration has never been reported [ based on pubmed search ] , and to the best of our knowledge , our case is the first to describe such a lesion occurring on this site . in addition to the various presentations of ad , the differential diagnosis of acantholysis on the vulva may include pemphigus vulgaris , herpes simplex virus , and hailey these entities are excluded in the absence of positive immunofluorescence in pemphigus , presence of viral changes in herpes , and more prominent acantholysis and less dyskeratosis in hailey acantholytic squamous cell carcinoma ( scc ) is differentiated by the absence of cytologic atypia in wd . interestingly , cystic dd , cystic scc , and keratoacanthoma with associated cystic sebaceous tumors in a muir torre syndrome patient have been reported . although rare and reported to occur at nongenital sites , these entities should be considered as the most important differential diagnoses for cystic wd . topical tazarotenic acid gel was reported to be effective in one patient . in summary , we present a rare case of ad that is unusual in its cystic presentation and genital location . this is thought , by the authors , to be a unique presentation of a follicular adnexal neoplasm , lying on the spectrum of wd or follicular dyskeratoma .

acantholytic dyskeratosis ( ad ) is a histologic pattern seen in darier 's disease or dyskeratosis follicularis , warty dyskeratoma , and transient ad . this pattern is characterized by suprabasilar clefting , acantholysis , and formation of corps ronds and grains . we present a case of ad that is unique based on its genital location and cystic architecture . a 53-year - old woman presented with an otherwise asymptomatic cyst on her left vulva of uncertain duration . on microscopic examination , there were fragments of cystic epithelium with areas of hypergranulosis , acantholysis , corps ronds , and corps grains formation . these features are felt by the authors to be a unique presentation of a follicular adnexal neoplasm .

malrotation of the midgut is an abnormality in embryological development of gastrointestinal tract . by the fourth intrauterine week the gastrointestinal tract is in the form of an endoderm lined tube divided into fore , mid- and hindgut . mid- and hindgut defined by their blood supply the superior and inferior mesenteric arteries respectively . by the fifth week of life the midgut begins a process of rapid enlargement , physiological herniation and rotation . with rapid expansion of liver and kidneys , expansion of the midgut intestinal loop can not be contained within the abdominal cavity ; this results in temporary physiological midgut herniation through the umbilical cord with superior mesenteric artery forming the axis . this process forms c of the duodenum and places it behind the superior mesenteric vessels . hernial reduction occurs by week 10 with the jejunum reducing first and lying to the left and subsequent distal portions lying progressively to the right . the ceacum descends from position in the right upper quadrant forming the descending colon with its mesentery gradually disappearing . a 17-year - old male was seen in the emergency department with 10-year history of abdominal colic which is relieved by vomiting , along with dehydration . there was no history of jaundice , fever , steatorrhea , or bleeding per rectum . the patient had been treated with proton pump inhibitors , prokinetic agents by general practitioners , without any relief . his ultrasound examination and standing x - ray of abdomen physical examination was normal except minimal abdominal tenderness in epigastric , right hypochondriac region along with mild dehydration . his liver function tests , renal function tests , amylase , hemogram , and urinalysis were normal . duodenum appeared connected to small intestine seen on right side giving a whirlpool appearance due to rotation of gut around the superior mesenteric artery . superior mesenteric vein was seen on left of superior mesenteric artery [ figures 1a and b ] . stomach and duodenum appear connected to small intestine seen on the right side giving a whirlpool appearance due to rotation of gut around the superior mesenteric artery . superior mesenteric vein is seen on the left of superior mesenteric artery surgical referral was made ; he was treated with four - port laparoscopic ladd 's procedure . the peritoneum to the right of the ascending colon and caecum was incised and the anteriorly situated bands were stripped to free the duodenum . he was discharged within 2 days eating a normal diet and made a good postoperative recovery . at 3 months he was gaining weight and had no further vomiting . midgut mal and nonrotation refers to failure in counter clockwise rotation of the midgut which results in misplacement of the duodeno jejunal junction to the right of the midline ; in addition the small bowel mesentery has narrow vertical posterior attachment which is prone to volvulus . other anatomical abnormalities include peritoneal ( ladd 's ) bands running from the right colon to the lateral abdominal wall and an extensively mobile ceacum that fails to descend . acute presentation is with volvulus of midgut or ileoceacum occurring most frequently in neonate with likelihood decreasing with age . in most of the reported cases of this presentation , patients present with bilious vomiting in the first month of life because of duodenal obstruction or a volvulus . pathophysiology of these chronic symptoms may relate to the compressive effects of peritoneal bands running from ceacum and ascending colon to the right lateral wall . the short mesentery allows the small bowel to twist around the narrowed sma pedicle to create a distinctive whirlpool appearance . surgical management of intestinal malrotation was first described by ladd in 1936 and remains mainstay of management today . it involves reduction of vovulus if present , division of abnormal peritoneal bands ( duodeno colic , dodenojejunal - ileocolic ) , and placement of the small bowel to the right of the abdomen and ceacum to the left . increasingly laparoscopic ladd 's procedures are being performed and have been shown to be effective where there is no acute volvulus.[79 ] this minimally invasive approach allows for earlier oral intake and discharge from hospital .

abnormalities in midgut rotation occur during the physiological herniation of midgut between the 5th and 10th week of gestation . the most significant abnormality is narrow small bowel mesentery which is prone to volvulus . this occurs most frequently in the neonatal period , less commonly midgut malrotation presents in adulthood with either acute volvulus or chronic abdominal symptoms . it is the latter group that represents a diagnostic challenge . we report a case of a 17-year - old male patient who presented with 10-year history of nonspecific gastro - intestinal symptoms . after extensive investigation the patient was diagnosed with midgut malrotation following computed tomography of abdomen . the patient was treated with a laparoscopic ladd 's procedure and at 3 months he was gaining weight and had stopped vomiting . a laparoscopic ladd 's procedure is an acceptable alternative to the open technique in treating symptomatic malrotation in adults . midgut malrotation is a rare congenital anomaly which may present as chronic abdominal pain . abdominal ct is helpful for diagnosis .

