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the diagnosis of mental retardation was based on diagnostic and statistical manual of mental disorders ( dsm)-4 diagnostic criteria and the intelligence quotient was assessed using binet - kamat method by the accompanying psychiatrist . the team consisting of an ophthalmologist and a postgraduate in the senior residency examined all the students . age , sex , residential address , antenatal and perinatal history , ocular complaints , and previous refractive error were noted . visual acuity was tested on snellen 's chart in the language preferred by the student . c-chart was used for children who were not able to read but can interpret the symbol . routine ocular examination was done on torch light in all students , ocular motility was tested using alternate cover uncover test , cycloplegic refraction , and ophthalmoscopy was performed in the indicated students . glasses were prescribed to all children having visual acuity less than 20/30 according to the retinoscopy . uncooperative and students needing special examination were referred to the hospital for the detailed examination . the data were entered in an excel sheet ( spss software , 13.0 , spss inc . fisher 's exact was used to study the strength of association between ocular problems and mental retardation . an association between the common ocular problems encountered in the study and the severity of mental retardation was studied by chi - square test for the linear trend . a p value less than 0.005 was considered significant . a total of 241 school children ( 6 - 16 years of age ) with mental retardation in special education schools were examined . eighty - one students had mild mental retardation and 32 ( 39.51% ) students had ocular problems . one hundred and fifteen students had moderate mental retardation ( 47.72% ) with ocular problems seen in 61 students ( 53.04% ) . students having severe mental retardation had more ocular problems ( 29 out of 42 ( 69.05% ) ) . the distribution of iq scores along with the number of students having ocular problems is presented in table 1 . refractive error ( 49.18% ) and strabismus 11 ( 18.03% ) were common ocular problems seen in moderate grade of mental retardation . strabismus ( 24.14% ) , nystagmus ( 6.90% ) , and corneal dystrophy ( 6.90% ) were common ocular problems seen in severe mental retardation . two children had ocular problems , one child had optic atrophy and other had anophthalmos . distribution of ocular disorder in children with learning disability myopia was a common refractive error seen in 30 ( 60% ) students , followed by hypermetropia ( n=10 , 20% ) , and astigmatism ( n=10 , 20% ) . the number of students having ocular problems in profound mental retardation was small in number ( 2/3 ) . significant statistical association was found between the severity of mental disability and ocular morbidities ( p=0.00144 ) . the risk of getting ocular morbidities among the severe and profound mentally retarded children was more as compared to mild mental retardation group ( odds ratio=3.39 ) . however , no association was seen between the level of mental retardation refractive error ( p=0.74 ) and the strabismus ( p=0.265 ) . association between severity of mental retardation and ocular morbidity a stormy perinatal history was seen in 55% children . the highlight of the study was the significant correlation between severity of the mental disability and the ocular morbidity suggesting high risk of getting ocular problems in severely mentally retarded children . however , no correlation was seen between the level of mental retardation refractive error and strabismus . this could be due to equal distribution of refractive error in mild and moderate grade of mental disability and less student having refractive error in the severe grade of mental retardation . they have found out significant correlation between strabismus with the increased level of mental disability . eighteen children ( 18/25 ( 72% ) ) had onset of squint during the first 6 months of life . this could be related to the stormy perinatal history , which was seen in 55% of the children . this was the first type of study in the region with sufficiently large sample size . the data could be useful to the governmental agency to plan and implement the national program in the region . however , children in the grade of moderate and severe mental retardation took more time for the examination . the draw back of the study is that , only children admitted to the special school for mental retardation were examined . we suggest visual function of the children should be assessed as soon as the child is admitted to the school . earlier assessment and correction of the visual problems will have greater chance of achieving potential and will prevent unnecessary visual impairment .
background : ocular problems are common in mentally retarded children . due to population growth these problems are increasing . prevalence rate is variable from region to region . data on ocular problems in mentally retarded school children is lacking in this region.aim:the aim of the present study was to identify the ocular disorders in children with mental retardation attending special schools in a district and to study their relationship with the degree of retardation.materials and methods : a total of 241 mentally retarded school children in the age group of 6 - 16 years attending special schools for the mentally retarded children in a district in central india were examined by a team of ophthalmologist , psychiatrist , and a resident in ophthalmology department of a medical college . complete ocular examination was done . ocular problems were identified and categorized according to the intelligent quotient.results:one hundred and twenty four children ( 51.45% ) had ocular problems . strabismus ( 10.37% ) and refractive error ( 20.75% ) were the common ocular problems seen in this study . an association was found between the severity of mental retardation and ocular problems ( p<0.005 ) . however , no association was seen between the severity of mental retardation and strabismus and refractive error.conclusion:a high prevalence of ocular problems was seen in mentally retarded school children . children with mental retardation should undergo annual ophthalmological check up . early detection and correction of ocular problems will prevent visual impairment in future .
while the exact mechanisms behind the relationship between elevated soluble vascular endothelial cell growth factor receptor-1 ( svegfr-1 ) levels and clinical outcome remain controversial , the finding in the current study by yang and colleagues that svegfr-1 is a promising sepsis biomarker is quite consistent with previous studies . in fact , in a recent investigation , we demonstrated that svegfr-1 performed with a diagnostic accuracy equal to , or exceeding , that of the commonly used sepsis biomarkers interleukin-6 and serum lactate . interestingly , preclinical studies have demonstrated that sepsis induces elevated levels of vascular endothelial cell growth factor ( vegf ) , as well as elevated levels of svegfr-1 . these studies have shown that vegf exacerbates sepsis and mediates morbidity and mortality , while it is thought that circulating svegfr- 1 binds and neutralizes the adverse / pro - inflammatory effects of vegf . this hypothesis is supported by the observation that supratherapeutic levels of svegfr-1 in murine models of sepsis deplete the blood of free vegf and protect against morbidity and mortality . if svegfr-1 is protective , then why are elevated circulating levels of svegfr- 1 associated with worse clinical outcome in sepsis ? the apparent dichotomy may be explained by one or more of the following hypotheses : 1 , svegfr-1 levels simply reflect the vigor of the anti - inflammatory response in sepsis ( akin to policeman at the scene of a crime - likely the more policeman , the worse the crime ) ; 2 , elevated svegfr-1 may cause profound immune depression by interfering with svegfr-1-mediated signaling in monocytes ; and 3 , svegfr-1 may interfere with endothelial repair by inhibiting vegf signaling in endothelial cells . as yang and colleagues point out , the existing literature concerning circulating vegf levels in sepsis is controversial . previous studies have shown that adverse outcomes in sepsis are associated with either high or low vegf levels . in the current study , vegf levels did not predict adverse clinical outcomes . however , it should be noted that the investigation used total vegf levels , which is different than measuring physiologically active free or unbound vegf . however , given the biological plausibility of a pathogenic role for vegf in sepsis pathophysiology ( by virtue of its pro - inflammatory , permeability - promoting and procoagulant effects at the level of the endothelium ) we believe that the vegf signaling axis remains a viable therapeutic target . to test this hypothesis , we are currently investigating the use of an anti - vegf antibody in patients with septic shock clinicaltrials.gov identifier nct01063010 ) . finally , we submit that while the exact mechanisms governing the observed association between urokinase - type plasminogen activator ( upa ) and outcomes in this study is unknown , the presence of this association underscores the importance and dynamic role of the endothelium in sepsis . the conversion of plasminogen to plasmin , which is primarily governed by upa , occurs at the level of the endothelium and is a primary regulator of the hemostatic balance in sepsis . as the evidence for a central role of the endothelium in sepsis builds , it is likely that a number of other endothelial - related biomarkers will emerge that reflect a ' broken endothelium ' . perhaps one or more of these markers can be leveraged in future trials to guide therapy with novel yet - to - be - determined endothelium - sparing agents . svegfr-1 : soluble vascular endothelial cell growth factor receptor-1 ; upa : urokinase - type plasminogen activator ; vegf : vascular endothelial cell growth factor .
the study by yang and colleagues examined 81 patients with septic shock due to pneumonia , along with 20 patients with pneumonia without organ dysfunction . their major findings were that circulating levels of soluble vascular endothelial cell growth factor receptor-1 ( svegfr-1 ) and urokinase - type plasminogen activator ( upa ) were associated with organ dysfunction and mortality , whereas vascular endothelial cell growth factor ( vegf ) levels had no such predictive power . yang and colleagues are to be complimented for a well - conducted study of a reasonably ( and helpfully ! ) homogeneous population of patients with sepsis that carefully and comprehensively analyzed the relationship between svegfr-1 , upa , vegf and clinical outcome . the study serves not only to provide evidence in support of new diagnostic biomarker targets in sepsis , but also to augment the growing evidence of an important role of the endothelium in sepsis in general , and the vegf signaling axis in particular .
epidemiology , clinical spectrum and outcome of the staphylococcal bacteremia / sepsis has been modified for years by different factors . in the era of antibiotic clinical course and outcome of this disease various studies have proven that the percentage of mortality is still up to 35% in some countries and it is associated with the acute renal failure , respiratory distress , shock and endocarditis ( 1 , 2 ) . considering the fact that the staphylococcus aureus bacteremia / sepsis is one of the most serious bacterial infection around the world , its importance is well recognized in developed countries and during the past years much effort was made in monitoring and prevention , especially infections caused by meticillin resistent strains ( 3 , 4 ) . the study included 87 patients , out of whom 57,5% ( 50 ) were men and 42.5% ( 37 ) women , age less than one to eighty years . all patients were hospitalized in the clinic for infectious diseases of the clinical center of the university of sarajevo during the period from 01.01.2000 . to 01.10.2011 . in a community acquired sepsis without comorbidity were 22 patients , with one were 19 , and with two 4 patients . in the study group of patients with hospital originated sepsis without comorbidity were 8 patients , with one comorbidity 5 , with two 4 and per 1 patients with four and five pre - existing diseases . the results of the analyzed group of patients with bacteremia showed that the group with outpatient originated infection have a slightly larger number of patients ( 6 ) with one comorbidity compared to patients without comorbidity ( 4 ) , while in the hospital occurred bacteremia the largest number of patients ( 4 ) had two comorbidity and ( 2 ) patients one and three , while ( 2 ) patients had no comorbidity . comorbidity related to disease and conditions specified in the table , which existed together with infection s. aureus / s . epidermidis or it is preceded , was registered in 51 of 87 patients , provided that for some patients at the same time noted two or more diseases / conditions . from the data in the table can be seen that the different diseases or conditions are variously associated with the investigated subcategories ( bacteremia / sepsis , hospital / outpatient infection , different strains of s.aureus/s.epidermidis ) . so for all the investigated comorbidities more frequent was staphylococcal bacteremia rather than staphylococcal sepsis . outpatient staphylococcal infections are more common in diabetes mellitus , neurological diseases malignant disease , and hospital infection are represented by the previous antibiotic , corticosteroid and chemo therapy , pressure ulcers , and different implants , while the number of patients for the other comorbidities more or less equally distributed between subcategories of hospital and outpatient infections . in all comorbidity most commonly was isolated s. aureus primarily strain mssa followed by mrsa strain which is more frequent in patients who were surgically treated ( comorbidity - various implants ) . staphylococci primarily the s. aureus and s. epidermidis , are one of the most virulent human pathogens with the possibility of colonization and infection in hospitalized patients with compromised defense capabilities or without , as well as healthy immunocompetent persons in the outpatient setting . in individuals with pre - existing diseases is always an increased risk for occurrence of infections because of damaged immune system , different drug therapies , exposure to diagnostic and therapeutic procedure and more frequent hospitalizations ( 5,6 ) . studies yamada and colleagues ( 7 ) and kempkera and colleagues ( 8) show that the most common comorbidity associated with staphylococcal infections are malignant diseases , cardiovascular diseases , diabetes mellitus , immunosuppression and in recent years hiv infection . in a study laupland and colleagues ( 9 ) the greatest risk of developing staphylococcal infections were registered in patients on hemodialysis , with transplanted organs , hiv infection , cancer and diabetes . in our study , 58,6% patients have a comorbidity , which corresponds with data from the literature where the staphylococcal infection is usually expected in patients with comorbidity . in the majority of patients 36,8% was found one pre - existing disease . the most common comorbidity registered in our study are consistent with those from the literature . following methicillin sensitivity was noted that meticillin sensitive strains were represented in the higher percentage compared to meticillin resistant strains which does not mean that the methicillin - sensitive strains are more pathogen but because in our study was greater representation of this strain in the total number of tested samples . the results show that patients with comorbidity have a higher prevalence to become sick with the bacteremia / sepsis , especially in hospital settings which suggests the importance of being mindful of the staphylococcal etiology of the bacteremia / sepsis in patients with comorbidities due to the selection of an adequate initial empirical therapy and reducing the risks of the septic shock .
summaryconflict of interest : none declared.introductionstaphylococcal bacteremia / sepsis is one of the most serious bacterial infections around the world . in individuals with pre - existing diseases , there is always an increased risk of infections occurring due to impaired immune system , a variety of drug therapy , exposure to a diagnostic and therapeutic procedure and frequent hospitalizations.objectivesto analyze the prevalence of comorbidity in a patient with the staphylococcal bacteremia / sepsis according to the diagnosis , the site of infection and according to the isolated agent.patients and methodswe analyzed the patients affected by the staphylococcal bacteremia / sepsis and treated in the clinic for infectious diseases during a ten - year period.results87 patients were included , out of whom 20 ( 23% ) with clinical signs of the bacteremia and 67 ( 77% ) of sepsis . in the analyzed sample , in 36 ( 41.4% ) were not registered comorbidity . hospital infections are represented by the previous antibiotic , corticosteroid and chemo therapy , pressure ulcers , and different implants . in all comorbidity , the most common isolated bacteria was s. aureus primarily strain mssa followed by mrsa strain which is more frequent in patients who were surgically treated ( comorbidity various implants).conclusionthe results suggest the importance of being mindful of the staphylococcal etiology of the bacteremia / sepsis in patients with comorbidities due to the selection of an adequate initial empirical therapy and reducing the risks of the septic shock .
this prospective interventional case series included patients with senile cataracts scheduled for phacoemulsification and intraocular lens ( iol ) implantation . for cataract grading , we matched the color and opalescence of the patients lens on the slit - lamp examination with the standard photographs of lens opacities classification system iii.8 the patients with any visible iris abnormality due to any cause were excluded from the study . the institutional review board of the iran university of medical sciences approved the study and all patients signed an informed consent . after creation of a paracentesis , 0.2 to 0.3 ml of preservative - free lidocaine 1% was injected into the anterior chamber . a quick chop method9 was performed using millennium phacoemulsifier ( bausch and lomb , ny , usa ) , hydroxypropyl methylcellulose 2% ( bausch and lomb , waterford , ireland ) as a viscoelastic agent , balanced salt solution ( bss ) as irrigation solution and one - piece foldable iol ( acrysof , alcon laboratories inc , fort worth , tx , usa ) in the bag implantation . , the horizontal diameter of the pupil was measured with a caliper under the operating microscope light . the light intensity of the microscope was the same during measurements and throughout all procedures . pupil diameter was measured again within 90 sec after intracameral lidocaine injection , when the surgeon was able to proceed for needle capsulorrhexis . all measurements were taken on the surface of the cornea and no magnification correction was made to know the actual size of the pupil . total surgical time was measured from the time of first stab incision to the end of operation when the blepharostat was removed . data were analyzed using spss for windows version 11 ( spss , chicago , illinois , usa ) . sixteen males and 15 females with a mean age of 67.39.9 years were included in the study . there were four diabetic and four pseudoexfoliative eyes . included eyes had nuclear opalescence and nuclear color grade 4 ( range 4 to 6 ) with variable amounts of cortical and posterior subcapsular cataracts . the mean pupil diameter after intracameral lidocaine was significantly greater than the baseline pupil size [ table 1 ] . there was no significant difference between the average male and female pupil diameters and between the average pupil size of diabetic and non - diabetic patients , both at baseline and after intracameral lidocaine injection measurements ( independent t test ) . at baseline there was no significant difference between average pupil diameter in patients with pseudoexfoliation ( 2.66 mm ) and those without ( 2.47 mm ) . however , after intracameral lidocaine injection , the average pupil diameter was significantly greater for patients without pseudoexfoliation ( 7.1 mm vs. 6.00 mm , independent t test and mann - whitney test , p<0.01 ) . the pupil dilation was satisfactory , persistent and stable up to the end of the surgery . one patient developed posterior capsular tear with vitreous loss at the time of cortical material removal that was managed properly with an iol in the ciliary sulcus , without the need for additional mydriatics . intracameral injection of preservative - free lidocaine is used widely for local anesthesia and discomfort relief in cataract surgery.1,10 lidocaine causes no additional inflammation and endothelial cell loss and its safety has been confirmed in previous studies.11 - 14 lincoff et al . reported the effect of lidocaine on iris paralysis and mydriasis.15 they noted pupil dilation after accidental intraocular injection of lidocaine without administration of any mydriatic drug . reported immediate pupil dilation after intracameral injection of preservative - free lidocaine 1% in previously undilated phakic eyes during trabeculectomy surgery.6 cionni et al . used intracameral lidocaine injection to induce mydriasis in phacoemulsification surgery , avoiding all preoperative dilating eye drops.7 however , in their report , epinephrine has been added to the infusion during routine procedure to maintain pupil dilation.7 this study shows that injection of 0.2 to 0.3 ml of 1% preservative - free lidocaine in anterior chamber can have a persistent , stable and satisfactory pupil dilation for a safe phacoemulsification and iol implantation in different patients including diabetic and non - diabetic and eyes with pseudoexfoliation . in the present study , despite the fact that no additional mydriatic drug has been used in infusion fluid , the pupil was adequately maintained dilated during the procedure . it doesnt need time - consuming preoperative program for pupillary dilation , doesnt have systemic side - effects of topical mydriatics and provides satisfactory pupillary dilation as well as simultaneous anesthetic effect in phacoemulsification cataract surgery . we measured the pupillary diameters on the surface of the cornea and did not consider the magnification factor of cornea to calculate the actual pupillary diameters . in this study , however , it is not clear how long the pupillary dilation will last after an intracameral lidocaine injection . although in the sole complicated case of this study , the pupil maintained its dilation up to the end of 25 min of the operation this could not be generalized . future studies are recommended to study the permanence of pupillary dilation after intracameral lidocaine injection as compared to the topical and intracameral mydriatics . we conclude that intracameral lidocaine is a safe and efficient alternative to traditional topical and intracameral mydriatics during phacoemulsification surgery .
topical and/or intracameral administration of anticholinergic and/or sympathomimetic mydriatic agents which are usually used for pupillary dilation during cataract surgery , have some disadvantages such as slow onset of dilation and adverse ocular and systemic effects . we evaluated intracameral injection of preservative - free 1% lidocaine without using any preoperative or intraoperative mydriatics to induce pupil dilation in 31 consecutive eyes scheduled for phacoemulsification cataract extraction and intraocular lens implantation . pupil diameter was measured before and 90 sec after intracameral lidocaine injection . after intracameral lidocaine injection , the mean pupil diameter was significantly greater than the baseline measurement ( p<0.001 ) . no additional mydriatics were needed up to the end of the operations . intracameral preservative - free lidocaine 1% has a rapid and effective mydriasis that could be a safe alternative to topical and intracameral mydriatics in phacoemulsification .
sarcoidosis is a systemic disorder of unknown etiologycharacterized by widespread development of noncaseating epithelioid cell granulomas in more than one organ system . a review of the literature suggests that the relationship between sarcoidosis and malignancy is controversial ; however , new onset sarcoidosis has been observed repeatedly and reported in oncology patients [ 2 - 4 ] . we report on a case of sarcoidosis mimicking cancer recurrence , which developed after chemo - therapy for ovarian cancer . a 52-year - old female was admitted to our hospital with a one - month history of abdominal pain on the left side . her abdominal computed tomography ( ct ) scan showed ascites , omental caking , and peritoneal thickening . she received combination chemotherapy comprising paclitaxel 175 mg / m+carboplatin 400 mg / m for eight cycles for seven months after surgery . however , four months later , she presented with a dry cough , and multiple painless subcutaneous nodules that had developed on her right arm and leg . a plain chest radiograph showed bilateral hilar enlargement compared with a previous film ( fig . computed tomography ( pet / ct ) scan for evaluation of cancer recurrence showed no uptake in the subcutaneous nodules , however , multiple areas of high uptake were observed in the enlarged paratracheal , left supraclavicular , retrocrural , and diaphragmatic lymph nodes ( the maximum standardized uptake value [ suvmax ] of the lymph nodes ranged from 12.3 to 21.5 ) ( fig . the same features were observed on both chest ct scan and pet / ct ( fig . 3a ) . however , her serum levels of carbohydrate antigen 125 ( ca125 ) and carbohydrate antigen 19 - 9 ( ca19 - 9 ) were unremarkable ; ca125 level was 9.42 u / ml ( reference range , 0 to 35.0 u / ml ) and ca19 - 9 level was 3.12 u / ml ( referencerange , 0 to 37 u / ml ) . to be certain of the diagnosis of cancer recurrence , biopsy of the mediastinal lymph nodes using endobronchial ultrasound - guided transbronchial needle aspiration and biopsy of the subcutaneous nodules ziehl - neelsen and polymerase chain reaction detection of mycobacterium tuberculosis in tissues were negative for mycobacterial infection , and a serum interferon - gamma release assay test was also negative . she had mild symptoms , including intermittent dry cough , and no specific treatment was required . she was observed closely at our clinic for three months , and the multiple painless subcutaneous nodules resolved spontaneously . her follow - up chest ct scan showed an interval decrease in the size of multiple mediastinal lymph nodes ( fig . however , newly developed perilymphatic and centrilobular nodules were detected in both lungs on the same ct scan ( fig . lymphangitic metastasis of ovarian cancer was suspected radiologically , therefore , she underwent video - assisted thoracoscopic wedge resection of the nodules in the right upper lobe . however , the pathological findings of the lung nodules showed noncaseating granuloma , which was similar to that of the mediastinal lymph nodes and skin ( fig . to the best of our knowledge , this is the first report of sarcoidosis that occurred following surgery and chemotherapy for ovarian cancer in south korea . in addition , this case demonstrates that the clinical presentation and radiological examinations , including a pet / ct scan , may not be able to discriminate between cancer recurrence and sarcoidosis . according to the literature , 4 - 14% of all patients with malignancy can exhibit histopathological evidence of sarcoidosis . in oncology patients , noncaseating granulomas have been observed most frequently in the lymph nodes draining the cancer . in the current case , development of noncaseating granulomas occurred not only in the mediastinal lymph nodes , but also in the subcutaneous tissue and lung parenchyma in the absence of recurrence of ovarian cancer , which led to diagnosis of sarcoidosis . it is difficult to explain why the reaction occurred only in distant lymph nodes and organs and not in regional lymph nodes . understanding of the pathogenesis of sarcoidosis associated with cancer remains incomplete . according to one hypothesis , localized sarcoidosis may be related to degenerative and necrotic changes within the tumor itself and that systemic sarcoidosis at sites not involved with the tumor may be mediated by humoral and t cell - mediated factors , and subsequent recruitment and activation of macrophages . development of sarcoidosis during or immediately after chemotherapy is rare and has been reported only in a limited number of cases of malignant lymphoma , osteosarcoma , and solid tumors [ 7,9 - 11 ] . the pathophysiological mechanisms of sarcoidosis during or after high - dose chemotherapy are unknown because chemotherapy inhibits the local immunological responses there by preventing granuloma formation and development of associated disease symptoms . it has been postulated that sarcoidosis can be caused by a microorganism because immunosuppression induced by antineoplastic therapy might spread this agent , leading to an immune response resulting ingranuloma formation . this phenomenon has been associated with several different chemotherapeutic agents ; the most common causative agent is the biologic modifier alpha - interferon . there is only one previous report on development of sarcoidosis associated with combination chemotherapy paclitaxel and carboplatin . reported a case of sarcoidosis that developed during induction chemotherapy comprising paclitaxel and carboplatin for lung cancer . in the current case , development of sarcoidosis occurred four months after chemotherapy ; however , the patient described by umezu et al . was diagnosed with sarcoidosis during chemotherapy . paclitaxel and carboplatin may have induced the reaction , however , conduct of further studies will be required in order to explain the pathogenesis of sarcoidosis during or after chemotherapy . as in the current case , in addition , the current case showed higher uptakes in the pet / ct scan ( e.g. , suvmax of lymph nodes , 12.3 to 21.5 ) than in previous reports , suggesting that the suvmax in the pet / ct scan is not accurate for distinguishing between sarcoidosis and malignancy . therefore , evaluation of other clinical parameters or the serum ace level might be necessary in order to discriminate between sarcoidosis and metastasis . in conclusion , when cancer recurrence is suspected despite successful chemotherapy for a neoplastic disease , the clinician should consider the possibility of sarcoidosis even if it occurs during or immediately after treatment . histopathological examinations can be useful for making a precise diagnosis and for appropriate decision making .
we report on a rare case of sarcoidosis that developed after chemotherapy for ovarian cancer , and mimicked a cancer metastasis . a 52-year - old female diagnosed with stage iii ovarian cancer underwent curative surgery and postoperative chemotherapy . four months later , her whole - body positron emission tomography and computed tomography ( ct ) scan showed high uptake in the mediastinal lymph nodes , and ovarian cancer recurrence was suspected . biopsy of the mediastinal lymph nodes and subcutaneous nodules revealed noncaseating granulomas . these lesions resolved spontaneously without treatment ; however , newly developed perilymphatic and centrilobular nodules were observed on follow - up chest ct . surgical biopsy of these lesions also showed noncaseating granulomas . she was finally diagnosed with sarcoidosis .
the placement of central venous catheter using seldinger 's technique , though a commonly performed procedure in the intensive care unit , has its own risks and complications . various complications have been reported with the use of guide wire as well as catheter . both malposition and entrapment of guidewire are known to occur during subclavian vein ( scv ) cannulation . we report a case of scv cannulation in which guide wire went to ipsilateral internal jugular vein ( i / l ijv ) and with subsequent passage of dilator it got kinked and directed to superior vena cava ( svc ) . though removal of entrapped guidewire in interventional radiology suite has been described as a possible method , kinks remote to entry site and its unraveling made the task difficult . as a result , combined transfemoral and subclavian route a 35-year - old , 70 kg , 180 cm , male patient with traumatic cervical spine injury was admitted to our intensive care unit with chief complaints of labored breathing and partial loss of power in all four limbs . after securing the airway with a tracheal tube , central venous cannulation was attempted using 7 f20 cm triple lumen catheter ( certofix , braun , germany ) . under strict aseptic precautions landmarks were identified and right scv was located infraclavicular at medial 2/3 and lateral 1/3 of the clavicle using introducer needle in single attempt . after confirming good backflow of the blood , guide wire ( 50 cm with j - tip ) , was introduced up to 15 cm mark without any resistance . after that tissue dilator was threaded over the guide wire followed by railroad of triple lumen catheter ( up to 13 cm ) over it , although the line was inserted in single pass but resistance was encountered during removal of guide wire . bed side chest roentgenogram revealed the guidewire going through right scv towards svc but after its entry it took sharp turn and entered ijv on the same side [ figure 1 ] . with bed side x - ray as a control we tried to remove the guide wire , pushing it caused the assembly to move inside the right atrium ( arrhythmia elicitation ) but attempts at removal resulted in decoiling at the site of insertion . further attempts at removal of the guidewire were deferred and patient was shifted to intervention radiology suite . the attempts at removal of guidewire by passing a tissue dilator to straighten the kinked portion were not met with success , so through percutaneous right transfemoral venous route a vascular snare was used to pull the guide wire out of ijv [ figure 2 ] . it was followed by successful railroad of long and large bore tissue dilator from the unraveled to the intact segment of guide wire from the insertion site . the incision at site of insertion was then deepened to remove the kinked portion [ figure 3 ] . another guidewire was passed through the same dilator followed by insertion of triple lumen catheter . portable chest x - ray showing looping of the guidewire a. into internal jugular vein b. superior vena cava guidewire dragged from internal jugular in the radiology suit ( arrow ) the unraveled guidewire after removal we present a case of malpositioning , kinking and unraveling of guidewire during scv cannulation . it seems that at the time of insertion , the guidewire got malpositioned into i / l ijv and the passage of dilator over it caused it to kink and enter svc on the same side [ figure 1 ] . catheter placement into ijv is the most frequent malposition during cannulation of scv using infraclavicular approach . in the current case kinking during subsequent passage of dilator was seen which is a rare finding . various maneuvers like turning the head to ipsilateral ( i / p ) side , compression of i / p ijv during insertion of the guidewire , keeping the j - tip directed downwards and electrocardiography guidance have been described to prevent it . the described management for removal of entrapped intravascular foreign body include application of slow gentle traction , surgical exploration or maneuvering in interventional radiology . in this case the application of traction resulted in unraveling of guidewire making the situation even more problematic . there is an inner single filament wire core which is surrounded by spiral covering welded to the core filament at both ends . according the testing guideline available ( iso : 11070 ) the minimum force at break for the guidewire used in central venous access procedure is 5 newton , which is pretty less . depending upon the manufacturer 's specifications the weld seams of outer covering at both the ends can withstand a pull strength of 17 n ( 1.73 kg ) before detaching from its core , whereas in the middle section guidewire can withstand a stress of up to 290 n. guidewire made by b braun , germany , used in this case can withstand a force of 24 n. we feel that application of firm pressure caused the surrounding coil to unwind ( like an overstretched spring ) ; however , the force was insufficient to break the inner filament . this may be the reason why we did not encounter negative consequences like breakage and embolization of the guidewire . such guidewire related complications can happen either due to manufacture defect or because of faulty technique . we believe that intention to remove a malpositioned and subsequently kinked guidewire caused its unwinding ; fortunately the pressure was inadequate to cause breakage of inner filament and subsequent embolization . due to less invasive nature percutaneous removal in interventional radiology suite is a preferred method as compared to open surgical removal so we opted for the former . there are case reports in which fluoroscopy was used for removal of kinked guide wire . however , in these reports periclavicular region was the most common site of kink and the passage of dilator to straighten it was key to success . we tried using this route but the unique course and kinking remote to the site of insertion required use of transfemoral loop snare to pull it out of ijv . this was followed by manual pulling of the kinked and broken wire towards the site of insertion and passage of wide bore tissue dilator over it and removal . intervenational radiology suite proved to be the safe and effective alternative for removal of the badly struck guidewire . hence to conclude , caution should be exercised during placement of guidewire . desperate attempts at removal of struck or kinked guidewire may result in loss of mechanical integrity , unraveling and breakage and possible embolization . do not thread a dilator over a guidewire which is suspected to be kinked for some reason . the use of fluoroscopic control in the form of bedside digital radiograph or intervention suit may help in the determination of condition and position of guidewire and should be preferred over to blind attempts at removal . the withdrawal of a kinked guidewire by means of interventional radiology is the procedure of choice .
the placement of central venous catheter using seldinger 's technique , remains a commonly performed procedure with its own risks and benefits . various complications have been reported with the use of guide wire as well as catheter . we report a unique problem during subclavian vein cannulation due to guidewire malposition which led to its kinking and difficult retrieval requiring removal in fluoroscopy suit . the probable mechanism of guide wire entrapment and possible bedside management of similar problems is described .
lung cancer is the leading cause of cancer - related mortality in both men and women throughout the world . it is also the most commonly diagnosed cancer worldwide with most of them at an advanced stage with a poor prognosis . a 78-year - old male smoker patient diagnosed to have non - small cell lung cancer ( nsclc ) , stage iv ( adrenal and liver metastasis ) was subjected to whole body bone scan to detect skeletal metastases , if any . the patient had received local palliative radiotherapy 20 gy/5 # for 1 week and was on chemotherapy with gefitinib since 12 days . increase in tracer uptake was noticed in the lower cervical vertebrae , right first rib , and medial cortical margin of the lower third of shaft of left femur [ figure 1a and b ] . hybrid single photon emission computed tomography / computed tomography ( ct ) localized the cervical uptake to left articular process of c6 and left transverse process of c7 vertebrae with dense sclerotic changes likely degeneration due to altered weight distribution . tc99m - methylene diphosphonate whole body bone scan in ( a ) anterior projection ( b ) posterior projection showing abnormal tracer uptake in the lower third of shaft of left femur ( arrow ) . hybrid single photon emission computed tomography / computed tomography ( ct ) localized the ct images ( c and d ) of the lower third of the left femur showed lysis of the anteromedial cortical margin ( arrow ) ct scan of the lower third of the left femur showed lysis of the anteromedial cortical margin with no soft tissue component [ figure 1c and d ] . on the basis of bone scan and pattern of cortical bone destruction , the diagnosis of a skeletal metastasis from a bronchogenic carcinoma was suggested . incidence of bone metastases in cancers of the breast , prostate , lung and kidney , is very high , approximately 70% of all patients . the reported incidence of bone metastasis from nsclc is around 15%-40% , most of which involves spine , rib , and pelvis and 6% in femur . bronchogenic carcinoma is an established entity in causing cortical bone metastases literature review also shows primaries in lung , breast , kidney , and pancreas as causes of cortical metastasis . of particular interest is the involvement of femur with studies reporting osteolytic cortical lesions preferentially located at the femur as typical of bronchogenic carcinoma . the reason for this special predilection for implantation in the cortical bone , particularly the femur , is still unknown . it is speculated that the vascular network , originating in the overlying periosteum of the long bones , serves as a pathway for metastatic deposits to destruct the cortical bone . literature review not only shows predominance of femoral involvement in bronchogenic carcinoma , but also patients with only pure cortical metastases had exclusive femoral involvement . four distinctive patterns of bone destruction in bronchogenic carcinoma have been described . a small focal intra - cortical lesions ( cookie - bite or cookie - cutter pattern ) ; large osteolytic lesions ; saucerized intra - cortical lesions with well defined periosteal reaction ; and lesions with predominant cortical destruction extending into the soft tissue as well as the medullary cavity . the term cookie bite metastasis was initially coined for a small intra - cortical lesion . although these lesions are not entirely pathognomonic , literature review shows that such lesion within the skeleton as initial diagnosis should alert the radiologist to the possibility of a primary neoplasm of the lung . our case further illustrates how a high degree of suspicion is needed in interpreting isolated uptake in bones scan in the femur in patients with bronchogenic carcinoma . moreover , as described in literature , the characteristic pattern of cortical destruction if found in a patient with carcinoma of unknown primary , then the search for an occult primary neoplasm should be started without delay .
detection of skeletal metastasis in patients with lung cancer is important from management point of view . we report the bone scan finding in a patient with non - small cell lung carcinoma showing isolated abnormal tracer in femur and having a characteristic appearance in computed tomography , highlighting the importance of bone scan in patients with bronchogenic carcinoma .
polar lepromatous leprosy is the anergic form of the disease characterized by marked lack of cell - mediated immunity against leprosy . macrophages become foamy in which mycobacterium leprae continue to grow and multiply and are transported to all parts of the body . our patient had old keratic precipitates , iris atrophy and 2-mm hypopyon in the anterior chamber . as reported earlier , the anterior chamber aspirate revealed plenty of afb on ziehl- nielsen staining confirming the clinical diagnosis of leprosy uveitis.5 the course of the disease is dependent mainly upon the individuals immunologic response to the bacilli . erythema nodosum leprosum is an acute and exaggerated immunological reaction in the chronic course lepromatous leprosy , it can be very severe and prove fatal.1 - 4 severe enl is termed as erythema necroticans when it presents as pustular or ulcerated lesions.1 patients with lepromatous disease and a bacterial index of > 4 + were reported to be at significantly increased risk , the usual triggers associated with enl reaction include intercurrent illness or anti - leprosy treatment.7 even though our patient received moderate doses of steroids in addition to mdt , he developed severe necrotic systemic enl . histopathologically , enl or type 2 reaction is characterized by an influx of neutrophils on a background of lepromatous granuloma in contrast to type 1 reaction which depict granulomas comprising of epithelioid cells , lymphocytes and langhans giant cells.8 the infiltration of neutrophils is specific to lepromatous enl while it is lymphocyte infiltration in erythema nodosum of other causes.9 chaudhary reported a case of lepromatous enl with a very high leucocyte count in the peripheral blood and he named it as a myeloid leukemoid reaction.10 in our patient , in addition to macrophages , numerous polymorphs with and without mycobacterium leprae were seen in almost all layers except the retina . perforation of the globe with influx of neutrophils during the generalized enl reaction confirmed ocular enl . this report depicts a case of histopathologically confirmed severe enl that resulted in post - therapeutic perforation of the globe . this case is reported to alert the ophthalmologists to the peculiar ocular complication of enl when a patient is treated with highly bactericidal drugs .
leprosy is a chronic granulomatous disease caused by mycobacterium leprae , clinically present either as tuberculoid , borderline or lepromatous type . erythema nodosum leprosum ( enl ) is an acute humoral response in the chronic course of lepromatous leprosy . although very severe enl reactions are known in systemic leprosy , such severity is rare in ocular tissues . a leprosy uveitis patient suffered from a severe form of post- therapeutic enl reaction which resulted in perforation of the globe at the site of preexisting subconjunctival leproma . painful blind eye was enucleated . histopathological study revealed infiltration of numerous polymorphs and macrophages packed with acid - fast bacilli in the conjunctiva , cornea , ciliary body , ora serrata and sclera . a profuse influx of neutrophils on a background of macrophages packed with m. leprae confirmed the ocular enl reaction . this case is reported to alert the ophthalmologists to a rare ocular complication of enl .
this case report reveals the importance of a rare complication of this common form of envenomation . a 40-year - old healthy male , who is a farmer by occupation , presented with burning pain and swelling all over the upper part of the body following multiple wasp stings . when the patient was cycling and going to his village , the nest of the wasps fell down from the weak branch of a tree and by this , the wasps were disturbed and around 50 wasps attacked him at once and caused the above injury and later after 2 days of this incidence , the patient developed progressive decrease in urine output . the patient did not have any other significant illness in the past including hypertension , or any history of nephrotoxic drug intake . examination revealed a pulse rate of 110/min , blood pressure of 150/100 mm of hg and had swollen and edematous scalp , left upper limb , and upper thorax . there were multiple sting marks over the head , neck , face and the left upper limb [ figure 1 ] . investigations revealed a blood urea of 120 mg / dl , serum creatinine which rapidly worsened from 1.2 mg/ dl to 4.5 mg / dl over next three days , the hematocrit was 50% and platelet count was 1.32 lakhs / mm . the urine was reddish brown colored and showed 3 + proteinuria and 100 of red blood cells ( rbcs ) . activated partial thromboplastin time ( aptt ) was prolonged ( 32 s with a control of 26 s ) . bleeding and the rest of the biochemical analysis showed serum creatinine phospho kinase ( cpk ) of 1 10 000 iu / l , lactate dehydrogenase ( ldh ) of 5250 iu / l , ast 1450 iu / l , alt 950 iu / l . meq / l , serum sodium 130 meq / l , serum calcium 9.0 mg / dl , serum phosphorous 6.2 mg / dl and serum uric acid 7.0 mg / dl . serum bilirubin was 1.9 mg% and urine myoglobin assay was more than 1000 ng / ml . the ultrasound of the abdomen showed normal sized kidneys with normal echotexture and preserved corticomedullary differentiation . the patient had progressively worsening renal failure and remained oliguric ( 250 - 300 ml / day ) for 3 days [ table 1 ] , in spite of adequate hydration . hence , the patient was initiated on intensive hemodialysis , and continued the antibiotics , antihistamines and corticosteroids till the signs of inflammation came down . after about 2 weeks , the patient entered a diuretic phase and his urine output started improving and serum potassium , aptt , and liver function tests became normal . three weeks later his renal functions gradually became normal ( serum creatinine 1.1 mg / dl ) . the pathogenic mechanisms responsible for the clinical sequelae following insect stings of the order hymenopterae ( which comprises wasps and bees ) include allergic reactions , rhabdomyolysis , hemolysis and direct tissue toxicity . the major causes of renal failure are acute tubular necrosis ( atn ) due to hypotension or pigment nephropathy resulting from rhabdomyolysis and intravascular hemolysis , and acute interstitial nephritis . their venom contains protein toxins , biogenic amines , and enzymes that allow the toxins to spread . the acute renal failure ( arf ) due to wasp sting bites the toxic principles include active amines like histamine , serotonin , kinins , phospholipase a2 , hyaluronidase , mellitin and apamine . the exact mechanism of rhabdomyolysis is not known but a direct toxic effect of venom on muscle is believed to be the main cause . however , other mechanisms postulated for renal damage due to wasp stings are : ( i ) direct nephrotoxicity due to toxin ; ( ii ) hypotension leading to ischemic tubular necrosis and ( iii ) nephropathy due to hemoglobinuria and myoglobinuria . the manifestations other than renal failure include myocardial necrosis and infarction , centrilobular necrosis of liver , and thrombocytopenia as a result of direct platelet toxicity.[68 ] our patient had a rapidly worsening renal failure with markedly elevated serum levels of cpk and urinary myoglobin suggesting rhabdomyolysis . the elevated ldh levels points towards a hemolytic process . in view of the prolonged aptt as the patient manifested all the stage of acute tubular necrosis and finally went to diuretic phase , the diagnosis of atn was made , though the renal biopsy was not done . ( 2005 ) reported similar case in southern part of india , in which patient had suffered from acute renal failure and multi organ dysfunction following wasp sting . 1992 ) have reported a case of acute renal failure without rhabdomyolysis and hemolysis implicating direct venom toxicity as the probable cause . thus the clinical sequelae of multiple wasp stings can result in multisystem involvement ranging from intravascular hemolysis , rhabdomyolysis , acute renal failure , hepatic dysfunction , and occasionally thrombocytopenia and coagulopathy . when progressive renal failure ensues , intensive hemodialysis results in good renal recovery with return of renal functions to normal in majority of the survivors . multiple hymenoptera envenomation is a serious condition and people with this are at high risk of developing multi - system involvement , predominantly acute renal failure . our case indicates timely intervention with intensive hemodialysis not only prevents mortality but also other organ complications .
the wasp stings usually cause local reactions and rarely anaphylaxis . however the multiple wasp stings may cause multisystem involvement . we report a case of acute renal failure ( arf ) following multiple wasp stings . a middle aged healthy gentleman presented with pain and swelling of the upper part of the body following multiple wasp stings . after 2 days , he developed progressive decrease in urine output with high colored urine . physical examination revealed the edematous and tender affected part . on investigating , it was found to have sequential elevations in renal function tests . the markers of muscle injury were grossly elevated and liver enzymes were deranged . these findings suggest multisystem involvement predominantly arf secondary to rhabdomyolysis . with the initiation of the intense hemodialysis , all the above parameters became normal . timely intervention of multiple wasp stings causing arf with multiorgan involvement by hemodialysis not only prevents mortality but also other complications .