it is more common in female with female - to - male ratio of 3:1 . not all the cases of necrobiosis are associated with diabetes ; hence the terminology necrobiosis lipoidica diabeticorum has been abandoned . the ulcers in nl are quite painful leading to impaired quality of life of these patients . we present a case of ulcerative nl , which failed to respond to traditional treatment modalities but responded quickly to thalidomide . a 58-year - old woman with 15 years history of insulin - dependent diabetes presented with nonhealing ulcers on her shin since 5 years . four months later , she noticed a pinhead sized lesion over the red area , which enlarged and ulcerated . three similar ulcers [ figure 1a and b ] appeared on the shin over the next one month . the ulcers were non healing and extremely painful but not associated with any constitutional symptoms . local wound care , topical corticosteroids , topical calcineurin inhibitors were tried along with oral antibiotics , dapsone , pentoxyphylline , and analgesics , without much relief . she had multiple discrete tender ulcers over a waxy , erythematous , atrophic plaque on the anterior aspect of her left leg , variying in size from 3 2 cm to 1 1 cm . the borders of ulcer were undermined , base was indurated , and floor was covered with necrotic debris . her hemogram , renal and liver function tests , and venous doppler test results were normal . pus culture grew staphylococcus aureus sensitive to linezolid , amoxycillin and clavulanic acid , and tazobactam piperacillin . skin biopsy from the edge of the ulcer revealed a hyperplastic , sclerotic epidermis and a dense superficial and deep dermal infiltrate of lymphocyte and plasma cells concentrated around the blood vessels and sweat glands [ figure 2a - c ] . we started the patient on oral linezolid 600 mg twice daily for 7 days and chloroquine 250 mg once daily for 4 weeks along with local dressings . topical human recombinant epidermal growth factor , oral clopidrogel and aspirin were added to the above regime and continued for another 2 months , with no improvement . pain reduced dramatically within 2 weeks and the ulcers healed completely after 4 weeks [ figures 3a and b ] . ( a and b ) multiple non healing ulcers over the shin ( a - c ) hyperplastic , sclerotic epidermis and a dense superficial and deep dermal infiltrate of lymphocyte and plasma cells concentrated around the blood vessels and sweat glands with several granulomas arranged in a horizontal manner in mid and lower dermis . ( h and e , 10 , 40 , 40 ) ( a and b ) complete healing and softening of skin after treatment with thalidomide there is no consistently effective therapy , and lack of uniform guidelines make treatment more challenging . many theories were put forward to explain the pathogenesis of nl : diabetic microangiopathy due to deposition of glycoprotein in the blood vessel wall could lead to impaired blood supply to the skin ; greater cross - linking of the collagen fibres in nl could lead to thickening of the basement membrane zone ; immune complex deposition in the dermal blood vessel walls could lead to vasculitis ; recently a role of disturbance in glucose transport by fibroblasts has been postulated . glut-1 is the human erythrocyte glucose transporter , which mediates facilitative transport of glucose across epithelial and endothelial barrier tissues . this protein was expressed in the sclerotic collagen of nl patients , indicating insulin resistance in these tissues . these include cutaneous blood flow enhancers , such as dypyridamol , clopidrogel , aspirin , pentoxyphylline ; topical and intralesional steroids , and topical calcineurin inhibitors ; wound healing enhancers such as epidermal growth factors , platelet - derived growth factors , collagen gel , hyperbaric oxygen ; surgery and pulse dye laser ; and immune modulators such as antimalarials , cyclosporine , and biologics . our patient was treated with various drugs but failed to respond . in several case studies , tnf - alpha inhibitors such as etanercept , adalimumab , and infliximab were shown to improve ulcerated nld . tnf - alpha has been found in high concentrations in the sera and skin of patients with nl thalidomide acts as an anti - inflammatory agent by suppressing tnf - alpha via degradation of its messenger rna and by decreasing the ratio of helper t cells to suppressor t cells . the ulcer healed rapidly and thalidomide was withdrawn over 12 weeks , with no relapse till date .

ulcerative necrobiosis lipoidica ( nl ) in diabetic patients is a rare , painful condition . it is a difficult - to - treat condition , impairing quality of life of patients . although various drugs have been tried , none of them is consistently effective . biologics in the form of tnf - alpha inhibitors show promising results in the treatment . but because of their high cost we chose thalidomide , which also has tnf - alpha inhibiting properties to successfully treat a long - standing case of ulcerative nl , which was refractory to various treatment modalities .

the experiments were performed on a 700 mhz bruker avance iii system equipped with a tci cryo - probe . the spin - noise - detected two - dimensional hmqc ( snhmqc ) spectrum shown was recorded on 99% c - enriched glucose in h2o ( 0.648 mol l ) in a 5 mm nmr tube using the scheme of figure 1 , with the following parameters : t0 = t2 = 27 ms , h spectral width 9.5 khz , maximum t1 = 3.78 ms , c spectral width 10.6 khz , 90(c ) pulse 12.6 s , repetition delay 250 ms . one pass through all 80 t1 values of the sequence thus takes 27 s. because no refocusing pulses could be used on the h channel ( to avoid generation of spurious coherence ) , all acquisition and evolution times are generally short as t2 * rather than t2 determines the loss of coherence in this particular experiment . for the spectrum shown in figure 2 , 6000 passes through the pulse sequence the data , topspin 3.1 c - programs and matlab scripts , which are available in the supporting information , were used . first , fourier - transformation along the direct dimension was performed in topspin 3.1 ( by the command xf2 ) . then , the cross - correlation of the two noise blocks , m0 and m2 , for each t1 time point was achieved by an in - house written au-program multiplying the transformed data blocks , point by point ( see eq 3 ) . fourier - transformation along the indirect t1 dimension and addition of 6000 different experiments were done using a matlab script .