epilepsy is associated with a two- to three - fold increase in mortality among patients compared with the general population . sudden unexpected death in epilepsy ( sudep ) is one of the most frequent causes of death among patients with epilepsy . there is strong evidence suggesting that sudden unexpected death in epilepsy ( sudep ) is a seizure - related phenomenon , , , . the first description of this phenomenon was introduced by russell in 1906 . since then , several cases have been reported in the literature presenting with a drop in heart rate or asystole during the seizure . bradycardia and asystole resulted from increased parasympathetic flow through the vagus nerve , which originates in the nucleus ambiguous and dorsal nucleus of the vagus in the medulla . on the basis of one study , the incidence of sudep ranges from 1:1000 and 1:2000 person - years to 1:200 person - years , . according to a recent revised definition , sudep consists of sudden , unexpected , witnessed or unwitnessed , nontraumatic and nondrowning death in patients with epilepsy , with or without evidence of a seizure , excluding documented status epilepticus ( seizure duration > 30 min or seizures without recovery in between ) and asphyxia ; if postmortem examination does not reveal a cause of death , the diagnosis is definite sudep , and if there is a preexisting condition before or after autopsy , which could have contributed to the death , it is classified as sudep plus . strong risk factors for sudep include young age , early onset of seizures , the presence of generalized tonic clonic seizures , male sex , and bedtime occurrence . less significant risk factors for sudep include the prone position , one or more subtherapeutic blood levels , sleep occurrence , and a structural brain lesion . the underlying pathophysiologic mechanisms for sudep are not completely understood , but autonomic dysfunction ; ictal arrhythmias , ictal bradyarrhythmia , and asystole , ; neurogenic pulmonary edema ; and ictal central or obstructive apnea , , were introduced in the literature . in this report , we describe two patients with seizure - associated asystole monitored by simultaneous video electroencephalography electrocardiography . a thirteen - year - old right - handed male with seizure disorder was admitted for a presurgical assessment . there was a history of neonatal hypoglycemia during an apparently normal vaginal delivery . he was started on antiepileptic drugs , but the second seizure appeared six months later . the seizures typically consisted of blurred vision and upward gaze followed by a loss of consciousness . his mri showed near symmetrical signal abnormality at parietooccipital regions bilaterally ( parasagittal aspect ) accompanied by mild gliosis and volume loss ( fig . , there was bilateral rhythmic activity maximum to the left associated with right - side clonic jerk and head and eye deviation to the right that secondarily generalized and was followed by 16 s of asystole at the end of the seizure ( fig . 1 , fig . 2 , fig . 3 ) . interictal abnormality consisted of bilateral spike - and - wave and bilateral slow activity maximum in the right posterior head region . at the end of monitoring , an anticonvulsant drug regimen of valproic acid and levetiracetam was started , and cardiology consultation was suggested . a 42-year - old right - handed male with seizure disorder was admitted for presurgical assessment . his epilepsy was due to penetrating head trauma in the left frontal lobe from a shell injury . since then , he had been experiencing episodes of intense fear followed by generalized tonic clonic movements . neurologic examination included mild paresis in the right upper extremity and in the distal part of the right lower extremity in the range of 12/5 . the very first clinical manifestation was after the initial eeg changes and consisted of a generalized tonic afterwards , the sa arrest took place and lasted about 1 min ( see fig . 5 , fig . 6 , the very first eeg change started with 5-hz spike slow waves over the left parasagittal area with the maximum amplitude on c3 and f3 . the interictal abnormality consisted of delta waves seen at p3 , c3 , and f3 . considering his cardiac arrest during the seizure , a cardiac consultation was done , and a pacemaker was implanted . during 24 months of antiepileptic regimen , the frequency of seizures was reduced remarkably . theoretically , these asystoles could have a role in the incidence of sudep , meaning that the presence of ictal bradycardia is a risk factor for sudep . furthermore , in cases of epileptic cardiac dysrhythmia , isolated eeg or ecg recording may prove insufficient , and prolonged simultaneous eeg / ecg monitoring may be required . only simultaneous eeg and ecg recording will reveal a possible cerebral origin of arrhythmias in these patients who are , typically , young . attaining the correct diagnosis is essential because appropriate treatment may prevent cardiogenic sudep , which is thought to be related to potentially lethal arrhythmias , such as asystole induced by epileptic seizures , and to prevent the cardiac side effects of specific antiepileptic drugs . in conclusion , cardiological investigation should be included in epilepsy management to search for abnormalities of hr or ischemic events . in addition , it can provide an appropriate guideline in pharmacotherapy since certain types of drugs including carbamazepine , phenytoin , benzodiazepine , and barbiturates should only be used with caution by patients with epilepsy who have cardiac dysfunction . increasing knowledge about sudep risk factors can have a significant preventive role . moreover , strategies such as taking a detailed cardiovascular history to get the comprehensive clinical picture including a detailed history of symptoms , risk factors , and prior cardiac findings should be undertaken .
ictal asystole is a rare , probably underestimated manifestation of epileptic seizures whose pathophysiology is still debated . this report describes two patients who had cardiac asystole at the end of their seizure . the first patient was a 13-year - old boy with complex partial seizures .. his mri showed symmetrical signal abnormality in the bilateral parietooccipital lobe accompanied by mild gliosis and volume loss . during a 3-day long - term video - eeg monitoring , he had cardiac arrest at the end of one of his seizures that was secondarily generalized . the second one was a 42-year - old veteran with penetrating head trauma in the left frontal lobe due to shell injury . during long - term video - eeg monitoring , he had one generalized tonic clonic seizure accompanied by bradycardia and cardiac asystole . asystoles could have a role in the incidence of sudden unexpected death in epilepsy ( sudep ) , meaning that the presence of ictal bradycardia is a risk factor for sudep . in cases of epileptic cardiac dysrhythmia , prolonged simultaneous eeg / ecg monitoring may be required . cardiological investigation should be included in epilepsy management .
enzinger et al . described for the first time in 1972 a distinct clinicopathological entity comprising a series of unique myxoid soft tissue tumors called , extraskeletal myxoid chondrosarcoma . many case reports and studies appeared subsequently in the literature , describing this rare tumor with distinct features . although now recognized as being unrelated to chondrosarcoma of the bone , emc was initially believed to be of cartilaginous origin . it is a slowly growing tumor , prone to local recurrences and sometimes , metastases.[35 ] this tumor behaves differently from other sarcomas as it is associated with a prolonged survival even after metastasis in some cases . however , a high rate of death has been described to occur eventually due to these tumors . this unusual tumor typically presents as an enlarging mass in the extremities of adults and rarely in various other locations . there have been only a few case reports on the cytological features of emc.[69 ] we describe here a case which was diagnosed based on fine needle aspiration cytology ( fnac ) and was later confirmed by histopathology . a 51 year - old male patient was referred to our fnac clinic with a gradually increasing swelling of ten years duration in the left thigh . local examination showed the swelling to be about 20 10 cm in size over the anterior and anterolateral aspects of the left thigh . the mass appeared to be intramuscular in location with maintenance of the plane between the neurovascular bundle and the mass . the mass was aspirated using a 22 gauge needle and the smears made were cellular and showed sheets and clusters of cells surrounded by a striking and prominent myxoid stroma [ figure 1 ] . some of the cells also had nuclear grooves [ figure 2 ] . at places , the cells formed small anastamosing cords and single - file arrangement pattern [ figure 1 ] . on the basis of the clinical , radiological , and cytological findings , the diagnosis was made of a myxoid sarcoma favoring extraskeletal myxoid chondrosarcoma . fnac smears showed monotonous cells in small clusters and singlefile pattern with a prominent myxoid stroma ( pap , 100 ) fnac smears showed cells with vesicular nuclei , some exhibiting grooves ( h and e , 400 ) resection revealed that the tumor was a well circumscribed and partly encapsulated mass measuring 2053 cm . the cut surface was gelatinous and showed glistening areas and multiple small cystic spaces filled with mucoid material . microscopy revealed a lobulated neoplasm with strands and groups of neoplastic cells embedded in an abundant myxoid and chondromyxoid matrix . the stroma stained positively with alcian blue at a ph of 2.5 . at places , the histopathological features were reminiscent of the cytomorphological features with anastamosing cords of cells in a myxoid matrix [ figure 3 ] . histopathology showed anastamosing cords of neoplastic cells in a myxoid matrix ( h and e , 40 ) emc is a rare soft tissue neoplasm of the extremities that was first delineated as a distinct entity by enzinger and colleagues . the distinct cytological features that were seen in the present case include the presence of uniform cells with the morphology of immature chondroblasts set in an abundant stroma that is myxoid . as seen in chondrosarcomas , chondrocyte - like cells embedded within lacunae have been described in some case reports , but not in the present case . the arrangement of cells in cords and columns within a myxoid background is an important clue to diagnosis . if available , fish analysis can also be performed on cytospin preparations to demonstrate the characteristic 22q12 translocation . other myxoid tumors of soft tissue that should be considered in the differential diagnoses on cytology include myxoid liposarcoma , soft tissue chondroma , myxoid variant of malignant fibrous histiocytoma , myxoma , and chondrosarcoma . thus , fine needle aspiration cytology has proved to be a useful tool in the diagnosis of adult myxoid sarcomas . myxoid liposarcomas show characteristic lipoblasts with scalloped nuclei and vacuolated cytoplasm and a delicate , branching capillary network against a myxoid background . the myxoid variant of mfh displays marked pleomorphism of the nuclei with histiocyte - like cells . chondrosarcomas typically possess a chondroid background and cartilage - like cells embedded within lacunae , rather than chondroblast - like cells lying loose within a myxoid stroma as seen in emc . this tumor shows a mixture of mature lipocytes and lipoblast - like cells embedded in a chondromyxoid matrix . none of these tumors display the distinctive cytological features of the cells of emc ; this aids the pathologist in confidently making a diagnosis of emc . the diagnosis may be supported on histopathological investigation by the immunohistochemical profile of the tumor . studies have shown that the tumor cells usually express vimentin , synaptophysin , s-100 protein , and epithelial membrane antigen . according to one of the studies which involved 23 cases , tumor size 10 cm , high cellularity , presence of anaplasia , and a mitotic activity of more than two per ten high power fields were all factors associated with decreased survival . ploidy status had no bearing on the prognosis in this study . in conclusion , although histopathology confirms the diagnosis of emc after excision , a preoperative diagnosis of emc can be arrived at confidently due to its distinctive cytomorphological features .
extraskeletal myxoid chondrosarcoma ( emc ) is a rare soft tissue sarcoma with limited literature available on its cytological features . we report here one such case where a diagnosis of emc was made based on fine needle aspiration cytology ( fnac ) . a 51 year - old male presented to our fnac clinic with a slowly growing mass in the left thigh , which was subjected to fine needle aspiration biopsy . radiological images showed no involvement of the underlying bone . magnetic resonance imaging was suggestive of a malignant neoplasm . the fna smears showed cell fragments and cords of monotonous cells embedded in abundant myxoid stroma . a diagnosis of a myxoid sarcoma favoring an emc was made in this patient . subsequent excision of the mass for histopathological examination confirmed this diagnosis . emc has distinctive cytological features that are helpful in confidently making a diagnosis in the appropriate clinical setting .
they are the main cause of thyroid cancer - related deaths , but are compatible with long - term survival in a large proportion of patients . however , nonregional lymph node metastasis , especially to the axillary nodes has been rarely reported in the literature , and it is not considered to be a typical site for metastasis from dtc . our case describes the image findings of iodine-131 ( i-131 ) uptake in i-131 whole - body scan ( wbs ) and hybrid single - photon emission tomography / computed tomography ( spect / ct ) in a case of papillary carcinoma with axillary lymph node metastasis . a 38-year - old female underwent total thyroidectomy with bilateral neck lymph node dissection , which revealed papillary carcinoma with lymph nodal metastasis . after 1-month , her thyroid stimulating hormone was > 100 miu / l , she was referred for i-131 wbs which showed residual thyroid in the anterior neck and lungs along with an intense uptake in the left lower neck and axilla [ figure 1 ] . spect / ct of the chest revealed apical group of left axillary lymph nodes lying just posterior to the upper portion of pectorlais minor [ figure 2 ] . she underwent radioiodine ablation therapy with the dose of 200 mci ( 7.4 gbq ) for lung metastasis and were on follow - up . whole - body iodine-131 scintigraphy shows uptake in thyroid remnant , lung metastasis , left neck and left axilla single - photon emission computed tomography - computed tomography of chest showing increased uptake localized to apical group of left axillary lymph nodes lying just posterior to upper portion of pectorlais minor thyroid carcinomas typically follow an indolent course , with excellent long - term survival rates . however , a small subset exhibits highly malignant behavior . larger primary tumor size , extracapsular extension , older age , certain histological variants , and distant metastases have all been identified as risk factors for the poorer prognosis , often with a cumulative effect . papillary thyroid carcin oma rarely metastasizes to axillary lymph nodes . in a report by nakayama et al . , the patient presented with cervical lymphadenopathy , and axillary nodal disease was also part of the initial presentation . the classical concept that the most commonly accepted route of ( regional ) lymphatic metastasis of papillary carcinoma is through the lymphatic system may be wrong at least in some patients because this approach may not work in unusual lymph node metastasis including axilla , and we should thus also consider the hematologic pathway as the route of metastasis to distant lymph nodes in addition to retrograde dissemination through lymphatic channels . there is a limited number of case reports describing the exceptional and rare metastatic spread of papillary thyroid carcinomas to the axilla , in those reports , axilla was involved as a late manifestation after multiple metastases to distant organs and lymph node or in recurrent papillary thyroid carcinoma and associated with poor prognosis . in another case , axillary metastasis was incidentally discovered during the diagnostic work - up of a calcification in the axilla detected by mammogram . integrated i-131 spect / ct imaging has an additional value in patients with thyroid cancer , for characterization of tracer uptake seen on planar imaging as well as for precise localization of malignant lesions in the neck , chest , and skeleton . this localization of i-131 uptake may have a clinical impact on patient management by influencing referral for i-131 treatment , tailoring of the administered radioiodine dose , and/or the addition of surgery or external radiation therapy when indicated . there was a report of a case described by damle et al . which detected axillary nodal recurrence in follow - up evaluation using spect / ct however , to the best of the authors knowledge , no report has yet detailed the axillary metastases from classic type of papillary carcinoma as initial presentation detected using spect / ct . there was another report that describes axillary lymph node metastasis of papillary thyroid carcinoma detected by fluorodeoxyglucose pet / ct in a thyroglobulin - positive patient with negative whole - body i scan . curative surgery if possible and/or 131-i therapy in cases that are radioiodine avid and have concomitant distant metastasis . it is well known that radioiodine uptake in a node is synonymous with metastatic disease from dtc , notwithstanding other extremely rare differentials . to further confirm our findings we also did an excision biopsy from the node , and she was treated with i-131 therapy for lung metastasis . to the best of our knowledge , this is the first case reported in literature where intense radioiodine uptake was seen in the left axilla as initial presentation and subsequently confirmed by spect / ct to be localized to the apical group of axillary nodes .
differentiated thyroid cancer is , usually , associated with an excellent prognosis and indolent course . distant metastases are rare events at the onset of thyroid cancer . among these presentations , metastasis to the axillary lymph nodes is even more unusual . only few cases of papillary carcinoma with axillary nodal metastasis were previously reported in the literature . we present a 38-year - old female who underwent iodine-131 whole - body scintigraphy , after total thyroidectomy and bilateral neck lymph node dissection for papillary carcinoma of thyroid , showed intense uptake in the remnant thyroid , lung metastasis , left cervical and left axillary lymph nodes . excision of left axillary lymph nodes confirmed metastatic papillary carcinoma .
the data deluge brought about by the genomic projects has fostered an unprecedented level of expectation for new medical , pharmacological , environmental and biotechnological discoveries . proteins mediate the majority of the functions of an organism , and all these functions are , by and large , determined by the proteins ' 3d structure . despite the progress achieved so far by structural genomics projects ( 1 ) , the exploration of the complete protein structure space through experimental techniques such as x - ray crystallography and nmr spectroscopy is still out of reach , because these techniques are time and resource consuming and not necessarily successful in all cases . consequently the gap between the numbers of known protein structures and sequences is steadily increasing . natural proteins spontaneously assume a unique 3d structure that , by and large , only depends upon the protein sequence . the problem of understanding the rules governing the folding process is very challenging and as yet unsolved . however , approximate methods for inferring the structure of a protein from its amino acid sequence are flourishing ( 2 ) . their results are of enormous relevance in many fields , from medicine to biology , from biotechnology to pharmacology . information derived from protein models has indeed proven to be useful by itself and in combination with experiments . protein models have been shown to be instrumental for the refinement of experimental structures ( 3,4 ) , the design of site - directed mutants ( 5 ) , the characterization of molecular function ( 6 ) and structure - based drug design ( 7 ) . not surprisingly , a growing number of scientific papers reporting the results of modelling experiments and their application to the design and interpretation of experiments are appearing in the literature . unfortunately , the models described in these reports are rarely publicly available and , in general , only accessible via direct interaction with the authors . the difficulty of mining the available structural model data leads to duplication of efforts and impairs the possibility of numerically evaluating the correctness of the models when the experimental structure becomes available . the establishment of public repositories for these protein 3d models can partly overcome these problems . specialized databases , such as modbase ( 8) and the swiss - model repository ( 9 ) , are already available for automatically built protein structure models . we have developed , and describe here , a protein model database ( pmdb ) where manually built models can be deposited and retrieved , together with their supporting information . pmdb ( interactively accessible at ) is a relational database of protein models submitted by users and obtained with different structure prediction techniques . the database is implemented on a linux server ( suse enterprise server 9 ) running apache , and the management system is mysql 4.1.12 . the queue management system is written in perl . php scripts and gd libraries are used for launching applications such as blast and for display , respectively . the current release contains > 74 000 models for 240 proteins , the majority of which are predictions submitted to the critical assessment of techniques for structure prediction experiment ( 2 ) . other models include those generated by our group ( 10,11 ) and models that we uploaded using published alignments ( 1215 ) . the database entry point is a protein target , for which one or more structural models can be present in the database . available information for each target includes the protein name , sequence and length , organism and , whenever applicable , links to the swissprot sequence database ( 16 ) . several models can be present for each target protein , or for different regions of the same target protein and the user can navigate through them using a graphical view shown in figure 1 . after the structure of a target is solved , the database entry is also linked to the experimental structure in the pdb ( 17 ) . models can be submitted in the form of a pdb file ( ts format ) or as an alignment to one or more known protein structures ( al format ) . in the latter case the coordinates of the backbone of the model are built using the al2ts program ( ) . when a user submits a model for a protein , the system verifies whether the target already exists ( i.e. there is already a model for some regions of the same protein ) . if not , the target is created and the model mapped to it . unless the target is an artificial or mutant protein , the target entry is linked to existing sequence databases [ at present the ncbi nr database ( 18 ) ] . the predictor can provide the ncbi i d of the protein ( in which case the system performs a sequence check ) , ask for a blast search in the database to retrieve the i d ( if more than one entry matches the sequence , the user is requested to select the correct one ) , or inform the system that the target is not expected to be present in any sequence database . the sequence of the target is derived from the submitted model pdb or alignment file . in the former case , if the distance between consecutive c is larger than expected for connected residues , the user is asked whether he or she wants to complete the target sequence ( figure 1 ) . the system also reports cases where atoms in the model are closer than the sum of their van der waals radii . the database stores information about the author of the model , a short description of the method used and supporting evidence , in the form , for example , of a multiple sequence alignment . submitters are also asked to assign a reliability value to their model(s ) and a literature reference that can also be provided at a later stage . models can be kept on hold upon request and made available to the general users after at most 6 months from deposition . at the end of the submission procedure , the user interface allows the model(s ) to be searched by protein or organism name , protein accession number ( in the nr database ) , author , pmdb model identifier , model type ( i.e. a complete coordinate set indicated by ts or an alignment to a known structure indicated by al ) . search results are displayed in the form of a table , listing the records satisfying all selected criteria ( figure 1 ) . each row refers to a target sequence and related models , along with summary information . every model that is not on hold can be downloaded or displayed through the 3d visualization program rasmol ( 20 ) . immediate future plans for the database include the possibility of using uniprot identifiers ( 21 ) for the protein targets and to perform more sophisticated searches . we also plan to add provisions for evaluating the models , other than the simple stereochemical checks performed at present , using tools such as whatcheck ( 22 ) , verify3d ( 23 ) and prosa ( 24 ) as well as tools to automatically evaluate the quality of models of proteins the structure of which is subsequently solved ( 25 ) . this will permit , in the future , to analyse the correlation between the actual quality of the models with the reliability values assigned by the authors and with those estimated by automatic verification tools . when a user uploads a model , its amino acid sequence is automatically retrieved from its coordinate file . residues for which coordinates are not available in the pdb model file , if any , can be manually inserted .
the protein model database ( pmdb ) is a public resource aimed at storing manually built 3d models of proteins . the database is designed to provide access to models published in the scientific literature , together with validating experimental data . it is a relational database and it currently contains > 74 000 models for 240 proteins . the system is accessible at and allows predictors to submit models along with related supporting evidence and users to download them through a simple and intuitive interface . users can navigate in the database and retrieve models referring to the same target protein or to different regions of the same protein . each model is assigned a unique identifier that allows interested users to directly access the data .
bone scintigraphy is a commonly used investigation for work - up of bone metastasis in patients with known malignancy . recently , introduction of hybrid single photon emission tomography / computed tomography ( spect / ct ) and its use in bone imaging has been shown to improve specificity of bone scintigraphy . we here present a case where non - ossifying fibroma ( nof ) was masquerading as metastasis on bone scintigraphy in an osteosarcoma patient . the present case report is about a 14-year - old male patient with parosteal osteosarcoma of the right proximal femur , who had undergone surgical resection with intramedullary nailing . at routine follow - up 16 months later , recurrence was noted at local site . the patient also gave a complaint of pain around the right knee . to rule out metastasis or infection three phase tc - methylene diphosphonate bone scintigraphy was carried out [ figure 1 ] . it showed mildly increased flow [ figure 1a , arrow ] and pool [ figure 1b and c , arrow ] activity in the region of right lower femur . delayed image revealed focal tracer uptake in lower shaft of right femur [ figure 1d and e , arrow ] . to characterize this lesion spect / ct was performed . ct [ figure 1f - h ] and spect / ct [ figure 1i - k ] images showed a sharply demarcated , asymmetrical , multiloculated , cortical based radiolucent lesion with sclerotic margins , located in the right femoral metaphysis and showing increased radiotracer uptake ( arrow ) . in addition , local recurrence was seen in right proximal femur [ figure 1d and e , broken arrow ] and no other site of skeletal metastasis was seen . as the nof was asymptomatic , it required no treatment . three phase 99mtc - methylene diphosphonate bone scintigraphy images of the patient . mildly increased flow ( a , arrow ) and pool ( b and c , arrow ) activity is seen in the region of right lower femur . on delayed image focal tracer uptake is seen in the lower shaft of right femur ( d and e , arrow ) . also noted was local recurrence in right proximal femur ( d and e , broken arrow ) . no other site of skeletal metastasis was seen . to characterize the lower femoral lesion single photon emission tomography / computed tomography ( spect / ct ) ct ( f - h ) and spect / ct ( i - k ) images showed a sharply demarcated , asymmetrical , multiloculated , cortical based radiolucent lesion with sclerotic margins , located in the right femoral metaphysis and showing increased radiotracer uptake ( arrow ) . furthermore , there is increased tracer uptake in the right lower limb joints proximal and distal to the femoral lesion - suggestive of associated sympathetic hyperactivity ( d and e ) nof are benign lesions , usually arising from the metaphysis of long bones of lower extremity . these are considered no touch lesions and treatment is observation . on three - phase bone scintigraphy appearance of nof is variable and could either have hyperemia or increased uptake on delayed phase . due to this non - specific appearance on bone scintigraphy , planar imaging appearance of nof can be confused with metastasis or infection . spect / ct has been shown to be useful for correctly characterizing various bone lesions seen on bone scintigraphy . as the ct appearance is pathognomonic of nof spect / ct might prove to be very useful for this purpose in nof . in the present case addition of spect / ct was able to correctly identify the lower femoral lesion as nof thereby avoiding unnecessary biopsy , as well as it ruled out bone metastasis . surgical excision is indicated only for lesions with concomitant or high risk of pathological fracture and very rarely for nof producing phosphaturic peptides , thereby causing tumor induced osteomalacia .
non - ossifying fibromas ( nofs ) are benign bone lesions with variable appearance on bone scintigraphy . single photon emission tomography / computed tomography ( spect / ct ) can help in accurate characterization of these lesions . we present a case of 14-year - old boy with recurrent osteosarcoma where nof was mimicking distant metastasis on 99mtc - methylene diphosphonate bone scintigraphy . spect / ct was able to correctly characterize the lesion as nof , thereby altering the management .
the recent tragic events unfolding in western africa have sparked a renewed interest in a deadly yet mysterious disease . the genus ebolavirus , known to cause ebola hemorrhagic fever , is a group of rna viruses that derives its name from the river next to which it was discovered . it is a member of the filovirus family and encompasses 5 species : zaire ebolavirus , sudan ebolavirus , bundibugyo ebolavirus , ta forest ebolavirus , and reston ebolavirus.1,2 while human - to - human transmission is known to occur through exposure to body fluids , the natural reservoir is most likely one or multiple species of fruit bats . exposure to and consumption of these bats as well as to nonhuman primates may be the method of animal - to - human transmission.1,3 as of august 2014 , the reported number of cases has reached 3,069.4 ebola hemorrhagic fever is known for its varied constellation of severe signs and symptoms . after an initial incubation period of 221 days , fever , extreme asthenia , diarrhea , nausea , vomiting , anorexia , abdominal pain , headaches , arthralgias , myalgia , and/or back pain may begin.5 fever is by far the most prevalent sign of active disease , occurring early and in as many as 94.1% of patients.6 however , ocular manifestations are also important to identify because they may aid in the diagnosis . ocular signs have been observed in both the acute and late settings.5,7 conjunctival injection has been reported in as many as 58% of infected patients and often presents bilaterally.8 subconjunctival hemorrhage as well as excessive lacrimation have been reported as well.7,9 the pathophysiology behind these manifestations is unclear . poor access to ophthalmic equipment and experienced practitioners as well as the danger posed by close - quarter examinations has limited the level of investigation.7 however , conjunctival injection and subconjunctival hemorrhage are easily recognizable , which makes the ophthalmic examination a rapid and simple method of identifying patients who are in need of further screening measures such as quantification of body temperature . ultimately , serologic testing ( viral rna or viral antibodies ) is necessary to confirm the diagnosis of ebola hemorrhagic fever.10 without this , patients with more common febrile illnesses such as malaria may be isolated and may contract ebolavirus if in close quarters with infected individuals.11 subacute / chronic ophthalmic manifestations are also of importance . patients who have been infected with ebolavirus follow one of two pathways : they may rapidly progress to a more intense hemorrhagic state where death is nearly inevitable or they may enter a convalescent phase . interestingly , patients who enter this phase are at risk for uveitic episodes . in a study documenting findings of the 1995 outbreak in what is now the democratic republic of the congo , 15% ( three patients ) of patients who entered the convalescent phase developed uveitis . each patient developed a unique variation of uveitis including anterior uveitis , posterior uveitis , and panuveitis . despite the complexity of findings , treatment required only topical steroids and cycloplegia . inflammation was quelled , but long - term sequelae were not investigated.7 further inquiry is necessary to identify whether this is in fact a causal relationship or simply an association . the current ebola hemorrhagic fever outbreak involves only the zaire ebolavirus species , and it has infected more people than any previously recorded outbreak ( figure 1 ) . in addition , it demonstrates a unique and perplexing geographic distribution ( figure 2 ) . these factors , in combination with the threat of transcontinental spread of the disease , make it increasingly important for medical professionals in every arena to become familiar with its signs and symptoms . those in the ophthalmic community may come in contact with patients who complain of fever as well as ocular signs such as injection and subconjunctival hemorrhage . if patients have a recent history of international travel or other risk factors , they should be transferred to a tertiary care center for serologic testing . topical steroids and cycloplegia have been proven to be effective , but further investigation of the natural history and long - term effects of this manifestation are warranted .
ebola hemorrhagic fever is a deadly disease caused by several species of ebolavirus . the current outbreak of 2014 is unique in that it has affected a greater number of people than ever before . it also has an unusual geographic distribution . nonspecific findings such as fever and generalized weakness have traditionally been very common early in the acute phase . ophthalmic manifestations have also been reported in significant numbers . conjunctival injection has been identified in both the acute and late phases . subconjunctival hemorrhage and excessive lacrimation have also been reported . various forms of uveitis have been associated with the convalescent phase of the disease . when identified in conjunction with other signs such as fever , acute findings such as conjunctivitis may contribute to the diagnosis of ebola hemorrhagic fever . ideally , serologic testing should be performed prior to isolation and treatment of these individuals . considering the prevalence of the current outbreak and the threat of transcontinental spread , ophthalmic health professionals need to be aware of the ocular manifestations of ebola hemorrhagic fever as well as the associated signs and symptoms in order to prevent further spread .
gastrointestinal ischemia arises due to a sudden reduction in intestinal blood flow , and it most commonly occurs due to mesenteric thrombosis , vasculitis and torsion of the intestine . in occlusive conditions , the collateral circuit of the intestine is occluded , which can be seen due to traumas , surgery on aorta or pelvic arteries , or can be caused by thrombosis / embolus in the mesenteric arteries . non - occlusive conditions are characterized by reduced flow , often due to hypotension such as septic or hypovolemic shock . ischemic colitis accounts for 5060% of cases of gastrointestinal ischemia and is associated with a high morbidity and mortality . in contrary , the rectum is only affected in 25% , a result of rich collateral blood supply of the rectum . symptoms include abdominal pain , gastrointestinal bleeding , diarrhea ( 68% ) , abdominal distention ( 63% ) and nausea / vomiting ( 38% ) . the disease can even be transient due to non - occlusive disorders , which makes it difficult to find the exact etiology of the ischemia and diagnosis may be delayed while exploring more common etiologies of the symptoms . risk factors for development of intestinal ischemia include age > 65 years , hypertension , renal failure , diabetes , history of irritable bowel disease , chronic obstructive pulmonary disease , constipation and atherosclerosis [ 68 ] . in this case report , we describe a rare case of ischemic proctosigmoiditis due to retroperitoneal hematomas in a patient with a pelvic fracture . an 87-year - old woman was admitted to the emergency unit due to abdominal pain and diarrhea . her previous medical history included prior to admission , the patient had fallen at home and was complaining about dizziness , lower abdominal pain , watery stool for the past 24 h and right - sided pelvic pain . initial assessment revealed following vital parameters : blood pressure 122/50 mmhg , pulse 63/min , respiratory rate 20/min , saturation 90% on room air and temperature 35.5c . during physical examination , the patient complained of tenderness over the pubic bone and lower abdomen , while no peritoneal reaction was found . biochemical measures revealed : hemoglobin 6.5 mmol / l ( 7.39.6 ) , white blood cell count 10.6 10/l ( 3.58.8 ) , platelets 102 10/l ( 145390 ) , creatinine 196 mol / l ( 5090 ) and c - reactive protein 29 mg / l ( < 8) . x - ray of the pelvic region revealed a right - sided nondisplaced pubic ramus fracture . after 9 h , the arterial blood gas was repeated and showed ph 7.42 and lactate 2.7 . due to near normalization of blood gas parameters upon i.v . fluid administration , the pelvic fracture was believed to be caused by a fall due to severe dehydration and diarrhea . twenty - one hours after admission , the patient s condition worsened and she was complaining of severe abdominal pain . a suspicion of intestinal ischemia arose , and without further radiologic examination , the patient was taken to the operating room for exploratory laparotomy . intra - operatively , necrosis of the sigmoid colon and proximal part of rectum were detected . additionally , another 10 cm area of non - vital ileum was detected 20 cm from the ileocecal region , which were adherent to the rectosigmoid junction . retroperitoneal hematomas were detected in the pelvic region , while no hematoma was identified in proximity to the inferior mesenteric artery . all non - vital intestinal segments were resected , and ileostomy and sigmoideostomy were performed . only minor bleeding was encountered during the procedure . postoperatively , the patient had severe sepsis and was kept at the intensive care unit . however , she recovered and was discharged 10 days after admission . histopathology of the resected tissue showed necrosis due to ischemia without any sign of vasculitis or thrombosis ( fig . 1 ) . we report a rare case of acute ischemic proctosigmoiditis where the origin of ischemia is difficult to establish . previously , only a few cases of ischemic proctitis have been reported in patients with pelvic fractures due to high - energy traumas , but no previous reports on ischemic proctosigmoiditis have been reported . the blood supply to the sigmoid colon arises from the inferior mesenteric artery through the sigmoid artery and its end branch , the superior rectal artery . the rectum has a rich blood supply which originates from the superior rectal artery and the middle and distal rectal artery , branches of the paired internal iliac artery . in addition , there is abundant collateral blood supply to the distal colon and rectum from the marginal artery of drummond that links the superior and inferior mesenteric arteries . in this case , we report ischemia of the distal part of sigmoid colon and proximal part of rectum . however , this patient had increased age , known hypertension and cardiovascular disease that could explain the increased susceptibility to ischemia . we assume that the origin of ischemia in this case was a pelvic fracture that caused retroperitoneal hematomas resulting in a local increase in retroperitoneal pressure and obstruction of the blood supply to the affected part of rectum and colon sigmoideum . possibly , early computed tomography imaging could have identified an expanding retroperitoneal hematoma and thus allowed for embolization by interventional radiologists . ischemia of colon and rectum are serious conditions associated with high morbidity and a mortality of 40% in patients with transmural necrosis of the rectum . our case illustrates that ischemia of the intestinal wall can arise due to pelvic fractures in patients with risk factors for vessel disease , and clearly states the difficulty in placing the correct diagnose of gastrointestinal ischemia .
gastrointestinal ischemia is caused by ischemic colitis in 5060% of cases and is associated with high morbidity and mortality among patients . ischemic proctosigmoiditis is a very rare disorder with only few cases reported . due to collateral blood supply the rectum is only affected in 25% of all cases of ischemic colitis . we report a rare case of ischemic proctosigmoiditis caused by a retroperitoneal hematoma due to a pelvic fracture .
a 68-year - old male presented with grade 4 nuclear cataract with visual acuity of 20/200 in the left eye . he had best corrected visual acuity of 20/200 in the right eye and examination of that eye revealed normal pseudophakia with age - related macular degeneration . stretch pupilloplasty was done for rigid pupil and intraoperative iris trauma was also recorded . at the end of surgery , hydration of the main incision and side ports was done . on first postoperative follow - up , inferior half corneal edema with descemet 's detachment from inferotemporal side port extending to lower one - third of cornea was noted . but persistent descemet 's detachment and corneal edema was noted on second and third postoperative day . 1.4% sodium hyaluronate was injected after which he developed an intraocular pressure ( iop ) of 40 mm of hg with persisting descemet 's detachment . on the fourth postoperative day , anterior chamber wash to clear viscoelastic was done and 0.2 ml of perfluro - n - octane was injected as a single bubble using a 26-gauge needle introduced through superotemporal peripheral cornea . the patient was given immediate head end elevated position which he was advised to maintain for 2 weeks . from the fifth postoperative day onward , the descemet 's membrane was found attached . initially , the area of descemet 's detachment was seen as faintly thickened [ fig . 1 ] which resolved at 1 week after perfluro - n - octane injection [ fig . 2 ] . fifteen days after perfluro - n - octane injection , the bubble was aspirated and thorough anterior chamber wash was given . at no point in follow - up after perfluro - n - octane injection , elevated iop or uveitis was noted . at 6 weeks the central corneal thickness by ultrasonic pachymetry was 517 m before phacoemulsification , 782 m before intervention with perfluro - n - octane and 520 m after removal of perfluro - n - octane . specular microscopy was performed at another center at 6 months follow - up with endothelial cell density of 2639 cells / mm in the right eye and 2481 cells / mm in the left eye . perfluro - n - octane bubble in anterior chamber with the area of descemet 's detachment seen as faint thickening . the superior extent of the area of descemet 's detachment is highlighted by the arrow heads haziness in the area of descemet 's detachment has cleared by 1 week . perflurocarbon liquids bubble is seen in anterior chamber one - month follow - up showing clear cornea with no evidence of descemet 's detachment descemet 's detachment can be instrument induced ( scroll like ) or due to injection of fluid ( attached to stroma all around the detachment ) . descemet 's membrane detachment can also be classified into planar type with the descemet 's membrane separation of less than 1 mm and non - planar type with a separation of greater than 1 mm . planar detachments are more likely to resolve spontaneously , and non - planar detachments should be repaired early . small localized descemet 's detachment usually resolves spontaneously , but large one needs intervention in the form of intracameral air , c3f8 or viscoelastic . since air or gas floats up , inferior descemet 's detachment extending to periphery of cornea may not get attached as in our case . perflurocarbon liquids ( pfcls ) were initially developed as blood substitute for their property to transport oxygen and their biologic inertness . pfcl is used because of high specific gravity and immiscibility with water for complex retinal procedures like giant retinal tear surgery . few reports about retained pfcl in anterior chamber after vitreo - retinal surgery are available in literature , which suggest inflammatory response and corneal toxicity after prolonged exposure of several months . toxicity with pfcl occurs due to hydrogen containing compounds , unsaturated carbon bonds and impurities like compounds with nitrogen bonds and hydrogen fluoride containing compounds . , based on their in vitro study on the effect of perflurodecalin on human retinal pigment epithelium and corneal endothelium , suggest the damage is caused by mechanical effect on cell function by impeding the normal metabolic exchange . caution against the use of pfcl for short- or long - term retinal tamponade in aphakic eyes considering the anterior segment toxicity of pfcl . but weinberger et al . followed up seven eyes with retained pfcl in anterior chamber for a mean follow - up of 9.4 months and showed that retained pfcl in anterior segment did not cause corneal toxicity or ocular inflammation . we have observed a patient with retained perfluro - n - octane bubble in anterior chamber following dislocated nucleus removal via anterior chamber for 3 months without inflammation or elevated iop ( not reported ) . based on the personal experience of that case , we decided to manage this patient with pfcl ( perfluro - n - octane ) . the difference in endothelial cell density between the two eyes was 158 cells / mm . in routine cataract surgery , an endothelial cell loss of 210% , and in complicated cataract surgery a loss of 1620% is possible . in the case being reported here , the difference in specular count between the two eyes was less than 10% and this loss can be explained by the descemet 's detachment as well as by the repeated manipulations . the central corneal thickness almost approximated the pre - operative thickness after removing pfcl though the pfcl bubble was in contact with nearly 50% of central cornea . hence , we consider that perfluro - n - octane was effective in attaching inferior descemet 's detachment and safe in anterior chamber for a period of 2 weeks in this patient intervened by us . this is the first case of inferior descemet 's detachment managed by intracameral pfcl to be reported as far as our knowledge is concerned ( pubmed key words used were descemet 's detachment + cataract surgery , repair of descemet 's detachment , descemet 's detachment + perfluro - n - octane , descemet 's detachment + pfcl , intracameral pfcl ) .
we report the case of a 68-year - old male who developed descemet 's membrane detachment after temporal clear corneal phacoemulsification which did not settle with air or viscoelastic injection . the descemet 's membrane was successfully reattached with restoration of 20/50 vision with the help of perfluro - n - octane liquid . to our knowledge , this is the first such case to be reported .
it is first described by malherbe and chenantais in 1880 as a calcified tumor originating from sebaceous glands and named as pilomatrixoma by forbis and helwig in 1961 . it is usually localized in the head and neck region and also on upper extremities . lesions located on the skin are usually bluish or reddish in color and could morphologically vary in bullous , anetodermic or perforated masses . recently , there has been only one dermoscopic study , which had focused on diagnosis of over skin located lesions . in this article , a 67-year - old female patient has been complaining of a painless , reddish mass on her right cheek . on dermatological examination , a nodular , well - marginated , and reddish lesion of 2 cm 2.5 cm in diameter was identified . dermoscopic evaluation demonstrated yellowish lobules on an erythematous background , surrounded by crown - like branching vessels [ figure 1a ] . ( a ) dermatoscopy showing yellowish lobules surrounded by crown like vessels ( gross tumor is seen in the right - upper corner of image , 10 ) . ( b ) bluish - white fishing net - like streaks is seen the entire image ( inset of gross tumor in the right - upper corner of image , 10 ) . ( c ) in the dermoscopic view , there are polymorphous vessels ( predominated linear irregular vessels ) , bluish areas ( left - middle side of image ) and ulcerations ( inset of gross tumor in the right - upper corner of image , 10 ) a 48-year - old male patient had a reddish nodule on his left eyebrow which had been growing slowly by 2 months . reddish , 1 cm 1 cm sized nodule with hairy surface was detected on the medial side of left eyebrow . dermoscopic examination revealed erythema and bluish - white streaks like a fishing net [ figure 1b ] . a 58-year - old male patient admitted with a reddish nodule on upper left side of the nose . physical examination revealed a reddish nodule on upper left side of nasal dorsum with an ulcerated area on its surface . dermoscopic examination demonstrated erythema , linear irregular vessels , hairpin like vessels , comma like vessels , ulceration , and bluish areas [ figure 1c ] . a 67-year - old female patient has been complaining of a painless , reddish mass on her right cheek . on dermatological examination , a nodular , well - marginated , and reddish lesion of 2 cm 2.5 cm in diameter was identified . dermoscopic evaluation demonstrated yellowish lobules on an erythematous background , surrounded by crown - like branching vessels [ figure 1a ] . ( a ) dermatoscopy showing yellowish lobules surrounded by crown like vessels ( gross tumor is seen in the right - upper corner of image , 10 ) . ( b ) bluish - white fishing net - like streaks is seen the entire image ( inset of gross tumor in the right - upper corner of image , 10 ) . ( c ) in the dermoscopic view , there are polymorphous vessels ( predominated linear irregular vessels ) , bluish areas ( left - middle side of image ) and ulcerations ( inset of gross tumor in the right - upper corner of image , 10 ) a 48-year - old male patient had a reddish nodule on his left eyebrow which had been growing slowly by 2 months . reddish , 1 cm 1 cm sized nodule with hairy surface was detected on the medial side of left eyebrow . dermoscopic examination revealed erythema and bluish - white streaks like a fishing net [ figure 1b ] . a 58-year - old male patient admitted with a reddish nodule on upper left side of the nose . physical examination revealed a reddish nodule on upper left side of nasal dorsum with an ulcerated area on its surface . dermoscopic examination demonstrated erythema , linear irregular vessels , hairpin like vessels , comma like vessels , ulceration , and bluish areas [ figure 1c ] . although it is widely used in diagnosis of melanocytic lesions , nonmelanocytic structures have also been observed recently . nonvascular , irregular , whitish formations ( 80% ) and streaks ( 70% ) were commonly observed . reddish homogenous zones ( 90% ) , hairpin like vessels ( 70% ) , linear irregular vessels ( 70% ) and dotted vessels ( 70% ) were identified as vascular structures . reported two studies which comprised of keratoacanthoma and squamous cell carcinoma patients and emphasized whitish unstructured zones on dermoscopy . in addition , white rings , targetoid hair follicles , central keratin accumulations and hairpin vessels are more likely to be seen in squamous cell carcinoma and keratoacanthoma . milky pink zones , linear irregular vessels and branching vessels were also demonstrated in merkel cell carcinoma . in case 1 , yellowish - white zones were surrounded by crown like shaped branching vessels . although streaks had n't been reported in cases out of pilomatrixoma , they could be seen in figures of some articles about merkel cell carcinoma . linear irregular vessel and two articles about merkel cell carcinoma which comprised of 10 and two cases also demonstrated these linear irregular vessels in all subjects . hairpin like vessels on the other hand , are commonly seen in nonmelanocytic lesions such as seborrheic keratosis , keratoacanthoma and squamous cell carcinoma . yellowish - white structures together with streaks , linear irregular vessels and hairpin like vessels are supportive dermoscopic findings of pilomatrixoma those are located on the skin however this is not enough for definitive diagnosis .
pilomatrixoma is a benign soft tissue neoplasm originated from follicular matrix of hair and is also known as malherbe 's calcified epithelioma . some lesions are located on the skin and are usually misdiagnosed . a small number of patients had been dermoscopically examined . in this article , three patients of pilomatrixoma who had three different dermoscopic views were evaluated and discussed in concordance with the literature .