we introduce two - dimensional nmr spectroscopy detected by recording and processing the noise originating from nuclei that have not been subjected to any radio frequency excitation . the method relies on cross - correlation of two noise blocks that bracket the evolution and mixing periods . while the sensitivity of the experiment is low in conventional nmr setups , spin - noise - detected nmr spectroscopy has great potential for use with extremely small numbers of spins , thereby opening a way to nanoscale multidimensional nmr spectroscopy .

hyperparathyroidism due to parathyroid abnormalities are classified into three forms : primary , secondary , and tertiary . parathyroid adenoma accounts for about 85% of all cases of hyperparathyroidism , considering all the forms : primary , secondary and tertiary . other less prevalent causes include parathyroid hyperplasia ( 15% ) and carcinoma ( 3 - 4% ) . however , acute primary hyperparathyroidism , or parathyroid crisis ( pc ) , is rare . parathyroid crisis is characterized by life - threatening hypercalcemia of a sudden onset in subjects with phpt . we describe a patient with pc secondary to a giant intrathyroidal parathyroid adenoma who presented with progressive neurological symptoms , nausea and vomiting without medical response to treatment . a 19-year - old female patient was admitted to the orthopedics department for supra - acetabular fibrous dysplasia treatment . after orthopedic surgery , during the second postoperative day she began to suffer from lethargy , nausea , vomiting , and abdominal pain . also , the patient reported muscle weakness , fatigue and a worsening of depressive symptoms . a blood analysis was performed which showed elevated ca ( 14.2 mg / dl , normal laboratory level 8.8 - 10.2 mg / dl ) . the serum phosphorus was low ( 3.99 ng / ml , normal laboratory level 4.0 - 19.0 ng / ml ) with an increased alkaline phosphatase level ( 171 ui / l , normal laboratory level 20 - 90 endocrine evaluation showed elevated parathyroid hormone ( pth ) ( 1207.0 pg / dl , normal laboratory 7 - 82 pg / dl ) . an urgent ultrasonography was requested and showed a 47 22 mm nodule in the left thyroid lobe . after two days of intensive medical treatment , the ca levels had not decreased and the clinical situation of the patient remained similar or even worse . because of life - threatening hypercalcaemia and a poor response to medical treatment , she directly underwent surgery after a consensus decision between the endocrinologists and the surgeons . a hemithyroidectomy was performed assuming it was an intrathyroiidal adenoma and without time for performing a fine needle aspiration because of the patient 's situation . during the surgical procedure , a superior parathyroid gland was observed and not resected . at the end of the hemithyroidectomy , the specimen was open and a clear intra - thyroidal adenoma surrounded by thyroid tissue was observed [ figure 1 ] . unfortunately , intraoperative pth and anathomopathological analyse were avaible but were not used according to the intraoperative findings . on the final anatomopathological examination , it had a maximum diameter of 3 cm and the whole specimen weighted 70 g. the postoperative course was uneventful . the patient received doses of calcium iv during the first day and oral intake began at day two . oral calcium was then given to the patient in the postoperative period until the fist visit at the outpatient clinic . during follow - up , the risk of developing this disease increases with a peak incidence between 50 and 60 years , whereas phpt in children and adolescents is a rare condition . parathyroid crisis is characterized by life - threatening hypercalcemia of sudden onset in subjects with phpt . it has been reported that changes in mental status are characteristic features of hypercalcemic crisis . moreover , she had muscle weakness . although neuromuscular manifestations have been described previously , the pathophysiology of these manifestations is not yet clear . moreover , in pc , as a potentially lethal condition , emergent parathyroidectomy has been advocated . certainly , it has been emphasized that expeditive parathyroidectomy is the cornerstone treatment of patients with pc . there is no clear relationship between surgery and the beginning of toxic , urgent hypercalcaemia . in surgical series there is an estimated reported prevalence of intrathyroidal parathyroid adenoma that varies from 1.4 - 6% . according to embryology , parathyroid glands originate from the third and fourth branchial pouches . after this , the glands caudally migrate to their final positions . any problems during this migration can lead to the glands settling in an anomalous location , even in the thyroid tissue . in a recent study , localization of most of the adenomas ( 70.6% ) was shown to be on the right side and on the lower third of the thyroid lobes . this study also reported that intrathyroidal parathyroid adenomas were present in 3% of patients submitted for parathyroidectomy . however , reports of a giant intrathyroidal parathyroid adenoma in the literature are exceptional . it has been noted that the weight of the adenoma correlates with the severity of the pc . gasparri et al . , found that in a patient with a serum calcium level higher than 15 mg / dl , the mean weight of the removed glands was 4.9 g , whereas it was 2.1 g in patients with serum calcium levels lower than 15 mg / dl . nevertheless , giant parathyroid adenomas are rare . , reported that adenomas heavier than 3.5 g accounted for 7.2% of their series of phpt . differential diagnoses , when very high levels of pth are present , must be established with parathyroid carcinoma . parathyroid carcinoma is a rare malignancy with an incidence rate of less than 1% in all patients surgically treated for primary hyperparathyroidism . to the best of our knowledge the clinical manifestations of hyperparathyroidism in parathyroid carcinoma are usually more severe than in patients with parathyroid adenoma . parathyroid crisis is an emergency because high levels of calcium pose a life - threatening situation for patients . . however , changes in mental status are prevalent , hence , the serum calcium level must be determined in any patient with unexplained central nervous dysfunction . once the patient with pc has been stabilized , expedited parathyroidectomy must be performed .