it is extremely rare for the spinal cord displaced out of the dura through a dural defect , which is known as a spinal cord herniation ( sch ) . the classification of the sch is known as traumatic , iatrogenic , and idiopathic according to the causes of dura defect6 ) . among those , surgical treatment of sch is usually followed by stabilization or improvement in the neurological symptoms , but still unfavorable postoperative outcomes are also reported3 ) . the release of the herniated portion of the spinal cord and subsequent repositioning of the cord into the normal anatomic position is advocated as essential . several surgical methods are introduced , such as patch closure after enlargement of the ventral dura defect and closure of the defect by direct suture12 ) . we treated a patient with spontaneous herniation of the midthoracic spinal cord through a dura defect manifesting paraparetic movement with decreased proprioception and sensory change . a 66-year - old female presented paraparesis with progressive gait disturbance and falling tendency to right side for 3 years . both sides of her quadriceps muscles and hamstring groups were atrophic , and unhealed scar on her right anterior lower leg was identified due to repeated fall . magnetic resonance imaging ( mri ) scan revealed anterior displacement of the spinal cord at t4-t5 vertebral level ( fig . intradural exploration of the spinal cord revealed dura defect about 25mm8 mm in size ( fig . spinal cord was fully released under microscope and dura defect was sealed with lyoplant ( aesculap inc . , motor power improved to nearly normal state 3 days after operation , so as radiating pain on both legs . neurogenic claudication also improved from 5 to 50 m , but touch and temperature sense under t6 level had been unchanged . among several different types of schs , spontaneous sch , which is an extremely rare disease , was firstly introduced by wortzman et al.10 ) , and increasing number of cases have been reported since then . middle aged - women have more tendencies to have the disease in the third and eighth thoracic level2 ) . the patient of our case report was in middle age and had shown cord herniation with dura defect at t4-t5 level , which certainly matches with the characteristic of the disease epidemiology . theoretically , the microscopic erosion of dural surface caused by repeated flexion and extension ; secondary dural damage due to arachnoid cyst or other congenital diseases ; or inflammatory change of dura by herniated discs could result in the incarceration of spinal cord , yet the pathophysiology of the formation of dural defect is still not clarified1 ) . in addition , thoracic sch could be closely related to the cardiopulmonary movements which cause the tissue adhesions or inflammatory reactions around the thoracic cord , so that it could finally lead to progressive pathological alteration of thoracic spinal cord18 ) . in our case , the dura defect located at the disc level of thoracic spine with clean margin , from which could be assumed that the defect could be formed spontaneously by degenerative change . according to groen et al.2 ) , progressive brown - sequard - like syndrome was present in 66% and paraparesis in 30% among 129 cases of anterior thoracic sch patients . the 75.9% of the patients with brown - sequard syndrome showed improvement after surgical treatment while only 55% of the paraparetic patients improved2 ) . most of the patients showed progressive aggravation of the symptom , so eventually it led to the surgical treatment . the mean operative interval was 5.2 years while our patient suffered from the progressive paraparetic symptom with defect of proprioception for 3 years before treatment , which had improved three days after the surgery . the displacement of spinal cord through dural defect could be confirmed at either t1 or t2 weight images . it could also be diagnosed with mri showing transdural cord herniation at t4/5 disc level in left anterior potion of dura in our case . no other radiologic evaluation was done for our patient except for simple x - ray of thoracic spine . additionally , computed tomography ( ct ) myelogram or simple ct also could help clarify the presence of cystic mass1 ) . surgical procedures introduced so far are the closure of dura defect by direct suture25 ) and the filling the dura defect by flat graft57 ) . direct closure is considered to be necessary to prevent postoperative cerebrospinal fluid collection and achieve realignment of spinal cord . also , promoters of this strategy state the effective prevention of recurrence of sch . on the other hand , using graft technique is believed to be safer and shows better outcome because of less cord manipulation than direct suture during operation . the patient in this case had relatively large size of dura defect with 25mm8 mm at the anterior portion of the spinal cord , so the graft technique was preferred because the overmanipulation of the spinal cord would be inevitable when directly sutured . because the incidence rate of the diagnosis of spontaneous anterior thoracic cord herniation has been increasing due to the frequent use of mri as diagnostic method , there would be more chance of encountering cord herniation patient of when diagnosing patients . moreover , most of the patients with surgical procedure showed satisfying results , which leads us to take surgical treatment into account when we encounter the patient with sch2 ) . patients showing myelopathic symptoms such as brown - sequard - like or paraparetic movements without traumatic events should be differentially diagnosed with sch . even though it is known to be rare , when a patient is diagnosed with the spontaneous anterior thoracic sch , a surgical approach could be considered as a primary treatment .
thoracic spinal cord herniation is a rare disease cause of progressive myelopathy . magnetic resonance image is a useful tool to diagnose preoperatively . operation is a treatment of option . sixty - six - year - old female visited dong - a university medical center for progressive gait disturbance with falling tendency to right side . she had radiating pain and tingling sense on both leg . sense of touch and temperature was decreased below t6 level . both hip and knee motor power were grade iv . magnetic resonance imaging scan showed anterior displacement of the spinal cord at t4-t5 vertebral level . under the diagnosis of thoracic spinal cord herniation with dura defect , operation was performed for the patient with intraoperative neuromonitoring . laminectomy at t4 and t5 level was done , and intradural exploration of the spinal cord revealed dura defect about 25mm8 mm in size . spinal cord was released under microscope and dura defect was repaired with lyoplant . the patient 's symptom improved after the surgical procedure , but touch and temperature sense under t6 level had unchanged .
dirofilaria is a species that are natural parasites of dogs , cats , and wild mammals . pulmonary lesions can form from d. immitis , whereas d. repens and d. tenuis cause ocular lesions . zooanthrophilic mosquitoes like culex , aedes , and anopheles have been regarded as vectors for dirofilariasis . microfilariae get transmitted from the blood of wild and domestic animals such as the dog and cat to humans by infected mosquito bites . humans are terminal host and the parasite will not produce any microfilariae in humans . human infection with dirofilaria repens is comparatively rare in india ; however , several cases have been reported in last few years , hence a proper knowledge of clinical pathological and microbiological features will help in prompt diagnosis and a proper management can be procured by the patient . we report a case of a 64-year - old female with subcutaneous dirofilariasis in the infraorbital region from kerala , south india . a 64-year - old female patient reported with a swelling on the left side of the face . swelling was noticed 6 months back and over the past week there had been a mild reduction in the size of the swelling . however , she was not on steroid inhaler for treatment of asthma . considering the zoonotic infections , she was asked about any contact with domestic animals or pets and the presence of mosquitoes in the area where she lived . however , she was not keeping any pets , but was living in a mosquito infested area . hematological examination revealed no abnormalities and chest x - ray was normal in appearance . on examination , a bony hard swelling was noticed on left infraorbital region measuring 3 2 cm in dimension and was non - tender on palpation . ( a - c ) clinical appearance showing swelling in the left infraorbital margin , and usg and ct appearance showing heterogeneous mass in the facial muscle over left maxilla both ultrasonography ( usg ) and computed tomography ( ct ) scan suggested a parasitic infection with granuloma formation in facial muscle over left maxilla with diffuse edema of adjacent soft tissues [ figure 1b and c ] . surgical excision of nodular mass was done and histopathological report was obtained [ figure 2a ] . multiple sections of fibrofatty tissue with dense inflammatory cells and foreign body giant cells were noted . cut section of filarial worm with thick laminated cuticle was also noted [ figure 2b ] however , we failed to attain a full worm as most of the dead worm got necrosed . ( a and b ) specimen and histopathology showing cut section of filarial worm with thick laminated cuticle and multiple sections of fibrofatty tissue with dense inflammatory cells and foreign body giant cells d. repens , d. immitis , d. tenuis , and d. ursi are some of the species commonly involved in human infections . there has also been reports of dirofilariasis from other countries like france , greece and israel . in india , d. repens is a natural parasite of carnivorous animals , primarily dogs , foxes and cats . in usa subcutaneous dirofilariasis normally affects the eyelids and periorbital region and rarely can occur subconjunctivally also . the most common symptoms found in dirofilariasis are localized pruritis , pain , and swelling . edematous , hyperemiac conjunctiva , with a sensation of movement under the skin or conjunctiva has also been reported . allergic reactions with fever , urticaria , and facial edema are reported as other symptoms . in a majority of instances parasites longitudinal ridges of d. repens are broader with less distinctly raised and it appears branched . microfilarias have never been reported in humans . in this case also repeated blood smears were negative for microfilaria and no eosinophilia reported . dirofilaria are identified by their thick laminated cuticle , broad lateral ends and large muscle cells . the length of female may vary from 8 to 13 cm and males from 4 to 4.8 cm . though the worms with external longitudinal cuticular ridges are taken as d. repens however , d. immitis can be differentiated from d. repens by absence of ridge in d. immitis . in our case also histopathological examination revealed a thick cuticle in the cut section of worm . surgical removal of the worm is the definitive choice of treatment . in our case also dirofilariasis is a rare zoonotic disease and only a few cases have been reported in india .
dirofilariasis is a parasitic infection transmitted by mosquitoes from domestic and wild animals . humans accidentally get infected and can present with a lung nodule , subcutaneous mass or an ocular lesion which will be either subconjunctival or periorbital . proper microbiological and histopathological examinations aid in proper diagnosis . herein , we present a case report of a 64-year - old female patient from kerala with an infraorbital mass diagnosed as dirofilariasis .
a 67-year - old male patient had undergone esophagectomy and intrathoracic esophagogastrostomy for carcinoma of the lower thoracic esophagus 27 months earlier . the original pathologic condition was determined to be moderately differentiated squamous cell carcinoma invading the lamina propria without lymph node metastasis ( clinical t1bn0m0 ) . he had undergone follow - up chest computed tomograph and esophagogastroduodenoscopy once or twice a year and a gastric carcinoma at the intra - abdominal part of the gastric conduit was found . the tumor was a 2a+2c type lesion and located in the posterior wall of the antrum . because of its morphology ( flat and depressed type with ulceration ) , size ( more than 10 mm ) and poor localization , endoscopic mucosal resection or partial gastric resection was not suitable ( fig . 1 ) , so we decided to perform a total resection of the gastric conduit . the whole procedures were performed in 3 stages : first thoracic , second abdominal , and third thoracic and abdominal approaches . because of previous intrathoracic esophagogastrostomy , it was necessary to check the intrathoracic condition and operability . the esophago - gastric anastomosis was located at the upper part of the paratracheal area ( figs . 2 , 3 ) . after dissecting the adhesions between the pleura , lung , and gastric conduit with blunt and sharp procedures , we resected the gastric conduit at the level of the previous anastomosis . the intrathoracic portion of the esophagus was about 4 cm segment long , so we saved it as long as possible and inserted the 28 mm anvil into it . the antral portion of the stomach was adhered to the diaphragm , liver , and surrounding structures . the length and vasculatures of the transverse and descending colon were considered to be suitable for an esophageal conduit . the left side of the colon with a pedicle of the left colic artery was prepared for esophageal reconstruction . the proximal sigmoid colon was connected ( end to side ) to the proximal part of the transverse colon with a 28 mm end - to - end anastomosis ( eea ) stapler . after the left - sided colon conduit into the thoracic cavity in the isoperistaltic manner , esophago - colonostomy was done with a 28 mm eea stapler . the distal part of the colon conduit was anastomosed at the side of the proximal jejunum with a 25 mm stapler in the manner of roux - en - y anastomosis . on the 10th postoperative day , an esophagogram showed no leakage or stenosis of the passage ( fig . the patient has received follow - up care for 7 months after surgery without any problems . after warren and gates reported on it in 1932 , the concept of multiple primary malignancies has been accepted widely and is supported by hypothesis of field cancerization . synchronous or metachronous multiple primary malignancies in esophageal cancer have since been reported with a frequency of about 10% . gastric conduit cancer is not only cancer arising in the remnant gastric conduit but also the second primary cancer following esophageal cancer . the incidence of gastric conduit cancer has been reported to be 0.2%-3.5% [ 2 - 4 ] . although the etiology of the secondary carcinoma in the gastric conduit is not well known , gastric conduit cancer has been reported increasingly because of survival prolongation of esophageal cancer patients after surgery . in our hospital , we have annually operated on about 30 esophageal cancer patients with gastric conduit and this surgical management of gastric conduit cancer was our first experience . because thoracotomy with adhesiolysis is necessary for performing a reoperation , total resection of the gastric conduit reconstructed via the posterior mediastinal route is very difficult . in addition , it is more invasive because it requires reconstruction with another organ such as the colon , jejunum , or skin , for the conduit . therefore , it is important to detect the lesion at an early stage so as to treat it with minimally invasive surgery such as endoscopic mucosal resection or partial resection . therefore , careful , periodic endoscopic evaluation after operation in the patient with esophageal cancer is needed . we had performed annual esophagogastoduodenoscopy evaluation and other radiologic studies twice in a year . in conclusion , a careful , periodic , and endoscopic evaluation after surgery for esophageal cancer is needed for early detection of other primary malignancies . in addition , surgical treatment of gastric conduit cancer after esophageal cancer surgery was found to be possible and safe in our experience .
we report a very rare case of surgery on gastric conduit cancer . a 67-year - old male patient underwent esophagectomy and intrathoracic esophagogastrostomy for squamous cell carcinoma of the lower thoracic esophagus 27 months ago . upon follow - up , a gastric carcinoma at the intra - abdominal part of the gastric conduit was found on an esophagogastroduodenoscopy . we performed total gastrectomy and esophagocolonojejunostomy in the manner of roux - en - y anastomosis . the postoperative course was not eventful and an esophagogram on the 10th postoperative day showed no leakage or stenosis of the passage . the patient was discharged on the 17th day with no complications .
morgagni hernias result from failure of the septum transversum to fuse with the thoracic wall and occur between the sternal and costal diaphragmatic attachments in the anterior mediastinum . they are the least common of the four types of congenital diaphragmatic hernias and account for 23% of all cases [ 1 , 2 ] . they may cause a number of potential complications , the latter usually being obstruction , bowel strangulation and volvulus , and if they are symptomatic they can cause a certain amount of distress to the patient . most morgagni hernias are discovered on chest radiograph or upper gastrointestinal study performed because of non - specific complaints , or they are diagnosed incidentally when the patients are being investigated for unrelated problems . repair of the defect is indicated in all cases because of the risk of viscera obstruction or incarceration . reports in the literature describe repair of the abnormality by the transabdominal or transthoracic approach with or without a mesh [ 3 , 4 , 5 ] . since the last decade minimally invasive techniques have also been applied in the management of morgagni hernias , and laparoscopic repair with the use of a mesh is now widely applicable . our report presents an interesting case of bilateral morgagni hernia in an elderly patient with a history of progressive onset of shortness of breath and occasional cardiac arrhythmias . our case is a 68-year - old male patient working as a farmer , who was admitted to a peripheral hospital for elective hydrocele repair . the routine plain chest x - ray revealed pleural effusion and the patient was referred to our department for further investigation . he reported an intermittent history of breath shortness and occasional cardiac arrhythmias , especially after hard work . on physical examination blood pressure and heart rate were normal , but respiratory sounds were found to be diminished at the right basal region on auscultation . the right liver appeared to be normal , but percussion on its left lobe revealed flatulence , indicating the presence of bowel . bowel loops were herniated into the right thoracic cavity through a right side anterior diaphragmatic defect ( fig . the patient was operated electively and subjected to a transabdominal approach through a bilateral subcostal incision . there was a right anterior diaphragmatic hernia approximately 10 cm in diameter with the ascending colon , the majority of the transverse colon and a huge amount of omentum , all within the hernia sac ( fig . the hernia extended approximately one half of the way up to the apex of the right lung . there was a second defect of approximately 4 cm , but with no hernia , in the left side diaphragm . 3 ) and the hernia sac was easily mobilised into the peritoneal cavity and excised . 1 prolene , ethicon , usa ) , between the posterior edge of the defect and the posterior rectus sheath ( fig . 4 ) . there was no tension on the repair and there was no need for mesh implantation on either side . no drains were placed , the postoperative course was uneventful and the patient was discharged on the 5th postoperative day . four types of congenital diaphragmatic hernias have been described : hiatal hernia with a defect at the esophageal hiatus , paraesophageal hernia with a defect adjacent to the hiatus , bochdalek hernia with a defect posterolaterally , and morgagni hernia with the defect being anteromedial or retrosternal . each type requires its own closing technique , with prosthetic reinforcement often needed for larger defects [ 7 , 8 ] . in 1769 , morgagni described for the first time a diaphragmatic hernia that originated from the sternocostal trigone . about 90% of morgagni hernias are located on the right side , with 2% located on the left and 8% bilateral [ 9 , 10 ] . morgagni hernias usually remain asymptomatic in infancy and childhood , but these hernias may progressively enlarge and become symptomatic , especially in adulthood . exercise and excessive work can cause symptoms which are related to dyspnoea . patients may also show signs and symptoms of acute incarceration or strangulation if their abdominal contents become trapped into the hernia sac . our patient was mostly asymptomatic with an intermittent history of progressive onset of shortness of breath and occasional cardiac arrhythmias , especially after excessive work . depending on the contents of the hernia , omentum , stomach , small intestine , or liver , it can appear differently on chest radiography and the diagnosis can be missed . differential diagnosis should be made between intrathoracic tumour , atelectasis , pneumonia , or pericardial cyst . this may affect the decision to operate and the type of operation carried out , those being the transabdominal and the transthoracic approach . contrast examination carried out for gastrointestinal symptoms can be perfectly normal , but computed tomography scan can be considered to be an accurate , noninvasive method of diagnosing morgagni hernia . moreover it can establish a diagnosis if the hernia sac is empty or contains omentum or part of the liver . magnetic resonance imaging has been also used as a noninvasive modality to distinguish morgagni hernia from other mediastinal masses [ 11 , 12 ] . in our case , diagnosis was made by physical examination and plain chest radiogram and confirmed by computed tomography . computed tomography showed a large diaphragmatic hernia with air - filled colon and omentum in the right chest cavity . although the majority of these hernias are asymptomatic , repair is recommended to avoid future complications . the thoracic approach is preferably used in cases where there is a question as to morgagni hernia and other thoracic abnormalities , such as a mediastinal cyst or mass or a bronchogenic carcinoma [ 12 , 13 , 14 ] , although with the advances in preoperative imaging the technique is less frequently applied . the transabdominal approach is favoured when the diagnosis is certain , as it allows easier reduction of the hernia , especially for bilateral hernias . furthermore , abdominal viscera within the hernia can be easily pulled down to their normal location into the abdomen [ 4 , 5 ] . since the introduction of the laparoscopic approach , the method is increasingly applicable . the hernia sac can be viewed through the laparoscope , and the contents can be easily reduced once the peritoneum at the perimeter of the defect is incised . the sac is usually not removed and the defect is closed with sutures and reinforced with a mesh stapled onto the diaphragm . in our patient we preferred the transabdominal approach , and a bilateral subcostal incision was made . the reason for this decision was the accuracy of the preoperative diagnosis and the existence of a large defect . follow - up after operative repair can be done with a chest radiograph and computed tomography . as of this writing , we have follow up our patient with chest radiography and computed tomography for 15 months so far with no sign of recurrence .
we present a case of bilateral morgagni hernia in a 68-year - old male with an intermittent history of progressive onset of breath shortness and occasional cardiac arrhythmias . diagnosis was made by clinical examination and the findings in a plain chest radiograph and was confirmed by computed tomography scan . the patient was operated electively and subjected to a transabdominal approach . a bilateral subcostal incision revealed a large right side anterior diaphragmatic defect with a hernia containing the ascending colon , the majority of the transverse colon and a huge amount of omentum . also a second smaller defect was found on the left side with no hernia inside . after large bowel and omentum had been taken down to the peritoneal cavity , both defects were primarily closed using interrupted nylon sutures without the use of a mesh . the patient recovered very well , had an uneventful postoperative course and was released on the 5th postoperative day . 15-month follow - up failed to reveal any signs of recurrence .
a 78-year - old average - built man with previous history of coronary artery disease was hospitalized with chest pain of few hours duration . thorough investigation had revealed carotid hypersensitivity ( 3 second pause after right carotid massage ) and mixed cardioinhibitory and vasodepressor response to upright tilt . the atrial lead was guidant ( 4480 - 318341 ) passive - fixation bipolar electrode and the ventricular lead was guidant ( 4457 - 39232665 ) passive - fixation bipolar electrode . pacemaker interrogation 26 days prior to the present admission showed normal function of the atrial and ventricular leads . on the day of admission , the electrocardiogram demonstrated sinus rhythm , st - segment elevation in the inferior leads , st - segment depression in lead v2 , normal av conduction , and no pr - segment depression . pacer stimuli occurred at 65 bpm , uninhibited by the prevailing sinus rhythm [ figure 1 ] . the tracing demonstrates sinus rhythm at 68 bpm , normal conduction , inferior current of injury and st - segment depression in leads i , avl , and v2 . atrial pacer stimuli occur at 65 pulse per minute , uninhibited by the prevailing sinus rhythm , indicating lead sensing failure . atrial pacing at maximum output ( 7.5 v , 1.9 ms ) demonstrated no atrial capture ( data not shown ) . after catheterization , interrogation of the pacer demonstrated no measurable p - wave : at sensitivity of 0.15 mv the atrial lead detected only a far - field qrs . it failed to capture the atrium at the highest programmable output ( 7.0 v at 1.9 ms ) . the device was programmed to the rate - adaptive , ventricular inhibited ( vvir ) mode . forty - five days later , he returned to the pacer clinic complaining of effort intolerance and fatigue . interrogation revealed normal atrial sensing ( p - wave = 3.0 mv ) and capture ( threshold = 0.8 v at 0.4 ms ) . follow - up pacer interrogations 2 months and 8 months later demonstrated stable function of the atrial lead [ table 1 ] . right coronary angiogram in the lao cranial projection before ( a ) and after ( b ) angioplasty of the proximal occlusion . timi grade 0 fl ow before angioplasty was restored to timi grade 3 . a large right atrial branch ( black arrow ) summary of atrial and ventricular lead testing before and after the day of acute inferior wall myocardial infarction ( 29th june , 2004 ) this case demonstrates transient malfunction of a permanent passive - fixation atrial pacer lead at the time of acute myocardial infarction . the infarction was due to total occlusion of the proximal right coronary artery at the take - off of the atrial branch . since malfunction of the lead preceded angioplasty , the stent placement in the right coronary artery we conclude that transient ischemia of the right atrial myocardium at the pacer - lead interface caused transient loss of sensing and pacing functions . the most plausible hypothesis to explain these observations is stunning of the atrial myocardium due to atrial ischemia . the atrial lead malfunction was found to have normalized 40 days after stent implantation of right coronary artery . this case demonstrates that transient malfunction of a permanent atrial pacer lead may result from occlusion of the right coronary artery proximal to the atrial branch . in the absence of other electrocardiographic features of atrial infarction , transient malfunction of an atrial pacing lead should be included as a criterion for atrial infarction in patients with pacemakers . in this clinical setting , a new atrial lead should not be urgently implanted ; the patient should be followed with the expectation that lead function may normalize over time . thus , should other physicians encounter similar patients , it would be informative to follow patients at close intervals to determine how quickly the function may normalize .
proximal right coronary artery occlusion caused transient loss of sensing and capture of the atrial lead of a permanent dual - chamber pacemaker . forty - five days after percutaneous revascularization , the atrial lead was discovered to be functioning normally . we hypothesize that ischemia of the right atrium caused stunning of the atrial myocardium at the pacer lead interface , which gradually improved following percutaneous coronary intervention ( pci ) , leading to return of lead function over time . so far only one similar case has been described in the literature .
during 20082013 , a total of 68 satellite or global positioning system radio collars were deployed on african buffalo captured in southern gonarezhou np ; in northern kruger np , south of the limpopo river ; and in zimbabwe , north of limpopo river ( on sengwe communal land ) . of the 68 buffalo , 47 were adult females , selected because their behavior is representative of core herd movements . two adult males were also equipped with global positioning system devices because males are believed to move between herds ( 7 ) ; however , these devices failed after a few weeks because the collars fell off . nineteen subadult female buffalo 2.54.5 years of age ( age determined by teeth eruption ) were also selected because individuals from this group are believed to disperse from their native herds ( 8 ; r. bengis , pers . blood samples were taken and stored appropriately for disease screening , and individually numbered ear - tags were applied . during the study , extraordinarily long - distance movements for 3 subadult females were plotted by satellite telemetry readings ( figure 1 ) . in january 2014 , a 2.5-year - old female buffalo collared in south africa walked a maximum direct distance of 95 km . in 6 days , she crossed into zimbabwe , then into mozambique , and into zimbabwe again to enter gonarezhou np , with localizations within the home range of the buffalo herd in which btb was first diagnosed in a female buffalo in 2008 ( 6 ) . this subadult buffalo later left the park and visited a commercial farm area before reentering gonarezhou np . in february 2014 , another 4-year - old female buffalo walked a direct distance of 64 km in 8 days . finally , in march 2013 , a 4.5-year - old female captured in july 2011 was sighted in a location deep into communal land at a distance of 96 km from her capture site . in contrast to these young female buffalo , no adult females collared in this study moved such long distances outside their home range during 20082014 . the long - distance travel of these 3 subadult females occurred over a few days during the rainy season ( figure 2 ) and included movements outside the gltfca boundary . cumulative home - range area of 15 buffalo collared with global positioning system devices in kruger national park , south africa , in november 2013 . only buffalo with collars that functioned for an entire year are displayed . data for 2 subadult female buffalo ( paths displayed in figure 1 ) are shown ( id65 and id68 ) separately from data for the 13 other buffalo ( subadult and adult females ) . our findings strengthen the hypothesis that btb was spread from kruger np to gonarezhou np through buffalo - to - buffalo transmission by subadult females that dispersed from their native herds . buffalo populations in kruger and gonarezhou nps are connected through long - distance movements of individuals , specifically prebreeding heifers . although this movement is important for buffalo conservation in tfcas , it could also facilitate the spread of animal diseases , including zoonoses , across borders . in 2010 and 2011 , btb , rift valley fever , and brucellosis were detected in kruger np buffaloes , although a previous study failed to detect brucellosis in the gonarezhou np population ( 9 ) ( table ) . buffalo id65 , which was initially captured in kruger np , was seen among a breeding herd in gonarezhou np , indicating the possibility of direct , buffalo - to - buffalo transmission of btb by dispersing infected individuals , without the need for bridge hosts ( e.g. , other wild or domestic ungulate species ) ( 10 ) . * individuals and herds belong to the same population ( see figure 1 , adult female home range ) . calf , < 2.5 y of age ; subadult , 2.54.5 y ; adult , > 4.5 y. laboratory tests used : for bovine tuberculosis , gamma - interferon test and isolation ; for brucellosis , rose bengal and complement fixation tests ; for rift valley fever , indirect elisa test . published results ( 9 ) for the same diseases tested in the gonarezhou national park buffalo population in zimbabwe are shown in parentheses . difference between kruger and gonarezhou population results for brucellosis was significant by fisher exact test for equality of proportion for small samples ( p<0.02 ) . additional ecological information on buffalo dispersion is needed : frequency of dispersion events ; size , age , and sex composition of the dispersing groups ; and information about whether dispersed individuals later return to their home ranges . subadult females appear to be particularly prone to dispersing behavior , unlike adult females , and we speculate that they may do so in small groups of individuals that are approximately the same age ( 8) . no record exists of subadult female buffalo mixing with male bachelor groups , which are also known to connect to adjacent herds ( 7 ) . . one possible explanation may be an out - breeding mechanism ( 11 ) that occurs before the start of reproduction ; subadult females may leave their native herd to begin their reproduction in a distant herd to minimize in - breeding . furthermore , abundant resources ( i.e. , water and grazing areas ) available during the rainy season maximize the probability of success of such behavior . we found that subadult females were infected with btb , brucellosis , and rift valley fever ( table ) , diseases with different mechanisms of transmission . age and social position in the herd may influence individuals rate of exposure to pathogenic infections and consequently may affect the dynamics of infection within and between herds . our results indicate that subadult female buffalo could play a role in the spread of diseases among distant populations , across protected areas and international borders , and during the rainy season . this seasonal pattern contrasts with the timing of most wildlife and livestock contact between adult females , which has been observed to occur predominantly during the dry season in the study area ( 4 ) . buffalo have been observed far outside the boundaries of protected areas , even outside the gltfca , in communal land where livestock farming is the main livelihood ; these observations considerably widen the wildlife livestock interface area where disease spread can occur ( 12 ) . livestock interfaces can encompass large areas , rather than being a fence or strip of land at the edge of protected areas . these data should assist in refining disease modeling by showing the importance of temporal and spatial considerations and by redefining variables ( e.g. , age and sex ) involved in risk for pathogen spillover or emergence ( i.e. , identifying super - spreaders ) ( 13 ) . our results suggest that the spillover of btb and other zoonoses at the wildlife livestock human interface constitutes a risk to animal and human health in the gltfca ( 9,14 ) . the health issue in tfcas can not be overlooked and must be part of any management decision . combining ecological and epidemiologic knowledge is necessary to understand disease dynamics in these complex agro - ecosystems .
we report on the long - distance movements of subadult female buffalo within a transfrontier conservation area in africa . our observations confirm that bovine tuberculosis and other diseases can spread between buffalo populations across national parks , community land , and countries , thus posing a risk to animal and human health in surrounding wildlife areas .
pancreatic fistulas are a heterogeneous group of disorders in which both disruption of the pancreatic dust and leakage of amylase and lipase - rich fluid to the skin are present . pancreatic fistula incidence is 20% after whipple procedure , 30% after distal pancreatectomy , and 40% after pancreatic necrosectomy . previously , pancreatic fistulas were managed through re - operation , which was associated with a high mortality rate . extensive effort has been applied to the management of patients with pancreatic fistulas in order to decrease the morbidity and mortality associated to this type of fistula . that included improving the nutritional status of patients and treating any underlying disorders that prevented the fistulas from healing . somatostatin is also used to enhance the process of fistula healing and to shorten the period required for closure . we report that the use of phenytoin enhances the closure of pancreatic fistulas by presenting two cases who underwent pancreatic surgery complicated with the development of a high - output fistula . a 49-year - old male patient presented with diffuse abdominal pain , fever and jaundice . the patient was taken to surgery , and a hand - assisted pancreatic necrosectomy was performed . intraoperatively , three duval sump drains were inserted , one at the head of the pancreas , another at the tail of the pancreas , and the last in the lesser sac , overlying the body of the pancreas . postoperatively , the patient developed a high - output pancreatic fistula from all of the drains . the patient was started on sandostatin on the first postoperative day , however , the drainage amount was still high in spite of the high dose of sandostatin at 600 g / day . the drain at the head was draining pancreatic fluid at around 400 ml / day , the drain at the tail of the pancreas was draining 150 ml / day , and on the tenth postoperative day , a controlled colocutaneous fistula also developed throughout the lesser sac drain . the amount of output was 100 ml / day of feculent material from a fistula that was found to originate from the transverse colon as diagnosed by a fistulogram . when two weeks of treatment with sandostatin showed minimal effect , we decided to start the patient on oral phenytoin at 300 mg / day through a nasogastric tube . ten days later , the pancreatic fistula at the tail of the pancreas and the colocutaneous fistula at the head both closed after all of these drains had been slowly extracted . the second case was a 53-year - old male patient who underwent whipple procedure for a pancreatic adenocarcinoma . postoperatively , the patient developed a high - output pancreatic fistula from the pancreatic - jejunal anastomosis . sandostatin was started with no significant reduction in fistula output after ten days of treatment . however , the amount of fistula output dropped from 300 to 40 ml / day . unfortunately , the patient developed significantly delayed gastric emptying that required him to have a prolonged hospitalization . the safety of pancreatic surgery has increased in recent years . in spite of the decrease in mortality after pancreatic surgery the overall incidence of pancreatic fistulas after whipple procedure is about 20% . in distal pancreatectomy , the incidence reaches up to 30% , whereas with pancreatic necrosectomy , the incidence is around 50% . the consequences can be life - threatening ( i.e. , bleeding , pancreatic fluid collection , abscesses ) and cause prolongation of hospital stay and an increase in costs . abdominal computed tomography is considered the initial study of choice for evaluating and assessing intra - abdominal postoperative conditions . in order to evaluate the status of the pancreatic duct , secretin - enhanced magnetic resonance pancreatography is considered the study of choice , as it also allows us to precisely locate the disruption without any risk of infection due to the external injection of contrast in fistulography . the treatment of fistulas requires multiple therapeutic modalities , which include conservative parameters as well as radiological , endoscopic , and surgical treatments . such a treatment will take anywhere from 4 to 18 weeks for the fistula to close , resulting in closure of the fistula in nearly 85% of cases . percutaneous embolization of the pancreatic duct with different sclerosing agents is a new method that can help facilitate the closure of fistulas in a short period of time . the sclerosing agents used are prolamine , fibrin glue , neoprene , and n - butyl cyanoacrylate . if there is incomplete disruption of the pancreatic duct , endoscopic stenting of the pancreatic duct can allow healing of the fistula . the use of transpapillary stents allows the surgeon to bypass the high resistance of the sphincter of oddi and ductal strictures , while subsequently reducing the intraductal pressure and the driving force behind the fistula . all of the modalities mentioned above depend on allowing the tract to fibrose and eventually close by decreasing the output of pancreatic secretion into the tract . the process of tract healing depends on the growth of connective tissue , which will promote closure of the fistula . multiple medical reports have promoted the use of phenytoin to enhance wound healing [ 2 , 3 ] . the mechanism by which phenytoin promotes wound healing is not fully understood , however , several theories have been proposed . the mechanism by which phenytoin induces wound healing includes stimulation of fibroblast proliferation , enhancing the formation of granulation tissue , decreasing collagenase activity , and inhibition of glucocorticoid activity as well as direct and indirect antibacterial activity by affecting inflammatory cells and neovascularization [ 4 , 5 ] . there is also evidence that phenytoin may play a role in anastomotic healing [ 6 , 7 ] . the use of somatostatin in both cases showed no decrease in the output of the fistula ; however , after four days of using phenytoin , there was a significant decrease in fistula output . of course , it is difficult to prove such an effect from phenytoin alone , but having a rapid response in terms of closure can not be attributed to the other conservative techniques that we used , such as nutritional support and somatostatin . the ability of phenytoin to promote closure of fistulas seems promising if we can prove its effect , but this will require further trials . in conclusion , the effect of phenytoin on healing has been proven in pilonidal sinus wounds , and such a positive effect on healing can be applied on gastrointestinal fistula healing .
pancreatic fistulas are among the most devastating complications after pancreatic surgery . their subsequent development can be lethal . we report two cases of pancreatic fistulas treated with phenytoin . fistula tract healing consists of several processes , including cell migration and the formation of a new extracellular matrix . multiple studies have shown that phenytoin can promote wound healing and induce faster fibrosis . we postulate that such a positive effect can be used to enhance fibrosis of the pancreatic fistula tract . we treated two patients who had developed high - output pancreatic fistulas after pancreatic surgery . the first underwent hand - assisted laparoscopic pancreatic necrosectomy and developed two high - output pancreatic fistulas and a colocutaneous fistula . the second occurred post whipple surgery . both were given oral phenytoin after failure to respond to other measures . in conclusion , oral phenytoin may have a positive effect in the treatment of fistulas . prospective studies are needed to indicate this possible effect of phenytoin on fistula healing .
a 59-year - old womon presented with an abnormal mass on chest x - ray that was discovered during a routine check - up . we obtained a biopsy though endobronchial ultrasound and pathologically diagnosed a thymoma ( type a ) . contrast - enhanced computed tomography showed a smooth , solitary homogeneously enhanced mass measuring 33.52 cm in the right paratracheal area , which had no calcification ( fig . the resected specimen was a firm light - gray tumor with a smooth capsule that measured 4.532.5 cm . postoperative follow - up proceeded without any problems , and the patient was discharged after 5 days . microscopy revealed that the tumor was a thymoma ( world health organization type a+b2 , masaoka staging i ) ( fig . the majority of thymomas are found in the anterior mediastinum . only a few thymic masses arising out of the anterior mediastinum have been described in the literature , and these have been found in an ectopic thymus location such as the neck , pulmonary hilus , or posterior mediastinum . the thymus arises embryologically from the third pharyngeal pouch and branchail cleft on each side . the thymic masses from each side then move toward each other and migrate from the midline to the anterior mediastinum and their final position . the incidence of ectopic thymic tissue is 3% to 5% in the retroinnomiate vein ( i.e. , a paratracheal site ) . surgical resection has been used for both a firm diagnosis and treatment in previously reported cases of a middle mediastinum thymoma . sakurai et al . reported that 18f - fluorodeoxyglucose positron emission tomography ( pet ) scan and 11c - acetate pet are useful for diagnosing a thymoma from the middle mediastinum as ectopic thymic tissue . castleman disease is an atypical lymphoproliferative disorder , and masses are commonly located in the chest . most patients are asymptomatic and lesions are accidentally found on chest x - ray as rounded mediastinal masses , often mistaken for a thymoma . thymomas rarely arise in the middle mediastinum , and thymoma is not considered in the differential diagnosis of middle mediastinum masses . but thymomas appear to have malignant potential , and the overall rate of thymoma recurrence is approximately 20% .
thymoma is a common anterior mediastinal mass , although thymomas have occasionally been found in the neck , pulmonary hillus , or posterior mediastinum . but a thymoma within the middle mediastinum has rarely been reported . we report a thymoma arising in the middle mediastinum with a review of the literature .
functional bowel disorders ( fbd ) , including the irritable bowel syndrome ( ibs ) are now recognized as common chronic bowel disorders that affect between 5 to 25 percent of populations ( 15 ) . of all fbds , ibs has received the most attention , in part due to its high prevalence ( 325% ) of adult sample populations in published series ( 68 ) . there are similar prevalences for ibs across western countries , but the prevalence may be lower in asian countries and in african americans ; however , there is a wide variation , even within individual countries ( 710 ) . in iran , the prevalence of ibs was reported as 5.8% in the general population and 3 - 18.4% in specific iranian populations ( 1114 ) . ibs can develop psychological stress or bacterial enteritis that causes injury to the bowel mucosa . due to an absence of any certain diagnostic clinical or paraclinical tests for this category of diseases ( 15 ) the diagnosis relies on criteria that have almost unanimous consensus ( 1518 ) . this pain may be accompanied by a reduced threshold for experiencing pain and an exaggerated pain response . certain factors can predispose to prolonged and exaggerated post - operative pain , these include ; the site and duration of surgery and the emotional well - being of the patient ( 19 ) . it is not known if abdominal pain or ibs results from gynecological surgery secondary to iatrogenic injury to abdominopelvic nerves ( 20 ) . however , the degree to which afferent injury resulting from surgery or central amplification due to psychosocial distress is operative is not completely clear . a potential model to test this question would be to prospectively evaluate patients undergoing a surgical procedure . so the aim of this study was to assess the incidence of pain or ibs in women undergoing hysterectomy or tubal ligation ( tl ) . it is a prospective study on 164 patients undergoing hysterectomy and 172 patients undergoing tl who were randomly selected in alzahra hospital and taleghani hospital , tabriz , iran from 2008 to 2009 . exclusion criteria included fulfillment of the rome ii criteria for painful functional gastrointestinal disorders including ibs or the presence of abdominal or pelvic pain ; chronic diarrhea or constipation ( requiring regular anti - diarrheal ) or laxative treatment , chronic pancreatitis , serious chronic co - morbidity including malignancy . all women undergoing hysterectomy and tl were followed for 12 months and screened for ibs based on the standardized questionnaire . all women were screened at 3 time points ( each 3 months in one year after surgery ) using standardized questionnaires to diagnose painful functional gastrointestinal disorders , including ibs , by rome ii criteria . demographic and clinical factors were also collected . statistical analysis performed by contingency tables and chi - square test . a total of 336 women entered to this study , 164 patients undergoing hysterectomy and 172 patients undergoing tl ( 6 patients from hysterectomy group have been excluded ) . the mean age of patients underwent tl was 37.85 5 years and the mean age of patients in hysterectomy group was 47.14 6.57 years . during 12 months after surgeries , 19 ( 11% ) patients in tl group and 19 ( 11% ) in hysterectomy group had abdominal pain ( > 12 weeks ) with at least two symptoms of ibs . ibs was confirmed in 9 ( 5% ) patients of tl and 13 ( 8% ) patients of hysterectomy group ( p>0.05 ) . the percent of symptoms associated with ibs appeared in table 1 . in both studied groups , the most prevalent symptoms along with abdominal pain were chronic constipation and abnormal bowel movement and the least prevalent were diarrhea and passage of mucus . there were not any significant differences in prevalence of symptoms among ibs positive patients between tl and hysterectomy group . also the distribution of abdominal pain with or without other symptoms of ibs was indicated in table 2 . frequency of symptoms associated with ibs in women undergoing hysterectomy and tl * ibs : irritable bowel syndrome , tl : tubal ligation abdominal pain with or without other symptoms of ibs * ibs : irritable bowel syndrome , tl : tubal ligation according to a new population based study in iran , the prevalence of ibs was estimated to be low ( 21 , 22 ) . so the result of this study indicates that the high incidence of abdominal pain and ibs symptoms that develops within 12 months of gynecologic surgery . surgery can cause postoperative pain , a unique entity with specific physiologic and clinical features ( 19 ) and patients with irritable bowel syndrome ( ibs ) have high surgical rates ( 23 ) . it is known that many women with irritable bowel syndrome ( ibs ) have had a hysterectomy ; possible explanations include misdiagnosis of ibs resulting in hysterectomy , ibs symptoms occurring as a result of hysterectomy , a single underlying disorder which produces symptoms in both gastrointestinal and genitourinary tracts , or a combination of these factors ( 24 ) . similar study showed that constipation and pain subtype ibs were more common in hysterectomy patients ( 23 ) . in contrast , a prospective study among women undergoing gynecological for non - pain indications the development of ibs was not significantly greater than controls . a limitation of this study is that we did not compare the surgical group with non - surgical healthy control . so it is recommended to do similar prospective study , using a carefully selected control group . in conclusion , these results suggested that gynecological surgeries ( tl and hysterectomy ) could facilitate ibs .
aimthe aim of this study was to assess the incidence of irritable bowel syndrome in women undergoing hysterectomy and tubular ligation.backgroundthe results of previous studies have shown an increased incidence of irritable bowel syndrome after gynecological surgeries.patients and methodsparticipants were patients of alzahra and taleghani university hospitals in tabriz . one hundred and seventy two women without gastrointestinal symptoms or a diagnosis of the irritable bowel syndrome underwent tubular ligation and 164 women underwent hysterectomy . patients were assessed every 3 month after hysterectomy and tubular ligation for 12 months . irritable bowel syndrome was diagnosed by a questionnaire based on rome ii criteria.resultsduring 12 months after surgeries , 19 ( 11% ) patients in tubular ligation group and 19 ( 11% ) in hysterectomy group had abdominal pain with at least two symptoms of irritable bowel syndrome . irritable bowel syndrome was diagnosed in 9 ( 5% ) patients in the tubular ligation and 13 ( 8% ) patients in hysterectomy groups ( p>0.05 ) . in both studied groups , the most prevalent symptoms along with abdominal pain were chronic constipation and abnormal bowel movement and the least prevalent were diarrhea and passage of mucus.conclusionthese results suggest that gynecological surgeries ( tubular ligation and hysterectomy ) may predispose to the development of the irritable bowel syndrome .
we analyzed reported data for the netherlands for december 2008december 2013 . reporting criteria included 1 hantavirus - associated symptom ( fever , renal insufficiency , or thrombocytopenia ) and virus detection in blood , or a major increase in igg titers or increases in igm or iga titers against hantavirus . for the seroprevalence study , a subset of samples from a large serum bank established for population - based serologic studies ( pienter 2 ) , particularly immunization program evaluations , was used . pienter 2 is a cross - sectional serosurvey conducted during 20062007 with a representative sample ( n = 7,904 ) of the population of the netherlands ( 7 ) . participants also completed a questionnaire that included basic demographic characteristics and behaviors and activities related to increased risk for acquiring infectious diseases . variables possibly related to hantavirus infection from the literature , such as age , sex , outdoor activities , and animal contact , were selected from the pienter 2 questionnaire . a total of 2,933 serum samples from 19 municipalities distributed across the country , including known high - risk areas , were included in the study and screened for antibodies against hantavirus ( figure 1 ) . municipalities sampled in the pienter 2 study and subset of municipalities included in the seroprevalence study of hantavirus infections , the netherlands . an elisa ( hantavirus igg dx select ; focus diagnostics , cypress , ca , usa ) that detects all known circulating hantaviruses in europe was used for initial screening of all serum samples . for confirmation , all elisa - positive samples were analyzed by using a puuv - specific indirect immunofluorescence assay ( ifa ) ( progen , heidelberg , germany ) . a randomly selected subset of elisa - negative samples were screened by ifa to correct for possible false - negative elisa results . we calculated odds ratios ( ors ) and 95% cis for variables putatively associated with hantavirus seropositivity by using mixed - effects logistic regression that included municipality as a random effect to account for clustering of samples . a p value 0.15 was used in the single - variable analysis to select variables for the multivariable model built in backward stepwise fashion . a total of 62 cases were reported during december 2008december 2013 ( figure 2 ) . most cases ( 63.0% ) were in men ( median age 48 y , range 1672 y ) . the highest number of cases ( n = 26 ) occurred in the region of twente ( figure 1 ) in the eastern netherlands . fifty - two case - patients ( 85.0% ) were hospitalized and seven ( 12.0% ) required dialysis . most cases ( 90.0% ) were acquired domestically . reported cases of hantavirus infection ( n = 62 ) by year , the netherlands , december 20082013 . a total of 154 ( 5.3% ) serum samples were positive by elisa . of the elisa - positive samples , 27 ( 17.5% ) were also positive for puuv igg by ifa and therefore considered samples with positive results . after we corrected for false - negative results , the overall seroprevalence was 1.7% ( 95% ci 1.3%2.3% ) . seroprevalence ( uncorrected ) in women was higher than that in men , albeit , not significantly . age was not associated with puuv infection . owning 1 or more dogs or any livestock the municipality - level random effect was significant ( p = 0.001 , by log - likelihood ratio test ) . most positive samples ( n = 10 ) were from the municipality of enschede ( twente region ) in which seroprevalence was 3.2% ( 10/309 ) ( figure 1 ) . * ifa , indirect immunofluorescence assay ; aor , adjusted odds ratio ; na , not applicable . the municipality - level random effect was significant ( p = 0.001 , by log - likelihood ratio test ) . adjusted for age , sex , and clustering at municipality level ( random effect ) . adjusted for age , sex , clustering at municipality level ( random effect ) , and the other covariates included in the multivariable model . we report that hantavirus seroprevalence in the netherlands is 1.7% for the years analyzed on the basis of a subset of samples from a large population - based serum bank . this seroprevalence was similar to estimates for neighboring countries ( 1.5% for belgium and 1%3% for germany ) ( 2,4 ) . the number of reported cases in the netherlands was low . given our seroprevalence estimate , although not entirely representative of the population of the netherlands , and the proportion of symptomatic puuv - infected persons ( 20%30% ) , some of whom seek medical care ( 1 ) , the number of cases in the netherlands ( 16.5 million resident population ) is expected to be higher . many cases with milder symptoms probably go unnoticed because of low awareness of hantavirus infection among physicians in the netherlands . hantavirus infections in the netherlands are most likely caused by puuv , although seov- or tulv - associated infections can not be excluded ; clinical seov infections have been reported from france and the united kingdom ( 8,9 ) . the proportions of hospitalizations and persons requiring dialysis were much higher than those reported in other countries . in finland , 52.0% of puuv - infected persons required hospitalization ( 10 ) . in germany , 64.0% of persons with reported cases of infection in 2010 required hospitalization ( 11 ) . the difference in hospitalization rates is probably associated with reporting bias because only the most severe cases are reported due to strict reporting criteria and possible higher thresholds for testing . bank voles are a forest - dwelling species , and risk for puuv infection is associated with vicinity of forests and the proportion of forested land cover ( 1,12,13 ) . seroprevalence and number of cases were highest in the twente region , a region to which puuv is endemic . this region borders areas in germany in which incidence is high ( 11 ) and is located near forests , which are scarce in the netherlands . however , it is highly unlikely that these factors reflect direct virus transmission from such domestic animals to humans , but represent proxies for lifestyle characteristics of dog owners and persons engaged in farming activities that predispose them to more frequent or more substantial contact with rodents . seroprevalence was higher in women , but the disease was most often reported in men . similar male : female ratios among persons with clinical puuv infection have been reported from other countries ( 10,14 ) . these data suggest that women have a higher proportion of subclinical or mild infections , although a recent study suggested that disease severity does not differ between men and women ( 15 ) . in conclusion , higher seroprevalence relative to the number of reported cases calls for further awareness of hantavirus infection among physicians in the netherlands . seroprevalence studies of persons with unresolved renal problems could further increase our understanding of the true incidence of hantavirus infection in the netherlands .
we report the recent epidemiology and estimated seroprevalence of human hantavirus infections in the netherlands . sixty - two cases were reported during december 2008december 2013 . the estimated seroprevalence in the screened municipalities in 20062007 was 1.7% ( 95% ci 1.3%2.3% ) . findings suggest that hantavirus infections are underdiagnosed in the netherlands .
a 64-year - old swiss pensioner presented to our hospital with a pustular rash and signs of systemic inflammation . the c - reactive protein was 62 mg / l and the differential blood count revealed leukocytosis ( 15.39 five days before , she had first noticed abrupt onset of sheets composed of small pustules on her stomach , subsequently spreading to affect the neck and the palms . the day before admission , lakes of pustules and papules formed on the whole body surface except the face and scalp . furthermore , the patient suffered from severe pruritus in the affected areas . in the 4 weeks preceding the admission , she had been treated with amoxicillin / clavulanic acid for a knee prosthesis infection caused by beta - hemolytic streptococci ( groups b and g ) , as well as with the anticoagulant rivaroxaban . a biopsy confirmed neutrophil infiltrates in the upper layers of the epidermis , as well as perivascular and interstitial neutrophil- and eosinophil - rich infiltrates mixed with lymphocytes and plasma cells [ 1 , 2 ] . microbiology swabs revealed neither bacteria nor fungi . according to the euroscar criteria [ 3 , 4 ] ( table 1 ) thus , the suspected offending drugs amoxicillin / clavulanic acid and rivaroxaban were replaced with clindamycin and fractionated heparin . topical clobetasol propionate 0.05% ointment was generously applied to all affected areas once daily and cold cream with 5% polidocanol , a local anesthetic , was given for the pruritus . development of new pustules ceased within 24 h ( fig . 1 ) and pustules shed within 72 h. the patient was discharged in good general condition after 7 days of hospitalization with only a faint , diffuse erythema with focal desquamation remaining ( fig . in over 90% of cases , agep is caused by medications . the patient has received both amoxicillin / clavulanic acid and rivaroxaban during the last 4 weeks before development of the disease . it is also atypical that the drugs had started to be administered 4 weeks before the onset of disease . it is widely accepted that in a minority of cases also infections can cause agep . our patient was suffering from an infected knee prosthesis , which was the target of the antibiotic treatment . strikingly , agep rarely manifests several weeks after the culprit drugs have been initially administered . the foremost intervention leading to clearing of agep - related pustules is the interruption of the offending drug . during the pustular phase , a local antiseptic treatment and moist dressings are useful , as well as topical corticosteroids . application of steroids more than once per day does not increase efficacy , but the onset of the therapeutic effect may be faster . in patients with systemic inflammation such as our case , systemic steroids themselves can induce pustulosis , which may be considered a reason to avoid them if possible . all reports on agep favor the application of parental steroids . our patient recovered in a very short time by stopping the medication intake and by the use of highly potent topical corticosteroids once daily . f.a . is funded by the hsm2 ( hochspezialisierte medizin ) and the kti grant . he has received payments from abbott , celgene , and is on the advisory board of leo pharma .
acute generalized exanthematous pustulosis ( agep ) is a dramatic generalized pustular rash of severe onset , which is considered a serious cutaneous adverse reaction to drugs . however , even though the clinical features are impressive and are often accompanied by systemic inflammation , it can be controlled quickly and safely by topical steroids subsequent to interruption of the offending drug . here , we describe the management of a case and the evolution of the pustular rash . an elderly woman consulted with a generalized crop of 23 mm , nonfollicular pustules on erythematous background . in the 4 preceding weeks , she had been using amoxicillin / clavulanic acid for a bacterial implant infection and rivaroxaban . the clinical euroscar criteria including the histology confirmed agep . her medication was stopped and topical clobetasol propionate was used . within 24 h , the development of new pustules ceased and the patient was discharged after 7 days of hospitalization with only a faint , diffuse erythema and focal desquamation remaining . this and many other cases in the literature suggest that topical steroids should be considered as a first - line treatment option , especially as systemic steroids themselves can sometimes induce generalized pustulosis .
the biomedical community has established the standards of good clinical practice as the cornerstone of medical research on humans . what are the standards for studying practices that overtly and intention ally fall short of good practice and are clearly discriminatory against the aged ? i find four ethical problems in the study on ventilated patients outside the intensive care unit ( icu ) . had this been an interventional study , omission of informed consent would have been unthinkable . but , unfortunately , in that hospital , and in many others , these patients would have been sent anyway to a medical floor . in some other countries , they would not have been ventilated at all unless an icu bed was secured for them in advance . the fundamental difference between the icu and a regular hospital floor lies in the capacity to monitor and to react . is it not likely that when an icu person collects all sorts of data on the participants , issues come to attention - such as wrong ventilator settings , a need for a different drug , and so forth ? intervention is incompatible with the methodology of the study ; non - intervention is grossly immoral . moreover , since icu beds might become available and patients might deteriorate , ventilated patients who can not be admitted to the icu on the day of hospitalization deserve reassessment for admittance later on . interestingly , no study patient was transferred from the medical floor to the icu . a third problem is related to the fact that in israel , as well as in many other places , the decision of whether to admit a patient to the icu is solely in the hands of icu doctors . it follows that this research was conducted in order to evaluate the safety of gatekeeping by the very people who serve as the sole gatekeepers . i wish the ethics committee of soroka hospital had set some provisory guidelines for triage and for care of ventilated patients in the medical floors prior to that hospital 's institutional review board 's endorsement of this non - interventional study . the authors themselves testify to their deviation from established ethical norms : the recommendation that ' chronological age per se is not a relevant criterion for hospitalization in an icu ' was not substantiated in the present study population . what the authors actually say is that the icu team in their hospital violates professional ethical guidelines protecting a vulnerable population , without any sort of reflection or policy endorsement . a study conducted in the united kingdom found that 12% of icu patients could be cared for in a regular ward and 53% of ward patients were better suited for icu care . healthcare expenditure , which is an explicit concern in the article , did not correlate with availability and accessibility of intensive care services . a meta - analysis of numerous clinical publications from all over the world has found age to be a factor in the triage of patients for critical care . the number of icu beds per capita varies substantially from one place to another , and a low bed / population ratio correlates with increased inhospital mortality overall . perhaps ageism rears its head when the ratio of icu beds to population is low , as is the case in israel . deliberate rationing of scarce health resources on the basis of age is highly controversial . like any other form of rationing , it depends on open deliberation for justification and legitimization , and not on inconclusive evidence and a motivation to save money . a serious confounding factor in the whole discourse on the allocation of intensive care is lack of clarity regarding the prognosis of ventilated patients . for some , icu care is plainly futile - but legal and psychosocial issues do not allow doctors to disconnect . a second group of patients is also sent to the regular floor , however , not because they do not need intensive care but because the person responsible for the icu does not have a bed for them . in the absence of conceptual differentiation of patients who need icu care from those for whom such care is futile , little may be said about the overall outcome in terms of mortality . those who are accustomed to seeing ventilated patients on the medical floors are not surprised to learn that more than one - quarter of them survived despite non - icu standards of care . doctors who avoid intubation of patients that have no chance of entry into the icu may reconsider this policy . in my eyes , this is the most important lesson to take from this publication . my second take - home message is that ageism is still prevalent in healthcare and clinical research . policy - makers should deliberate more openly the role of age in distributive justice in healthcare , while boosting awareness of existing ethical guidelines and of every doctor 's commitment to protect the vulnerable .
four ethical issues loom over the study by lieberman and colleagues - the absence of informed consent , the study being non - interventional in situations that typically call for life - saving interventions , the bias involved in doctors that study their own problematic practice and monopoly over intensive care unit triage , and ageism . we learn that the israeli doctors in this study never make no - treatment decisions regarding patients in need of mechanical ventilation . they are complicit with botched standards of care for these patients , however , accepting without much doubt an ethos of scarce resources and poor managerial habits . the main two practical lessons to be taken from this study are that , for patients in need of mechanical ventilation , compromised care is better than a policy of intubation only when the intensive care unit is available , and that vigorous efforts are needed in order to extirpate ageism .
a 35-year - old female with a previous history of long - term high - dose benzodiazepine use presented in 2001 following an overdose of ibuprofen . she had concurrent symptoms of depression , persecutory ideas , and bizarre , chaotic behavior ( eg , wandering the streets claiming she was being poisoned ) . physical examination was normal , except for an extensor left plantar reflex , as were her urea and electrolytes ( u&es ) , full blood count ( fbc ) , thyroid function tests ( tfts ) , liver function tests ( lfts ) , and random blood glucose ( rbg ) . ct and mri brain scans and an eeg were reported as normal , and hence an organic etiology was excluded . a diagnosis of severe psychotic depression was made and she was treated with a variety of antidepressant , antipsychotic , and mood stabilizing agents for over twelve months , together with electroconvulsive therapy without significant response . she displayed behavioral difficulties including poor self care , agitation , and she was intrusive and demanding . twelve months later , a second opinion was sought at which time the patient had intermittent paranoid ideas , mood congruent auditory hallucinations , and psychological pillow . a diagnosis of schizophrenia was made and clozapine was commenced , again with no significant impact . in july 2002 , her family relocated and she was transferred to another psychiatric hospital . her presentation was unchanged except for the emergence of hypochondriacal ideas and worsening behavioral difficulties such as disinhibition , coarsening of social skills , and deteriorating self care . the patient was referred for a further opinion in november 2003 at which time her main difficulties were a mixture of depressive , psychotic , and anxious symptoms with marked behavioral disturbance . physical examination revealed a left extensor plantar reflex , a pout , and palmomental primitive reflexes but no other neurological signs . a battery of investigations was arranged , including u&es , fbc ( including screen for acanthocytes ) , tfts , lfts , rbg , c - reactive protein test , calcium , phosphate , copper , ferritin b12 and folate , pituitary hormones , autoantibodies , chest x - ray , and ecg . an eeg recording revealed a slightly slow posterior rhythm suggestive of a mild non - specific cortical disturbance . the previous ct scans from 2001 were reviewed , and despite a normal report , there were significant atrophic changes which had progressed ( figures 1 and 2 ) . the initial report did not identify any abnormality , but mild atrophy particularly in the frontal regions is evident . a lumbar puncture revealed normal cerebrospinal fluid , glucose , protein , angiotensin - converting enzyme , and cytology . on neuropsychological assessment she scored 68/100 on the extended mental state examination , having deficits in mental reversal , orientation , verbal fluency , and recall . a clinical diagnosis of frontotemporal dementia ( ftd ) was made , in accordance with the consensus diagnostic criteria ( neary et al 1999 ) . arnold pick described clinical syndromes associated with frontal and temporal lobe degeneration over a century ago , but for many years these conditions were unrecognized as specific subtypes of dementia . in recent years , these conditions have been rediscovered ( gustafason 1987 ) and diagnostic criteria have been developed . ftd comprises a pathologically heterogeneous group of disorders characterized by behavioral change . only a minority of patients exhibit pick - type histological changes , which includes loss of large cortical nerve cells with widespread gliosis and swollen neurons or inclusions positive for both tau and ubiquitin . is the official classification for these forms of dementia in icd-10 and dsm - iv . ftd is the most common form of primary degenerative dementia after alzheimer s disease that affects people in middle age . it occurs most commonly between the ages of 45 and 65 years ( snowden et al 2002 ) . there is an equal incidence in men and women ( gustafason 1987 ) . from the onset of symptoms the delay in diagnosis has been reported to be on average 3 years , and death usually occurs within 6 years of the development of symptoms ( hodges et al 2003 ) . when viewed with the wisdom of hindsight , the emerging clinical characteristics of ftd in this case are clear . however , the age of onset is unusual , and we highlight the complexity of reaching a diagnosis in the absence of clear physical signs and when investigations are reported to be normal . this case draws attention to the problems inherent in the traditional functional organic divide that continues to characterize investigation and diagnosis in modern psychiatry . the presenting symptoms in this case caused diagnostic uncertainty , and a variety of functional diagnoses were made , even though the symptomology was atypical and did not fit neatly into any diagnostic category . although clinicians may be reassured of the absence of an underlying organic etiology following normal neuroimaging and electrophysiological studies in psychiatric patients presenting with atypical features , this is clearly not always the case . once the momentum behind a functional diagnosis has been established , consideration of alternative etiologies is often abandoned and reappraisal of the biological underpinnings of the disorder may be neglected . we advocate that physical investigations are repeated if patients continue to present a diagnostic challenge . the reevaluation of the results of previous normal investigations , in the light of the developing clinical picture , may further aid the diagnostic process .
frontotemporal dementia ( ftd ) is the most common form of primary degenerative dementia after alzheimer s disease that affects people in middle age . the average delay in reaching an accurate diagnosis has been reported to be around 3 years . we report a case of ftd in a 35-year - old female who presented with complex symptoms and no clear physical signs . this case draws attention to the problems inherent in the traditional functional organic divide that continues to characterize investigation and diagnosis in modern psychiatric practice , and highlights the importance of reevaluating the results of previous normal investigations in the light of the developing clinical picture .
this study was done to verify whether the national immunization team has achieved its objective or not by a small checking survey which was conducted over 2 days as a door - to - door survey immediately after the immunization days . stratified two - stage sampling was done in the population of al - khobar area . the total sample size was divided into different sectors using bowleg 's proportional allocation scheme . at the first stage , housing blocks were selected using simple random sampling procedures as the blocks are similar in nature . at second stage , two houses from each block were selected in 1 or 4 order ( each block having 8 to 10 houses ) . all saudi and non - saudi nationals living in these houses were screened using a pre - tested questionnaire administered in a face - to - face interview by trained interviewers . the questionnaire covered areas of placement of stickers on immunized households , the accuracy of information displayed in these stickers , the number of children vaccinated and not vaccinated , the reason for non - vaccination and whether or not the immunized children received certificates . the cooperation of the authorities in the various government institutes was secured through ample discussions pertaining to the objectives of the survey and the mechanics of screening of the targeted households . the total population surveyed was 527 people including 213 adults and 314 children , 152 ( 48.4% ) of the children were aged five years or less . one household occupied by a senior citizen and another household were not available initially . upon re - visit by screening team , they were informed that the occupiers had gone abroad . in all the households surveyed , all children of eligible age , ie . , 5 years or less were vaccinated ( n=152 ) . only 2 households received the vaccination at a primary care health center but all others had been vaccinated by the national immunization programme teams . , 100% of households surveyed had stickers placed on the outside door by the national immunization programme teams , 95% of the stickers were placed out of the reach of children . none of the surveyed households had follow - up visits by the national immunization programme teams . such a goal was obtained in the americas through case surveillance , recognition and immunization.68 the incidence of poliomyelitis infections in other parts of the world is steadily dropping.913 to achieve similar objectives a national immunization programme was undertaken in saudi arabia . this was done on a door - to - door basis . following the national immuni - zation programme days , this pilot study also shows that 101 ( 95% ) of household had stickers placed outside the door identifying them as having been visited by the national immunization programme teams . although a lower percentage was required ( 80% ) to achieve the national immunization programme objectives , it seems that the immunization programme in al - khobar had achieved a higher percent - age coverage ( 100% ) . through re - infection by the live attenuated oral polio vaccine and auto - immunization , the number of immune individuals to polio virus will exponentially rise . this may ultimately help the goal of eradicating polio virus from the kingdom .
objectives : to verify whether the first national campaign for poliomyelitis vaccination achieved its objectives.setting:al-khobar area in the eastern province of the kingdom of saudi arabia.materials & methods : randomized samples have been obtained using the bowleg 's proportional allocation scheme . at the first stage , housing blocks of 8 - 10 houses were selected using random sampling procedure . at a second stage , 2 houses from each block were selected in a 1 or 4 order . both saudi and non - saudi nationals living in these households were screened using pretested questionnaire administered at a face - to - face by trained interviewers . the questionnaire covered areas that included number of children vaccinated , reasons for non vaccination , whether or not the vaccinated child has received certificate of vaccination as well as placement of stickers on the door of the vaccinated households.result:a total of 107 households were surveyed in al - khobar . the total populations surveyed was 527 people including 213 adults and 314 children ( 152 of the children were aged 5 years or less ) . in all the households surveyed , all eligible children age 5 years or less were vaccinated . all of the vaccinated children obtained a vaccination certificate.conclusion:the overall percentage coverage in al - khobar was 100% . this denotes the success of the campaign . a nationwide survey is suggested to corroborate these findings .
patent foramen ovale ( pfo ) is an increasingly recognized cause of cryptogenic stroke and peripheral embolism . contrast transesophageal echocardiography ( tee ) was established as a simple , accurate , and safe procedure for the diagnosis of interatrial communication . paradoxical embolism is main pathophysiology via pfo , but this is mostly a presumptive diagnosis , while definite demonstration of a thrombus crossing a pfo is rare . detection on thrombus - in - transit by echocardiography , the best management is controversy until now . a previous healthy 53-year - old man was hospitalized with progressive dyspnea and cough for 2 weeks . initial physical examination revealed a blood pressure of 126/81 mm hg , respiratory rate of 20 per minute , and pulse rate of 103 per minute . on auscultation , the electrocardiography showed sinus tachycardia and inverted t wave on the anterior and inferior lead . except for a plasma d - dimer level over 5,000 ng / ml ( upper normal limit : 500 ng / ml ) , other laboratory findings were normal . initial arterial oxygen pressure was 73 mm hg , arterial carbon dioxide pressure 29 mm hg , and oxygen saturation 94% at room air . pulmonary computed tomography showed multiple filling defects of segmental branches of pulmonary arteries ( fig . intravenous heparin was started . in recalled previous history , he denied any predisposing conditions like as airplane travel or deep vein thrombosis . transthoracic echocardiography ( tte ) found wormlike , free - floating masses in the both atria ( fig . systolic pulmonary artery pressure was 80 mm hg , and the inferior vena cava was dilated . no central pulmonary artery emboli or thrombi in the inferior vena cava were visible . left ventricular ejection fraction was 61% . right ventricular pressure overload resulted in d - shaped left ventricle and right ventricular systolic function was slightly depressed . interatrial septum was thin and mobile serpentine thrombi wedged through a pfo and lodged in both atria were demonstrated ( fig . 3b , supplementary movie 2 ) and color doppler indicated right to left shunt ( fig . 3c , supplementary movie 3 ) . immediately after tee , oxygen saturation was decreased down to 84% , but with oxygen supplementation through a facial mask , it was elevated up to 95% . due to a very high risk of paradoxical systemic embolism with potential disastrous consequences , he underwent emergent intravenous thrombolysis . operation was not considered for his situation , because we could n't trace the route of missing thrombus . dyspnea was markedly relieved and follow - up chest computed tomography showed dissolved pulmonary thromboembolism . further evaluation of hidden malignancy , all levels of tumor marker was normal range including carcinoembryonic antigen 1.4 ng / ml ( normal 0 - 5 ng / ml ) , prostate - specific antigen 2.2 ng / ml ( normal 0 - 3 ng / ml ) and ca19 - 9 1.1 u / ml ( normal 0 - 37 u / ml ) . repeated chest computed tomography showed decreased size of multiple filling defects in both pulmonary arteries without evidence of lung cancer . evaluation for hypercoagulable condition was not found any abnormal finding - protein c 82% ( normal 70 - 140% ) , protein s 118% ( normal 70 - 140% ) , and antithrombin iii 94% ( normal 80 - 120% ) . and serologic test for rheumatologic problem revealed normal range ( anticardiolipin antibody : negative , lupus anticoagulant : negative ) . the patient was discharged from hospital with uneventful recovery and he has been doing well without additional embolic events after discharge and maintenance 24-month anticoagulation therapy . paradoxical embolism was first described by connheim in 1877 and is defined as the embolic entrance of venous thrombosis into the systemic circulation through a right to left intracardiac shunt , like as presence of pfo.1 - 3 ) this is mostly a presumptive diagnosis , while definite cases demonstrating a thrombus traversing the pfo are very few.4 - 6 ) a thrombus from the venous system into the right heart may easily cross into the left heart through a pfo if the right atrial pressure exceeds left atrial pressure , which can occur in the setting of pulmonary hypertension.2)4)6 ) echocardiography , especially tee , plays a main diagnostic tool in recognizing this potentially life - threatening , but treatable , condition.7 ) patients with a right - to - left intracardiac shunt via a pfo , agitated saline contrast - enhanced tee is helpful . cough test is known to be superior to valsalva maneuver in the delineation of right to left shunt.8 ) valsalva maneuver is contraindicated in patients with thrombus - in - transit via pfo - the risk of another embolism . the best treatment for a pulmonary embolism with a thrombus straddled across pfo is unclear . basically , the therapeutic options are cardiac surgery , thrombolysis , or anticoagulation with heparin , or a combination of all these treatments . currently , surgical embolectomy and closure of pfo has been done as a preferred option.9 - 11 ) in our case , we decided the intravenous thrombolysis because of the patient 's missing thrombus , although fragmentation of the thrombus followed by pulmonary or systemic embolization may be a theoretical risk of this treatment . some previous reports described anticoagulant therapy can be used as an alternative treatment in this condition . 4)12 ) we described a case of highly suspected paradoxical embolism with pulmonary embolism , in which a thrombus straddled and disappeared through pfo was observed in transesophageal echocardiography . in conclusion , thrombus - in - transit can be demonstrated by tee in patients with pulmonary embolism and if detected , immediately treatment can prevent systemic embolism .
pulmonary embolism and concomitant right atrial thrombus entrapped in a patent foramen ovale ( pfo ) is a rare , unusual finding in echocardiography . the diagnosis of paradoxical embolism is usually presumptive when pfo is detected by echocardiography . we herein reported a case of a 53-year - old patient presenting with pulmonary embolism in which a thrombusin - transit through a pfo was found and disappeared during transesophageal echocardiography .
an 18-year - old woman patient was admitted to hospital with complaints of abdominal distension , and the patient had experienced intermittent mild abdominal pain since birth . the abdominal girth had slowly increased in diameter over the previous 18 years , and the patient 's general condition of health was poor , presenting symptoms including fatigue , fever , nausea , vomiting , decreased appetite and weight loss . clinically , the diagnosis of a giant hydronephrose ( gh ) was made , and upon physical examination , an extremely distended abdomen was revealed , with a palpable mass in the all - flank region ( fig . the blood laboratory analyses revealed a concentration of blood urea nitrogen of 30.1 mg / dl , and a level of creatinine of 1.56 mg / dl . the level of hemoglobin was 12 g / dl , and the hematocrit was determined to be 45.7% , whereas tumor markers were all revealed to be in normal range , the results of the urine analysis revealed a red blood cell count of 32/high power field ( hpf ) a white blood cell count of 6/hpf and a yeast cell count of 5/hpf . blood sugar , phosphate , liver function , serum calcium , serum amylase and electrolyte analyses all yielded results within the normal limits . a diagnostic aspiration performed from the area of abdominal swelling revealed the presence of urine . ultrasonography revealed the presence of a massive hypoechoic lesion occupying almost the entire abdomen , which exerted pressure on the bowel on the left - hand side . a cystic lesion revealed multiple septations , which were likely to have arisen from the right kidney . a computerized tomography ( ct ) scan revealed a large abdominal mass of water density in the all - abdomen area , which comprised a huge cystic mass ( 41.530.530.5 cm ; fig . c ) that , in the right kidney , occupied almost the entire abdominal cavity . this mass had displaced the small intestine , colon , pancreas , spleen and the left kidney . after admission , the cystic mass was punctured , and a total of ~24 l urinal fluid was drained by urethral catheterization over a period of 8 days ( 3 liters each day ) . there was an improvement in the general condition of the patient : the distended abdomen decreased in size ( fig . a right nephrectomy was performed : when an incision was made in the right lumbar region , the cyst presented as a retroperitoneal soft cystic dilatation mass , lacking in definition , measuring almost 302015 cm ( fig . 3 ) , which was able to adhere to adjacent organs , including the liver and the pelvic cavity , lifting the cecum and the ascending colon . the ureter was normal , and ureteropelvic junction obstruction ( upjo ) was established as the cause of gh . gh is a rare urological condition , which occurs in patients of all ages , defined as the presence of > 1 liter of fluid in the collecting system ( 1 ) . symptomatic nephrolithiasis and hydronephrosis more frequently present as clinical conditions , and gh is a rare clinical phenomenon . although numerous cases of gh have been reported in the literature , only a few of them contained > 2 liters of fluid ( table i ) . in the present case report , the hydronephrotic kidney contained 24 l urine , and ghs seldom fill the entire abdomenal space , as had occurred in our patient . the most common cause of gh , as detailed in the literature , is a condition caused by upjo , although stone disease , trauma , renal ectopy and ureteral tumors have also been reported ( 2 ) . in the present study , the clinical symptoms of gh are non - specific , and may present with vague symptoms , including an increased abdominal girth due to the presence of a mass in the flank . gh is a slowly progressive disease , and a huge abdominal mass or distended abdomen may cause pain , and be symptomatic of hematuria , recurrent urinary tract infection or other symptoms or complications described in the literature , including nausea , fatigue or dyspepsia , urinary tract infection , loss of weight , renal insufficiency , gross hematuria resulting from trauma in the area , the compression of surrounding structures , or even rupture of the kidney ( 3 ) . ultrasonography and ct scans have facilitated the diagnosis of hydronephrosis , and a case of gh may be defined as the presence of hydronephrosis occupying a hemiabdomen , which meets , or extends beyond , the midline , and which extends at least five or six vertebral bodies in length ( 4 ) . however , in a number of cases , a differential diagnosis between gh and other cystic formations proves to be difficult . therefore , as demonstrated in the present case report , an accurate diagnosis of gh in individual cases remains challenging . contrast - enhanced ct of the abdomen and the pelvis provides the gold standard for an accurate diagnosis of gh , and other useful diagnostic imaging techniques include abdominal radiography and intravenous urography . the ideal treatment for gh is nephrectomy , and this is the procedure of choice ; other treatment options in a functional kidney include percutaneous nephrostomy , reduction pyeloplasty with nephropexy calycoureterostomy , and calycocystostomy ( 5 ) . in spite of the widespread use of prenatal ultrasound and the development of new diagnostic techniques , a puncture / drainage procedure may be performed in cases where the condition of the patient does not allow other treatments to be performed , or where hemodynamic changes may occur following a sudden abdominal decompression ( 6 ) . in the present case study , the patient underwent a right nephrectomy and was discharged on postoperative day 8 . in conclusion , gh is a rare condition , which is associated with the occurrence of cystic abdominal masses , and the puncture / drainage procedure or nephrectomy provides the most suitable method for surgical intervention .
the hydronephrotic kidney , resulting from a ureteropelvic junction obstruction ( upjo ) , presents commonly as a clinical condition , with the presence of usually no more than 12 liters in the collecting system , but a very small number of cases of giant hydronephroses ( ghs ) has been reported in adults . a gh is defined as the adult renal pelvis containing > 1 liter of urine , or at least 1.5% of the body weight . in the majority of cases , the range of the hydronephrotic kidney remains restricted to the renal area . however , the patient described in the present case report had a range for the hydronephrotic kidney which occupied almost the entire abdominal cavity ( ~24 l ) , and cases such as these are rarely presented ; therefore the aim of the present case study was to document a clear case of gh resulting from upjo , also accompanied by a review of the current literature .
four samples of pork liver sausage ( designated sausages a d ) collected at the final production stage from 4 independent manufacturers in 3 locations in southern france were found to be hev positive by using real - time reverse transcription pcr ( rt - pcr ) . samples were tested in 2 institutes , the animal health and veterinary laboratories agency , united kingdom , and wageningen university and research centre , the netherlands . three - dimensional ( 3d ) cell culture propagation was performed to investigate the presence of infectious hev particles . plc / prf/5 hepatocarcinoma cells ( american type culture collection 8024 ) were cultured as a monolayer ( 2d ) at 37c in gtsf-2 medium ( 8) in a 5% co2 environment . cells were trypsinized at 95% confluence and resuspended in fresh medium to a density of 2 10 cells / ml . fifty milliliters of cell suspension was then introduced into a rotating wall vessel with 10 mg / ml of porous cytodex-3 microcarrier beads ( collagen type i coated porous microspheres , average diameter 175 m ( sigma , dorset , uk and zwijndrecht , the netherlands ) and incubated at 37c in 5% co2 . the inoculum was prepared by homogenizing a 2.5-mg fragment of each sample with mortar and pestle in 5 ml of culture medium . the homogenate was centrifuged at 8,000 g for 3 min , and the supernatant was filtered sequentially through 1.2-m , 0.45-m , and 0.2-m filters to reduce the risk for bacterial contamination . the medium was removed from the rotating wall vessel , and 2.5 ml of inoculum was incubated with the cells for 2 h at 35.5c ; at that point , 47.5 ml of fresh medium was added . subsamples of medium ( 140 l ) were collected in duplicate , added to 560 l of lysis buffer ( qiaamp viral rna mini kit ; qiagen , crawley , uk , and venlo , the netherlands ) , and stored at 20c until rna extraction was performed . both institutes performed real - time rt - pcr by using primers and probe as described ( 9 ) , using the superscript iii platinum one - step quantitative rt - pcr system ( invitrogen , paisley , uk , and bleiswijk , the netherlands ) . negative ( water ) and positive ( extract from positive fecal sample , genotype 3 ) controls were included . hev rna was detected in the 3d cell culture supernatants of all 4 sausage samples up to 8 d postinfection ( dpi ) . thereafter , hev rna was detected only in the cells inoculated with the sample a homogenate ( figure 1 ) ; it was assumed that the signals from the other 3 samples represented residual inoculum or an abortive infection . the sample a culture showed a cycle threshold ( ct ) value of 27 on the day of inoculation ( dpi 0 ) that increased to 35 on dpi 5 , continued to increase until dpi 11 , when it peaked at 38 and then began to decrease . cycle threshold ( ct ) values detected by real - time reverse transcription pcr for hepatitis e virus positive supernatant of 3d cells infected with homogenate of pork sausages from france . diamonds indicate testing of sausage a in the united kingdom ; squares indicate testing of sausage a in the netherlands ; triangles indicate testing of progeny of sausage a in the united kingdom . to evaluate the infectivity of progeny viruses from the primary inoculation , supernatant positive for hev by rt - pcr from dpi 16 was used to infect fresh 3d plc / prf/5 cultures according to the protocol described for the primary inoculation . cells infected with the supernatant from the original sample a homogenate cultures had positive hev rna test results on most days after inoculation ; ct values remained relatively constant at an average of 37 from immediately after inoculation ( dpi 0 ) to the end of the experiment at dpi 35 . to compare the cultured virus to the inoculum , a partial fragment of the open reading frame 2 of hev extracted from culture subsamples ( dpi 16 , dpi 55 ; progeny subsample at dpi 35 ) was sequenced as described ( 10 ) . hev rna sequences detected in the culture subsamples were characterized and demonstrated 100% identity with that of the inoculum ( 304 bp of open reading frame 2 ) but differed from the control strain used . further confirmation of the presence of viable virus particles in cell culture was sought by using electron microscopy . supernatants of the cell cultures were applied to pioloform / carbon - coated , 400-mesh copper grids ( plano gmbh , wetzlar , germany ) for 10 min , fixed with 2.5% aqueous glutaraldehyde solution for 1 min , and stained with 2% aqueous uranyl acetate solution for 1 min . the specimens were examined through transmission electron microscopy by using a jem-1010 microscope ( jeol , tokyo , japan ) at an 80-kv accelerated voltage . hev particles were observed in the supernatant of the sample a culture collected at dpi 33 ( figure 2 , panel a ) . for additional proof by immunoelectron microscopy , an escherichia coli expressed , his - tagged hev genotype 3 capsid protein derivative harboring amino acid residues 326608 ( 11 ) was used to generate hev - positive serum in a rabbit by 3 subcutaneous inoculations at 4-week intervals ( p. dremsek et al . the immunoelectron microscopy examination of this serum and goat anti - rabbit igg linked with 5-nm gold particles ( bbinternational , oconomowoc , wi , usa ) confirmed the presence of hev particles ( figure 2 , panels b d ) . hepatitis e virus ( hev ) particles in the cell culture supernatant of pork liver sausage sample a , collected at 33 dpi . a ) transmission electron micrograph of negatively stained hev particles 33 and 34 nm ( arrowheads ) . b d ) hepatitis e virions 28 ( b ) , 33 ( c ) , or 32 ( d ) nm in diameter , identified by using an hev genotype 3specific rabbit hyperimmune serum and a gold - labeled secondary antibody . we confirmed that 1 sample of pork liver sausage that had positive test results for hev rna by real - time rt - pcr contained viable hev . we cultured 4 sausage samples in 2 different institutes by performing 3d cell - culture propagation of hev ; hev replication was detected in the same sample independently in both institutes , and the replicated virus was shown to be infectious . we observed entire , cell - free virus particles by transmission electron microscopy at dpi 33 , providing further proof of in vitro replication of the virus that contaminated the pork liver sausage . we conclude that pork liver sausages can contain infectious hev and that consuming these products should be regarded as a risk factor for hev infection . furthermore , we have shown the potential of the 3d culture system to correlate the presence of hev rna with the presence of infectious hev particles .
we investigated viability of hepatitis e virus ( hev ) identified in contaminated pork liver sausages obtained from france . hev replication was demonstrated in 1 of 4 samples by using a 3-dimensional cell culture system . the risk for human infection with hev by consumption of these sausages should be considered to be high .
multiple myeloma is a malignant monoclonal gammopathy characterized by proliferation of myeloma cell clones responsible for their osteolytic lesion appearance ( favoring pathological fractures and nerve compression ) , bone marrow infiltration ( with the advent of bone marrow failure ) , abnormal protein production m component ( responsible for damage kidneys , the hyperviscosity syndrome and secondary amyloidosis ) , installing of immune deficiency ( favoring infections ) . infections are the most common complications in multiple myeloma , followed by bleeding complications , neurological , renal , hypercalcemia related complications and amyloidosis . the mechanism of developing these infectious complications is mixed and involves several pathophysiological links like : - proliferation of myeloma cells in the bone marrow resulting in decreased normal hematopoiesis with bone marrow failure and peripherals cytopenias including leukopenia and granulocytopenia ; - monoclonal immunoglobulins secreted by malignant clone can adhere to leukocytes causing their functional capacity decrease ( phagocytosis , bactericidal activity ) or to some fractions of complement , inducing abnormal opsonized capacity ; - humoral immune deficiency - increased monoclonal component myeloma - is accompanied by a decrease in other normal immunoglobulin with polyclonal hypogammaglobulinemia installation . the decrease in number and functional capacity of polyclonal b lymphocytes affects the ability of b lymphocytes to respond to specific antigens and inhibition of antibody formation in the primary and secondary immune response and thus decrease the body 's defense capacity . - cellular immunity dependent on t lymphocytes is less affected - expressed in vitro by the decrease of reactivity of t cell lymphocytes to mitogens . abnormal t lymphocytes , nk cells and monocytes increase humoral immune deficiency - immunosuppression due to corticosteroid and cytostatic treatment increases the risk of infections . myeloma cells may be extramedullary located due to extramedullary plasmacytoma or extramedullary dissemination of mm . extramedullary plasmacytoma involves submucosal lymphoid tissue of the nasopharynx or paranasal sinuses without affecting the red bone marrow . a 60 years old man , smoker , with no history of respiratory hospitalization and relative good past health was admitted in our hospital with fever , chronic cough , significant weight loss and progressive dyspnea in the previous two months . physical examination then showed pale skin , fine crepitation over bilateral lung bases , pain in some small joints , blood pressure was normal but he had sinus tachycardia ( 132/min ) , sao2=90% . chest x - ray evidenced diffuse , bilateral lower - zone reticulo - nodular shadowing . repeated exams of sputum smear were negative for mycobacterium tuberculosis ( both microscopy and culture ) . his hemoglobin was half of the normal value but renal function and calcium level were within normal limits . chest computed tomography revealed scattered ground glass opacities in both lungs that were suggestive of interstitial lung disease and no suspect thoracic lymph nodes . so the next step was bronchoscopy and bronchoalveolar lavage ( bal ) fluid was examined and it revealed the presence of monoclonal plasmatic cells in bal . we transferred the patient to hematology where he was further investigated and treated for his severe anemia . he was diagnosed with multiple myeloma igg type stage iii subclass a. after this the pacient had a rapid decrease of cardiac and respiratory function and the chest x - ray after 4 months showed the extension of the interstitial infiltrates in lower fields of both lungs . because of his cardiac condition ( ischemic heart disease , cardiomyopathy and sinus tachycardia ) he could nt start specific chemotherapy for mm so his prognosis is severe . extramedullary dissemination involves the spleen , liver , lymph nodes , thyroid , adrenal , ovary , testis , lung , pleura , pericardium , gastrointestinal tract and skin . antemortem diagnosis of extramedullary dissemination in the lungs can be determined by bronchoalveolar lavage ( monclonale plasma cells are found ) or lung biopsy ( interstitial infiltrate of plasma cells ) . pulmonary parenchyma is an uncommon site of extramedullary involvement in multiple myeloma ; only isolated cases with histological proofs have been reported in the literature . one study described 13 cases with lung involvement of multiple myeloma , of which six had pneumonia , two had mass lesions , two had multiple nodular lesions , and three had interstitial infiltrates . approximately 10% of patients with multiple myeloma demonstrate pulmonary findings during the course of their disease . the findings most commonly described include bacterial and fungal infections , pleural - based plasmacytoma , pleural effusions , and well - circumscribed pulmonary plasmacytoma . identifying malignant plasma cells in bal fluid from multiple myeloma patients may be difficult , especially when the plasma cells are mature in appearance or low in number . diffuse pulmonary myelomatous involvement therefore may be more frequent than has previously been reported . a high index of suspicion is required because infection , hemorrhage , idiopathic pneumonia , edema of the lung , and plasma cell infiltration may have identical radiologic manifestations . citologic examination of the sputum and bal fluid and an analysis of cytoplasmic immunoglobulin dna provide a simpler means of confirming diagnosis and may obviate the more invasive needle biopsy or open lung biopsy . we present one such case in which diagnosis was established from bronchoalveolar lavage ( bal ) fluid demonstrating the presence of monoclonal plasma cells . diffuse parenchymal infiltrates in the lung due to mm are rare but should be considered when finding pulmonary infiltrates . analysis of bal fluid for plasma cells is a noninvasive method to establish a diagnosis . pulmonary mm is associated with rapid progression of the disease unlike primary pulmonary plasmacytomas that has good prognosis .
introduction : mutiple mieloma ( mm ) cells are rarely found in extramedullary sites . the sites of extramedullary dissemination reported in the literature are spleen , liver , lymph nodes , kidneys , thyroid gland , adrenal gland , ovary , tests , lung , pleura , pericardium , intestinal tract and skin . we report a case in which the myeloma was diagnosed after we discovered the presence of monoclonal plasma cells in the bronchoalveolar lavage fluid ( bal ) . matherial and method : a case in which diagnosis was established from bronchoalveolar lavage ( bal ) fluid demonstrating the presence of monoclonal plasma cells in craiova pneumology departament . results : analysis of bal fluid for the presence of plasma cells and for cytoplasmic immunoglobulin dna provides a noninvasive means of establishing the diagnosis . conclusions : pulmonary parenchyma is an uncommon site of extramedullary involvement in multiple myeloma . interstitial lung disease as pulmonary manifestation of multiple myeloma is even rarer ; only isolated cases with histological proofs have been reported in the literature .