primary hyperparathyroidism ( phpt ) is not an uncommon endocrine disorder . however , acute primary hyperparathyroidism , or parathyroid crisis ( pc ) , is a rare clinical entity characterized by life - threatening hypercalcemia of a sudden onset in patients with phpt . we describe a patient with pc who presented with acute worsening of depressive symptoms , nausea and vomiting , and required emergency surgery . serum calcium , alkaline phosphatase , and parathyroid hormone were elevated and serum phosphorus was low . an emergency hemithyroidectomy was performed because of none medical control of hypercalcemia . a giant intrathyroidal parathyroid adenoma was diagnosed . phtp can be a life - threatening situation for patients , requiring immediate surgical treatment . a giant intrathyroidal parathyroid adenoma is an uncommon cause of pc .

trabeculectomy remains the surgery of choice in patients with advanced glaucomatous optic neuropathy ( gon ) . in conventional trabeculectomy , efforts have been made to improve the outcomes and reduce the complications of trabeculectomy . in this regard , the ex - press glaucoma implant was introduced as a nonvalved miniature stainless steel device , designed with the intent of offering a simple and safe alternative to classic trabeculectomy , obviating the need for tissue excision or removal . express is easier than but not superior to trabeculectomy ; the problem is , that it is a very expensive alternative which the majority of patients in developing countries can not afford . therefore , we tried to use a low cost alternative which is universally available and can function as an alternative to classic trabeculectomy without the need for tissue excision or removal . herein , we describe an innovative , simple and cost effective technique using a 24 gauge ( 24 g ) polytetrafluoroethylene ptfe ) intravenous cannula ( ivc ) as an alternative to the classic trabeculectomy . the first patient was a 50-year - old man with intraocular pressure ( iop ) of 16 mmhg and 32 mmhg on maximal tolerable medical therapy in his right and left eyes , respectively . best corrected visual acuity ( bcva ) was 20/20 and light perception in his right and left eyes . the second patient was a 72-year - old male subject with iop of 12 mmhg and 38 mmhg on four topical ocular hypotensive medications , in his right and left eyes , repectively . bcva of the second patient was 20/20 and no light perception in the right and left eyes , respectively . both patients underwent glaucoma filtering surgery using a 24 g intravenous cannula ( ivc ) together with application of 0.1 mg / ml mitomycin - c subconjunctivally for 3 min under peri - bulbar anaesthesia . we specifically selected these end - stage patients due to unknown long - term results and complications of the surgery . an 8 - 0 polyglactin ( vicryl ) traction suture on a spatulated needle was placed through the superficial cornea near the superior limbus . consequently , a standard fornix - based conjunctival incision was made to gain exposure to the scleral bed adjacent to the limbus . gentle cautery was performed in this area . a limbus - based rectangular scleral flap ( 4 mm 4 mm ) of about half scleral thickness mitomycin - c ( mmc ) 0.1 mg / ml soaked pledgets were applied subconjunctivally for 3 min after coating the cornea with viscoelastic . a rectangle of deep sclera ( deep scleral groove ) measuring 1 mm 4 mm was made perpendicular to the limbus , leaving a thin layer of deep sclera covering the choroids [ figure 1 ] . a 24 g ivc with an internal diameter of 0.7 mm ( bd neoflon iv cannula , becton dickinson , helsingborg , sweden ) with bevel of the trocar facing up , was inserted into the anterior chamber ( ac ) at the limbal end of the deep scleral groove and at the center of the blue line , parallel to the iris plane and directed towards the pupil [ figure 2 ] . the trocar was completely withdrawn when the cannula was 1 mm inside the ac and the ptfe cannula was cut 4 mm from the site of insertion . the tube was then secured in the preformed deep scleral groove using 10 - 0 nylon sutures [ figure 3 ] . the function of the tube was ensured by noting the dilution of trypan blue dye applied over the cut end of the cannula . the scleral flap was sutured using four 10 - 0 nylon sutures and the conjunctiva was repaired meticulously with 8 - 0 polyglactin sutures . intra - operative photograph showing the 24 g cannula inserted into the anterior chamber ( ac ) through the centre of the blue line at an angle parallel to the iris plane and directed towards the pupil . intra - operative photograph showing the 24 g cannula placed in the preformed deep scleral groove , secured to the scleral bed using 10 - 0 nylon sutures . postoperatively , iop of the first patient at 3 , 6 and 9 months ranged from 12 mmhg to 15 mmhg with a well formed anterior chamber and a functioning diffuse bleb . gonioscopy and anterior segment oct showed the presence of the tube [ figure 4 ] . iop of the second patient at 6 weeks and 3 months ranged from 12 to 14 mmhg . anterior segment oct at 9 months follow - up , showing the presence of the tube ( 24 g cannula ) in situ . however , some of the problems associated with trabeculectomy are attributed to the need for tissue excision and lack of standardization of filtration . new drainage devices such as the express shunt are being used in an attempt to standardize the technique . the restricted internal diameter of these devices provides certain consistency and standardization of the filtration procedure and flow regulation is largely governed by the sutures applied to close the scleral flap . tissue excision or removal is not required , thereby reducing inflammation and complications associated with trabeculectomy . the major disadvantage of these devices is the added cost of surgery , especially in developing countries like ours . therefore , we present the use of an inexpensive and widely available 24 g intravenous cannula made of ptfe which is known to entail no toxicity in humans to date . the cannula functions by diverting aqueous humor through the implant from the ac to the intrascleral and subconjunctival spaces , similar to the express device , and offers the advantages of standardization of filtration without the need for any tissue excision , unlike in trabeculectomy where a sclerostomy and an iridectomy are mandatory . our technique is a simple modification of conventional trabeculectomy aimed at making the surgery simpler and reducing immediate postoperative complications by using a small ostium without any need for iridectomy . we have introduced a low cost innovation which will help standardize surgery , make it easier and also decrease its learning curve ; this procedure also avoids the need for a sclerostomy or iridectomy thereby minimizing tissue injury . in summary , the use of a 24 g intravenous cannula is a cost effective and simple alternative to glaucoma drainage devices in developing countries like ours , alongwith decreased learning curve of the procedure . we recommend further studies to evaluate the long - term safety and efficacy of this technique .