classical exstrophy of the bladder is a rare anomaly with an incidence of 1 in 30,000 - 50,000 live births . there are a few case reports of covered exstrophy with anorectal malformation and the complete duplication of bladder , urethra , and the mullerian structures . however , covered exstrophy associated with duplication of introitus and the lower vagina has not been reported earlier in the english literature . a 2.6 kg , 2-day - old female child , delivered by cesarean section , was brought to us with malformed external genitalia and a swelling over the lower part of the abdomen and the perineum . physical examination revealed a lobulated bowel projection with a skin - covered swelling extending over the lower part of the abdomen and the perineum . the umbilicus was low set , situated just above the upper extent of the swelling . the pubic symphysis was widely separated with a bulging urinary bladder occupying the space in between . the introitus was replaced by the swelling which was continuing on to the lower abdomen . there was duplication of the introitus with two openings , each flanked by the single labia majora located laterally , in the groin area [ figure 1a ] . the two orifices on either side were in communication with each other as a catheter introduced from one orifice exited through the other . the urethral orifice could not be seen from the outside ; however , both the introitus openings were discharging urine intermittently . an infantogram and cross - prone - lateral x - ray showed a high type of anorectal malformation and the absence of calcification in the area of accessory bowel loop . ( b ) neoanus after excision of accessory bowel segment the baby was resuscitated and undertaken for surgery . a midline laparotomy showed that the small bowel and large bowel were normal and in continuity . the internal genitalia were female type and the uterus was unicornuate with normal fallopian tubes and the ovaries . the accessory bowel loop was attached to the lower part of the swelling and the lumen was in continuity with lumen of the terminal part of the rectum . the extra piece of the bowel was excised and the terminal part of rectum fashioned as a neoanus [ figure 1b ] . a suprapubic cystostomy ( spc ) was also inserted as the anatomy of the bladder neck and urethra was still not clear . the rectus sheath in the upper part of the abdomen was closed primarily and the lower part containing the urinary bladder in subcutaneous location was left as such . the histopathologic examination of the sequestered loop revealed colonic mucosa . the baby was put on oral feeds after 48 h and the spc was clamped . the baby was decompressing well from the perineal openings and was discharged from the hospital after removal of the spc catheter . , the baby underwent genitoscopy examination which showed single urethral orifice with duplication of the lower part of the vagina . at present , the patient is waiting for the genital reconstruction at a later age and is continent for urine . variants of the bladder exstrophy complex are quite uncommon and constitute about 8% of all the cases . these variant lesions , also called split symphysis variants , have all the musculoskeletal findings of classical exstrophy , but the bladder is closed with varying degrees of skin and the subcutaneous covering , and the urethra and sphincter mechanism may be intact . exstrophy variants have been classified into four major subgroups , namely pseudoexstrophy , superior vesical fissure , duplicate exstrophy , and covered exstrophy with visceral sequestration . classical bladder exstrophy is believed to result from the persistence or overdevelopment of an abnormal infraumbilical cloacal membrane that acts as a wedge and prevents the medial progression of the lateral mesoderm between its ectodermal and endodermal layers . the exstrophy variants are explained by the incomplete rupture or persistence of this abnormal infraumbilical cloacal membrane . , however , explained the occurrence of exstrophy and its variants to be the consequences of persistence of an abnormal caudal insertion of the body stalk on the embryo . covered exstrophy seems to differ from the classical exstrophy not only in the degree of exposure of the bladder and urethra , but also by frequent presence of the abnormalities of the external genitalia . a partial diphallus with incomplete urethral duplication and laterally displaced phallus with widely separated scrotal halves associated with covered exstrophy have been reported by mildenberger et al . genital abnormalities observed in females with covered exstrophy included bifid clitoris ( 67% ) , labial cleft ( 11% ) , hypoplastic labia ( 11% ) , and stenosed duplicate vagina ( 11% ) . covered exstrophy with complete duplication of the genital and the lower urinary tract with visceral sequestration have also been reported . the embryological process in variants of exstrophy may not affect the development of the lower urinary tract significantly , but the so - called wedge effect may prevent fusion of superficial paired genital swellings as happened in our case too . the vaginal duplication in our case involved the introitus and the lower part of the vagina only , sparing the developmentally different upper vagina and the uterus . covered exstrophy differs from pseudoexstrophy variant in that , there is an associated isolated ectopic bowel segment present on the inferior abdominal wall near the genital area . this segment is usually a segment of the colon and has no communication with the underlying gastrointestinal tract . however in the present case , the segment was communicating with the rectum and this has not been described earlier in the literature . it has been suggested that this condition is a result of the secondary closure of the cloacal membrane which isolates ectopic embryological colonic cells . ten cases ( six male and four female ) of covered exstrophy with visceral sequestration have been reported in the world literature . none of the cases showed the presence of anorectal malformation . as the continence in these patients is generally good , the treatment is required only for associated gastrointestinal or genital anomalies .
covered exstrophy is a rare variant of the exstrophy - epispadias complex . we report a female newborn with covered exstrophy , absent anal opening and duplication of the introitus and the lower vagina . this rare , previously unreported , combination of anomalies highlights the complexity of the embryological events in the caudal area during separation of the hindgut and allantois .
a 20-year - old woman visited the neurology clinic for generalized stiffness and frequent falling episodes secondary to tactile stimuli . she was born at term , and her antenatal and birth history were not remarkable . there was no developmental delay or neurologic deficit ; however , her parents had noticed sudden falling events since she was five years old . in response to unexpected tactile stimulation , she felt her body become rigid for a few seconds , which resulted in injurious falling down events with spared consciousness . she usually kept indoors and walked cautiously in order to avoid unexpected falling accidents . in childhood , the frequency of her falls was approximately four or five times per year , but after her teenage years , the frequency decreased to once or twice per year . her father and older sister had similar symptoms ( figure 1a ) . on physical examination whole exome sequencing with genomic dna extracted from peripheral blood identified a heterozygous missense mutation c.896g > a ( reference sequence : nm_001146040.1 ) in glra1 . no mutations were found in other genes known to cause familial hyperekplexia , such as glrb , slc6a5 , gphn , and arhgef9 . the change in the patient s glra1 sequence alters the arginine codon at 299 to a glycine codon ( p.arg299gln ) . the same mutation was also found in her sister , who was symptomatic ; however , we could not perform a genetic study on her parents . clonazepam was administered at a dose of 0.5 mg per day , which resulted in an improvement in the startle response . hyperekplexia , known as a hereditary startle disease , is characterized by an exaggerated startle response and neonatal hypertonia . this disorder is a rare neurogenetic condition , but it is potentially treatable . the symptom spectrum can vary from an exaggerated startle response to infantile apnea spells and even injurious falls . a previous case study reported a possible association with sudden infant death syndrome . in patients with hyperekplexia , no abnormalities are observed on routine blood tests , urinalysis , brain imaging studies , or eeg . hyperekplexia could be misdiagnosed as epilepsy , cerebral palsy , anxiety disorder , or conversion disorder and therefore can be mistreated . early diagnosis and treatment are important , as they not only prevent injuries but may also influence the quality of life of a patient . to conduct a systematic review of the literature regarding hyperekplexia cases caused by mutation in the glra1 gene , we retrieved articles from the pubmed database using the keywords hyperekplexia and glra1 , english and hyperekplexia and case , english . clinical features , ethnicity , types and locations of mutations and mode of inheritance , as obtained from the retrieved literature , are summarized in table 1 . most patients showed neonatal hypertonia ( 76% ) and an exaggerated startle response ( 73% ) . most patients ( 64 out of 66 cases with the nose - tapping test ) exhibited a hyperactive brainstem reflex , which was found with the nose - tapping test . exaggerated head retraction reflexes in response to the nose - tapping test indicate exaggerated brainstem reflexes and provide an important clue to diagnose hyperekplexia . the patients also suffered from severe complications , such as developmental delay ( 16.8% ) and apnea spells ( 20.7% ) . external abnormalities , such as umbilical ( 13.9% ) and inguinal hernia ( 11.2% ) , hip dislocation ( 6.8% ) and club foot ( 1.9% ) , were not uncommonly observed ( figure 1c ) . these findings are consistent with a previous case series that is not included in our analysis . clinical features that helped differentiate hyperekplexia from epilepsy included unexpected stimulus - inducing falling accidents , short episodes lasting only a few seconds , and spared consciousness , with no other abnormal movements accompanying the event . cautious gait , face lacerations and family history may be helpful for differentiating hyperekplexia from conversion disorder . frequent falls were observed in 39.8% of cases for which information was available , and cautious gait was reported infrequently ( 4% ) . data regarding falls and gait might have been biased by patients age , and therefore , there is a potential for missed information . six out of 161 reviewed patients exhibited a wide - based and stiff gait due to considerable fear of an unexpected falling event , and two patients lacked confidence in outdoor environments , resulting in impaired social behavior . presentation of a cautious gait resulting from unexpected falling episodes might be an indication of hyperekplexia . clonazepam , which enhances gaba - gated chloride channel function and presumably compensates for defective glycine - gated chloride channel function , has been considered the first choice for the treatment of hyperekplexia . antiepileptic drugs , including carbamazepine , phenytoin , valproate , and vigabatrin , have also been used for treatment . in this review , 60 out of 70 cases ( 85.7% ) showed good response to clonazepam , which is similar to a previous study . among genes causing familial hyperekplexia , glra1 is the most common causative gene , accounting for 80% of hereditary cases . our patients carried a heterozygous mutation , p.arg299gln , which was inherited in an autosomal dominant fashion . missense mutation of the arginine at codon 299 , which was previously reported as codon 271 , is the most common ( table 1 , figure 1d ) . both autosomal dominant and recessive inheritance have been reported in familial hyperekplexia caused by glra1 mutation . our analysis showed that dominant inheritance ( 73.9% ) was 3-fold more commonly reported than recessive inheritance ( 26.1% ) . interestingly , most dominantly inherited mutations were located between codons 290300 of the glra1 gene ( figure 1d ) . distribution of ethnicity in the reviewed hyperekplexia cases was caucasian ( 42% ) , asian ( 33% ) , turkish ( 14% ) , arabic ( 8% ) , and african ( 3% ) ( figure 1e ) . in a genotype - ethnicity correlation , 8 asian families ( including isolated cases ) and 7 caucasian families demonstrated the p.arg299gln mutation of the glra1 gene . these findings support the notion that the arg299 amino acid site is vulnerable to hyperekplexia in ethnically disparate cases .
familial hyperekplexia , also called startle disease , is a rare neurological disorder characterized by excessive startle responses to noise or touch . it can be associated with serious injury from frequent falls , apnea spells , and aspiration pneumonia . familial hyperekplexia has a heterogeneous genetic background with several identified causative genes ; it demonstrates both dominant and recessive inheritance in the 1 subunit of the glycine receptor ( glra1 ) , the subunit of the glycine receptor and the presynaptic sodium and chloride - dependent glycine transporter 2 genes . clonazepam is an effective medical treatment for hyperekplexia . here , we report genetically confirmed familial hyperekplexia patients presenting early adult cautious gait . additionally , we review clinical features , mode of inheritance , ethnicity and the types and locations of mutations of previously reported hyperekplexia cases with a glra1 gene mutation .
admission to the intensive care unit ( icu ) is only part of the course that a patient makes during their illness . intensive care is not a gatekeeper speciality and patients therefore generally have their first contact in a hospital with emergency physicians , surgeons , internists , cardiologists , and so forth . after discharge from the icu , most patients will return to the gatekeeper speciality - surgery , internal medicine , or the like . following discharge from the hospital , patients will return to their homes and therefore the contact with their lifetime physician from their home situation , the general practitioner ( gp ) , is of utmost impor tance . the gp is also expected to have longstanding knowledge of the home situation of the patient . whenever decisions in terms of end - of - life decisions have to be taken during icu admission , the intensivist should be well informed . not only is medical professional judgement important , but also the will and wishes of the patient . to gather all this information , the intensivist should contact doctors who have been involved in the treatment of the patient so far , including the gp , as well as the patient and relatives if possible . it should therefore be stressed as crucial that treating intensivists have ( regular ) contact with gps . etesse and colleagues report in the present issue of critical care about the relationship between gps and intensivists in a part of southeastern france . the response rate was very low ( 20% ) and this will influence the results and conclusion . only one - half of the gps rated their contact with the intensivist ( on a scale from 1 to 100 ) at > 57 , and only 25% rated as > 77 . the conclusion that gps are not very satisfied by communication with intensivists is therefore an under statement . to which extent the general dissatisfaction of gps influences the results is not addressed in this french study . data from the netherlands suggest that overall professional satis faction is worrisome , especially in the older gps , and that 34% of all gps want to stop working as a gp before the age of 60 . it is of note that this general dissatisfaction is in line with the specific finding of the authors . the authors did not address whether the icus involved were using any organizational structure within the depart ment for communication with the gps . it should be understood that in cases of end - of - life decisions where the gp has previous good contact with the family and patient , and therefore the best knowledge of the premorbid situation , the gp should be contacted and consulted . some major changes in the position of the gp , however , have occurred over the past decades . the solo - working gp , working 7/7 days , has disappeared since most gps nowadays work in a team . a significant proportion of gps work part - time , so the old perception of the gp who knows all his patients from birth to death is outdated . in addition , gps are not always easily reached in a timely fashion , which seriously hampers the communication process . although we are not aware of any data , it is not unreasonable to assume that a significant proportion of patients in the icu do not know their gp very well . in such circumstances it is unlikely that the gp can add to the information required to make important decisions in the icu . the information from the intensive care department regarding admission to the icu should undeniably be given in all cases , and should be timely . the authors correctly point out the consequences of icu admission of the patient for their family and loved ones . symptoms of anxiety and depression are very common in the relatives of critically ill patients . psychiatric illness ( depres sion , anxiety disorders or complicated grief disorders ) can occur in as much as 30% of relatives who were confronted with death in an icu . gps can play an important role in this respect , provided that they are well and timely informed . etesse and colleagues must be congratulated on their effort , especially since they distillate tangible advice from their data that can be easily implemented in a communication structure of intensive care - including systematic telephone calls to the gp on admission of the patient to the icu , good communication with the family , and instant information for the gp at the moment of discharge from the icu . in our view , the end - of - life decisions are to be made primarily by the intensivist and the team involved , using all available and relevant information .
for many reasons it is crucial that treating intensivists have ( regular ) contact with general practitioners ( gps ) . information about the premorbid condition of the patient , their will and wishes , is of importance to be able to set appropriate treatment goals . the gp is the doctor who is responsible for the patient once discharged from the hospital . additionally , the gp can play an important early role in the support of relatives , provided the gp is timely informed . this kind of communication should be organized in a structured way within the intensive care unit department .
thoracic injuries are most commonly caused by penetrating trauma ( stab or gunshot wounds ) or blunt trauma ( traffic accidents , falls from heights ) [ 2 , 3 ] . this paper presents two cases of penetrating chest wounds treated at the cardiac surgery department , trauma surgery department , and intensive care unit of the jzef stru multidisciplinary hospital in pozna . the patients were two young men , aged 29 and 36 , who were admitted to the trauma center in 2013 after suicide attempts . the strategy for managing these life - threatening situations , designed by a multidisciplinary team of consultants , enabled the choice of optimal treatment and diagnostic methods for both patients . the 29-year - old patient had been undergoing psychiatric treatment for several years ; his mental condition deteriorated , as evidenced by increasing aggression and significant psychomotor agitation . the patient 's housemate decided that it was necessary to call the police and emergency services . during the ensuing negotiations with police officers , the patient stabbed himself in the chest above the sternum with a knife . he was admitted to the emergency department with a penetrating chest wound located at the level of the jugular notch ( fig . , the patient was conscious and did not suffer from circulatory or respiratory failure ( blood pressure 130/80 , heart rate 90 bpm ) ; his speech was coherent , but he had trouble swallowing . imaging diagnostic procedures were started immediately , including a chest x - ray examination ( fig . 2 ) and computed tomography of the chest and neck . initial evaluation revealed left - sided pneumothorax and injury of the left thyroid gland lobe ; the blade of the knife extended from the jugular notch to the left transverse process of the th5 vertebra without passing through important structures . therefore , the patient was transferred to the operating theater , where , under general anesthesia and under the supervision of a cardiac surgery consultant , suction drainage was introduced to the left pleural cavity , and the knife was removed from the puncture site . as there was no significant bleeding or air leakage , the wound was cleaned and closed with surgical sutures ( ethilon 4/0 ) . after the procedure , the patient was transferred to the intensive care unit ( icu ) , where analgosedation and mechanical ventilation were continued . during the 1 day after the intervention , the patient was in a stable circulatory and respiratory condition with periodic drops of saturation to 90% . in the afternoon , the drainage of red fluid from the left pleural cavity increased . due to increasing symptoms of respiratory failure , a decision was made to employ mechanical ventilation and sedation . computed tomography of the chest visualized a massive hematoma in the left pleural cavity ; no contrast extravasation was observed ; the distal segment of the drain had no contact with the hematoma . furthermore , the examination revealed slight right - sided pneumothorax with fluid accumulation ; the left lung was compressed by the hematoma , and edema was visualized on the left thyroid gland lobe . in light of these findings , a trauma surgeon was consulted ; the surgeon decided to introduce another drain into the left pleural cavity , but found no indications for thoracotomy . on the next day , chest x - ray examination demonstrated that the amount of fluid in the left pleural cavity was reduced , the left lung was aerated , and the drains were properly located . over the subsequent postoperative days , the patient 's clinical condition stabilized , and he was extubated on the 8 day . on the 10 day after the removal of the pleural drains , the patient was transferred to the regional hospital for nervous and mental diseases in gniezno in a stable circulatory and respiratory condition . the knife penetrating the patient 's chest at the level of the jugular notch lateral photograph of the chest . the blade of the knife extending from the jugular notch to the left transverse process of the fifth thoracic vertebra ( th5 ) the 36-year - old patient without previous psychiatric history stabbed himself with a knife in the left side of the precordial area during an argument with his fiance . the patient came to the hospital on his own ; during admission , he claimed that the wound was superficial because it occurred while performing everyday kitchen chores . during his stay at the emergency department , cardiac surgical consultation and imaging examinations were recommended . during the consultation , chest x - ray examination revealed an increase in the width of the mediastinal shadow on the left side ; the urgently performed computed tomography of the chest revealed signs of bleeding into the mediastinum as well as atelectatic changes in the dorsal region of the left lung . dissection of the pericardial sac revealed a large amount of fluid blood and isolated thrombi causing cardiac tamponade . an incised wound , 2 cm in length , was observed on the anterior surface of the pulmonary trunk ; it was immediately repaired with continuous prolene 4 - 0 sutures . evaluation of the pleural cavity demonstrated no lung damage or bleeding ; however , injury of the interior chest wall and rupture of the left internal thoracic artery were observed . the patient was transferred to the icu in a relatively severe general condition ( no coherent speech , mechanical ventilation , intravenous catecholamine infusion ) . for 4 days after the procedure , the patient required intensive medical care , catecholamine support , mechanical ventilation , and blood product transfusion . during the patient 's stay at the icu , his condition stabilized gradually , and he was extubated on the 5 postoperative day . during subsequent days thanks to intensive rehabilitation , the conscious patient was transferred to the trauma surgery department on the 10 postoperative day in a stable circulatory condition in order to undergo further treatment . the presented cases of young men with penetrating chest wounds confirm that all patients with thoracic injuries require intensive and individualized diagnostics and therapy . the applied multi - specialist approach resulted in successful outcomes . in the first case , the circumstances of the injury ( visible handle of the knife which was almost completely sunk into the patient 's chest ) initially suggested that extensive surgical intervention would be required . however , the use of imaging diagnostics enabled the exclusion of serious injuries within the chest ; the treatment could , therefore , be limited to removing the knife in the operating theater . in the case of the second patient , the small size of the penetrating chest wound did not reflect the extent of the damage caused by the blade . computed tomography revealed serious injuries within the chest , which were promptly treated with cardiac surgery . thoracic injuries should always be approached with the utmost caution , and advanced diagnostics should be employed as soon as possible in order to confirm or exclude damage to vital organs .
thoracic injuries are usually caused by penetrating or blunt trauma . the primary method of treatment is surgery . this study describes two cases of male patients with stab wounds of the chest resulting from suicide attempts . the first case involved a 29-year - old patient transported and admitted to the hospital with a knife still in his chest ; its blade extended from the jugular notch to the 5th thoracic vertebra but did not damage any important structures . the applied treatment , limited to evacuating the knife , resulted in a satisfactory outcome , and the patient was discharged from the intensive care unit ( icu ) in good condition . the second patient reached the hospital on his own . on admission , he did not reveal the real cause of the wound ; however , in view of his deteriorating condition , he admitted that the knife penetrated deeply into the mediastinum . in this case , sternotomy was necessary to stop the bleeding of the pulmonary trunk and internal thoracic artery . after completion of treatment , the patient was discharged in good condition . the described management of life - threatening situations conducted by a multidisciplinary team of consultants enabled the choice of optimal treatment methods and resulted in successful outcomes .
although spontaneous spinal epidural haematoma ( sseh ) is a low incidence condition , it is widely recognised throughout the literature as a cause of myelopathy [ 5 , 15 ] . the relationship between sseh and anticoagulant therapy is well known and the probable cause of bleeding is thought to be the rupture of the venous epidural plexus during a sudden elevation of thoracic or abdominal pressure [ 4 , 6 , 8 , 10 , 11 , 13 , 15 ] . radicular involvement is very rare and , when it appears , it mostly affects the lumbar spine , producing ciatalgia [ 1 , 3 , 11 ] . although the currently suggested approach is an urgent surgical decompression [ 9 , 10 ] , conservative treatment is recommended when there is an objective improvement of the neurological status [ 2 , 11 , 12 ] . in general , the originality of this report arises in three points : the case involved the cervical spine producing simple radiculopathy.it was solved spontaneously.anticoagulation therapy was not discontinued . a 64-year - old white man undergoing anticoagulant therapy because of cardiac valve prosthesis arrived at the emergency room of our hospital suffering from a sudden onset of cervical pain and a left c5 brachial pain and weakness . based on the suspicion that it was a case of disc herniation with left c5 root involvement , symptomatic treatment by means of non - steroid anti - inflammatory drugs and painkillers was indicated . a cervical mri was scheduled and the patient was invited to undergo further examination at the spinal surgery unit . owing to the fact that the first physician was not a spinal surgeon and therefore not aware of the relationship between anticoagulation and sseh , discontinuation of anticoagulant therapy was not indicated . two days later , when the patient was examined by the spinal surgery unit , the pain had been completely relieved and the weakness had also decreased . the mri revealed a left posterolateral ovoid mass compatible with a haematoma extending from c4 to c5 ( fig . 1 ) . once the specialist knew the real cause of the radicular syndrome , the haematologist was consulted and they preferred not to suspend the anticoagulant therapy ( despite an adequate level of anticoagulation , inr 2,7 ) in order to prevent thromboembolism . . a , c isointense appearance of the haematoma on the mri t1-weighted sagittal and axial images . b , d hyperintense appearance of the haematoma on the mri t2-weighted sagittal and axial images mri 2 days after the onset of symptoms . a , c isointense appearance of the haematoma on the mri t1-weighted sagittal and axial images . b , d hyperintense appearance of the haematoma on the mri t2-weighted sagittal and axial images seven days later , the patient was free of weakness and the mri showed a decrease in the haematoma size ( fig . 2 ) . successive mri results were obtained 1 month ( fig . 3 ) and 1 year later on ( fig . the haematoma is smaller nevertheless it is located at the same c4c5 level mri 1 month later : disappearance of the haematoma mri 1 year later : normal mri groen and van alphen reported that 4.5% of a series of 320 sseh cases treated surgically presented radiculopathy ; all cases affecting the lumbar spine . recently , groen reported that 9% of a series of 64 sseh cases treated conservatively presented an isolated radicular compromise ; only one of his cases was located on the cervico thoracic spine . our case adds little in terms of number but the fact that it may be the only report of its kind on purely cervical radiculopathy due to sseh , which may be considered anecdotic , as well as interesting . although the spreading of the haematoma throughout the epidural space is the most likely hypothesis proposed for spontaneous recovery in case of neurological impairment [ 7 , 14 ] , our case does not support that theory because the haematoma did not spread throughout the epidural space . conservative management is currently indicated in uncommon situations or when neurological symptoms improve before medical evaluation . geographic isolation , initial inaccurate diagnosis , neurological improvement pending an adequate coagulation level prior to surgery , high surgical risks and several other reasons had been referred to as the likely causes that led to the opportunity for spontaneous recovery [ 4 , 7 , 9 , 11 , 16 ] . our case can be categorised as wrong diagnosis initially , but we have to admit that the first non - specialised consultation spared our patient from the operation . if the patient had first consulted a more skilled specialist , following the state of the art , he would have probably been operated on , thus obtaining a good result and a proud surgeon . finally , the main interest of our case would be focused on the controlled maintenance of the anticoagulant therapy in patients with mild neurological compromise such as radiculopathy due to sseh in order to avoid thromboembolic risk .
introduction : spontaneous spinal epidural haematoma ( sseh ) is widely recognised throughout the literature as a cause of myelopathy , radicular compression being very rarely reported . surgical management is almost always recommended , especially in the cases of spinal cord compression . conservative treatment is reported as a curiosity and only in the case of spontaneous improvement . this report presents the particular case of a 64-year - old patient undergoing anticoagulant therapy that had a cervical radiculopathy due to a sseh confirmed by mri . the patient improved spontaneously and symptoms were solved with unconventional conservative treatment and without stopping the anticoagulant therapy . conclusions : spontaneous epidural haematoma must be kept in mind when patients undergoing anticoagulant therapy have a sudden onset of cervicobrachialgia . even though most spinal surgeons advocate surgical treatment , a conservative approach may lead to a complete recovery and may be considered as a good option in the case of radicular involvement . discontinuation of the anticoagulant therapy may not always be needed , especially when the clinical syndrome improves spontaneously .
we are currently modifying the database structure such that entries can be annotated by sequence and domain content . as the data grow , this step will be key to advancing refold as a research tool . although the advanced query tool allows searching across much of the database , we are developing a custom interface that will allow user - configurable queries against the whole dataset as well as user customization of how the results are displayed . we are developing functionality that will integrate data from other sources , such as the comprehensive annotation of proteins in uniprot ( 13 ) supplied in xml format . it is important to note that a typical user of refold is an experimentalist who most probably does not possess the skills ( or indeed time ) to navigate the vast array of protein sequence ( e.g. pfam and uniprot ) and structure ( e.g. scop and pdb ) information available . in order to facilitate this task , we will leverage data integration technologies set out in the efamily project ( ) . accordingly , we will also make refold data available in xml format for consumption by others . in the first instance , we are developing an xml schema that will provide a standard format for exchange . in particular , graphical methods allowing the visualization of relationships between parameters , such as ph , pi , etc . this means that the data becomes readily available to the community and amenable to analysis . some validation logic is already built into the deposition process , providing both a useful service to the depositor as well as an indication on data quality to users . the ongoing development of refold with the clear aim of providing fast and user - friendly access to refolding data , integrated with a wide array of protein resources , will serve a worldwide community of life scientists faced with the demanding challenges of producing pure , active protein . ( b ) graphical representation of data . portions of the chart can be selected to reveal the underlying data . ( ( b ) search results can also be shown as a tree - view , which structures the data hierarchically according to structural class / family / protein . data deposition form , split into logical sections of protein ( top ) , expression ( middle ) and refolding data ( bottom ) .
a large proportion of proteins expressed in escherichia coli form inclusion bodies and thus require renaturation to attain a functional conformation for analysis . in this process , identifying and optimizing the refolding conditions and methodology is often rate limiting . in order to address this problem , we have developed refold , a web - accessible relational database containing the published methods employed in the refolding of recombinant proteins . currently , refold contains > 300 entries , which are heavily annotated such that the database can be searched via multiple parameters . we anticipate that refold will continue to grow and eventually become a powerful tool for the optimization of protein renaturation . refold is freely available at .
most of the cases are asymptomatic with the exception of a few that may present with mild abdominal pain and nutritional deficiencies . intestinal obstruction is an acute presentation that has been observed in a number of cases where there is an increased worm burden that may partially or completely block the intestinal lumen.1,2 we present a case that proved difficult to diagnose as the child s symptoms of subacute intestinal obstruction did not pair up with the radiological results , forcing us to take unusual measures for diagnosis . a 3-year-4-month - old child presented to the emergency department with the complaints of greenish vomiting and abdominal pain for 3 days . his vomiting bouts were continuous with a frequency of five to ten episodes a day he had a history of mild constipation for 1 year , for which he was being managed with a high - fiber diet . on examination , though mildly tachycardic ( heart rate of 125 beats per minute ) , his respiratory rate , temperature , and blood pressure were within reference range . laboratory investigations showed an increased leukocyte count ( 14.510 cells / l ; neutrophils , 79.9% ; lymphocytes , 10.9% ; eosinophils , 2.2% ; basophils , 0.1% ) without any abnormalities in serum concentrations of electrolytes , amylase , and lipase . supine x - ray of the abdomen was performed suspecting intestinal obstruction , which showed no abnormalities ( figure 1 ) . computed tomography ( ct ) of the abdominal region was done to rule out any anatomical or positional abnormalities of the intestine , with suspicion of a partial obstruction in the small intestine . the child was withheld oral food and fluid ( nil per os ) , and nasogastric tube was kept on low volume suction . barium meal and follow through were hence scheduled , which revealed growth of intestinal worms in the upper intestine at various sections , which were found to be the cause of partial intestinal obstruction , as shown in figure 2 . the number of worms found was quite low , leading to the inability of reaching a definitive diagnosis earlier . upon resolution of the patients vomiting bouts and the development of healthy bowel sounds , he was initially started on a liquid diet and his diet was progressed further . follow - up after 6 days showed complete resolution of symptoms with confirmation that the worms had been passed during defecation . this study was conducted with the written informed consent of the patients guardian , and was approved by the ethical review board of the aga khan university hospital . intestinal nematode infection is one of the leading five causes of intestinal obstruction in a third of the world s population . ascaris lumbricoides ( al ) is the most common form of helminthic infection responsible for several medical problems , especially in the developing countries.2 though a vast majority of cases are asymptomatic , infected patients may present with a potentially severe variety of pulmonary and gastrointestinal disturbances.13 ascaris helminthic infection is predominantly found in areas of poor sanitation and is associated with malnutrition , anemia ( iron deficiency ) , and impairments of cognition and growth . al usually affects the pediatric age group patients residing in low socioeconomic areas with malnutrition and immune deficiencies . the worms reside near the ileocecal valve without causing serious symptoms , unless the environment becomes too hostile for their survival , which may result in their migration to a more tolerable region in the intestinal tract . al migration may cause serious complications such as pulmonary ascariasis , pancreatitis , liver abscesses , cholecystitis , volvuli , intussusception , and intestinal obstruction.14 diagnosis with clinical symptoms , hematological investigations , and biochemical profile is usually inconclusive . testing the stool for ova is one of the initial tests that are done which aid in establishing a diagnosis . abdominal x - rays , ultrasound , and ct scans may be used to rule out other differentials that might give a similar presentation and visualize the al itself.5 in the case of our patient , following an inconclusive abdominal x - ray and ct scan , barium studies were planned and a barium meal and follow through were scheduled . barium studies were performed due to the clinical presentation of the patient in which upper intestinal obstruction was more likely the cause of discomfort . an abdominal x - ray and whole abdomen ct were within reference range , indicating that the large intestine had no abnormalities that could lead to the green vomitus the patient had initially presented with . exactly 250 ml of contrast was given to the patient and a series of images were taken thereafter . the first picture taken was right after the patient had completely ingested the contrast , and the last image was taken 1 hour after its consumption . a low - count al infestation usually presents as a diagnostic dilemma , as reported by lakshmi et al6 as the small group of worms keep migrating to more favorable areas , or the worm burden is very low to begin with . low worm counts may mimic several diseases , confounding the original diagnosis , hence delaying the treatment to relieve acute intestinal obstruction . x - ray of the abdomen in intestinal obstruction may show several air fluid levels . mortality rate of intestinal obstruction of children under the age of 10 years is below 6% . partial obstruction of the intestines from al infestation usually resolves spontaneously with conservative treatment which includes bowel rest , intravenous fluids , and nasogastric decompression.7 if the mechanical obstruction persists , the worms act as a fixed point leading to intussusception or volvulus formation.1,8 al may excrete toxins , leading to small bowel spasticity and inflammation . volvulus , intussusception , or increasing pressure to the intestinal wall will inevitably cause intestinal necrosis . if intestinal necrosis occurs , resection and primary anastomosis are a mandatory step in the management of the disease . medical therapy consists of treatment with antihelminthic drugs , mainly albendazole and mebendazole.7 a single dosing is usually curative in 85% of the patients . multiple dosing can be administered taking into account the worm count , efficacy , and adverse effects of the drugs.9 al should be kept in mind in preschool children with symptoms of sudden acute intestinal obstruction . barium studies are effective , simple , and a cheap alternative to ct abdomen in the diagnosis of acute - onset intestinal obstruction secondary to al infestation , if other imaging studies turn out inconclusive . the patient should be managed medically if bowel necrosis has not yet occurred . moderate to severe al infestation causing partial to almost complete intestinal obstruction managed medically results in a lesser hospital stay and lower mortality rates , in comparison to surgical management . al infestation is easier to diagnose in cases of a heavy worm count causing intestinal obstruction . partial bowel obstruction may often present as a confounding mix of several diseases , none with a characteristic diagnostic marker to reach a definitive diagnosis .
ascariasis is a common infestation in developing countries where there is poor hygiene . a majority of the cases are asymptomatic , with a few cases presenting with mild abdominal pain and nutritional deficiencies in the long term . here we present a case of a young boy who presented as a diagnostic dilemma , with signs of acute intestinal obstruction without any supporting radiological evidence . a barium study revealed the presence of low - burden ascaris infestation that was managed medically .
long - term indwelling urinary catheterization is done in patients who have spinal cord injury , neurological or musculoskeletal disorder impairing walking movements or bladder control of person , and in elderly with urinary retention who are unfit for surgery . it is a widespread practice in medical field and its well - recognized complications are ( 1 ) urinary tract infection including urethritis , cystitis , pyelonephritis , epididymitis , periurethral abscess , and transient bacteremia ( 2 ) mechanical - bladder and peritoneal perforation , bladder spasms ( 3 ) catheter - associated penile fracture ( 4 ) urethral stricture ( 5 ) paraphimosis ( 6 ) cellular toxicity from catheter ( 7 ) urinary bladder malignancy associated with catheter ( 8) catheter blockage due to concretions deposition over catheter lumen . an 80-year - old male , bedridden , known case of bilateral club foot presented to us with the complaints of fever for 3 days . the patient had a high grade , intermittent fever , associated with chills and rigors . he was treated for urinary tract infection 2 months ago and that time had normal urogenital anatomy both clinically and radiologically except for prostatomegaly . he was discharged from the hospital with indwelling foley 's catheter as he complained of severe weakness on getting up from bed . on physical examination , he was febrile ( 101f ) and had tachycardia ( pulse - 110/min ) . urogenital examination showed in situ foley 's catheter , normal scrotum , eroded penis ( glans penis and penile shaft ) along with ventrally cleaved penile shaft by indwelling catheter , scanty nonfoul smelling pus discharge , and no lymphadenopathy [ figure 1 ] . investigations revealed hemoglobin - 11 g / dl , total leukocyte count - 16,800/cumm , differential leukocyte count showing neutrophilic predominance , platelet count - 1.39 lakh / cumm , and erythrocyte sedimentation rate - 46 mm / h . the uncommon complications of prolonged foley 's catheterization include aberrant foley 's catheter placement , urethral diverticula , ischemic necrosis of penis , pseudoaneurysm of bulbar artery , and iatrogenic hypospadias . prolonged indwelling urethral catheter produces downward pressure which may be due to larger size of foley 's catheter or improper technique of securing catheter , which interferes with blood supply of urethra causing ischemic effects . small caliber catheters are preferred as they do not put pressure on urethral mucosa or glands . in our case the time interval between urethral catheterization and detection of hypospadias in our patient was 2 months and reported literature showed that hypospadias can develop anytime between 1 month and 16 years after urethral catheterization . several different classifications have been described , but most surgeons use the classification proposed by barcat and modified by duckett . the described locations include anterior ( glandular and subcoronal ) , middle ( distal penile , mid shaft , and proximal penile ) , and posterior ( penoscrotal , scrotal , and perineal ) . the location is 50% in anterior , middle in 20% , and posterior in 30% cases ; the subcoronal position is most common among all . the surgical guiding principle is aiming at multiple goals including penile straightening ( arthroplasty ) , urethroplasty , glansplasty , meatoplasty , scrotoplasty , and preputial reconstruction . in order of frequency , urethrocutaneous fistula is a most common complication of surgical repair of hypospadias , followed by meatal stenosis , narrow neo urethra , and receding urethra . although not a life - endangering condition , hypospadias has significant mental and physical consequences , as it involves surgical correction , and even after correction , individuals may experience sexual dysfunction and psychosocial difficulties later in life . urinary catheterization is a simple technique that can be performed by the physician or nurse . because of a variety of possibly severe complications , it must be done only when unquestionably necessary . nevertheless , it has to be remembered that although inserting urinary catheter is a simple procedure , yet proper insertional technique has to be followed such as aseptic precautions , balloon inflation with proper amount of sterile fluid , choosing appropriate catheter size , and securing the catheter to abdomen or thigh without tension on tubing to prevent complications such as hypospadias .
long - time urethral catheterization may be responsible for various complications such as urethral stricture , urethral fracture , urinary tract infections , and hypospadias . hypospadias is the most common congenital anomaly of male external genitalia . however , urethral catheter - induced iatrogenic hypospadias is a rare entity . in this article , we describe a case of an elderly male who was found to have iatrogenic hypospadias 2 months after urinary catheterization .
a 75-year - old caucasian female , awaiting elective laparoscopic cholecystectomy , complained to her general practitioner of persistent cough , dyspnoea and low - grade fever that had lasted for more than one month . she presented to the emergency department , where a chest radiograph showed areas of consolidation in the lower lobes and in the right apex . another course of antibiotics was prescribed and her symptoms improved . to ensure resolution of the pneumonia , prior to laparoscopic cholecystectomy , twenty days later the patient had a high resolution computed tomography ( hrct ) scan in our institution . ct images showed the resolution of the pneumonia , but revealed an anomalous supernumerary bronchus directly arising from the right side of distal trachea corresponding to tracheal bronchus ( trb ) . tracheal bronchus ( trb ) is a congenital supernumerary bronchus to the right upper lobe that arise directly from the lateral wall of tracheal , generally located 3 cm proximal to the carina ( fig . 1 ) . trb is an incidental finding in man , with a low prevalence ( 15% ) , conversely it is a normal finding in pigs and for this reason it is also called it can be classified into two types : ( 1 ) supernumerary , as an accessory bronchus , and ( 2 ) displaced when the entire upper lobe ( usually right side ) is supplied by this bronchus . the congenital abnormality is usually asymptomatic : the accessory bronchus is discovered , as incidental finding , during radiological investigation or bronchoscopy . trb may also be related to inflammatory conditions due to retained secretions and may cause recurrent pneumonia , chronic bronchitis and atelectasis as in the case discussed . multidetector ct permits a direct diagnosis of trb , non - invasively , and is able to correctly identify the origin of the right upper lobe bronchus with 3d airway reconstructions ( fig . 2 ) . the use of volumetric hrct allows multiplanar reconstructions to accurately evaluate the bronchus path and calibre and to guide management ( video ) . supplementary video 1 related to this article can be found , in the online version , at doi:10.1016/j.ejro.2014.10.001 . video 1ct virtual bronchoscopy video shows an endoscopic reconstruction of the distal trachea , from which an anomalous ct virtual bronchoscopy video shows an endoscopic reconstruction of the distal trachea , from which an anomalous in fact , special care is required during intubation : if trb anomaly is not diagnosed , endotracheal tube could provoke the occlusion of the bronchus , which could lead to the collapse of the lobe , , .
a 75-year - old female underwent a high resolution computed tomography ( hrct ) scan for recurrent bronchitis and cough . hrct images showed an anomalous supernumerary bronchus to the right upper lobe directly arising from the right side of distal trachea , corresponding to tracheal bronchus ( trb ) . trb can cause recurrent right upper lobe pneumonia and special care is requiring during endotracheal intubation .
spontaneous sphenoidal encephaloceles are uncommon entities and the presence of lateral sphenoidal encephalocele is a rare congenital anomaly3 ) . sternberg 's canal is a lateral craniopharyngeal canal resulting from incomplete fusion of different sphenoid bone components5 ) . congenital skull base abnormalities are believed to be the result of incomplete or premature fusion of the normal bony elements involved in the complex process of central skull base ossification . due to their rarity , these lesions are seldom discussed in the medical literature and , to date , only isolated reports and small series are found4 ) . the persistence of these abnormalities is often associated with cerebrospinal fluid ( csf ) leak and encephaloceles8 ) . the efficacy and safety of the trans - sphenoidal approach for the resection of pituitary tumours and the treatment of associated csf rhinorrhea has encouraged surgeons to use the transnasal route for the repair of all csf leaks in the middle cranial fossa and sphenoidal sinus region1,7,10 ) . in the present case , we report the effectiveness of surgical treatment of a persistent sternberg 's canal by an endoscope - assisted trans - sphenoidal approach . a 45-year - old female presented with complaints of intermittent right - sided watery nasal discharge for one year , without headache , nausea or fever . the symptom was worsened by clinostatic position : during the night , the patient referred watering fluid in the pharynx . 2-transferrin - analysis and albumin / prealbumin tests of the nasal discharge indicated cerebrospinal fluid . the high resolution computed tomography of facial massix scans , including coronal reconstructions , revealed a particularly extended sphenoidal sinus on the right side ( fig . we decided to approach the lesion by an endoscope - assisted trans - sphenoidal approach , supported by neuronavigation , and the parasellar bony defect was confirmed ( fig . , the endoscope - assisted observation permitted to individuate cerebrospinal liquor outflow , under brain pulsation ( fig . the herniated brain material was removed under endoscopic control , until the individuation the bone defect on anterior wall of accessory cell . after abdominally fat and muscle graft by a right paraombelical incision , under intraoperative microscopic control , a sphenoidal accessory cell duraplasty was accomplished by inserting grafts of abdominal fat into the " bottleneck " of the meningocele . with the aid of neuronavigation , reduction of the right sphenoidal encephalocele was obtained and the persistent canal was sealed with the aid of fat , muscle graft and fibrin glue . the nasal discharge stopped immediately after surgery and postoperative computed tomography demonstrated the complete reconstruction by a duraplasty ( fig . spontaneous csf leaks have been estimated an infrequent entity , but recently this idea has changed9 ) ) . an overpneumatized sphenoid sinus , especially in its lateral wall , is often a site where a csf leak may emerge13 ) . in fact , pit holes in the floor of the middle cranial fossa have been indicated as causes of encephaloceles , especially when associated with large pneumatization . in the presence of sufficient pneumatization , sternberg 's canal may act as the site of origin of congenital meningoceles or csf fistulas12 ) . furthermore , normal spikes of csf pressure , and evidently hydrocephalus , may facilitate the development of small holes with herniation of the dura / arachnoid or brain tissue13 ) . the treatment of spontaneous sphenoidal csf fistulae has historically involved both extra- and intracranial approaches . the extracranial approaches are desirable from a noninvasive standpoint ; however , they fail to provide control of possible lateral extension of the sphenoidal sinus , where several of these fistulae arise8 ) . the transnasal approaches to the sphenoid sinus have been proven to be safe and effective in the treatment of csf rhinorrhea associated with post - trans - sphenoidal pituitary surgery , postoperative anterior cranial fossa cranial base surgery , and even medial intrasphenoidal and sellar encephalocele1,6 ) . the endoscope - assisted observation provided the treatment of sellar and parasellar lesions , although pure endoscopic trans - sphenoidal approach have been proposed for this purpose2,4,11,14 ) . surgeons performing endoscope - assisted microneurosurgical procedures have to see through an operative microscope during specific microsurgical approach . the visualization of the same structures as seen through a different tool , also considering that scope with a different viewing angle provides quite different vision . during microsurgical procedures , however it allows detailed view only of superficially located structures requires manipulation and retraction of intact superficial anatomical sectors , which unavoidably results in iatrogenic traumatism . this may be obviated combining microsurgical techniques to the use of the endoscope , which allows atraumatic vision of the structures located beyond and behind the superficial structures " around the corner " . endoscope - assisted management is technically challenging , nevertheless its advantages are a good view of the surgical field while being less traumatic than pure trans - sphenoidal or transcranial approaches . a case of middle cranial fossa spontaneous csf fistulae , involving a temporal encephalocele through lateral excision of this sinus , has been presented . our experience shows how the endoscope assisted trans - sphenoidal approach allows a wide , safe , and direct route to the lateral recess of the sphenoid sinus . compared with traditional transcranial routes , this approach provides a good exposure of the bony defect with minor morbidity and considerable advantages .
we report an uncommon case of a 45-year - old woman who presented with spontaneous rhinorrhea . a computed tomography ( ct ) scan of the head revealed an abnormally large sphenoid sinus associated with a parasellar bony defect ( sternberg 's canal ) through which magnetic resonance imaging could detect an encephalocele of the right temporal lobe . an endoscope - assisted trans - sphenoidal approach was performed and , with the aid of image guided surgery , reduction of the encephalocele was obtained and followed by surgical repair of the dural and bony defects . the postoperative course was uneventful and the cerebrospinal fluid fistula was closed as confirmed by the postoperative ct scan and by the absence of rhinorrhea . after three years of monitoring the patient remained asymptomatic .
a 56-year - old male with a history of deep vein thrombosis located in left common iliac vein and hypertension underwent thromboembolectomy in july 2015 . contrast - enhanced computed tomography ( ct ) scan revealed a renal cyst with internal enhancing structure in the upper pole of the right kidney , which measured 1.6 cm . on follow - up ct scan after 6 months , the largest diameter of the renal cyst increased to 5.6 cm . he had an uneventful peri- and postoperative course . on a thorough clinical workup , he had no history , signs and clinical findings suggestive of sarcoidosis or tuberculosis . gross examination revealed a 5.3 cm 4.0 cm 2.8 cm - sized well - encapsulated cortical cyst . the cystic space was mainly filled with old blood but also displayed a tan - colored solid mass measuring 2.2 cm 1.4 cm 1 cm ( fig . multiple non - necrotizing granulomas of various sizes with multinucleated giant cells were scattered within the tumor stroma ( fig . 2 ) . a few granulomas contained foci of coagulative necrosis , but they were devoid of caseation necrosis that is characteristic of tuberculosis ( fig . as the performed procedure was a partial nephrectomy , evaluation of uninvolved renal parenchyma was limited . on ziehl - neelsen and grocott s methenamine silver stains , no acid - fast bacteria or other microorganisms were identified . polymerase chain reaction for mycobacterium species yielded negative result . on a follow - up ct scan taken 5 months after the operation , any evidence of recurrence was not detected . tumor - related slr is postulated to occur due to t cell mediated reaction against various cancer - associated antigens . those antigenic factors are believed to be expressed directly by the cancer cells or shed as soluble antigen during tumor destruction . as those antigenic factors are conveyed by nearby lymphatic system , studied distribution of various kinds of inflammatory cells in lymph nodes with tumor - related slr . in this study , tumor - related slr mainly appeared in lymphoid sinuses and t - cell zones in a continuous growth pattern . this finding supports the hypothesis that tumor - related slr occurs because of tumor - shed antigens drained into lymph nodes . only fewer than 15 cases have been reported in the english literature since early 1990s [ 3,7,8,10 - 13 ] . , there was a male preponderance ( 9 males and 4 females ) . in reports which included the side of affected kidney , seven out of 10 cases occurred in the right kidney . most cases manifested as clear cell type , except for one case of sarcomatoid renal cell carcinoma . fuhrman nuclear grade ranged from 1 to 4 , but there was only one case with grade 4 histology which was the sarcomatoid renal cell carcinoma case . of 12 ccrccs with tumor - related slrs , three cases had documented the absence of sarcomatoid component . all of the cases exhibited epithelioid granulomatous reaction related to the tumor . in the majority including this study ( 8 out of 13 ) , granulomas existed only in tumor stroma . four cases had granulomas both in the tumor stroma and fibrous stroma immediately surrounding the tumor . reported a case of sarcomatoid renal cell carcinoma ( rcc ) containing granulomas only in the periphery of the neoplasm . the patient with sarcomatoid rcc in the report of piscioli et al . died from multiple disseminated metastatic disease 6 months after the diagnosis . rest of the cases maintained disease - free status until the last follow - up . tumor - related slr was reported to be associated with better prognosis or lower possibility of metastasis in hodgkin s lymphoma , gastric adenocarcinoma and breast cancer . in contrast , tomimaru et al . found that sarcoid reaction identified in regional lymph nodes did not lead to better prognosis in lung cancer . as granulomatous reaction is thought to give protective function against infectious agents , tumor - related slr might also aid in destruction of tumor cells . likewise , tumor - related slr may be a immunologic defense mechanism against tumor cells , decreasing the metastasis rate and expansion of the tumor . however , tumor - related slr may only be secondary phenomenon due to tumor - shed antigens . in table 1 , there is a case without recurrence or distant metastasis after a long follow - up period ( 4 years ) , but this does not support the hypothesis that tumor - related slr is associated with better prognosis . in the reviewed cases , only one patient died of distant metastases during the follow - up period ( 6 months ) . however , that patient was diagnosed with a sarcomatoid rcc , which is known to show aggressive clinical course . thus , the relationship between sarcoid reaction and tumor prognosis is yet to be defined . in summary , we describe a rare case of ccrcc with intratumoral granulomatous reaction in a male patient , without evidence of systemic sarcoidosis or tuberculosis . further research is required to clarify the mechanism of tumor - related slr and its impact on prognosis .
granulomatous reaction associated with clear cell renal cell carcinoma ( ccrcc ) is a rare finding , and only a few cases have been described in the literature . it is postulated to occur due to cancer - related antigenic factors such as cancer cells themselves or soluble tumor antigens shed into the blood . herein , we describe a case of a 56-year - old male patient diagnosed with ccrcc with intratumoral granulomatous inflammation .
he had underwent an operation for an enormous lipoma , approximately 20 cm in diameter , on his back in our clinic when he was 1.5 years old , and plastic surgery was performed following amputation for macrodactyly of his right first and second toes when he was 4 years old . during this time , he had no abdominal scanning . on physical examination , he had an abdominal mass almost 30 cm in diameter , a right lower extremity overgrowth , two amputated right foot fingers , and an incision scar on his back . abdominal magnetic resonance imaging revealed a giant hypodense lesion , stretching from the inferior of the liver to the inguinal region covering the right kidney and the right ureter . the density of the masses were approximately 100 hu , in accordance with lipid density , both lining intra- and extraperitoneally ( fig . a giant mass was found , surrounding all the abdominal organs , including the liver , kidney , ureter , mesocolon , colon , omentum , and intestines on the right side and extending to the inguinal canal . liposuction was planned to remove the remainder of the adipose tissue in the right lower extremity . abdominal magnetic resonance imaging revealed a giant hypodense lesion , stretching from the inferior of the liver to the inguinal region covering the right kidney and the right ureter . proteus syndrome ( ps ) is an extremely rare sporadic disorder that manifests as an asymmetric , disproportionate overgrowth of any connective tissues , such as bone , fat , or epidermal nevi , in a mosaic or patchy pattern . this hamartoneoplastic syndrome was first described by cohen and hayden.1 its prevalence is approximately 1 per 1,000,000 live births , and intra - abdominal expansion has been reported in no more than 20 cases in the literature.2 diagnosing ps is a challenge due to its different presentations and the overgrowth of different tissues and localizations . in 1999 , biesecker et al developed diagnostic criteria ( table 1 ) and evaluation guidelines to clarify the diagnostic confusion.3 the coexistence of the syndrome with other manifestations such as scoliosis , central nervous system anomalies , and thoracic deformity anomalies has also been described.4 connective tissue nevus disproportionate overgrowth ( one or more ) vertebrae megalospondylodysplasia specific tumors before the end of the second decade ( either one ) parotid monomorphic adenoma bilateral ovarian cystadenomas dysregulated adipose tissue ( either one ) regional absence of fat vascular malformations capillary / venous / lymphatic malformation minor downslanting of palpebral fissures and/or minor ptosis wide or anteverted nares source : adapted from biesecker et al.3 some overgrowth syndromes including ps , klippel trenaunay syndrome , and hemi - hyperplasia - multiple lipomatosis syndrome have been delineated . sapp et al defined a new overgrowth syndrome resembling ps named clove ( congenital lipomatous overgrowth , vascular malformation , epidermal nevus ) syndrome.5 our case differed from the clove syndrome because there was no vascular malformation , epidermal nevi growth , or the presence of skeletal overgrowth . biesecker et al have been working in the field of overgrowth and presented recent diagnostic criteria for ps.6 7 8 our case showed all the major diagnostic criteria of the syndrome , that is , mosaic distribution , a progressive course , and sporadic occurrence . as in our case , lipomas in ps are composed principally of mature adipocytes , and the adipose tissue shows dysregulation.3 our patient also had macrodystrophia lipomatosa , which is characterized by the enlargement of one or more fingers or toes with predominantly fibroadipose tissue . macrodystrophia lipomatosa can be part of an overgrowth syndrome such as ps,8 as in our patient , or cloves syndrome,5 or it can be found as an isolated abnormality in an otherwise normal patient.9 10 the syndrome is so rare that there are few case reports in the literature . in 2002 , lublin et al11 described three patients with ps and conducted a review of the literature regarding abdominal , otolaryngological , and thoracic manifestations and surgical complications . one of the patients , a male , required an emergent exploration for an acute abdomen secondary to a twisted necrotic portion of the right iliac . one of the other two patients with retroperitoneal ps was treated conservatively , and the other underwent an operation for extension to the inguinal canal , as in our case.11 the surgical approach to ps with intra - abdominal and retroperitoneal extension is primarily conservative , with surgery reserved for cases that are invasive in nature or where the pathology causes compressions or complications . any organ or tissue can be affected in ps ; therefore , the secondary complications are highly variable . it is associated with a range of tumors , pulmonary complications , progressive skeletal deformities , invasive recurrent lipomas , and a striking predisposition to deep vein thrombosis and pulmonary embolism.12 for the last 1 year , our patient is under careful follow - up to detect local relapse with abdominal ultrasongraphy in every 6 months . by presenting a case with ps , we aim to draw attention to the diagnosis , the treatment modalities , and the place of surgical intervention in this rare disorder . despite their histological appearance , intra - abdominal lipomas surgical intervention is indicated when the overgrowth causes physical compression of the surrounding organs , as in our case .
proteus syndrome ( ps ) is an extremely rare sporadic disorder that manifests as an asymmetric , disproportionate overgrowth of any connective tissues , such as bone , fat , or epidermal nevi , in a mosaic or patchy pattern . this hamartoneoplastic syndrome was first described by cohen and hayden . its prevalence is approximately 1 per 1,000,000 live births , and intra - abdominal expansion has been reported in no more than 20 cases in the literature . the phenotypes of the patients differ because of the variation in the pattern of the overgrowths , making diagnosis difficult . extremely large subcutaneous lipomas and internal lipomas , which occur rarely , are one of the presentation phenotypes . here , we present the second patient in the literature with ps involving the epiploon .
we present an unusual case of sbo secondary to the omental encasement without previous history of abdominal surgery or acute inflammatory process in the abdomen . a 42-year - old man was admitted to our hospital in emergency department with complain of abdominal distention and obstipation from last 10 days . a plain abdominal x - ray film showed several dilated small bowel loops with multiple air - fluid levels . patient had no sign of improvement after 24 h management than emergency laparotomy was performed through a midline incision , revealing small amount of ascites in the pelvic cavity . greater omentum forms an encircling band around the mid ileum that causes small bowel obstruction . distal small bowel loop shows sever inter - bowel adhesion that was difficult to separate . previous history of anti - tubercular treatment present so we prefer to consider that these adhesion may respond well to anti - tubercular treatment . the reported prevalence of these obstructions varies in different series depending on the type of practice and the population of patients . about 60% of intestinal obstructions are caused by adhesions , 25% by hernias , and 510% by neoplasm . opening the peritoneal cavity leads to the formation of potentially obstructive structures ( adhesions or bands ) in almost 95% of patients . with the increased incidence of abdominal surgery , congenital hernias occur most commonly in infancy and childhood but rarely can be seen in adults . the most common congenital hernias in adults include obturator ( little old ladies hernia ) , paraduodenal , transmesenteric , and transomental hernias . in advanced abdominal and pelvic malignancy , 551% of patients with ovarian malignancies and 1028% of patients with gastrointestinal cancer will suffer from intestinal obstruction . while , there are many possible causes of sbo , the clinical presentation rarely indicates the exact etiology . computed tomography ( ct ) has been shown to be useful in determining the site , level , and cause of obstructions . identification of adhesion as a cause of sbo remains a diagnosis of exclusion that must be based on the finding of an abrupt change in bowel caliber without evidence of another cause of obstruction . the characteristic ct features of internal hernia include abnormally located cluster of bowel loops and mesenteric vascular abnormalities such as whirling and twisting . preoperatively , it is often difficult to identify the cause of the ileus when laparotomy has not been carried out . even though , there is no history of previous laparotomy , intensive diagnostic investigations and early surgical intervention should be considered in any adult patients with signs and symptoms of acute sbo to avoid possible complications of bowel strangulation and gangrene .
highlightssmall bowel obstruction ( sbo ) is a common occurrence in adult surgical procedures.about 60% of sbo are currently accounted for adhesion mainly due to previous laparotomy.acute , non - postoperative sbo is less common.preoperatively , it is often difficult to identify the cause of the ileus .
in the present issue of critical care , pettila and colleagues report the first single - centred pilot randomized controlled trial of activated protein c ( apc ) in alcoholinduced acute pancreatitis of moderate severity but without infection . after screening 215 patients , 32 patients satisfied the trial inclusion criteria and were randomized to either placebo or apc at 24 g / kg / hour for 96 hours , in addition to standard therapy for acute pancreatitis . the study - powered to evaluate the effect of apc on the change in organ dysfunction , measured using the sequential organ failure assessment score as the primary outcome - failed to show any benefit . in acute pancreatitis , severity is defined by the occurrence of organ failure and/or peri - pancreatic complications . severe acute pancreatitis ( sap ) is characterized by the presence of an overwhelming inflammatory response , with unregulated activation of the coagulation system . evaluation of the coagulation and the endogenous protein c / antithrombin iii ( at iii ) system shows that nonsurvivors in sap have significantly lower levels of protein c and at iii activity , and higher levels of d - dimer and plasminogen activator inhibitor-1 , than survivors . these changes mirror the patterns seen in severe bacterial sepsis that suggest exhaustion of fibrinolysis and coagulation inhibitors , thereby identifying a possible role for apc in sap independent of the need to diagnose severe sepsis . prior to the study by pettila and colleagues , the literature was limited to animal studies and to subgroup data from the prowess trial of 62 patients with acute pancreatitis and severe sepsis , where there was a trend to reduced mortality in those treated with apc ( 24% vs. 15% ) . the consensus guidelines thus recom mended that careful consideration is given to apc therapy in those patients with sap and infection , given the theoretical but unproven concern of retroperitoneal haemorrhage . sap is a devastating disease with an attributable mortality of around 30% , and thus interventional trials are required to find a potential therapy to improve outcome . although commendable , this pilot trial of apc in pancreatitis must be interpreted with caution . as the authors point out , the study is underpowered to detect any meaningful difference in the primary outcome - change in the sequential organ failure assessment score - as a surrogate for apc effect . the authors also report no difference in bleeding complications , yet serious bleeding is actually a relatively infrequent event in patients treated with apc . in a meta - analysis of 10,679 apc - treated patients , this equates to approximately one serious bleeding event per 30 patients ; consequently , in a study involving only 16 apc - treated patients it is difficult to draw any clinically relevant conclusions ( good or bad ) with respect to bleeding . the mortality benefit with apc is best shown in patients with severe sepsis and high risk of death ; for example , patients with multiple organ dysfunction , patients with acute physiology and chronic health evaluation ( apache ) ii score 25 , or those in shock . there has always been debate regarding benefit in the patients at low risk of death , such as those in the lowest quartile of the prowess trial ( that is , apache ii score < 17 ) . the severity of illness and risk of death due to acute pancreatitis in this pilot trial was low ( mean age of 47 , mean apache ii score of 14 and zero mortality in the control arm ) . moreover , 34% of the patients never required invasive ventilation and 37% never developed shock requiring a vasopressor . indeed , for subsequent trials with apc the inclusion criteria have all focused on ensuring high severity of illness , including the important ongoing prowess - shock trial . identifying individual patients who are likely to benefit the most and to suffer the least morbidity from an intervention is a challenge , and there is an increasing role for biomarkers to assist in this endeavour . in the context of apc , serial measurements of protein c may be the best biomarker in establishing those most at risk of poor outcome in severe sepsis , may highlight those patients most likely to benefit from apc , and also may prove useful in monitoring therapy . as protein c deficiency has been demonstrated in experimental early sap , this trial might have been more relevant had it measured protein c as a biomarker . in addition , the observed low mortality in the study highlights the importance in study design of selecting a population who are genuinely at high risk of death and are potential ideal candidates for what is an expensive and debated therapy . this pilot study is underpowered to contribute significantly to the baseline data necessary to inform the design of a future interventional trial assessing apc as a potential therapy in sap . if further trials with apc are undertaken in this disease , it is essential they have the scope and ability to capture a genuinely high - risk population and provide the intervention in a timely manner . consideration should be given to selecting patients at highest risk of progressive organ dysfunction defined by at least moderate protein c deficiency apache : acute physiology and chronic health evaluation ; apc : activated protein c ; sap : severe acute pancreatitis . in the last 5 years dw has contributed to a number of trials of activated protein c in severe sepsis which have been sponsored by eli lilly . he has also received honoraria from eli lilly ( the manufacturers of activated protein c ) for speaking at educational meetings .
severe acute pancreatitis ( sap ) is characterized by an unregulated systemic proinflammatory response secondary to activation of trypsin within the pancreatic tissue , resulting in multiple organ failure . this dysregulated inflammation leading to organ dysfunction also characterizes severe sepsis . activated protein c ( apc ) has pleotropic effects on the immune , coagulation , inflammatory and apoptotic pathways , and has been postulated to benefit acute pancreatitis - although concerns of possible retroperitoneal bleeding remain . currently , experimental studies and subgroup data on patients with pancreatitis from a randomized controlled trial of apc in severe sepsis form the literature on the possible role of apc in sap . we review the first randomized controlled trial of apc in acute pancreatitis published in the present issue of critical care .
as part of a broad effort to define the human virome , we performed high - throughput sequencing ( genome analyzer iix ; illumina inc . , san diego , ca , usa ) on several plasma samples from children with febrile illness ( k.m . the human research protection office , washington university ( st . louis , mo , usa ) approved this study . the case report in this article describes results generated from study of a 20-month - old boy with a history of transient and resolved neutropenia . louis children s hospital for petechial rash ( 3-day history ) , fever < 40c ( 1-day history ) , cough , and nasal congestion . the evaluation included a leukocyte count , with results ( 7.8 10 cells / mm ) within the reference values and with a differential count of 26% bands , 59% neutrophils , 8% lymphocytes , 6% monocytes , and 1% atypical lymphocyte ; blood culture results were negative . nasopharyngeal swab specimen was negative for respiratory syncytial virus , influenza types a and b , parainfluenza , and adenovirus by fluorescent antibody testing , and culture results were negative for respiratory viruses . chest radiograph was interpreted as showing mild peribronchial thickening , which may represent a viral process . in addition , plasma or blood samples from the patient were subjected to a battery of pcr screenings for the following viruses , the results of which were all negative : adenovirus ; enteroviruses ( enterovirus asr ; cepheid inc . , sunnyvale , ca , usa ) ; human herpesvirus 6 and 7 ; parvovirus b19 ( realstar parvovirus b19 pcr kit 1.0 ; altona diagnostics , hamburg , germany ) ; human bocavirus ; cytomegalovirus ( whole blood ) ; epstein - barr virus ( whole blood ) ; and jc , bk , wu , and ki polyomaviruses . total nucleic acid was extracted from 100 l of the patient s plasma by using the roche ( indianapolis , in , usa ) magna pure system and randomly amplified by using a sequence - independent pcr strategy as described ( 12 ) . amplicons were sheared and , following standard library construction , were sequenced by using the genome analyzer iix ( illumina inc . ) according to the manufacturer s protocol . when present in the sheared amplicons , the primer used for random amplification was removed , resulting in 83-nt sequences . from all reads , 238 had > 80% nt identity to the partial mlb2 sequence in genbank ( gq502192.1 ) when aligned by using cross_match software ( www.phrap.org/phredphrapconsed.html#block_phrap ) . in addition , 374 sequence reads with similarity to anelloviruses were detected in this sample by alignment of the reads to the genbank nt and nr databases , using cross_match and blastx ( http://blast.ncbi.nlm.nih.gov/blast.cgi ) , respectively . anelloviruses are commonly detected in human blood ( 13 ) and have no known disease association . no reads aligned with any other viruses , except endogenous human retrovirus sequences . given the number of sequence reads from the plasma sample that could be aligned with the 3,280-nt sequence of mlb2 ( accession no . gq502192.1 ) in genbank , we reasoned that additional reads were likely to be present from parts of the mlb2 genome that had not yet been sequenced . to provide a complete reference genome for such an analysis , we sequenced the complete mlb2 genome from a previously described isolate ( genbank accession no . gq502192.1 ) ( 3 ) from a stool sample by using a combination of reverse transcription pcr ( rt - pcr ) , 3 and 5 rapid amplification of cdna ends , and pyrosequencing on a genome sequencer ( roche ) as described ( 4 ) . the complete mlb2 genome of 6,119 nt , excluding the polya tail , was confirmed by sanger sequencing of overlapping rt - pcr amplicons and has been deposited in genbank ( accession no . comparison of the high - throughput sequencing reads from the plasma to the complete genome yielded an additional 199 reads with > 80% nt identity . assembly of all reads yielded 10 contigs , with an average length of 305 bp , which aligned throughout the mlb2 genome ( figure ) . conventional rt - pcr and quantitative taqman rt - pcr independently confirmed the presence of mlb2 in the plasma sample . the complete sequence of the capsid ( open reading frame 2 ) of this plasma - derived mlb2 strain was obtained by rt - pcr ( genbank jf742760 ) by using primers designed from the stool - derived mlb2 strain . the capsid of the plasma - derived mlb2 strain has 99% nt identity with the stool - derived mlb2 strain . because of limited quantities of the plasma sample , we were unable to sequence the complete genome of the plasma - derived mlb2 . map of 10 plasma - derived astrovirus mlb2 strain contigs generated by high - throughput sequencing ( genome analyzer iix ; illumina inc . orf , open reading frame . to quantify the mlb2 virus load in the plasma specimen , we developed a quantitative rt - pcr taqman assay targeting the capsid ( forward primer lg0169 5-acaactggccctacattgaattc-3 , reverse primer lg0170 5-ccgacacgcacatctcgat-3 , and probe fam - tcgggtcttggcgcgcgat - tam ) . we used the maxiscipt kit ( ambion , austin , tx , usa ) to generate in vitro transcribed rna from a plasmid containing the region of interest to establish a standard curve for the assay . on the basis of the results of this assay , this sample has 4.5 10 copies of mlb2 per ml of plasma . to evaluate how frequently astroviruses may be present in human plasma , we screened archived plasma samples from 90 children with fever and 98 afebrile controls by using an astrovirus consensus rt - pcr ( 3 ) . all 188 plasma samples were negative , which suggests that astrovirus mlb2 viremia is relatively rare , at least in the cohort analyzed . the role of novel astrovirus mlb2 in human health and disease and the clinical consequence of mlb2 viremia are not yet known . this case report raises the possibility that astrovirus mlb2 may be a cause of febrile illness . in addition , the finding of mlb2 viremia suggests that astrovirus mlb2 may have effects outside the enteric system . these data , combined with the recent detection of an astrovirus in brain tissue of an immunocompromised patient ( 14 ) and the brain tissue of mink with shaking mink syndrome ( 15 ) , demonstrate a broader distribution of astroviruses in the body than previously recognized . for example , no other known pathogen was detected in the patient in this case report , and he had mostly upper respiratory signs , raising the possibility that mlb2 may play a role in respiratory illness .
astroviruses cause diarrhea , but it is not known whether they circulate in human plasma . astrovirus mlb2 was recently discovered in diarrhea samples from children . we detected mlb2 in the plasma of a febrile child , which suggests that mlb2 has broader tropism than expected and disease potential beyond the gastrointestinal tract .
acute kidney injury ( aki ) occurs in one third to two thirds of patients in the intensive care unit ( icu ) [ 1 - 7 ] . the majority of clinical studies in icu patients have found that aki was associated with increased in - hospital mortality . we can only speculate why even small increases of serum creatinine lead to worse outcomes . plausible causes for this are volume overload , inflammation of and adverse effects on other organs ( so - called ' organ cross - talk ' ) , and inadequate drug dosing . the adverse effects of an episode of aki may also persist for longer follow - up times . in their center in florida , hobson and bihorac and their colleagues found that , in specific icu cohorts such as those who are recovering from major surgery or cardiac surgery , aki leads to worse outcomes over a period of years . in a large cohort of hospitalized veterans , mortality in patients who had 90-day survival was higher in patients with aki , and there was a stepwise increase for increasing aki severity class . in this issue of critical care , gammelager and colleagues nicely demonstrated similar findings in a large cohort of general icu patients recruited in a large region in denmark . among patients surviving for 30 days , 1-year mortality rates were 20.5% for the aki - risk group , 23.8% for the aki - injury group , and 23.2% for the aki - failure group in comparison with 10.7% for the patients without aki . the strengths of the study are the relatively large sample size , the multicenter setting , and the complete follow - up data . the increased risk for long - term worse outcome persisted in different subgroups , making it even more plausible that the increased mortality observed is not caused by a confounder but indeed is associated with the study variable aki itself . how can we explain this increased long - term mortality after an episode of aki ? the data presented in the article by gammelager and colleagues do not provide an answer for this . we can only speculate on the possible link between an episode of aki and long - term mortality . one of the most claimed reasons for long - term worse outcomes after aki is incomplete renal recovery , which covers a spectrum from patients whose glomerular filtration rate is discrete lower than their kidney function was beforehand to patients who remain dependent on dialysis . incomplete renal recovery is most likely in patients who had the most severe stage of aki ( that is , aki treated by renal replacement therapy ) , and the effects on mortality will be most pronounced in patients who remain dependent on dialysis . unfortunately , the authors could not provide data on renal recovery , such as a serum creatinine or estimated glomerular filtration rate at the time of hospital discharge or , for example , 3- or 6-month follow - up time . however , we question whether incomplete renal recovery did play an important role in their findings . in the study , a total of 987 patients with aki were treated with renal replacement therapy . when icu mortality of these patients is comparable to that of patients in other western countries , approximately 50% , or 493 , of the former will have died . of the survivors , 10% to 25% ( or 50 to 123 ) so non - dialysis - dependent incomplete renal recovery would account for 80% of the additional deaths . although every nephrologist will confirm that decreased kidney function has an impact on long - term outcomes , the implications are measurable only when observed over a period of several years . in other words , incomplete renal recovery may explain only in part the impressive mortality between day 30 and 1 year as observed in this study . careful analysis of the cumulative mortality curves reveals that there was a biphasic pattern : a steep increase in mortality from icu admission until day 50 and a relatively flat curve afterwards . importantly , the second half of the curve for patients with aki is very similar to that for patients without aki . as 25% of patients with aki were still hospitalized after day 30 , it is very likely that the steep curve still represents in - hospital mortality . in other words , patients who survived the immediate 1-month period after aki died in the second month after aki diagnosis - a period still linked to the original disease that lay at the origin of aki . modern - day intensivists are challenged by the care for patients who survived a first episode of critical illness and stay in the unit and hospital for several weeks . why patients with aki do still worse in this period is not clear and should be evaluated in future studies . apart from this , several lessons can be learned from these observations . first , in modern - day icu care , we should aim for endpoints that are more relevant , such as 60- or 90-day survival instead of the classic 30-day survival . second , we are challenged by care for initial icu survivors , who remain fragile and in moderate organ dysfunction for longer periods of time . the challenges that we face for these patients are not fully understood . in the icu , we probably need to develop new therapies that are in contrast to the immediate ' point and shoot ' approach of the old - style intensivists . when these patients are discharged to a step - down ward , they should be cared for by a team that is specialized in their care . as a consequence , there is a growing consensus that other issues of long - term outcome , such as quality of life , are worth studying . in conclusion , we should increase our knowledge of these initial icu survivors , try to elucidate why patients who had an episode of aki do particularly worse , and develop integrated care with a focus on immediate , but also long - term , outcomes .
acute kidney injury ( aki ) is associated with worse outcome in the acute phase of acute illness but also in the chronic phase . in a large danish study in this issue of critical care , 1-year mortality was higher in patients with aki than in patients without aki . mortality was most important during the first 50 days after admission to the intensive care unit ( icu ) , whereas after 2 months the survival curves of patients with aki and those of patients without aki were similar . the reasons for this observation are not clear , but protracted critical illness and fragility after acute critical illness probably play important roles . because we see more and more of these patients , they should be the focus of icu research . consequently , icu and post - icu care for these patients requires focus and a more integrated approach to the specific problems of these survivors of acute critical illness .
partial anomalous pulmonary venous connection ( papvc ) is relatively uncommon congenital anomaly and covers 0.51% of congenital heart diseases . the quite rare combination ( 35% ) of papvc with hypoplasia of the right lung and dextroposition of the heart is designated as scimitar syndrome . the term scimitar , referring to a turkish sword , was first used to describe the shape of the vein by nicholas halasz . in two - third of cases , the scimitar vein ( sv ) provides drainage for the entire right lung , but in others for only the lower portion . via sv pulmonary veins are discharged into the inferior or superior vena cava , or directly to right atrium . so a left - to - right shunt is established and this shunt result in long - standing right ventricular volume overload and induce increased risk of right ventricular failure . the diagnosis frequently can be established with a chest radiograph and heart doppler ultrasound . recognizing the clinical and radiographical features of this rare disease is important when dealing with patients presented with cardiac and pulmonary complaints . a 38-year - old man was referred to our hospital with abnormal findings on plain chest roentgenogram and he presented with complain of cough for 10 days . the patient appeared well on chest examination respiratory rate was 18/min and breath sound was normal . chest x - ray showed a sloping opacity at the right heart border [ figure 1a ] . the contrast - enhanced computed tomography ( ct ) demonstrated that the left pulmonary venous drainage was normal , but mild hypoplasia of right lung , mild dextroposition of heart , and left renal agenesis were detected [ figure 1b and c ] . the abnormal right pulmonary venous drainage from the right lung via a large characteristically curved anomalous vein draining into inferior vena cava at just superior to diaphragm [ figure 2a ] and resembling to the curved turkish sword scimitar [ figure 2b ] . artery blood gas analysis revealed ph of 7.44 , po2 of 72 , pco2 of 37 , and o2 saturation of 95% . while expected alveolar - arterial po2 gradient of the patient was 10.5 mmhg , the measured a - a gradient was 22.4 mmhg . transthoracic and transesophageal echocardiogram revealed an ejection fraction of 65% , systolic pulmonary artery pressure of 30 - 35 mmhg , slight ra and rv dilatation , and a one - fourth tricuspid regurgitation were also detected . ( a ) chest x - ray demonstrates a sloping opacity at the right heart border . ( b ) axial ct image shows mild hypoplastic right lung and mild dextroposition of heart . the arrow shows that hypoplastic right pulmonary veins do not flow to the left atrium ( c ) axial ct image shows the absence of left kidney ( a ) reconstructed coronal oblique image of contrast - enhanced computer tomography demonstrates that the scimitar vein was connected to the inferior vena cava . ( b ) the scimitar , which is a short , curved turkish sword based on chest roentgenogram and contrast - enhanced ct findings , scimitar syndrome was diagnosed and renal agenesis was detected . we investigated in literature but did not detect any case both with scimitar syndrome and renal agenesis . the sv must be present in scimitar syndrome , but rarely the only abnormal findings . other anomalies together with the sv are : abnormal right lung lobation and hypoplasia ( almost 100% , with widely varying degrees of hypoplasia ) ; dextroposition of the heart ; hypoplasia of the right pulmonary artery ( 60% ) ; systemic arterial blood supply to the right lower lung from the infradiaphragmatic aorta ( 60% ) ; asd of secundum type . in our case ; sv , mild hypoplasia of right lung , mild dextroposition of heart , and left renal agenesis were determined . most patients are symptomatic infants , but there are sparse reports of adults with scimitar syndrome . in 82% of adult patients , left to right shunt is less than 50% and in 77% of these patients pulmonary artery pressures are normal and in 23% are slightly elevated , and these patients lead a normal life span without surgical correction . however , in cases with shunt more than 50% , dyspnea and pulmonary artery hypertension ( pah ) develop . but his artery blood gas analysis showed some hypoxemia ( po2 of 72 ) and spirometric investigation showed mild restrictive ventilatory defect . the corrective surgery was not performed because of his refusal and the absence of both pah and symptoms . patients with scimitar syndrome generally undergo comprehensive evaluation with ct scans and cardiac angiography to characterize the anomalous vessels . recently cardiac magnetic resonance imaging has been proposed to assess the congenital abnormalities . in our case ; chest radiograph , contrast - enhanced ct , transthoracic and transesophageal echocardiogram were sufficient to diagnose and eliminate the other anomalies . the incidence of unilateral renal agenesis is about 1 in 1300 births and the prognosis is excellent . in renal agenesis this process starts at approximately 5 week and ceases at approximately 36 week of gestation . in normal lung development , primary blood supply is formed after the 7 week and pulmonary venous drainage to the left atrium is settled by 11 week . since both organogenesis take place around the same period , in our case , we thought that during fetal period there might be some factor effecting both cardiovascular and urinary system . we submitted the case because of being asymptomatic male adult patient which is seen quite rarely and for the first time to be determined scimitar syndrome and renal agenesis together .
partial pulmonary venous connection anomaly is relatively uncommon form of congenital heart diseases . the quite rare combination of this anomaly with hypoplasia of the right lung and dextroposition of the heart is designated as scimitar syndrome . most cases are presented in infantile period and adult presentation is exceedingly rare . our patient , a 38-year - old man , was admitted to a doctor with flu - like complaint and because of abnormalities on chest x - ray he was sent to our clinic . he did not have any chronic complaints such as shortness of breath and fatigue . after investigation , scimitar syndrome was diagnosed . left renal agenesis was determined with abdominal examination . best of our knowledge in literature we did not detect any case both with scimitar syndrome and renal agenesis , and we wanted to report the asymptomatic adult scimitar syndrome case with left renal agenesis .
kawasaki disease ( kd ) is an acute febrile illness affecting infants and young children , causing vasculitis of the small and medium size arteries , and its etiology is still unknown . most concerned are the coronary arteries showing aneurysm formation , thrombosis with occlusion , rupture and myocardial infarction , which often cause death . we report a six - month - old infant presented with croup from enlarged retropharyngeal lymphnode , which is an unusual presentation . later on , he developed symptoms of typical kawasaki disease and formed a giant aneurysm and thrombosis of the left anterior descending and right coronary artery . a six - month - old male presented at the comer children s hospital ( chigaco , il , usa ) with history of fever , cough and stridor . he was treated in an outside hospital with antibiotics without any response . on transfer to our hospital he developed maculopapular rashes over the trunk , dry red swollen lips and bilateral non - purulent bulbar conjunctivitis . the x - ray findings and the persistent fever required a computed tomography ( ct ) scan of the neck , which showed several enlarged retropharyngeal lymphnodes . he was treated with dexamethasone and 3 doses of racemic epinephrine , then the fever defervesced within 12 h. he continued to have stridor at rest with sub costal retractions , nasal flaring and remained febrile . pertinent laboratory findings are : erythrocyte sedimentation rate ( westerngreen ) , 145 mm / h ; c - reactive protein , 84 ( nl<5 ) ; platelets , 1.244 million ; serum albumin , 3.0 gm / dl ( nl 3.55.0 ) . an echocardiogram demonstrated dilated coronary arteries with thrombus ( figures 1 , 2 and 3 ) ; left anterior descending ( lad ) artery measured 119.4 mm ; right coronary artery ( rca ) measured 4.14.8 mm . figure 1giant aneurysm of the left anterior descending coronary artery measuring 1411 mm with a thrombus within the lumen measuring 3.96.0 mm . giant aneurysm of the left anterior descending coronary artery measuring 1411 mm with a thrombus within the lumen measuring 3.96.0 mm . figure 2small aneurysm of the right coronary artery with a small thrombus measuring 2.72.4 mm . small aneurysm of the right coronary artery with a small thrombus measuring 2.72.4 mm . figure 3computed tomography scan showing color flow to the left main coronary artery with aneurysm of the left anterior descending coronary artery . computed tomography scan showing color flow to the left main coronary artery with aneurysm of the left anterior descending coronary artery . initially , he was treated with intravenous immunoglobulin ( ivig ) 2 gm / kg and a high dose of aspirin . because of recurrence of fever and giant aneurysm of the lad with thrombus , he was treated with a 2nd course of ivig . he still remained febrile after receiving a course of remicade ( infliximab ) , therefore heparin infusion was started . later on , he was started on plavix ( clopidogrel ) , warfarin ( coumadin ) and lovenox . during his hospitalization the thrombus within the lad and rca dissolved , but the lad giant aneurysm still remained and the ct scan of the coronary arteries ( figure 4 ) confirmed the echo finding of giant aneurysm . figure 4computed tomography scan of the left anterior descending coronary artery showing a giant aneurysm . the follow - up echocardiogram at the age of one year , showed a giant aneurysm of the lad measuring 98.1 mm and a cardiac catheterization revealed giant aneurysm of the lad ( figures 5 and 6 ) , and no evidence of stenosis . figure 5frontal view coronary artery angiogram showing the giant aneurysm of the left anterior descending coronary artery . frontal view coronary artery angiogram showing the giant aneurysm of the left anterior descending coronary artery . figure 6lateral view of the coronary artery angiogram . lateral view of the coronary artery angiogram . in patients with kd , aneurysm of the coronary artery develops in 20% of the untreated patients and in 48% of children treated with gamma immunoglobulin . giant aneurysm ( > 8 mm ) of internal diameter of the coronary arteries is rare and has greatest risk of thrombosis and myocardial infarction , causing sudden death . therefore , it is recommended a close follow - up of these patients and a very aggressive anticoagulation therapy with multiple agents . surgical intervention ( e.g. coronary artery by - pass ) should be considered when the aneurysm size increases significantly in spite of maximal medical therapy .
we report a six - month - old febrile infant presenting with stridor . later on , he developed typical kawasaki disease with giant aneurysm of the coronary artery with thrombosis that resolved with an aggressive anticoagulation therapy . the giant aneurysm still persisted a year later . respiratory illness with stridor is an unusual presentation of kawasaki disease .
on may 8 , 2012 , acute symptomatic tbev infection was diagnosed in a kidney transplant patient in slovenia ( table , patient 1 ) . a possible link between the infection and consumption of raw goat milk was revealed , triggering a detailed investigation of possible sources of infection and of 3 other persons who , together with patient 1 , had consumed 2 l of raw milk ( colostrum ) from the same goat on april 18 ( table ) . two days after the milk was consumed , fever , fatigue , and malaise developed in 3 of the 4 person , including patient 1 , who also had headache and myalgia . * except for patient 4 , no patients were vaccinated against tick - borne encephalitis . for patients 1 and 2 , the incubation period was 2 d ; for patient 3 , the incubation period was 23 d. patient 1 refused hospitalization and was treated as an outpatient ; patient 2 was hospitalized for 8 d ; patient 3 did not seek medical care . tbev , tick - borne encephalitis virus ; nt , neutralization test ; rrt - pcr , real - time reverse transcription pcr ; pos , positive ; neg , negative ; nd , not done . patients 1 and 2 were examined in the emergency department of the local general hospital on april 20 . laboratory test results were in the reference range , with the exception of mild leukopenia in both patients and mildly elevated liver enzyme levels for patient 1 . patient 3 remained well , but a second phase of disease developed in patients 1 and 2 approximately 14 days after the milk was consumed . the second phase was characterized by high fever , headache , nausea ( and vomiting in patient 1 ) , tremor , and mild disturbances of concentration and consciousness . results of cerebrospinal fluid laboratory tests for patient 2 revealed abnormalities consistent with aseptic meningitis ( reference values are in parentheses ) : leukocytes 29 10/l ( < 5 10/l ) , neutrophilic granulocytes 9 10/l ( < 5 10/l ) , lymphocytes 20 10/l ( < 5 10/l ) , protein concentration 0.39 g / l ) , glucose concentration 3.27 mmol / l ( 2.53.9 mmol / l ) . patient 1 , who refused lumbar puncture diagnostic testing and hospitalization , was treated as an outpatient . the course of disease in patients 1 and 2 was moderately severe , and the outcome was favorable . a detailed epidemiologic history revealed that none of the 3 patients recalled a recent tick bite and that patients 2 and 3 consumed raw goat milk rather often , believing it was healthful . for all 3 patients , tbev infection was confirmed by 1 ) elisa ( enzygnost anti - tbe / fsme virus [ igg , igm ] ; siemens , marburg , germany ) demonstrating specific igm and igg against tbev in serum and by 2 ) the presence of neutralizing antibodies against tbev . real - time reverse transcription pcr of serum samples did not detect tbev rna ( table ) ( 8) . he received his basic vaccination ( 3 doses ) during 19951996 , the first booster dose in 2000 , the second in 2005 , and the third in 2010 . serologic test results showed the absence of specific igm and high levels of specific igg . an antibody concentration of 912 u / ml in the first serum sample , obtained 27 days after he consumed raw goat milk , and of 672 u / ml in the second serum sample , obtained 3 weeks later , together with a high relative avidity index ( 85% ) , suggested a recent booster response . patient 3 , the owner of a small farm with 9 sheep and 9 goats , including the goat whose milk was consumed , consented to a virologic investigation of serum , blood , and milk samples from his farm animals . by using an indirect immunofluorescent assay , we detected tbev - specific antibodies in 5 of 9 goat serum samples ( titer range 201,280 ) and in 1 of 4 goat milk samples . quantitative real - time reverse transcription pcr for tbev was performed on all serum and blood samples and on 4 goat milk samples ( 9 ) . tbev rna was detected in serum ( 1.50 10 rna copies / ml ) and milk ( 1.88 10 rna copies / ml ) of the goat whose milk was consumed , confirming the source of infection . our investigation of illness among 3 of 4 persons who consumed tbev - infected raw goat milk revealed that all 4 persons were infected with the virus . febrile illness developed in 3 of the 4 persons 23 days after the milk was consumed ; the fourth person , who had been vaccinated against tbe , remained healthy . the course of the illness was biphasic in 2 of the 3 symptomatic persons : leukopenia ( a characteristic finding for the initial phase of tbe ) was present during the initial phase , and the second phase was clinically indicative of meningoencephalitis . even though 1 of these 2 patients received immunosuppressive therapy because of renal transplantation , the course of the disease was only moderately severe , and the outcome was favorable . a prospective clinical study of patients in the initial phase of tbe who were monitored for the appearance of the second , encephalitic phase of the disease , showed that an abortive form of tbe ( i.e. , an isolated initial phase not followed by the meningoencephalitic phase ) , as seen in the third patient in our study , is a rare event ( 10 ) . after a tick bite , the incubation period for tbe is a median of 8 days ( range 428 days ) ( 2 ) . the incubation period can be shorter for exposure by the alimentary route ( 5 ) and was found to be only 2 days in the patients in our study . these findings might suggest that drinking tbev - infected raw milk ( colostrum ) may result in tbe within a shorter incubation period than when tbe is associated with consumption of infected milk products ( e.g. , cheese ) . therefore , short incubation should not be an exclusion criterion for the diagnosis of tbe , but in proven cases of tbev infection , a short incubation period is likely a clue for alimentary transmission of tbev . previously reported tbe outbreaks caused by alimentary transmission of tbev lack definitive evidence of the virus having been present in milk or dairy products . however , in our study of 4 tbev - infected persons , the source of infection was proven by direct demonstration of tbev rna and a corresponding virus load ( concentration ) in serum and milk samples from the goat whose milk was consumed . the outbreak described herein could have been avoided if the milk had been pasteurized or boiled before consumption or if the persons who became ill had been protected by vaccination , as was the fourth person who drank the tbev - infected raw milk but did not become ill . the increasingly fashionable natural lifestyle encourages the consumption of raw milk and products made of unpasteurized milk . even though alimentary transmission of tbev is rare , the risk of such exposures could be reduced through education campaigns that encourage persons to consume only milk that has been boiled or pasteurized and only dairy products made from pasteurized milk . in addition , tbe vaccination , which effectively protects against tick bite associated and dairy product associated tbev transmission , should be encouraged in areas where tbev is highly endemic .