we describe an innovative technique for performing standardized low cost glaucoma filtration surgery using a polytetrafluoroethylene ( ptfe ) intravenous cannula . the trocar of a 24 gauge ( 24 g ) ptfe intravenous cannula was used to create a trabeculectomy ostium and its tube was inserted under a partial thickness scleral flap in 2 patients with advanced glaucomatous optic neuropathy , in whom intraocular pressure ( iop ) was not controlled on maximal tolerable hypotensive therapy . postoperatively , iop of the operated eyes at 3 , 6 and 9 months follow - up ranged from 12 to 15 mmhg with a well formed anterior chamber and a diffuse bleb .

the spectrum of chronic hbv infection ranges from asymptomatic infection to chronic hepatitis with progression to cirrhosis , end - stage liver disease and hepatocellular carcinoma . a 30-year - old man presented with recent hypertension , severe headache and lethargy on a background of recently diagnosed hbv infection . urine microalbumin / creatinine ratio was 31.6 mg / mmol of creatinine ( < 2.5 mg / mmol ) with a urine protein excretion of 350 mg / day . other abnormal investigations included alanine transaminase 108 u / l ( 5 - 55 ) , aspartate transaminase 76 u / l ( 5 - 55 ) and erythrocyte sedimentation rate 46 mm / h ( 030 mm / h ) . hbsag and hepatitis b e antigen ( hbeag ) were positive , while igm hepatitis b core antibody was negative . hepatitis b viral dna was quantitated at 364 million iu / ml . electrophoresis of serum and urine were negative for paraprotein . antinuclear antibody , antineutrophil cytoplasmic antibody , extractable nuclear antigen , complements and anti - double - stranded dna were negative . three months after diagnosis , he continued to have high viral load suggesting a chronic hbv infection . mol / l with an estimated glomerular filtration rate ( egfr ) of 57 ml / mt . glomeruli were normal on light microscopy with evidence of tubulointerstitial nephritis ( figure 1a ) . there was interstitial oedema and dense mononuclear cell infiltrate comprising lymphocytes , plasma cells and occasional eosinophils . trace deposits of granular iga ( < 1 + ) were seen on immunofluorescence microscopy . nuclear abnormalities in the form of condensed chromatin and round structures with a dense core surrounded by a clear halo were observed in some of the tubules ( figure 1b and c ) . ( a ) light microscopy view from the first renal biopsy showing degeneration of tubular epithelium ( arrows ) and associated interstitial nephritis ( lhs of image ) 20 ; ( b ) electron microscopy showing necrosis of tubular epithelium cells ( asterisk ) , ( c ) virus - like particles in the nucleus of a tubular epithelial cell . tubulo interstitial nephritis ( tin ) was attributed to chronic hbv infection , and he was initiated on entecavir 0.5 mg twice a day . although the viral dna load decreased significantly over the next 4 weeks ( 429 copies / ml , 58 iu / ml ) , renal function deteriorated ( serum creatinine rose 210 mol / l egfr 35 ml / mt ) . on a repeat renal biopsy , nine months after initiation of entecavir , egfr improved to 45 ml / mt ( serum creatinine 165 extra hepatic manifestations associated with chronic hbv infection can be varied with renal , skin , joint and nervous system manifestations . the renal manifestations of chronic hbv infection usually include glomerulonephritis , essential mixed cryoglobulinaemia and polyarteritis nodosa ( pan ) . the most characteristic presentation is nephrotic syndrome , with membranous nephropathy being the most common pathological finding . children have a good prognosis with up to 95% achieving clinical resolution within 7 years . adults , in contrast , may have a relentless course with 30% progressing to renal failure and 10% eventually requiring haemodialysis . pan is a rare complication of chronic hbv infection , occurring in 1% to 5% of patients . human immunodeficiency virus , cytomegalovirus , epstein barr virus , hanta virus , polyoma and adenovirus are some of the viruses associated with tin . we are not aware of any published report of hbv associated tin in the english literature . the renal biopsy findings of tin in our patient were quite unexpected as the usual renal manifestation of hbv nephritis is glomerular . however , the electron microscopic findings of viral structures akin to hbv along with positive immunohistochemical staining for hbv antigen in the tubules , and a progressively worsening renal function with increasing viral load further strengthened our belief of hbv infection as the cause of tin . although the majority of patients spontaneously clear the viraemia , active treatment was initiated considering progressive renal impairment . entecavir , a potent nucleoside analogue with low risk of antiviral resistance , was chosen , as lamivudine and interferon were considered to be less effective in the presence of very high viral load . improvement of egfr with prolonged entecavir treatment further supports our view that hbv infection is the cause of tin in this patient . an interplay of host , virus and other environmental factors is necessary for nephropathy to develop . this is supported by the observation that only few patients with chronic hbv infection have renal disease . much of the available evidence points to a direct cytopathic effect by virus , immune complex deposition , viral induced cytokine injury or a specific immunological effector mechanism induced by hbv as probable explanations for renal injury . in summary , we conclude that , in our patient , tin was associated with chronic hbv infection . we suggest that tin should be considered as a possible cause of renal impairment in hbv - infected patients . our case exemplifies that aggressive antiviral treatment in such patients would stabilize and improve renal function . the exact pathogenesis of tin in association with hbv infection needs to be elucidated by future experimental work and further clinical observations .