tick - borne encephalitis ( tbe ) developed in 3 persons in slovenia who drank raw milk ; a fourth person , who had been vaccinated against tbe , remained healthy . tbe virus rna was detected in serum and milk of the source goat . persons in tbe - endemic areas should be encouraged to drink only boiled / pasteurized milk and to be vaccinated .
colonies grew rapidly at 30 on mea , attained a diameter of 65~67 mm after 2 days . sporangiophores mostly sympodially branched , grew to width of 6~12 m and a variable length . sporangia measured 24.5~49.5 22.5~48 m , were globose to subglobose , light yellow , and were multispored . the columellae measured 17.5~30 16~29.5 m , and globose to ellipsoidal , with a collarette . the sporangiospores were variable , mostly ellipsoidal and measured 3.0~8.5 2.5~7.0 m in diameter . the colonies grew slowly than on mea , attained a diameter of 62~64 mm and 57~59 mm after 2 days at 30oc , respectively . the colony morphology and culture characteristics of the eml - puki12 - 1 isolate on mea was compared with the previous description . morphology of the present isolate was generally similar to the previous description of m. irregularis . colonies grew rapidly at 25 on mea , filling the petri dish after 4 days of incubation . sporangia measured 25~65 23.5~60 m , were globose to subglobose , light yellow , and multispored . the columellae measured 17~29 15.5~26.5 m , and ellipsoidal to conical , with a collarette . the isolate produced abundant mycelia on pda agar ; and the sporulation was excellent on pda agar and sma agar , respectively . comparing the colony morphology and culture characteristics of the isolate on mea medium , with previous descriptions , the present isolate was generally similar to those of m. fragilis . the isolates were observed to grow over a wide range of temperatures with varying growth rates on mea , sma , and pda ( fig . the average growth rates of eml - puki12 - 1 and eml - puki06 - 1 on mea , sma , and pda were 28 and 27 mm / day , 25.5 and 32 mm / day , 26 and 27 mm / day at 25 , respectively . the optimal growth temperature range was 25~30. among the different temperature and culture media , the best mycelial growth was found at 30 on mea media for eml - puki12 - 1 and 25 on sma media for eml - puki06 - 1 . on all media , the isolates grew slowly below 10 , rapidly at 25~30 , and could grow well at temperature 35. despite the wide intraspecific variation found among some taxa , the rdna its region has been used as a critical barcode marker for identification of mucoralean fungi at the level of species . the results of our molecular data analysis of the two mucoralean species were consistent with the phylogeny presented by walther et al . . in the its tree , our strains : eml - puki12 - 1 , eml - puki12 - 2 , eml - puki06 - 1 , and eml - puki06 - 2 completely matched the irregularis and fragilis clade , respectively . although some kinds of new and undescribed zygomycetous fungi in korea have been reported in several recent studies , data relating to the diversity of zygomycetous fungi in korea are still lacking . therefore , m. irregularis and m. fragilis isolated from the gut of insects and described as new record can contribute to the knowledge of diversity of zygomycetous fungi in korea .
while surveying the diversity of fungi of the order mucorales , two isolates , eml - puki12 - 1 and eml - puki06 - 1 , were obtained from the gut of soldier fly larvae inhabiting the bulrush at a pond located in the chonnam national university arboretum , gwangju , korea . the isolates were confirmed as mucor irregularis and mucor fragilis species , respectively , based on the morphological characteristics and phylogenetic analysis of rdna internal transcribed spacer region . such mucoralean species belonging to undiscovered taxa has not previously been described in korea .
percutaneous endoscopic gastrostomy tube placement is a commonly used procedure to improve the nutritional state and rehabilitation capability of patients with central nervous system disorders2,4,5,7,8,10,18,19 ) . it has a very low complication rate which , in most cases , is related to shunt infection occurring a few days or weeks after the procedure , and the procedure has been accepted as being safe6,11,15,16 ) . there have been few reports of mechanical failure of the ventviculoperitoneal ( vp ) shunt after percutaneous endoscopic gastrostomy ( peg ) tube insertion without shunt infection . the authors report a unique patient who had developed shunt malfunction immediately after peg tube placement without any signs of shunt infection . a 50-year - old male underwent a decompressive craniectomy and evacuation of intracranial hematoma because of his subdural hematoma and severe cerebral edema , caused by a motor vehicle accident . his right kocher 's point was used for ventricular entry , and the peritoneal catheter was placed through a paramedian subcostal incision . he was transferred to the rehabilitation department and had been in a semicomatous state for 8 months . a percutaneous endoscopic gastrostomy tube was placed six months after the vp shunt by a senior gastroenterologist who had more than 10 years of endoscopic experiences . the tube was introduced into the stomach through the epigastrium with a standard endoscopic pull - through technique . as usual , the patient received prophylactic antibiotics , and the procedure was performed with special attention and using a gentle technique so as not to interrupt the intraperitoneal shunt catheter . after a few hours , the scalp covering the surgical defect of his skull , which had been made by decompressive craniectomy , began to swell up . on the next day , it became worse and we took a brain ct scan which showed severely enlarged intracranial ventricles ( fig . furthermore , no red blood cells or white blood cells were found in his cerebrospinal fluid . skull x - rays were taken to confirm that the valve setting of the vp shunt had not been changed . the postprocedural abdominal film showed no gastrointestinal complications such as pneumoperitoneum or ileus , but kinking in the intraperitoneal shunt catheter was observed compared to the catheter being smooth before the procedure ( fig . we made a small incision in the subcostal area and pulled out the intraperitoneal catheter . the externalized catheter was carefully observed and no obstruction or disconnection was found . after we confirmed the adequate csf drainage of the catheter , we put it into his peritoneal cavity again . postoperatively , the swollen scalp depressed down and the sizes of the intracranial ventricles normalized in the follow - up ct scan . after a few days of close observation , the patient was transferred to the rehabilitation department . percutaneous endoscopic gastrostomy tube insertion is performed in patients who have difficulty with swallowing or problems with mentality . sometimes these patients already have had a vp shunt inserted . because shunt malfunction can cause severe complications to the patient , it is important to know if the peg tube placement is safe for patients with a vp shunt . there have been eight published papers supporting the safety of peg tube placement in patients with a vp shunt1,6,11,12,14,15,16,17 ) . it has thus been accepted as a safe procedure because it does not increase the infection rate of vp shunt . in the 126 cases reported in the eight articles , all the complicated cases were related to shunt infection and there were no cases with a simple mechanical failure of the vp shunt without shunt infection . our case is quite different from those cases in terms of the occurrence time and the presence of infection . to our knowledge , this is the first reported case of acute vp shunt malfunction after peg tube placement without any signs of shunt infection . however , in the case of percutaneous fluoroscopic gastrostomy , which is technically similar to peg tube placement , one case was reported in 2006 . jea et al.9 ) reported a 68-year - old woman who developed massive pneumocephalus caused by the inflation of gas into the peritoneum during fluoroscopic gastrostomy tube placement . chan et al.3 ) reported a 70-year - old male whose peritoneal catheter extruded through a removed gastrostomy scar which happened two years after peg tube removal . in addition , in 2013 , niewiadomski et al.13 ) reported a case of a 54-year - old female whose intraperitoneal catheter of vp shunt penetrated into her stomach 3 months following the peg insertion . those two cases were not related to shunt infection , but they were different from our case in terms of the occurrence time . the authors think that the intraperitoneal shunt catheter was mobilized or forced to make a kink by the movement of the stomach , the small bowel or the omentum during the procedure and that it eventually caused obstruction of the shunt catheter and acute aggravation of the hydrocephalus . though the patient was in a semicomatous state which made it difficult to detect the early neurological symptoms of hydrocephalus , he had a surgical skull defect , so we could find out the problem immediately and took steps to make the patient better . therefore , although peg tube placement has been accepted as a safe procedure in patients with a vp shunt , clinicians should be aware of the possibility that acute shunt malfunction can occur , even if there is no infection . furthermore , gastroenterologists who perform the procedure should be careful about the shunt catheter and need to use gentler techniques when the patient has a vp shunt . after the procedure , it is important to monitor not only the patient 's laboratory data but also the neurological status and postprocedural abdominal x - rays as well .
percutaneous endoscopic gastrostomy tube placement is often performed in patients with a ventriculoperitoneal shunt and it has been accepted as a safe procedure . the authors report a case of a 50-year - old male who developed acute exacerbation of the hydrocephalus immediately after the percutaneous endoscopic gastrostomy tube placement without any signs of shunt infection , which has not been reported until now . after revision of the intraperitoneal shunt catheter , the sizes of the intracranial ventricles were normalized .
worldwide , tumours account for the majority of cases of large bowel obstruction with diverticular disease and volvulus accounting for the remainder . volvulus commonly affects the sigmoid , caecum and transverse colon in the decreasing order of frequency with caecal volvulus accounting for < 1% of causes of large bowel obstruction . additionally , the bascule subtype accounts for < 10% of cases presenting with caecal volvulus . we present the case of a 58-year - old patient who presented with symptoms of intestinal obstruction and had characteristic radiological findings of a caecal volvulus a 58-year - old female with a past medical history of a large intrathoracic hiatus hernia presented to her general practitioner and was subsequently referred to our surgical department with symptoms of sudden onset severe epigastric pain associated with vomiting and abdominal distension . her abdomen was generally tender , maximally in the epigastric region , although she was not peritonitic . the admission erect chest radiograph showed a large hiatus hernia and an elevated right hemidiaphragm with a large loop of distended bowel ( fig . 1 ) . subsequent computed tomography ( ct ) scan demonstrated an almost completely intrathoracic stomach with a degree of volvulus , and the liver medially displaced by a loop of large bowel felt to be caecum ( fig . 2 ) . since the patient remained otherwise well , bidirectional endoscopy was performed , and given that a volvulus point could not be identified and the ileocaecal junction was not identified , contrast studies were requested ( figs 35 ) which demonstrated an incomplete obstruction caused by a volved caecum rotated anteriorly and cephalad . figure 1:erect chest radiograph , showing a large hiatus hernia and large loop of bowel elevating the right hemidiaphragm . figure 2:ct coronal section demonstrating caecum medially displacing the liver and causing an upward pressure on the right hemidiaphragm . figure 3:a large bowel contrast study showing the flow of contrast from the collapsed transverse colon into the dilated , upwardly displaced caecum . figure 4:coronal section of contrast - enhanced ct demonstrating contrast filling upwardly displaced caecum . figure 5:axial section of contrast - enhanced ct demonstrating whirl sign at the point of volvulus . erect chest radiograph , showing a large hiatus hernia and large loop of bowel elevating the right hemidiaphragm . ct coronal section demonstrating caecum medially displacing the liver and causing an upward pressure on the right hemidiaphragm . a large bowel contrast study showing the flow of contrast from the collapsed transverse colon into the dilated , upwardly displaced caecum after the contrast study partial resolution of symptoms occurred but given the development of free fluid in her contrast ct scan , the decision was taken to manage her surgically . a midline laparotomy was performed , with the finding of a freely mobile , grossly distended caecum flipped anteriorly over the ascending colon as demonstrated on her ct scan ( fig . 6 ) . additionally , there was no evidence of a gastric volvulus ( suggesting partial twisting ) and since her stomach was completely intra - abdominal , there was no need to address her hiatus hernia at that point in time . the patient was admitted to the high dependency unit post - operatively , made a good recovery and was discharged home within a week of surgery . caecal volvulus accounts for 1% of all causes of large bowel obstruction [ 1 , 2 ] . the mechanism of caecal volvulus has been well documented and can be explained by the presence of an excessively mobile caecum or incomplete fixation of the ascending colon to the retroperitoneum during embryogenesis . additionally , caecal volvulus can be described as organoaxial ( true caecal or caecocolic volvulus ) or mesenteroaxial ( caecal bascule ) . the latter describes the folding of the caecum anteriorly over the ascending colon and accounts for 10% of all caecal volvulus , while the former describes the twisting of the terminal ileum , caecum and ascending colon . interestingly , our patient had an incidental finding of a gastric volvulus on imaging , the aetiology most likely related to her large hiatus hernia , a known risk factor for gastric volvulus . the embryological theory as highlighted above for volvuli affecting the large bowel may also be applicable to the stomach . a review of 561 patients with caecal volvulus found that they commonly presented with symptoms of bowel obstruction abdominal pain , abdominal distension , constipation , nausea and vomiting . physical findings included abdominal distension , hyperperistalsis , peritoneal signs , abdominal mass and absent bowel sounds . it is reasonable to assume that patients with the bascule subtype may have intermittent subacute obstruction due to the flopping of the caecum back into its anatomical position . additionally , this subtype is less likely to strangulate as the mesentery is not frequently twisted when compared with the organoaxial subtype . risk factors commonly described for caecal volvulus include previous abdominal surgery , high fibre intake , chronic constipation and distal obstruction [ 57 ] . . found that ct findings of whirl , ileocaecal twist , transition points , x marks the spot and split wall have high specificity for caecal volvulus . additionally , they found that the absence of distal colonic decompression made the diagnosis of caecal volvulus unlikely . diagnostic confusion may exist between sigmoid and caecal volvulus ; however , the location of the mesenteric twist ( ct whirl sign ) is highly accurate in distinguishing the two . swirling strands of soft tissue attenuation within a background of fat attenuation and gives the appearance of a hurricane on a map. barium enema has been historically used to confirm the presence of a volvulus , assess the distal colon and may also have a therapeutic effect . factors such as patient co - morbidities and the viability of the bowel should be taken into account when managing caecal volvulus . the authors agree that right hemicolectomy is associated with the lowest recurrence and complication rates compared with detorsion alone , detorsion and caecopexy and caecostomy . additional options when faced with non - viable bowel and where primary anastomosis is not suitable include ileostomy and mucus fistula . minimally invasive procedures may play a role in the management of patients without perforation or gangrenous bowel and provides a reasonable treatment option for this condition .
caecal volvulus is a rare cause of intestinal obstruction , with the bascule subtype accounting for < 10% of all cases of caecal volvulus . it is associated with significant morbidity and mortality if left undiagnosed . we present the case of a 58-year - old female who presented to our surgical department with symptoms of intestinal obstruction . she had various radiological investigations , which supported the diagnosis of a caecal volvulus of the bascule subtype . she was subsequently managed surgically and had a right hemicolectomy and ileocolic anastomosis . her recovery was uneventful and she was discharged within 1 week of having her operation . fortunately , caecal volvulus of the bascule subtype is rarely encountered ; however , clinicians need to be aware of its presentation and subsequent management options so that clinical outcomes are improved .
nonpuerperal vulvar hematomas are uncommon and are often the result of blunt trauma like overzealous coitus , physical assault , and straddle - type injury resulting from such activities as bicycle riding , using recreational devices like the mechanical bull , cross - country skiing , and riding a go - cart . we present here a case of vulvar hematoma , which occurred after laparoscopic ovarian cystectomy . the patient , a 23-year - old , para 0 , underwent a laparoscopic ovarian cystectomy for an asymptomatic 12 x 8-cm mass . the mass was filled with 2 to 3 large loculated areas with clear , serous colored fluid . although the surgery was uncomplicated and with minimal blood loss,the patient presented to the clinic the first day after her surgery with complaints of nausea , vomiting , and an episode of syncope . the patient was orthostatic with a blood pressure of 80/40 and a pulse of 108 . the physical examination was significant for a soft , nondistended abdomen with mild - to - moderate tenderness , with no rebound or guarding . a laparoscopic incision with bloody discharge existed , and the perineum had a vulvar hematoma ( figure 1 ) . , bleeding could be seen from both the left superficial epigastric vessel and from a large ventral wall subcutaneus hematoma . also , bleeding was noted at the base of the prior cystectomy , and approximately 2,000 cc of blood was present in the abdomen . the subcutaneus tissue of both vulva and vagina are loose and can accumulate a large amount of blood before obvious signs and symptoms of a hematoma become apparent . although not common , vulvar and vaginal hematomas may cause significant morbidity . the case presented here may be the first to describe an association between operative laparoscopy and vulvar hematoma . failure to recognize intraoperative bleeding during laparoscopic procedures can be attributed to the tamponade effect of pneumoperitoneum and to decreased venous return due to the steep trendelenburg position . to underline the challenges of laparoscopic postoperative bleeding , wong et al described 3 cases of intra - abdominal bleeding following laparoscopic adnexal surgery . in 2 of the 3 cases , postoperative exploratory laparotomy failed to identify the source of bleeding . to address the abdominal vascular wall injury at laparoscopy , balzer et al , studying the vascular anatomy of the abdominal wall in 21 human cadavers , found that from 36 trocar incision sites , recommended commonly in the literature , half incur the risk of vascular injury . to minimize the danger of causing lesions in the large abdominal vessels , balzer et al suggest locating the trocars in the ventral midline or ion , a zone 5-cm in width lateral to the lateral border of the rectus sheath
few cases of intraoperative or postoperative complications associated with laparoscopic adnexal surgery have been reported in the literature . we describe a case of laparoscopic abdominal vascular injury and persistent bleeding in the matrix of the ovary following laparoscopic cystectomy . during the first postsurgical day , the patient was syncopal . the physical examination showed a vulvar hematoma and minimal bleeding from a laparoscopic incision in the abdominal wall . vulvar hematoma and an unstable patient may signal serious vascular bleeding .
burning mouth syndrome ( bms ) is defined as the sensation of burning or pain on the oral mucous membrane , without evidence of abnormalities in the direct inspection , clinical exam or subsidiary investigation . besides pain and tingling , bms is often characterized by dry mouth and taste perversion [ 1 , 2 ] . bms is more prevalent among postmenopausal women , affecting from 1.5 to 5.5% of this population . several factors are reported as being associated with burning mouth symptoms , including xerostomy , allergies to dentistry materials , oral candidiasis , systemic nutritional deficiency ( e.g. vitamin b1 , b2 , b12 , folic acid and zinc ) , diabetes , hormonal disturbances , oral ulcer and periodontitis . some studies suggest that bms is a specific form of cranial neuropathy , based on the pattern of sensorial findings ( hypoesthesia ) , reduction of the nociceptive threshold , as well as on neurophysiologic findings ( suggestive of a small fiber neuropathy ) . other studies suggest central nervous system abnormalities , including changes in the blink reflex , larger availability of dopamine receptors in the putamen and reduction of 6-[f ] fluorodopa recaptation on putamen . anecdotal reports suggest the benefit of alfa - lipoic acid , an antioxidant used in diabetic neuropathy , amisulpiride , a neuroleptic of second generation that in low dosages presents dopaminergic activity , and of topic clonazepam [ 1013 ] . herein we report one case of refractory bms with complete improvement with pramipexol , a non - ergotic dopaminergic agonist commonly used for parkinson s disease . as exposed below , this medication was chosen based on some clinical features of our patient that resembled what is commonly seen in restless leg syndrome . pramipexol has not been previously reported as potentially effective for bms . accordingly , because the patient was very refractory , and also because of some peculiar clinical findings , we speculate on the pathophysiology of the disease . a 68-year - old woman reported daily continuous burning feeling on the lateral and anterior site of her tongue and lower lip for 3 years . the burning was of moderate intensity ( 7 on a 10-point visual analogical scale ) , worsening by the end of the afternoon . she reported important improvement when speaking and moving the lower lip , as well as when drinking cold water . she noticed change in her taste sense , with lower tolerance to heavy meals and increased burning after acid meals . she did not complain of xerostomia and therefore the salivary flow rate was not measured . she denied other medical antecedents or use of medication . over the past 3 years , before seeking care with us , the patient previously consulted four gastroenterologists . she had no improvement of the burning sensation after proper treatment ( table 1 ) . table 1diagnostic criteria for burning mouth syndrome according the international classification of headache disorders ( 2004 ) description : an intraoral burning sensation for which no medical or dental cause can be found.diagnostic criteria : ( a ) pain in the mouth present daily and persisting for most of the day . diagnostic criteria for burning mouth syndrome according the international classification of headache disorders ( 2004 ) the patient also had consulted five dentists . they changed her dental prosthesis twice , tried periodontal treatment and artificial saliva without meaningful results . she used wear superior total denture and inferior removable partial denture , both in good conditions , without vertical dimension lose or instability . blood analysis was performed and blood routine , as well as screening for vitamin deficiencies and ferritin were within normal limits . we established a diagnosis of refractory bms and initiated treatment with pramipexol ( 0.125 mg at night follow - up information was obtained prospectively using a headache calendar ( measuring frequency and severity of pain , use of rescue medications , etc . ) and was contrasted to the baseline . after 1 week of treatment the patient reported a 20% improvement ( a decrease from seven to five on visual analogical scale ) and around 25% diminution in the frequency of the symptoms . , the symptoms relapsed around 6 days after , with an intensity of pain around 60% of the baseline . since then , she has been using 0.75 mg at night , with total control of the burning sensation . the patient was followed for a year and she remains pain free , with no significant tolerability issues . this case of bms showed two peculiar clinical characteristics : ( 1 ) it worsened by the end of the day ; ( 2 ) it improved with the movement of the tongue . the circadian fluctuation of the symptoms is a known feature of the bms ; however , the relief by moving the tongue is not a notorious manifestation of this disease . therefore , this pattern of symptoms seemed similar to what is seen in restless leg syndrome ( rls ) , a condition secondary due to a dopaminergic dysfunction of the central nervous system . rls is characterized by lower limbs dysesthesias that worsen by the end of the day and improve when moving the legs [ 16 , 17 ] . the prevalence of rls in patients with headache is high , , however , there are no data correlating rls with bms . although the relationship between bms and parkinson s disease is not universally accepted , the comorbidity has been suggested . bms is frequent among patients with parkinson s disease ( pd ) . in a study of 115 patients with pd , burning mouth happened in 24% of them , incidence prevalence 30 times greater than expected by chance only . non - specific disturbances on the dopaminergic system have been documented in patients with bms [ 8 , 9 ] . the association of bms with rls and pd , besides the improvement with amisulpiride , point to a dopaminergic dysfunction in bms . pramipexol is a non - ergotic dopaminergic agonist , with predilection to dopaminergic d-2 receptors [ 23 , 24 ] . our anedoctal finding should be confirmed by a pilot study , and , if positive , by clinical trials . as a cautionary note , since this is a report of a single case , we can not exclude that the therapeutic response to pramipexol happened as a placebo effect , or merely as a coincidence .
burning mouth syndrome ( bms ) is characterized by burning discomfort or pain in otherwise normal oral mucosa . it is usually refractory . treatment modalities are scarce . herein we report one case of primary disabling bms , previously refractory to multiple regimens , with complete and persistent improvement with pramipexol , a nonergot dopamine agonist which has high selectivity for dopaminergic d2 receptors . we discuss potential pathophysiological implications of our findings .
a 44-year - old lady presented to us with complaints of dull aching continuous pain in the right flank region for 1 year . she had intermittent acute exacerbations of pain associated with nausea / vomiting requiring hospitalization for pain relief . there was no associated fever , hematuria , lithuria , or lower urinary tract symptoms . on examination a large renal mass ultrasonography revealed a large cystic hypoechoic area in the region of renal hilum suggestive of either upjo with large extrarenal pelvis and gross hydronephrosis , or a parapelvic cyst causing extrinsic obstruction at ureteropelvic junction . an intravenous urogram and cect abdomen were done to further characterize the mass [ figure 1 ] . it confirmed an 11 12 10 cm simple cystic mass at renal hilum with proximal gross hydronephrosis and pushing the kidney superolaterally . the right kidney showed delayed excretion of contrast with poor function and relatively thin parenchyma . ( b and c ) contrast enhanced ct scan showing a large cystic parapelvic right renal lesion with hydronephrosis . there was no apparent solid component inside the mass an informed consent was taken for right retrograde pyelography and exploration . retrograde pyelography confirmed gross hydronephrosis with a malrotated kidney ; however , pelvis was not dilated to the size equivalent to that of the cyst on cect , excluding the diagnosis of upjo . a rib - cutting extraperitoneal flank approach was used through the 12 rib bed for excision / deroofing of the parapelvic cyst [ figure 2 ] . the cyst was densely adherent to the psoas fibers and sheath posteriorly , which were separated by a sharp dissection . the cyst was aspirated first to collect sample for cytology and then electively decompressed in a controlled fashion using suction canula . care was taken to avoid any spillage of fluid by packing the surgical field with betadine soaked sponges . the fluid was straw colored and clear . there was no lamination of the cyst wall , unlike a hydatid cyst . the cyst opening was closed and further dissection done to separate it from ureter , pelvis , and lower pole of kidney . the cyst was excised in toto and sent for histopathological review which surprisingly revealed a cystic schwannoma typically positive for s-100 protein on immunohistochemical staining [ figure 3 ] . ( a ) operative photograph showing the tense cystic mass at the right renal hilum . ( b ) completely excised specimen ( a ) hande stained 40 photomicrograph of the specimen showing a predominantly cystic tumor bounded by fibroconnective tissue capsule . the tumor was composed of antoni a areas of compact spindle cells with focal nuclear palisading and few antoni b areas of spindle to ovoid cells in a loosely textured matrix . ( b ) 200 photomicrograph showing typical s-100 immunoreactivity of the schwannoma . at 3 months follow - up retroperitoneum is a relatively uncommon site ( 0.7 - 3% ) for schwannoma to occur . given the large loose areolar space available in the retroperitoneum , tumors in this location often present late with vague complaints related to compression of surrounding structures and often develop degenerative changes inside them . such tumors which acquire histological degenerative changes over long standing duration are often referred to as cystic change is a fairly common ( 50 - 60% ) association with this type of tumor in the retroperitoneum . although there are several existing case reports of a schwannoma occurring at the otherwise quite rare location of renal , hilar , or perirenal region,[59 ] most of these have presented as either complex cystic or solid masses mostly confusing as renal cell carcinoma in the preoperative diagnosis . to the best of our knowledge , the presentation of a schwannoma mimicking a simple cyst has not been reported earlier . to this extent , the index case had a unique and atypical presentation , the histopathological diagnosis being a complete surprise . most cases are confirmed only on histopathology , since there are no specific features of schwannoma on imaging studies . the degenerative changes in ancient schwannomas may produce inhomogenous enhancement and complex cystic appearance in an otherwise well circumscribed mass on ct scan . mri with gadolinium enhancement may provide a clue to the site of neuronal origin but provides no marked benefits over cect . overall , the case represented a rare diagnosis , that too with an atypical presentation posing a diagnostic dilemma .
schwannoma is a benign soft tissue tumor of neural origin arising from the schwann cells of the neural sheath . it has rarely been reported in renal or perirenal region . the preoperative diagnosis has mostly been confused with renal cell carcinoma in this location in most previous reports . we report a case that presented with a large simple cystic mass at the renal hilum . the preoperative differential diagnosis included hilar renal cortical cyst , renal sinus cyst , ureteropelvic junction obstruction ( upjo ) , or even a hydatid cyst . the final diagnosis was clinched only on histopathological examination .
horner syndrome ( hs ) was characterized first in humans by johann friedrich horner in 186911 ) . hs results from interruption of the oculosympathetic pathway at anywhere along its course between the hypothalamus and the orbit1 ) . hs is characterized by the classic triad of ipsilateral eyelid ptosis , miosis and facial anhidrosis . there are many causes of hs , but herniated cervical disc ( hcd ) is a very rare cause among them . hcd is a common cause of spinal cord compression , but hs associated with hcd has been described just in very few literatures . there were only two cases of herniated thoracic disc at t1 - 2 as the causes of hs2,6 ) . in addition , the occurrence of hcd as the etiology of hs has not been reported . a 41-year old male patient awoke to find sudden onset of left ptosis , right side numbness without identifiable history of trauma or physical stress . he first visited local medical center and had brain magnetic resonance imaging ( mri ) taken , however , no specific findings were found . when he was referred to our neurosurgery department via emergent department , neurological examination presented left side ptosis , miosis and anhidrosis of left half of face which are typical symptoms of hs , and there was numbness below right side of the t8 dermatome . , pupils measured 5 mm on the right eye and 3 mm on the left eye , and the left pupil showed dilatation lag . sweat test showed only right side starch powder on the face changed to dark brown color , which means left side anhidrosis ( fig . 1 ) . the cervical spine mri demonstrated an upward migrated and large left paramedian disc herniation with severe unilateral spinal cord compression at the c4-c5 level which is high signal intensity with internal low signal intensity on t2wi and intermediate signal intensity on t1wi . was doubted , we conducted a standard microsurgical anterior approach to the c4-c5 interspace on 2 days after admission . posterior longitudinal ligament had been ruptured and spinal cord was severely compressed especially at left side . the extruded disc material was migrated to upward including part of the cartilaginous endplate . after complete decompression of neural structures , postoperatively , right side numbness was improved but left miosis and ptosis remained until discharge . however , after two months from the operation , miosis , ptosis and anhidrosis were completely recovered , too . horner syndrome , or oculosympathetic paralysis , was characterized first in humans by johann friedrich horner in 186911 ) . it is resulted from interruption of the oculosympathetic pathway between the hypothalamus and the orbit . the clinical findings of hs include ipsilateral miosis , ptosis , and facial anhidrosis1 ) . the oculosympathetic pathway begins with a first - order neuron at the posterior lateral aspect of the hypothalamus and through the brain stem extends down the spinal cord from c8 to t2 . second - order ( preganglionic ) neuron , which is located in the intermediolateral gray substance of the spinal cord at the level c8-t2 ( ciliospinal center of budge - waller ) , then exit the spinal cord via the ventral roots and enter the paravertebral sympathetic chain . the preganglionic pathway passes over the apex of the lung and ascends in the cervical sympathetic chain to the superior cervical ganglion . the third - order ( postganglionic ) neuron , which is superior cervical ganglion located at the level of c2-c3 , posterior to the carotid sheath and anterior to the longus colli muscle , follow the carotid plexus into the skull , join with the ophthalmic nerve , and enter the orbit . hs may occur as a result of injury anywhere along this pathway1,5,9 ) . based on localization of the oculosympathetic pathway interruption , a hs is often classified as central ( first - order neuron ) , preganglionic ( second - order neuron ) or postganglionic ( third - order neuron ) . the causes of central hs are brain stem ischemia , brain tumors , demyelinating diseases , syringomyelia and transverse myelitis1,4 ) . preganglionic hs is resulting from thoracic or neck tumor , direct spinal cord trauma , herniated disc at c8-t1 , iatrogenic disruption of the sympathetic pathway from radical neck dissection or selective nerve root block , carotid angiography , stenting or endarterectomy , spontaneous carotid dissection , and aortic aneurysm to various malignant conditions that directly or indirectly affect the normal sympathetic innervations3,5,7,8,9,10,12,13 ) . the causes of postganglionic hs are vascular headaches , tumor or aneurysm in cavernous sinus , nasopharyngeal tumor , and trauma with basal skull fracture . and most frequently it is seen as a consequence of carotid artery dissection or during cluster headache anhidrosis is rarely conspicuous , and in the postganglionic subtype , it is virtually absent1,9 ) . , the fibers of first - order neurons travel in budge 's center immediately lateral to the dorsal gray matter and synapse in the spinal cord gray matter9 ) . the possible cause of hs in this case is that the hcd directly compresses spinal cord producing an insult to the first - order neuron of the sympathetic pathway within the spinal cord at the level of c4-c5 . mr imaging is the most reliable investigative procedure and should be accepted as an initial diagnostic tool for hs associated with hcd . patients with hcd commonly have neck pain , cervical radiculopathy , myelopathy and a combination of these symptoms . however , when patients have no cervical symptoms initially , or completely lack cervical symptoms , this unusual presentation can lead to a delayed or incorrect diagnosis in many wrong directions . in particular , as the patient had no cervical symptoms initially , the physician suspected a cerebral stroke first . careful history - taking and detailed neurologic examinations are indispensable steps for early diagnosis of hs associated with hcd . for the treatment of it , early diagnosis and prompt surgical decompression careful history - taking and detailed neurologic examinations are indispensable steps for early diagnosis of hs associated with hcd . for the treatment of it , early diagnosis and prompt surgical decompression
horner syndrome ( hs ) occurs when there is interruption of the oculosympathetic pathway . the causes of hs are various , but hs originated from herniated cervical disc is very few . hs attributable to the lesion of the first - order neuron of cervical spinal cord is extremely rare . a 41-year old male was admitted for sudden onset of left ptosis and right side numbness . neurological examination revealed ptosis , miosis and facial anhidrosis on the left side . mri and ct scans demonstrated large left paramedian disc herniation with cord compression at the c4 - 5 level . the herniated disc was removed through anterior approach and his symptoms were improved after the operation .
spontaneous hematomas are more common than post - traumatic epidural hematomas . the annual incidence of spontaneous epidural spinal hematoma has been reported to be 0.1 in 100,000 in the general population , whereas , in the pediatric population , this incidence is significantly lower . only 40 pediatric cases have been reported in the literature , of which 34 were spontaneous and the rest were traumatic . according to the author 's literature search , only 7 cases of post - traumatic epidural hematoma have been reported in pediatric patients till now , and the present case is the youngest . although the lesion is located in the thoracolumbar region in older patients , the cervicothoracic site is more common in children . presenting symptoms in children early surgical intervention has been reported to result in more favorable outcomes in pediatric patients as compared to adults . however , the present case of an 8-month - old child with post - traumatic cervical epidural hematoma though diagnosed and treated late , resulted in a complete recovery . an 8-month - male infant had a history of a fall from the crib a fortnight prior to admission . the mother noticed a progressive decrease in movement in both the lower limbs of the child , which gradually worsened to paraplegia . on examination , magnetic resonance ( mr ) scan of the cervical spine with screening of the entire spine showed an epidural hematoma from c4 to d1 vertebral levels with severe cord compression [ figures 1 and 2 ] . t1-weighted sagittal image of the cervicodorsal spine shows an hyperintense epidural hematoma with severe cord compression t2-weighted axial image at the c6 - 7 vertebral level demonstrates severe anterior cord displacement at surgery in prone position , c6 and c7 laminoplasty was performed . the patient had a dramatic recovery of the lower limb function in the 48-hour postoperative period and started moving both lower limbs well . the anterior part runs fairly constant and is tightly attached to the posterior longitudinal ligament via hofmann 's ligaments . on the other hand , most authors agree that the rupture of this valveless epidural internal venous plexus is the primary source of an epidural hematoma . the sudden elevation of pressure induced by crying , coughing , voiding , straining , and trauma can cause a bulk of backflow into the valveless venous system , which makes the loosely supported posterior part of the vertebral venous plexus prone to rupture . thus , the majority of spontaneous bleeds occur in the posterior aspect of the spinal canal in both adults and children . gaining head control is an important neuromuscular development milestone during the first 4 - 6 months of life . during this period therefore , the combined effect of the weight of the head and increased cervical mobility in the presence of undeveloped muscular capacity of the neck predisposes the cervical spine to injury with sudden flexion and extension movements . hence , although epidural hematomas are located in the thoracolumbar region in older patients , the cervicothoracic site is more common in children . the clinical presentation depends on the speed of blood accumulation , location , and extent of the hematoma . the infant initially presents with irritability , crying , neck stiffness , and limited range of motion of the neck , followed by motor neurological deficits as noted by decreased ability to roll over and decreased limb movements . presenting symptoms in children are usually nonspecific , making the diagnosis difficult . however , an early diagnosis followed by surgical intervention has been reported to result in a more favorable outcome as compared to adults . spinal mr imaging clearly delineates the location of an epidural hematoma and identifies an associated vascular malformation . as noted in our case , the hematoma is seen as a posterior high - signal intensity lesion on t1-weighted image . on t2-weighted images , the clot appears as a low - signal intensity lesion suggesting a subacute - type hemorrhage of more than 3 days duration . t1-weighted mr images are most valuable because of the pathognomonic signal shift from isointensity with the cord in the early period to hyperintensity in the intermediate stage of clot resolution . cervical spine radiographs with anteroposterior , lateral , and odontoid views are useful to identify associated traumatic fractures . early surgical evacuation of the clot is recommended as a treatment of choice for symptomatic traumatic spinal epidural hematoma . in exceptional cases where the neurologic deficit is minor , conservative management may be undertaken , especially with the coexistence of a serious coagulopathy and associated serious medical disease . a laminoplasty is favored compared to laminectomy in the pediatric population to avoid postlaminectomy deformity in the growing spine . early diagnosis and surgical intervention have the most favorable outcome for spinal epidural hematomas . in infants , as noted in our case , even a delayed diagnosis and treatment results in a good outcome probably due to the plasticity of the infant nervous system .
an 8-month - old male infant had presented with a history of a fall from the crib a fortnight ago . he had developed progressive weakness of both lower limbs . on examination , the infant had spastic paraplegia . magnetic resonance ( mr ) imaging of the cervical spine showed an epidural hematoma extending from the fourth cervical ( c4 ) to the first dorsal ( d1 ) vertebral level with cord compression . the patient had no bleeding disorder on investigation . he underwent cervical laminoplasty at c6 and c7 levels . the epidural hematoma was evacuated . the cervical cord started pulsating immediately . postoperatively , the patient 's paraplegia improved dramatically in 48 hours . according to the author 's literature search , only seven cases of post - traumatic epidural hematoma have been reported in pediatric patients , and our patient is the youngest . the present case report discusses the etiopathology , presentation , and management of this rare case .
isolated abducens nerve palsies caused by vertebra artery ( va ) aneurysm have been rarely reported1 - 3,7 ) . nerve compression due to aneurysm , cisternal hematoma , vascular insufficiency , increased intracranial pressure ( icp ) have been suggested as potential mechanisms for isolated abducens nerve paralysis following ruptured va aneurysm . the patients usually presented with bilateral or ipsilateral gaze palsy to the pathologic lesions . to the best of our knowledge , we describe the first case of dissecting va aneurysm presenting with contralateral abducens nerve palsy and discuss its possible mechanisms . a 38-year - old man was admitted for management and evaluation of a headache that had persisted 6 days . the patient was discharged receiving analgesic and further radiologic tests were recommended . on the present admission , 1b ) disclosed a fusiform dilatation of the right vertebral artery involving the proximal portion of the posterior inferior cerebellar artery ( pica ) . the patient suddenly suffered from severe headache with diplopia a day before the scheduled cerebral angiography . emergent intracranial ct demonstrated a high density area in the basal cistern and prepontine cistern without ventriculomegaly ( fig . 2a , b ) revealed a dissecting aneurysm of right distal vertebral artery compromising proximal pica with blood supplying to the left proximal portion of pica through glomus - like vascular channel from right va . in particular , the vertebrobasilar artery was remarkable flexed to the inferior on the left side . no diffusion restriction was found in follow - up magnetic resonance imaging ( mri ) of the brain stem ( fig . 2d ) illustrated the upward displacement of the vertebrobasilar artery compared with previous images and a ct scan ( fig . full recovery of left abducens nerve paresis was achieved in 11 weeks after onset ( fig . the incidence of isolated abducens nerve palsy following va aneurysm is rare . to our knowledge , only three cases of va aneurysm ( two ruptured and one unruptured ) presenting with unilateral sixth nerve paralysis have been reported1 - 3 ) . the possible mechanisms of the isolated nerve palsies related to aneurysm of va can be divided into several groups : direct compression by aneurysm1,2 ) , brain stem or nerve compression due to thick cisternal hematoma , especially prepontine cistern7 ) , stretch nerve injury on the petrous apex triggered by increased icp6 ) , and vascular insufficiency to the abducens nuclei by vasospasm9 ) . the chance of the direct nerve contact with aneurysm could be ruled out in our patient because the patient suffered left sixth nerve palsy after ruptured right dissecting va aneurysm . coppeto and chan1 ) and dumas and shults2 ) showed ipsilateral sixth nerve palsy to the same direction of va aneurysm . thick prepontine cisternal hemorrhage was found in an intracranial ct scan , but the possibility of nerve paresis was thought to be low , considering the prepontine segment of the abducens nerve , because direct compression by cisternal hematoma causes only bilateral abducens nerve palsy . morioka et al.7 ) illustrated two cases of bilateral isolated sixth nerve palsy compressed by cisternal hematoma . regarding the increased icp , no prominent brain swelling or acute hydrocephalus was seen on brain ct . in addition , the neurologic deficit sings associated with facial nuclei or medial longitudinal fasciculus were not noted9 ) . therefore , for this patient , a thick blood clot in the prepontine cistern ( fig . 2a ) and may lead to compression of the abducens nerve on the opposite side . a similar mechanism has been suggested as a cause of isolated sixth nerve palsy on the contralateral side . elongation of vertebra artery8 ) and prominent flexion of basilar artery related to aneurysm of superior cerebellar artery5 ) can lead to isolated sixth nerve palsy on the contralateral side by compression of the vertebral artery of the opposite side . opening of liliequist 's membrane and cisternal irrigation of hematoma may seem to be effective for clinical improvement4 ) . complete recovery of nerve palsy was observed in three cases ( two bilateral and one unilateral)3,7 ) . in this case , although , mri with spoiled gradient recalled echo images was not obtained for the confirmation of direct abducens nerve contact with vertebral artery due to the limited time , the follow - up mri may support the mechanism of nerve compression by shifted vertebrobasilar artery . isolated contralateral abducens nerve palsy following ruptured va aneurysm may develop as a result of nerve compression secondary by displaced verterobasilar artery due to primary thick clot in the prepontine cistern .
isolated abducens nerve paresis related to ruptured vertebral artery ( va ) aneurysm is rare . it usually occurs bilaterally or ipsilaterally to the pathologic lesions . we report the case of a contralateral sixth nerve palsy following ruptured dissecting va aneurysm . a 38-year - old man was admitted for the evaluation of a 6-day history of headache . abnormalities were not seen on initial computed tomography ( ct ) . on admission , the patient was alert and no signs reflecting neurologic deficits were noted . time of flight magnetic resonance angiography revealed a fusiform dilatation of the right va involving origin of the posterior inferior cerebellar artery . the patient suddenly suffered from severe headache with diplopia the day before the scheduled cerebral angiography . neurologic examination disclosed nuchal rigidity and isolated left abducens nerve palsy . emergent ct scan showed high density in the basal and prepontine cistern compatible with ruptured aneurismal hemorrhage . right vertebral angiography illustrated a right va dissecting aneurysm with prominent displaced vertebrobasilar artery to inferiorly on left side . double - stent placement was conducted for the treatment of ruptured dissecting va aneurysm . no diffusion restriction signals were observed in follow - up magnetic resonance imaging of the brain stem . eleven weeks later , full recovery of left sixth nerve palsy was documented photographically . in conclusion , isolated contralateral abducens nerve palsy associated with ruptured va aneurysm may develop due to direct nerve compression by displaced verterobasilar artery triggered by primary thick clot in the prepontine cistern .