hepatitis b viral infection is usually a self - limiting disease in immunocompetent individuals . chronic infection can be seen in up to 5% of infected patients . renal manifestations of chronic hbv infection are usually glomerular . we describe here an uncommon presentation of a patient with chronic hbv infection with very high viral load and rapidly progressive renal failure . renal biopsy showed features of tubulointerstitial nephritis and tubular epithelial inclusion bodies suggestive of hbv infection . entecavir treatment slowed down the progression of his renal disease . tubulointerstitial nephritis should be considered as a part of the differential diagnosis in patients with hbv infection . early antiviral treatment may halt the progression of renal disease .

the art of body piercing is an age old process for the people in the developed countries . however , nowadays , it has evolved as a part of their fashion process and the ear being the most common body part pierced . although it is a routinely performed procedure , it is not without complications such as oedema , haematoma , infection and keloid formation . most commonly used in dental surgery , the laser scalpel is nowadays used almost in all procedures due to its safety , precision , ease of use , haemostasis , less post - operative pain and oedema and lesser scarring . in this article , we would like to describe a novel method of laser ear piercing using the diode laser . an 18-year - old female patient visited the plastic surgery outpatient department , with a desire to get an ear piercing . the patient already had pierced ear lobule and wanted a second piercing above the first one . after routine blood investigations had been done , the procedure was carried out in the department laser operation theatre . the chosen site for piercing was marked , and non - contact cooling was applied . after ensuring adequate cooling and local anaesthesia , doc medica diode laser ( doc medica srl , corso casale , torino , italy ) with a power of 2.0 w in continuous mode was used to make a tract from the anterior to posterior direction [ figure 1 ] . once the tract was made , a gold stud was introduced through the tract [ figures 24 ] . the same procedure was repeated on the opposite side . applying diode laser and making the tract from anterior to posterior direction golden ear studs being inserted through the tract with accuracy and hemostasis screw is tightened to the stud with proper visibility ear stud in place , at the exact place marked pre - operatively intraoperatively , it was noticed that there was very minimal bleeding . the probe was almost the same size as the earring stud , and the long tip was comfortably tunnelled to the other side . there was no need for any traction to the ear lobule and the anatomy was maintained , so the direction of the tunnel was made in the desired way . the patient was advised to apply an antibiotic ointment at the site of piercing every day for 1 week . the wire technique which necessitates serial dilatation of the tract until the suitably sized ear stud can be placed is a painful process . piercing guns used very commonly among jewellers did not gain much acceptance among doctors due to the higher incidence of infection . the most common technique used is the railroading method , wherein an 18-gauge needle is railroaded over a 26-gauge needle over which the tip of the earring is guided through . a newer method of ear lobe piercing was described by lamba and gupta , in which an 18-gauge bd insyte - w intravenous catheter was used for piercing . the co2 laser has been used for ear piercing by chang et al . in 2010 . the electric current is the pump source which produces photons which are conducted through a laser active medium . it is cheap , small in size and has a long flexible fibre cable which makes the diode laser scalpel easy to use . the use of diode laser for ear piercing has many significant advantages over the conventional techniques . the procedure can be carried out with topical local anaesthesia combined with various pre - cooling methods used in conventional laser therapy such as cold gel application and cryospray application . this avoids the need for an injection before the procedure and can be useful in children . the laser scalpel instantly ablates the tissues it cuts causing excellent haemostasis . in the ear , where there is rich blood supply and bleeding is a common problem , the use of laser scalpel for piercing holds this excellent advantage . this causes very minimal chances of post - operative perichondritis or wound infection reducing the chance for keloid formation . studies have shown that the use of piercing gun or 16-gauge cannula for piercing cause stripping of the perichondrium from the cartilage which is more at the exit site . this damage to the relatively avascular cartilage makes the ear more prone to infection . as the diode laser probe is precise , causing less tissue injury the chance of damage to cartilage is reduced greatly and hence the chance of post - procedure keloid formation is also reduced . chang et al . conducted a study in 2010 , comparing co2 laser and spring loaded gun for ear piercing . the study concluded that though there was pain intraoperatively when the laser was used for piercing , the post - operative pain was significantly lesser than when spring loaded gun was used . we have noticed that tip of the diode laser is almost of the same size of the earring studs and its penetrating property with minimal coagulation will be more ideal for the purpose . after the procedure , it was found to have numerous advantages compared to the conventional methods and the co2 laser method . the diode laser is a fast , safe , easy to use and highly effective way of ear piercing . the advantages we noticed while using the diode laser over conventional methods were more precision , minimal trauma with less chances of hypertrophy and keloids , no bleeding with coagulation effect of laser , less time taken compared to conventional method and less chance of infection due to thermal heat effect of laser . thus , we recommend its use for all ear piercings . there are no conflicts of interest .

earlobe piercing is a common office room procedure done by a plastic surgeon . various methods of ear piercing have been described . in this article , we describe a novel method of laser ear piercing using the diode laser . an 18-year - old female patient underwent an ear piercing using a diode laser with a power of 2.0 w in continuous mode after topical local anaesthetic and pre - cooling . the diode laser was fast , safe , easy to use and highly effective way of ear piercing . the advantages we noticed while using the diode laser over conventional methods were more precision , minimal trauma with less chances of hypertrophy and keloids , no bleeding with coagulation effect of laser , less time taken compared to conventional method and less chance of infection due to thermal heat effect of laser .

pneumatosis intestinalis ( pi ) is a rare and unexpected disorder defined as accumulation of gas in the submucosa or subserosa of the large or small intestine . it is associated with a variety of diseases , and its pathogenesis is still obscure . , it has been reported in 12 cases after pediatric orthotopic lt [ 1 - 5 ] , although nearly none has been reported in adult liver recipients . we report three cases of adult liver transplant recipients in whom the diagnosis of pi was made by computer tomography ( ct ) and simple x - ray ( table 1 ) . the patients in case 1 and 2 were unremarkable during post - operative period , and they were discharged home . the patient in case 1 experienced high fever and watery diarrhea after two months and the patient in case 2 complained of watery diarrhea after four months . the patient in case 3 experienced high fever and watery diarrhea one month after the transplantation without discharge . at admission , all patients were stable in blood pressure , pulse rate , and respiratory rate , but in case 1 and 3 , patients had high body temperature ( 38.8 and 38.5 ) . the physical examination showed severely distended , non - tender abdomen , with hypoactive bowel sound and tympanic percussion in case 1 . the laboratory findings of all cases did not reveal abnormal findings except mildly elevated c - reactive protein , ranging 0.38 to 1.59 mg / dl ( normal range , < 0.3 mg / dl ) . serial blood tests of case 1 were positive twice for cytomegalovirus ( cmv ) antigenemia , but other cases showed all negative results . the peak titer of cmv antigenemia was 10/400,000 and colonoscopic finding did not show abnormal finding in case 1 . the cytotoxicity assay of the all patient 's stool was negative for clostridium difficile toxin . stool cultures and special stains for micro - organisms were all negative in all patients . abdomen simple x - rays and ct in abdomen revealed diffuse pneumatosis intestinalis of the colon , ileus , and free air under diaphragm , but no air in the portal vein in case 1 and 2 ( fig . 1 ) . an abdominal ct of case 3 demonstrated pneumopericardium , diffuse linear pneumatosis intestinalis of the colon and extraluminal air within retroperitoneum and mesentery but no free intraperitoneal air ( figs . 2 , 3 ) . all patients were conservatively managed with bowel rest , parenteral nutritional support , intravenous antibiotics ( cefotaxime , ampicillin / sulbactam , and metronidazole ) because of no signs of peritonitis . the patients in case 1 was treated with additional antiviral agent ( ganciclovir ) for positive cmv antigenemia and nasogastric tube decompression for free air , but other patients did not receive antiviral agent and did not have nasogastric tube decompression . the patients in case 1 and 3 did not receive immunosuppressive agent for 1 week , but the patient in case 2 received reduced immunosuppressive treatment with fk506 ( 2 mg daily ) , methylprednisolone ( 2 mg daily ) , and mycophenolate mofetil ( 500 mg daily ) . he remained in excellent general condition from the admission and had a sense of well - being during hospitalization . the abdominal distension of case 1 resolved and diarrhea improved within a week and antibiotics were discontinued . all patients were given a diet , and were discharged home after the pi resolved clinically and radiographically . the common factor linking pi to organ transplant recipients is steroid treatment . in this report , one patient did not receive steroid treatment and the other two had steroid treatment but the amount was only 4 mg / day . drug concentrations of fk506 and mycophenolate mofetil ( mmf ) in all patients have low levels as well . it might be possible that immunosuppression including fk506 , steroid , and mmf did not cause of pi in our cases . symptoms that have been attributed to pi are in decreasing order of frequency : diarrhea , bloody stool , abdominal pain , abdominal distension , constipation , weight loss , and tenesmus . in this report , three recipients had watery diarrhea , and two had high fever . the physical examination of recipients did not show specific findings except abdominal distension in one case . the patients did not have abdominal pain , tenderness , rebound tenderness , and rigidity of abdominal wall . there was no clear evidence of peritonitis despite of pneumoperitoneum and air density in mesentery and retroperitoneum in simple x - rays and ct scan of abdomen . we assumed that there was no direct communication between lumen and peritoneum , therefore conservative treatment should be performed rather than surgical exploration . however , patients with white blood cells of more than 12,000/mm with or without the symptoms of clinical obstruction such as emesis , vomiting , and pain , age over 60 years were considered to be candidates for surgical intervention . based on this report , pi should be considered as the differential diagnosis of adult after lt who suffers from watery diarrhea and fever . pneumoperitoneum , air - density in mesentery and retroperitoneum in patients with pneumatosis intestinalis without signs of peritonitis improved with conservative management .

pneumatosis intestinalis is an uncommon disorder characterized by an accumulation of gas in the bowel wall . we described three cases undertaking liver transplantation . the patients developed diarrhea in three cases and high fever in two . an abdominal x - ray and computed tomography scan demonstrated extensive pneumatosis intestinalis in the colon with pneumoperitoneum mimicking hollow organ perforation . however , the patients had no abdominal symptoms and there was no evidence of peritonitis . the infection work - up was negative except one case with cytomegalovirus antigenemia . after one week of conservative management including bowel rest and antibiotic therapy , their pneumoperitoneum resolved spontaneously without any complication . pneumatosis intestinalis should be considered as a differential diagnosis after adult liver transplantation with patients suffering from watery diarrhea and fever . pneumoperitoneum , air - density in mesentery and retroperitoneum in patients with pneumatosis intestinalis without signs of peritonitis improved with conservative management , which included bowel rest and antibiotic therapy .

an 88-year - old woman with a history of hypertension , atrial fibrillation , and unstable angina suddenly developed left - side motor weakness and right - side eyeball deviation . her diagnosis was right middle cerebral artery ( mca ) infarction and she was given tissue plasminogen activator ( tpa ) . seven days later , she suddenly developed dyspnea and unstable vital signs : blood pressure 80/40 mmhg , heart rate 150 beats / min , respiration 44 breaths / min , body temperature 39.1 , and o2 saturation 94% . under the impression of septic shock , she was intubated and left subclavian venous catheterization was attempted because of the need for continued fluid management . then , the left subclavian vein was identified after several attempts using an infraclavicular approach . a seldinger - type central venous catheter set was used ( two - lumen central venous catheterization set with blue flextip , arrow , usa ) . the guidewire was passed through the introducer needle , which had been placed with venous return . minimal resistance was encountered during guidewire insertion , and the guidewire was advanced approximately 30 cm . at that point , the guidewire could be advanced no further and subsequent attempts to withdraw the guidewire were failed despite moderate force . a chest x - ray showed that the guidewire was knotted and kinked and the patient was transferred to our hospital for surgical exploration . after admission to our hospital , a portable chest x - ray and subclavian venogram were performed for further evaluation . the x - ray revealed that the guidewire was knotted and kinked in the area of the middle third of the left clavicle and that part of it had folded back on itself completely and entered the mediastinum ( fig . the subclavian venogram revealed that the guidewire had not perforated the subclavian vein and was knotted , kinked , and extended extravascularly . 2 ) . following discussion with orthopedic and chest surgeons , surgical exploration under general anesthesia was decided . in the operating room , the patient 's initial vital signs were blood pressure 100/50 mmhg , heart rate 134 beats / min , respiration 25 breaths / min , and o2 saturation 96% . although noninvasive blood pressure ( nibp ) cuff had been applied on the right arm , an arterial catheter was placed in the left radial artery to continuously check the blood pressure and to promptly detect and monitor the blood pressure changes in case of the damage to the left subclavian artery during operation . and a 7-cm incision was made just medial to the entry point of the guidewire , parallel to langer 's line . the tangled part of the wire was kinked between the sternocleidomastoid muscles and the vessels were not damaged . we cut the wire to untangle it , and pulled out the wire that had entered the mediastinum . the entire guidewire was removed successfully , as confirmed under an image intensifier in the operating field . postoperatively , the patient was transferred to the surgical intensive care unit , from which she was discharged to a ward the next day without further complications . we experienced a case of left subclavian venous catheterization complicated by extravascular knotting , kinking , and entrapment of the guidewire . while some studies have reported on intravascular knotting or looping of guidewires [ 6 - 9 ] , a few cases of extravascular knotting , kinking , and entrapment of guidewires have been reported internationally and we believe that our case is the first domestic report . wang and einarsson experienced a similar case , but on surgical cutdown , the wire was discovered to perforate the proximal subclavian vein and knotted behind the clavicle . their patient had a history of previous subclavian catheterization thus they postulated that damage to the vein from the previous catheterizations predisposed the vessel to perforation by the guidewire . in another similar case , surgical cutdown revealed that the wire had passed through a nearly occluded subclavian vein and into the anterior scalene muscle , where fibrous tissue redirected the wire upward and into a loop . that patient also had a history of five previous subclavian catheterizations . in our case , the patient had no history of subclavian catheterization and surgical exploration which means that the vessels were not perforated or damaged . the guidewire was located just beneath the subclavian vein and the tangled part of the wire was kinked between the sternocleidomastoid muscles . furthermore , the first 12 cm of the guidewire from the j - tip was doubled back on itself and extended 6 cm into the mediastinum ; no damage occurred to the j - tip . from these facts , we inferred that the guidewire was initially inserted extravascularly , although the introducer needle had been placed with venous return . the intact subclavian vein and venogram findings supported this supposition . when the j - tip guidewire was inserted into the extravascular connective tissue area , it had folded into a u - shape , which then folded completely upon itself into an i - shape . finally , this i - shaped wire passed through the connective tissues between the vessel and muscle into the mediastinum , while the rest of the wire , which was under continuous force , became kinked , knotted , and entrapped in the middle clavicular area . this likely resulted from the connective tissue around the subclavian vein and attachment of the vein to the surrounding structures . anatomically , the path of the subclavian vein is not straight , as it loops over the first rib to descend into the superior mediastinum . furthermore , a bottleneck exists between the clavicle and first rib , which can impede the anterograde threading of the wire and might contribute to looping and knotting . in our case , consequently , we emphasize that a guidewire should not be advanced if any resistance is encountered , as originally pointed out by seldinger . guidewires are not very rigid structures and if any force is applied they are likely to kink , moreover , further application of force after kinking might result in knot formation . if we pull out the knotted guidewire back , the guidewire might be entrapped . to avoid these complications , if there is any resistance , guidewire should not be advanced and resistance encountered during insertion of the guidewire , it is necessary to check the chest x - ray to determine the position of the guidewire instead of pulling it back . our case shows the consequences of inserting a guidewire despite of minimal resistance and pulling it backward when the guidewire is not advancing . in conclusion , initially , the guidewire was passed through extravascular connective tissue and then the wire became knotted , kinked , and entrapped extravascularly . physicians should be aware of these rare potential complications when a guidewire is advanced if any resistance is encountered .

various complications of central venous catheterization have been reported , some of which are well - known , while others are described as a sporadic events . we experienced a case of left subclavian venous catheterization complicated by extravascular knotting , kinking , and entrapment of the guidewire and the guidewire was removed surgically . although minimal resistance was encountered during guidewire insertion , the guidewire was advanced approximately 30 cm . physicians should be aware of these rare potential complications when a guidewire is advanced if any resistance is encountered .