in general , large variations exist in the cancer profile among different geographical regions , between countries even within the country depending on environmental and genetic factors , dietary and addiction habits , infection load and different socio- economic factors . patterns observed in a referral cancer institution mostly reflect leading cancer types in specific regions . in this study , we surveyed a cancer pattern among patients admitted for treatment at swami ram cancer hospital and research center , haldwani , a referral center for cancer patients of the kumaun region of uttarakhand . this hospital caters patients from hill , foothill , and also from adjoining areas of neighboring state , uttar pradesh . the study included cancer patients confirmed by tissue diagnosis and admitted at the hospital from january 2010 to december 2010 . information regarding age , sex , and residential area was retrieved from the patient case sheets maintained in the record section . a total of 354 confirmed cases were identified in this period of which 206 were males ( 58.19% ) and 148 were females ( 41.80% ) . among all the cancer cases lung cancer was the most prevalent type ( 17.23% ) overall . separately in females leading cancer type was breast cancer ( 22.29% ) followed by cervix cancer ( 14.86% ) and ovarian cancer ( 13.51% ) . lung cancer was most common type ( 26.21% ) followed by larynx cancer ( 11.16% ) and cancer of oropharynx ( 9.7% ) . such types include prostate cancer ( 2.82% ) , stomach cancer ( 2.54% ) ; laryngopharynx and cancer of urinogenital system ( each with 1.97% ) ; cancer of pancreas ( n = 5 ) , liver cancer ( n = 3 ) , cancer of nervous system ( n = 3 ) ; cancer of intestine , nasopharynx , skin , anal canal , and uterus ( each with two cases ) . a single case was reported for each of testis , penis cancer , male breast cancer , soft tissue sarcoma , extraskeletal myxoid chondro - sarcoma , osteogenic sarcoma , thymoma , gastroespohageal junction , lacrimal gland , salivary gland , and inguinal node cancer . distribution of major cancer types overall half of the cases ( 49.71% ) were in age group of 4160 years followed by the age group of 6180 years ( 32.20% ) . rest were in between 21 and 40 years , and few cases ( n = 7 ) were below 21 years and above 80 years ( n = 7 ) . distribution of age in major cancer types the majority of patients were from nainital ( 46.89% ) , us nagar ( 40.96% ) , and pithoragarh ( 8.75% ) districts . it was noted that 10.16% of the patients came from adjacent parts of uttar pradesh . this study although is a brief one ( 354 patients ) , it clearly indicates that in this region of uttarakhand , lung cancer had much higher preponderance . in addition , the majority ( 88.52% ) of lung cancer patients were males ( male : female ratio 7.7:1 ) . this data corroborate with that of rawat et al . in their retrospective study including 203 lung cancer patients at dehradun , another district of uttarakhand , they reported that 89.16% patients were males . smoking tobacco ( active or passive ) is an established risk factor for lung cancer . according to a survey conducted by indian council of medical research involving 4932 households in urban and rural area of uttarakhand and 5443 individuals , a considerable portion of male population ( 35% ) was smokers and the mean age of initiation of smoking was 19 years of age . other than lung cancer , smoking is an undoubtedly most important etiological factor and accounts for male preponderance in cancer of larynx and esophagus . in our study , a high male over female ratio was found for cancer of larynx ( 11.5:1 ) . for esophageal cancer the male : female ratio was 2:1 . chewing tobacco is an important risk factor for oral and oropharyngeal cancer . in uttarakhand , 21% of males were found to use smokeless tobacco while 2% of females were addicted to it . for oropharyngeal cancer the male to female ratio alcohol consumption enhances the risk of mouth , tongue , pharynx , larynx , esophagus , and liver cancer . except in liver cancer , in all these alcohol - related cancers smoking a considerable portion of male population of this state ( 32% ) was found to be addicted to alcohol intake , which possibly contributed in part to a high number of pharynx cancer cases ( n = 32 , including oropharynx , laryngopharynx , and nasopharynx ) , and larynx cancer cases in the present piece of work . in an earlier study gaur et al . also observed that tobacco- and alcohol- related cancers were predominated in males at dehradun . breast cancer is the commonest cancer among women worldwide ( 26% of all female cancers ) . although this is the leading female - type cancer in developed countries , developing country like india is experiencing a steady increase in breast cancers over the years . in present analysis breast cancer had highest prevalence in females followed by cervical and ovarian cancers , respectively . one study from north india reported that majority of breast cancer patients ( 83.9% ) were in the fourth to sixth decade of their life . in our study , one - third of the patients were between 21 and 40 years of age and approximately half of them were between 41 and 60 years of age . average age of breast cancer patients was 48 years which corroborates with a delhi - based study ( 47.73 years ) . in this study , an attempt was made to observe major cancer types in nainital and its adjoining districts of uttarakhand , which may be useful to develop a cancer prevention program for prevalent types in this region as well as to plan research strategies . however , a larger study spanning more years is required to get more clear idea about the cancer pattern in the state .
a survey was conducted to determine the cancer profile in nainital and adjoining districts of uttarakhand . epidemiological information was collected from the records of patients with confirmed cancer cases . a total of 354 cases were studied for the year 2010 . lung cancer was found to be leading cancer type ( 17.23% ) overall . breast cancer was most prevalent in females ( 22.29% ) followed by cervical ( 14.86% ) and ovarian cancers ( 13.51% ) . men were mainly suffering from tobacco- and alcohol - related cancers , e.g. , lungs ( 26.21% ) , larynx ( 11.16% ) , oropharynx ( 9.7% ) , oral cavity ( 6.79% ) , and esophagus ( 6.79% ) . cancers of unknown primary site ( 1.41% ) were also detected .
he was taking warfarin for deep vein thrombosis and had a hemoglobin level of 4.5 g / dl . he had undergone low anterior resection and partial bladder resection with adjuvant chemotherapy to treat sigmoid colon cancer 3 years earlier . the anemia and the prolongation of the prothrombin time were easily corrected ; thus , continuous bleeding was not suspected . nevertheless , in order to rule out gastrointestinal bleeding as a cause of the severe anemia , abdominal computed tomography ( ct ) was performed . however , there was no evidence of intra - abdominal bleeding . instead , a 55-mm abdominal aortic aneurysm with eccentric bulging combined with a right common iliac artery aneurysm ( fig . the aneurysm size increased by 10 mm compared with the size 2 years previously . because severe intra - abdominal adhesion and wound problems after laparotomy were expected , endovascular aortic aneurysm repair ( evar ) was recommended instead of open surgery . evar was started with the deployment of a bifurcated main body using an aortic stent graft of 303040 mm and an inner bare stent 323250 mm in size ( s&g biotech inc . , subsequent extension of the right graft limb was performed uneventfully using two covered stents ( 121280 mm and 121260 mm ) to exclude the right common iliac artery aneurysm . dreadful complications occurred during the last procedure of the left graft limb extension . after the selection of the left graft limb and the guide wire exchange to a lunderquist stiff wire ( cook inc . , bloomington , in , usa ) , a 16f introducer sheath was inserted for the delivery of a limb extension graft , but the tip of the sheath perforated the left proximal external iliac artery during the advancement of the sheath ( fig . sheath - pushing against the tortuous iliac artery caused the guide wire to slip back into the sheath , and the dilator tip punctured the arterial wall . although the extension of the left graft limb was scheduled to end at the distal common iliac artery , the extension was promptly determined to be lengthened to the external iliac artery to cover the perforated site . the left graft limb of the main body was reselected , and a stent graft of 1212100 mm was deployed . however , subsequent aortography showed an occlusion of the left graft limb , and acute thrombosis was suspected ( fig . a prompt balloon thrombectomy was attempted after the left superficial and deep femoral arteries were clamped to prevent distal embolization . a 6f balloon catheter was advanced through the occlusion and withdrawn carefully not to cause disjunction of the overlapped stent grafts . fresh thrombus was successfully removed , and the graft flows became excellent without any endoleak upon the completion of angiography ( fig . 3 ) . at the 3-month follow - up , the patient was doing well without any symptoms like buttock claudication . although the surgery reduces the risk of rupture of the aortic aneurysm , the mortality of surgery has been reported to be approximately 2% to 8% . in fact , in the high - risk group for surgery , the morbidity is as high as 30% . since the first successful endovascular treatment of an abdominal aortic aneurysm in 1991 , this procedure has been performed with increasing frequency for high - risk patients . recently , with the development of smaller and more precise devices , evar has been accepted as a useful treatment method for patients at high risk for postoperative complications and patients refusing surgery . however , evar also has many complications related to the procedures , such as ruptures , thrombi within stent grafts , endoleaks , dislocation of the stent grafts , and infections . we experienced combined iliac artery rupture and graft limb thrombosis , which would cause a fatal outcome unless addressed immediately . although the main cause of the iliac artery perforation in this case was the fact that the push of the introducer sheath was careless and jerky , one of the keys for successful endovascular repair of aortic aneurysm is adequate vascular access through the femoral and iliac arteries . calcification , diminished diameter , and severe tortuosity of the iliac arteries have been associated with an increased incidence of iliac injury during evar . however , even after careful preoperative assessment , inadvertent iliac rupture can occur and be a source of morbidity and mortality . this case calls attention to the importance of a careful manipulation of wires and catheters during the endovascular procedure . with respect to the management of acute iliac artery rupture , endovascular repair with a stent graft intraoperative invasive arterial blood pressure monitoring , maintenance of stiff wire access , readily available intra - aortic occlusion balloons , and an inventory of iliac stent grafts are the prerequisites for the prompt endovascular management of a ruptured iliac artery during evar . almost all the previous literature reported delayed or late stent graft thrombosis rather than acute or intraoperative thrombosis as in this case . the incidence rates have been reported to be 2.7% to 23.8% , and underlying stenosis or kinks have been pointed out as the main causes . however , the main cause of the acute graft limb thrombosis in this case was thought to be inadequate anticoagulation during the management of the ruptured iliac artery . the goal of the management of the stent graft thrombosis is the restoration of appropriate blood flow to the lower extremities . therefore , the available treatment methods are thrombectomy , catheter thrombolysis , and extra - anatomical bypass , such as axillo - femoral or cross - femoral bypass . according to the eurostar ( european collaborators on stent / graft techniques for aortic aneurysm repair ) registry , as a secondary procedure after evar , 11% of the patients required femoro - femoral bypass grafting . in this case , thrombectomy was performed rather than thrombolysis or extra - anatomical bypass because thrombolysis right after the event of iliac artery perforation was inappropriate , and extra - anatomical bypass should be a second - line treatment option considering that the thrombus was fresh . the limitations of thrombectomy are mainly associated with technical difficulty in the introduction of a catheter into the lumen of the stent graft , particularly in the case of late thrombosis and with the possibility of stent migration or disconnection of graft elements , which can lead to type i or type iii endoleaks . to prevent thrombosis in the stent graft , adequate anticoagulation during and after the procedure is necessary . in summary , we report a rare case of combined iliac artery rupture and graft limb occlusion complicating evar , which were successfully managed without additional open surgery . careful manipulation of catheters and guide wires , as well as maintenance of optimal anticoagulation throughout the procedure , is fundamental but the most important requirement for an endovascular procedure . to decide on the best treatment option among the various surgical and endovascular modalities
for high - risk patients , endovascular aortic aneurysm repair ( evar ) is a good option but may lead to serious complications , which should be addressed immediately . a 75-year - old man with a history of abdominal surgery underwent evar for an aneurysm of the abdominal aorta and iliac arteries . during evar , iliac artery rupture and graft limb occlusion occurred , and they were successfully managed by the additional deployment of an iliac stent graft and balloon thrombectomy , respectively . we , herein , report a rare case of the simultaneous development of the two fatal complications treated by the endovascular technique .
the most common causes of lower gastrointestinal bleeding are known to be vascular disease , intestinal neoplasm , inflammatory bowel disease , ischemic colitis and hemorrhoid [ 1 , 2 ] , and appendiceal bleeding is known to be an extremely rare cause . the reported causative factors of appendiceal bleeding to date are angiodysplasia , appendicitis , diverticulum , endometriosis , intussusception , carcinoid , lymphoma , and crohn 's disease [ 4 - 11 ] . the appendectomy is commonly known to be the most effective treatment for appendiceal bleeding , but the authors successfully performed endoscopic clipping without any complications in patient with acute massive lower gastrointestinal bleeding caused by appendiceal bleeding and report this case with a literature review . a 70-year - old man was admitted to our hospital complaining of hematochezia that had developed 2 days earlier . he had been diagnosed with hypertension 5 years earlier at a private clinic and was taking antihypertensive medications , but had never taken anti - platelets such as aspirin . his blood pressure was 90/60 mmhg , his heart rate was 110 beats / minute , his respiration rate was 24 breaths / minute , and his body temperature was 36.7 when he was admitted to our hospital . the lung sound and the heart sound were within normal limits , but the bowel sound was increased on auscultation . the abdomen was soft , and the liver and the spleen were not palpable on physical examination . the leukocyte count was 12,500/mm , hemoglobin was 10.5 g / dl , and the platelet count was 234,000/mm on the blood test . there were no abnormal findings on the chest x - ray , and there were no specific abnormalities on the abdominal x - ray either . suspecting acute lower gastrointestinal bleeding , we performed emergency endoscopy and sigmoidoscopy . there was no evidence of upper gastrointestinal bleeding on endoscopy , but there was a large amount of fresh blood from the rectum to the sigmoid colon on sigmoidoscopy ( fig . however , the bleeding source was not identified because it was difficult to secure a clear visual field and to advance due to feces . the colonoscopy was done up to the terminal ileum , and active bleeding from the appendix was observed ( fig . endoscopic hemostasis was attempted using a clip ( ez clip ; olympus , tokyo , japan ) , and the procedure was completed after clipping twice and confirming successful hemostasis ( figs . 3 , 4 ) . the computed tomography ( ct ) scan showed no evidence of acute appendicitis or any other specific lesion around the appendix ( fig . the blood test showed no reduced hemoglobin , and his blood pressure and hematochezia were improved . a follow - up colonoscopy was performed after 3 days , evidence of neither further bleeding nor post - procedural appendicitis were found , so the patient was discharged . the prevalence of lower gastrointestinal bleeding is known to be about 0.02% and it is higher in men and elderly populations . the common causative factors are vascular disease , intestinal neoplasm , inflammatory bowel disease , ischemic colitis , and hemorrhoid [ 1 , 2 ] . appendiceal bleeding is known to be a rare cause of lower gastrointestinal bleeding , and it can be caused by angiodysplasia , mucosal erosion associated with acute appendicitis , appendiceal diverticulum , appendiceal endometriosis , intussusception of the appendix , carcinoid , lymphoma , and crohn 's disease [ 4 - 11 ] . generally , submucosal vessels are exposed when appendicitis occurs in these diseases described above , and this can cause bleeding . reported that appendiceal bleeding should be differentiated from crohn 's disease , mucocele , diverticulum , fistula connected to the aorta , endometriosis , angiodysplasia , dilation of submucosal vessels , granuloma , intussusception , ulcer secondary to aspirin , and gastrointestinal stromal tumor . the appendectomy is the most common treatment for appendiceal bleeding , but percutaneous tanscatheter arterial embolization is known to be useful for appendiceal bleeding caused by angiodysplasia , and successful cases have been reported nationally . however , percutaneous transcatheter arterial embolization should be attempted selectively when primary endoscopic hemostasis has failed because it can cause complications such as necrosis of normal mucosa and perforation . the authors successfully performed endoscopic clipping without any complications in a patient with acute appendiceal bleeding after confirming that there was no evidence of acute appendicitis or other specific lesions around appendix on abdominal ct and colonoscopy . this report is unique because endoscopic clipping was attempted initially instead of an appendectomy and the procedure was successfully completed without complication whereas the appendectomy is known to be the most common treatment for appendiceal bleeding based on a review of the literature to date . in other words , there have been a number of case reports regarding the causes of appendiceal bleeding , but none of them reported successful endoscopic clipping to treat appendiceal bleeding . therefore , this report is significant in that it suggests to skillful endoscopy specialists a new therapeutic strategy that enables them to manage appendiceal bleeding swiftly by using endoscopic clipping , which is both simple and safe . the appendiceal bleeding in our patient was thought to be caused by angiodysplasia because the patient did not have any history of aspirin therapy . endoscopic clipping is judged to be a safe and effective therapeutic method before considering surgery when there are no other specific findings except appendiceal bleeding on abdominal ct and colonoscopy . the authors attempted to ligate by locating one wing of the clip towards the inside of the appendix opening and the other wing towards the outside after predicting the direction where the blood might leak , instead of ligating the opening completely , to prevent post - procedural appendicitis ( fig . this ligation method was thought to prevent post - procedural appendicitis , as well as provide effective hemostasis . there is a total lack of evidence regarding endoscopic clipping related to appendiceal bleeding internationally because appendiceal bleeding is a rare disease and because in the literature , surgery is described as the most effective therapeutic method , but much evidence exists in the literature describing endoscopic clipping as an effective therapeutic method for diverticular bleeding [ 14 , 15 ] . therefore , further research regarding its safety and complication associated with the procedure should be conducted in the future .
lower gastrointestinal bleeding is a common disease among elderly patients . the common sources of lower gastrointestinal bleeding include vascular disease , crohn 's disease , neoplasm , inflammatory bowel disease , hemorrhoid , and ischemic colitis . however , bleeding from the appendix has been reported very rarely in patients with lower gastrointestinal tract bleeding . in general , after a colonoscopic diagnosis of appendiceal bleeding , a laparoscopic or surgical appendectomy would be recommended . we report a case of successful colonoscopic treatment of appendiceal bleeding without complications by endoclips . this report suggests that colonoscopic clipping is a safe and effective means to treat bleeding from appendiceal lesions . further study is needed to evaluate procedure - related complications and to confirm the procedure 's safety and efficacy .
geriatricians in canada have long looked to our colleagues in the united kingdom for inspiration and guidance . a recent report by david oliver , catherine foot , and richard humphries on behalf of the king s fund raises many issues regarding current problems with the care for older adults in existing health - care systems and also suggests ways forward . given our long association and shared history ( the debt that we owe includes many of our pioneering clinicians ) , there is reason to read making our health and care systems fit for an ageing population with care . here we hope not to summarize a lengthy and comprehensive review of many aspects of the health - care system , which are either problematic or promising in the care of frail older adults , but rather to highlight a few key issues which may help guide our thinking about the future of geriatric medicine in canada . one key shift in mindset argued for by oliver and colleagues is an increased focus on community - based and intermediate care as alternatives to acute - care hospitalization . hospital - centred care has such a strong culture that sometimes avoiding it can be more effective than trying to reform it . the framework of discharge to assess versus decide to admit presents a useful alternative to our current discharge to assess model , acute health needs are the focus of acute care encounters . patients are then discharged home , as soon as their condition has stabilized , for rapid follow - up of ongoing care and support needs by community - based clinicians . alternatively , clinicians can actively decide to admit patients who require admission for specific medical therapies , rather than having admission be the default path of least resistance . much stands to be gained from designing our systems of care to focus on care in the community and in the least acute setting possible , rather than subjecting older adults to the harms that are well - known in acute care and that typically have defied reform . solid systems of care in communities can also potentially streamline assessment processes and avoid duplication of efforts . some jurisdictions in the uk have even recognized that the assessment and therapy needs of older people living in their own homes do not take weekends off , leading to implementation of seven day care at home . ( as wayne gretzky famously said ) and get out in front of the move to community - based care . the important role remains for clinical specialists working in tandem with primary care systems and clinicians and ( in an ideal world ) an organized form of so - called intermediate care . such facilities aim to offer an environment in which rehabilitation and recuperation can be the primary focus , rather than settling onto the treadmill of yet more investigations which may add less benefit to frail patients then do solid clinical acumen , and a focus on improving function and meeting goals that are truly patient centered . it also means making routine care less hazardous for example , by avoiding the twin evils of over sedation and inadequate pain treatment and promoting early mobilization , proper nutrition , interprofessional collaborative practice , and early involvement of patients and families in setting goals of care . when it was introduced , intermediate care was quite controversial , as it appeared to harken back to the regrettable era in which geriatric medicine was viewed as an undertaking of care for second - rate patients in second - rate facilities by second - rate doctors . in a sharply worded 2001 bmj commentary , raymond tallis and grimley evans argued that in the operationalization of the uk national service framework for older people , the good intentions of clinical experts were sidetracked by the political agenda to keep old people out of hospital by reducing their inappropriate use of hospital beds , an agenda largely driven by cost savings but which had undertones of value judgments on the patient population , as well . in any event , renewed focus on good intentions and the evidence base ( of reducing harmful interventions and environments which thrive in acute care hospitals ) allows the benefits of intermediate care , with its focus on sensible medical care , rehabilitation , and re - enablement , to be realized . the idea of intermediate care might sound pejorative still , but it should be recognized that it can be either a ( from care at home ) or a step down ( from acute care hospital ) . here , less is more : ideally , intermediate care is provided close enough to acute care that clinicians have an easy commute , but far enough away to allow them to avoid the temptation to over - investigate and treat what need not be investigated or treated . for many frail older people , the trap of access to invasive investigations that are too easy and too often - used , again by default , is a deep one . still , in an era in which patients labelled as social admissions have a high risk of death , we need to be alert that words matter , especially when they are meant to designate whole areas of activity ; they re not called brands for no reason . in recent years there has been an increasing focus on efforts to keep frail older adults out of emergency departments . while these efforts may be well intentioned , emergency health systems , including paramedics , ambulance services , and emergency departments , remain an important point of access to care when need is greatest . in consequence , we should seek not to turn people away who need help , but rather to create better pathways of care once they have reached out for assistance . for example , once a 911 call is made , if paramedics are trained and equipped to provide assessment and care on - site , a transfer to hospital may be avoided . even so , this can only work sustainably if care in the home does not end when the ambulance pulls away from the curb . clinical care programs focusing on rapid follow - up of older patients discharged from emergency departments ( e.g. , home visits by specialized clinicians trained in the principles of home - based comprehensive geriatric assessment [ cga ] ) show great promise in canadian settings and should be a focus for implementation and , importantly , evaluation and research . our current models of care for frail older adults have been heavily hospital - based . given that hospitals can do more harm than good for frail older adults , this is not un - problematic . alternatives include : keeping them out of hospital in the first place ( geriatricians will have a pivotal role here , with familiar principles of cga and optimizing function , to which we would add a key role for advance care planning);making care less hazardous in the hospital ( here we see a great role for intermediate care and attention to cultures of care which minimize iatrogenic and environmental harms ) ; andattention to the interface between acute and long - term care this will have both clinical and administrative aspects with an emphasis on home supports that are flexible enough to meet the needs of users . keeping them out of hospital in the first place ( geriatricians will have a pivotal role here , with familiar principles of cga and optimizing function , to which we would add a key role for advance care planning ) ; making care less hazardous in the hospital ( here we see a great role for intermediate care and attention to cultures of care which minimize iatrogenic and environmental harms ) ; and attention to the interface between acute and long - term care this will have both clinical and administrative aspects with an emphasis on home supports that are flexible enough to meet the needs of users . the king s fund report challenges us to identify early priorities for change and quick wins . we also need to focus on what our brand will be , if we are to prosper , and not just compete , as canadian health care comes to grips with population ageing . what would be our quick win in a canadian context ? community - based care , while perhaps not entirely quick , could be that win .
a report from the united kingdom on making health and care systems fit for an ageing population proposes a range of interventions to make care better for older adults , especially those who are frail . here , we discuss the proposed shift for the acute care hospital to other models of care . the key for these models of care requires a fundamental shift to care that addresses the full range of an individual s needs , rather than being based around single diseases . how this might apply in the canadian context is considered . we emphasize strategies to keep people out of hospital but still receive needed care , make acute hospital care less hazardous , and improve the interface between acute and long - term care .
a seventy year - old woman presenting gradual evolution pruritus gets worse recently . at clinical examination , both labia were observed as white plates , dispersed , irregular borders and sanded surface , suggestive of high - grade intraepithelial vulvar neoplasia . she was undergone to a vulvar polyp that revealed a high - grade vulvar intraepithelial neoplasia , so it was decided superficial vulvectomy . the pathological examination revealed vulvar intraepithelial squamous neoplasia of high - grade , without evidence of invasion of the underlying stroma with signs of hpv infection , diffusely involving both lips , which developed in condyloma plan context , coexisting neuroendocrine carcinoma of high - grade small cell with lymphatic tumour embolization . discrete parakeratosis and dense inflammatory infiltrate , predominantly lymphocytic , was observed in both lips . the neuroendocrine lesion is a mass of intermediate size cells with high nucleus / cytoplasm ratio , hyperchromatic nucleus , some pleomorphism , with necrosis and figures of mitoses . the lesion is surrounded by inflammatory infiltrate mononucleate ( figure 1a ) . the immunohistochemistry was positive for ae1/ae3 , ck7 , ema , cam5.2 and berep4 , and negative for ck5 , p63 , ck20 and ttf1 . it is also observed immunostaining for neuroendocrine markers synaptophysin and chromogranin a ( figure 1c and d ) , with a proliferative activity assessed by ki67 about 100% . pathological examination of the surgical specimen revealed squamous intraepithelial lesion of low grade , with no evidence of residual carcinoma or neuroendocrine tumour lymphatic embolization , and solid metastasis in 1 of the 6 right inguinal nodes of neuroendocrine carcinoma small cell without contralateral inguinal lymph node metastasis ( figure 2 ) . neuroendocrine tumours have an aggressive behaviour and are a diagnostic and clinical challenge , due to their rarity and the lack of standardized therapeutic approaches . they are rarely found in gynaecology , however they comprise approximately 2% of all tumours of the gynaecological tract . most of them occur in the cervix , but they can also arise in other sites including vulva , vagina , uterus , and ovary . these tumours are a heterogeneous group of neoplasms that show various histologic findings and biologic behaviours . neuroendocrine small cell carcinoma is included in the group of high - grade neuroendocrine carcinomas . small cell carcinoma , arising in other organs has the same histopathologic characteristics , as the ones originated in the gynaecologic tract . recent genomic analyses of small cell carcinoma of the lung have revealed potential driver genomic alterations . some authors believe that the comprehensive genomic characterization of gynaecologic small cell carcinomas may lead to the identification of markers , that result in an improvement of diagnostic reproducibility of small cell carcinomas of the gynaecologic tract and of molecular aberrations , that may be exploited therapeutically in subgroups of the disease . the uterine cervix is the most frequent place in the female genital tract of this cancer and high - risk human papillomavirus ( hpv ) infection . regardless of the location , small cell carcinoma in the gynaecologic tract displays an aggressive clinical behaviour , with few - reported long - term survivors . this tumour has been observed in women with vulvar intraepithelial neoplasia or squamous cell carcinoma . the small - cell carcinomas of the vagina and vulva need to be distinguished from merkel cell cancers , a neuroendocrine carcinoma of the skin . attention to the histologic features of neuroendocrine differentiation and the immunohistochemical staining of neuroendocrine markers is necessary to reach a correct diagnosis . microscopically , they are composed of small , oval - spindle cells , which are arranged like a sheet , trabecular or nested pattern . rosette - like or acinar formation may be seen . the cells have high nuclear to cytoplasmic ratio , scanty cytoplasm , and hyperchromatic nuclei with inconspicuous nucleoli . small foci of squamous and/or glandular differentiation can be seen but they usually are less than 5 - 10% of the total volume of the tumour . immunohistochemistry : neuroendocrine differentiation can be proved with neuroendocrine markers , such as , chromogranin s , synaptophysin , cd56 , cd57 , neuron specific enolase , protein gene product 9.5 and synaptic vesicle protein 2 . these markers recognize antigens that are expressed independently of the specific hormones secreted by neuroendocrine cells . chromogranin - a , synaptophysin and cd56 are the most commonly used neuroendocrine markers in most practices . reactivity for neuron - specific enolase and cd56 is usually present , although it is known to be a less specific neuroendocrine marker , compared to chromogranin and synaptophysin . the frequent coexistence of neuroendocrine carcinoma and epithelial tumours alltogether with the monoclonality of the two components implies a common cellu lar origin of the neuroendocrine and epithelial components . better survival is associated with fewer genetic aberrations , but tumour - related mortality occurs in approximately one - third of patients . neuroendocrine tumours show highly aggressive clinical behaviour , regardless of the site of origin . despite the potential differences in etiology and risk factors , small cell carcinoma from different sites of the gynaecologic tract , have similar morphologic appearances and clinical behaviour . early stage disease has varied treatment approaches , based on the site of malignancy , but systemic chemotherapy with or without radiation , plays a role in the adjuvant setting to mitigate the risk of recurrence . similar to small - cell cancers arising in other sites , it appears that the regional therapy is not a sufficient treatment , for this tumour .
neuroendocrine tumours are rare in the gynaecologic tract , comprising approximately 2% of all gynaecological tumours . they have an aggressive behaviour and are a diagnostic and clinical challenge , due to their rarity and the lack of standardized therapeutic approaches . there are a few case reports . it is defined as a high - grade carcinoma exhibiting neuroendocrine differentiation . the authors describe the case of a 70-year - old woman , with vulvar neuroendocrine small cell carcinoma after superficial vulvectomy . the patient was submitted to a surgery with wide local excision and adjuvant radiation therapy . a review of the literature on this topic is also presented .
quadricuspid aortic valve ( qav ) , a rare congenital heart defect with an incidence range of 0.008 % to 0.033%,1 , 2 was first reported by balington et al.3 in former years , qav was an accidental finding during surgery or necropsy , but recent improvements in echocardiographic imaging techniques have conferred an easier and earlier detection of qav . herein , we report a case of qav diagnosed via transesophageal echocardiography ( tee ) . a 44-year - old woman presented with a history of dyspnea on exertion ( new york heart association functional class ii ) starting 7 years previously and aggravated in the previous 23 years . her past medical history revealed hypertension and hypothyroidism , without a positive family history of heart diseases . she had been under observation for the past 3 years and undergone two transthoracic echocardiography ( tte ) examinations , in which only moderate to severe aortic insufficiency was detected . because of the deterioration of her dyspnea , she was referred to tehran heart center for tee evaluations . on physical examination , she had a grade ii / vi decrescendo diastolic murmur in the second intercostal space of the right sternal border with no radiation and a wide pulse pressure . blood pressure was 170/70 mmhg with a normal pulse rate , and lung auscultation was normal . chest radiography showed a ct ratio at the upper limit of normal ; and normal sinus rhythm , normal axis deviation , and left ventricular ( lv ) hypertrophy were detected on the electrocardiogram . angiography reported an ejection fraction ( ef ) of 50% , severe aortic insufficiency , mild aortic root dilatation , lv pressure of 150/0 - 15 , aortic pressure of 150/80 , and normal coronary artery . in the treadmill exercise test , she developed fatigue in stage iii of the bruce protocol ( after 6 minutes ) . in tte , there was mild lv and left atrial dilation with normal lv systolic function ( left ventricular end - diastolic diameter = 57 mm , left ventricular end - systolic diameter = 41 mm , left ventricular ef = 55% ) , moderately severe aortic insufficiency with no aortic stenosis , moderate mitral regurgitation , normal right ventricle size and function , mild tricuspid regurgitation , and pulmonary artery pressure = 36 mmhg . tee was performed for further evaluations of the aortic and mitral valves with regard to the severity and mechanism of the insufficiency of the aortic and mitral valves . after precise evaluation , tee confirmed moderate to severe aortic insufficiency ( figure 1 ) and moderate mitral regurgitation and also revealed qav ( figure 2 ) and the prolapse of both mitral leaflets as the mechanisms of the valvular regurgitation , respectively . apart from a small patent foramen ovale , no dilation of the sinus of valsalva or ascending aorta was visualized . a rare congenital heart defect , qav has an incidence range of 0.008 % to 0.033%.1 , 2 it was first reported by balington et al . in 1826.3 qav used to be an accidental finding during aortography,4 surgery , or autopsy ; currently however , tte and tee are the methods of choice in the detection of qav . it is deserving of note that similar to our case , tte is reported to only detect aortic regurgitation and not qav in some patients.5 according to the leaflet morphology , 7 types of qav are described by hurwitz and roberts , named from a to g.2 because of 1 large cusp , 2 intermediate - sized cusps , and 1 small cusp , our patient was set in type d. in this case , the placement of the left coronary artery was normal . holm et al . reported a case with a large left main coronary artery originating unusually low in the aortic root near the posterior margin of the left cusp , which produced a 4-leaf clover appearance.6 and kaminishi et al . reported a widely patent left coronary ostium in their case.7 qav is a single malformation which is rarely associated with other congenital defects , including paroxystical supraventricular tachycardia , right double kidney with double renal pelvis and double proximal ureter , hypertrophic obstructive and non - obstructive cardiomyopathy , patent duct , atrial septal defect , ventricular septal defect , pulmonary valve stenosis , bicuspid pulmonary valve , and malformation of the mitral valve.812 associated mitral valve anomalies which are reported include severe mitral regurgitation , prolapsed mitral valve causing a mild regurgitation , mitral regurgitation resulting from annulus dilatation and thickening of the anterior leaflet , severe mitral regurgitation due to infective endocarditis , ruptured mitral valve aneurysm , and hypoplastic anterior mitral leaflet.1216 in the case presented herein , there was a concomitant patent foramen ovale and eccentric moderate mitral regurgitation due to prolapsed mitral valve leaflets . aortic stenosis is rare , and most dominant abnormalities due to qav are different degrees of aortic insufficiency because of malcoaptation of the leaflets.11 , 17 similarly , aortic valve leaflets could not meet each other centrally in our patient . qav is an uncommon congenital anomaly which was accidentally found in surgery or autopsy in former years . nowadays , in tandem with advances in echocardiographic imaging , tee helps us to identify qav earlier and to manage it more appropriately .
quadricuspid aortic valve ( qav ) is an uncommon congenital anomaly which was an incidental finding during surgery or autopsy in the past . we present the case of a 44-year - old woman with moderately severe aortic regurgitation due to unequal cusp size qav diagnosed via transesophageal echocardiography . due to echocardiographic imaging improvement , the diagnosis of qav is now easier and earlier than the past .
( a ) centromeres ( white boxes ) nucleate a specialized set of proteins called the kinetochore ( blue ) , including cenp - a and cenp - c ( green ) , which in turn interact with the spindle microtubules ( red lines ) . as centromere sequences diverge , either cenp - a or cenp - c has evolved adaptively ( darker green ) in different lineages . ( b ) changes in the sequence or organization of centromere dna ( yellow boxes ) may create stronger centromeres ( indicated , for convenience , by a greater number of microtubule interactions ) . selective segregation of stronger centromeres into egg cells could , theoretically , lead to meiotic drive ( see text for details ) . ( c ) meiotic drive in female meioses could generate imbalances in subsequent male meioses . such imbalances might in turn be neutralized by adaptive evolution ( circular arrows ) of kinetochore proteins such as cenp - a or cenp - c .
centromere function is remarkably conserved between species , yet the satellite sequences that make up centromeric dna are highly divergent . proteins that bind these sequences appear to be evolving under positive selection , supporting a model wherein the interplay between centromeric repeats and the proteins that bind them creates an opportunity for an intriguing phenomenon known as centromere - based meiotic drive .
one of the few well - understood features of intensive care unit ( icu ) delirium is its association with poor patient outcome . risk factor stratification is essential to the understanding , prevention and treatment of any disorder , and is a cornerstone of scientific endeavor in clinical research . the largest study on icu delirium risk factors to date is published in the previous issue of critical care . delirium in the critical care setting is said to occur in 22% to over 80% of patients . such broad variations in delirium incidence may be partly attributable to differences in predisposing risk factors , which may differ between icus . co - morbidities present prior to icu admission have particularly seldom been considered in icu delirium studies ; several of them are discussed in the paper by van rompaey and colleagues . physicians hold opinions on the risks and benefits of environmental factors ( for example , physical restraints ) affecting the patient once in the icu ; however , little is known about these factors . human interaction , such as the effect visitors may have on delirium , remains unexplored in the critically ill patient . these environmental features , discussed in van rompaey and colleagues ' paper , are important not only because they are inexpensive but also perhaps because they less likely to harm patients than poorly studied pharmacological interventions . the patients described in the study were no longer intubated and thus were perhaps less ill ; in addition , not all centers collected all risk factor data . van rompaey and colleagues nevertheless provide confirmation that several previously identified risk factors remain significant in this multicenter study , challenge other risk factors , and add several risk factors not previously described . alcohol use is not screened for routinely , validated alcohol withdrawal scales remain under - used , and intervention for withdrawal is seldom incorporated into treatment plans . this knowledge is all the more surprising because the relevant studies identify alcohol abuse as a significant risk factor for developing delirium . whether alternatives to routine care ( that is , benzodiazepines titrated to symptoms , rather than antipsychotics ) in these high - risk patients are of any benefit is unknown . age has been considered a risk factor for delirium in non - icu populations and by some icu investigators . when van rompaey and colleagues consider pre - existing cognitive dysfunction , tobacco use and alcohol , age does not appear to confer an additional risk for delirium . if dementia is a risk factor but age is not and studies considering pre - icu admission co - morbidities would indicate this is true prevention or prophylaxis of this common and morbid disorder in the icu should consider different interventions than , say , similar initiatives on the wards . several previously described predisposing factors ( psychoactive drugs , sedatives and opiates ) are revisited and their relative contribution to the risk of icu delirium is tempered by the case mix , and the addition of other variables . the most novel elements in this study , however , are the environmental risks for icu delirium . visible daylight , visitors and not being physically restrained seem to result in a lower incidence of delirium . it is refreshing to know that in the high - tech environment of icus , someone still asks the question ' which inexpensive and nonmorbid approach is useful ? ' combinations of pharmacotherapy and nonpharmacologic interventions such as psychotherapy are used in a broad range of psychiatric disorders . in some situations , mental health is purportedly based on the ' functioning of a high - order nervous system in constant and complex relation with the personal and social environment ' . several of the points raised by the authors ( such as the benefit of company ) offer simple venues for nonpharmacologic intervention . the rise in odd ratios for delirium in physically restrained patients yet make sense when one considers how fearful delirious patients are . other reports have recently corroborated that nonpharmacologic interventions may impact on delirium ; early physiotherapy and ambulation appears to be associated with a decrease in delirium days in a recently conducted randomized , controlled study . all recommended or clinically used antipsychotic drugs have been validated for the treatment of schizophrenia in young populations over short periods of time . the potential for harm with these drugs is all the more worrisome given that their effectiveness and side effect profiles in older or critically ill populations are largely unknown . there is an urgent need for carefully conducted studies differentiating cognitively intact patients from those with subsyndromal delirium or frank delirium , which will integrate stratification based on risk factors such as those described by the authors . only then can we move forward with the disorder , which arguably causes patients , their families and their caregivers the most distress during critical illness .
icu delirium is associated with poor patient outcome . risk factor stratification is essential to the understanding , prevention and treatment of this disorder . alcohol consumption , smoking and prior cognitive impairment appear strongly correlated with delirium risk . several potentially modifiable associations deserve prospective study : these include administration of sedatives and opiates ; multiple catheters ; as well as minimizing physical restraints and enabling visitors .
the oral mucosa is constantly subjected to external and internal stimuli and therefore manifests a spectrum of diseases that range from developmental , reactive and inflammatory to neoplastic . these lesions represent a reaction to some kind of irritation or low - grade injury like chewing , trapped food , calculus , fractured teeth and iatrogenic factors , including overextended flanges of dentures and overhanging dental restorations . diagnosis of each lesion from the groups is aided by their clinical and radiographic features , but histopathology is the key for final diagnosis . the most common is chronic inflammatory gingival enlargement , when the gingiva presents clinically as soft and discolored . this is caused by tissue edema and infective cellular infiltration , caused by prolonged exposure to bacterial plaque , and is treated with conventional periodontal treatment , such as scaling and root planing . situations in which the chronic inflammatory gingival enlargements include significant fibrotic components that do not respond to and undergo shrinkage when exposed to scaling and root planing are treated with surgical removal of the excess tissue . risk factors include poor oral hygiene as well as physical irritation of the gingiva by improper restorative and orthodontic appliances . a female patient aged 38 years reported with a chief complaint of swelling in the left front region of the gums . the lesion was nodular , circumscribed polypoid lesion measuring about 1.5 cm 1.2 cm , pinkish to reddish in color , which bled easily , and was painless . this lesion involved the marginal and interdental gingiva on the facial surface of the maxillary left canine and 1 premolar . pre - operative photograph oral hygiene instructions were given and scaling and polishing were done on the first visit . then , the patient was recalled for surgical excision of the lesion . after excision , residual calculus was removed and root planing was performed [ figures 2 and 3 ] . the patient was motivated to maintain oral hygiene and was asked to rinse her mouth with 0.2% chlorehexidene mouthwash twice daily for 1 week . post - operative photograph microscopic examination revealed hyper - parakeratinized stratified squamous epithelium with ulceration and acanthosis of the stratum spinosum . the underlying dense fibrous connective tissue stroma showed severe chronic inflammatory cell infiltrate consisting of lymphocytes and plasma cells and a moderate number of endothelial - lined blood vessels suggestive of chronic inflammatory fibrous hyperplasia [ figure 4 ] . photomicrograph of the specimen figures 5 and 6 show the post - operative photograph of the same lesion after 1 and 3 weeks , respectively . reactive hyperplasia comprises a group of fibrous connective tissue lesions that commonly occur in the oral mucosa as a result of injury or chronic irritation . chronic trauma can induce inflammation , which produces granulation tissue with endothelial cells and chronic inflammatory cells and , later , fibroblasts proliferate and manifest as an overgrowth called reactive hyperplasia . these tumor - like lesions are not neoplastic but indicate a chronic process in which an exaggerated repair occurs ( granulation tissue and formation of scar ) following repair . inflammatory fibrous hyperplasia or fibrous hyperplasia is a benign soft tissue response to a local irritant . it can be due to calculus , a sharp tooth , a broken filling , excessive plaque and other irritating factors . the color ranges from normal to white or reddish depending upon whether or not the surface is ulcerated , keratotic or both or neither . it can be soft or firm in palpation histologically , inflammatory fibrous hyperplasia is made up of a mass of hyperplastic connective tissue with dilated blood vessels , usually with chronic inflammatory cells such as lymphocytes and plasma cells , but it can also be made up of solid connective tissue with minimum to no inflammatory cells , the latter called fibrous hyperplasia . the surface epithelium ranges from normal to acanthotic , ulcerated , keratotic or a combination of two or more of these features . surgical excision is the preferred treatment of choice , with removal of local irritants to prevent recurrence . for hyperplastic lesions , follow - up of the patient is needed as it exhibits a tendency to recur .
gingival enlargement , the currently accepted terminology for an increase in the size of the gingiva , is a common feature of gingival disease . local and systemic factors influence the gingival conditions of the patient . these factors results in a spectrum of diseases that can be developmental , reactive and inflammatory to neoplastic . in this article , the history , etiology , clinical and histopathological features , treatment strategies and preventive protocol of inflammatory hyperplasia are discussed .
genital self mutilation is a rare and a severe form of self - injurious behavior . it is usually described in psychotic disorders , mostly in schizophrenia as a result of delusions and hallucinations.[13 ] in most cases , penile mutilation is common . risk factors of genital self mutilation include commanding hallucination , religious delusions , substance abuse , and social isolation . genital mutilation has been ascribed to sexual conflicts and offences , body image preoccupation and distortion , expression of internalized aggression and suicidal intent , and a means to get relieved of urinary symptoms . this phenomenon has been described in schizophrenia , affective psychosis , alcohol intoxication , and personality disorders . a review of literature suggests that genital self mutilation is usually associated with psychotic illness . greilsheimer and groves in a group of 52 cases of genital self mutilation found 87% to be psychotic and 13% to be non - psychotic . the non - psychotic cases included personality disorders , transvestism , and complex religious or cultural beliefs . a series of 14 patients of self - inflicted genital injuries found 65% to be psychotic and 35% to be non - psychotic . there have been sporadic cases of non - psychotic genital self mutilation in the literature . various forms of psychopathology have been postulated in such cases . in the present case , genital self mutilation of scrotum in a 28-year - old male , a case of delirium , due to withdrawal of alcohol , a 28-year - old male working as a tractor driver was brought to the emergency surgical ward by his wife with complaints of cutting off at the left side at the base of his scrotum with a kitchen knife . he started with 200 ml of arrack and slowly increased the amount to 1l over a period of 6 years as he was not getting the desired effect he used to get with 200 ml . on occasions , when he did not have the money or opportunity to drink , he used to experience dysphoric state including severe anxiety , palpitations sweating , restlessness , and tremors of hands . he also did not have any control over the amount of alcohol he is taking and the money he is spending on it once he started to drink . he also neglected his duties both at work and at home due to this habit , for which he had frequent quarrels with his wife and has also ended up in financial crisis with a big debt . due to above mentioned problems two days before admission , patient decided to stop drinking alcohol and stopped all of a sudden and he has developed abnormal behavior like talking to self , not feeding self , not recognizing family members , and talking irrelevantly . he started to complain that he is hearing voices of male and female when no one around . on the morning of the day of admission , patient went to the kitchen and cut his scrotum at the base with a knife on left side . his wife after one hour saw blood stains on floor and on his groins and brought him to casualty . the mental status examination of the patient showed that the patient is not co - operative , not oriented to time , place , and person , hence cognitive functions could not done . he was diagnosed as mental and behavioral disorders due to alcohol withdrawal state , complicated with delirium . the patient was admitted in psychiatry ward , all the investigations done like complete blood picture , liver function tests , blood urea , serum electrolytes , and computed tomography ( ct ) scan brain to rule out any organic cause . they started on tablet chlordiazepoxide 200 mg in divided doses , tablet thiamine 150 mg both given orally in divided doses to prevent korsakoff 's psychosis and injection lorazepam 2 mg i m on sos basis to prevent any agitation . the patient became oriented on the 4 day of admission and then stared to complain of pain at injury site for which he was started on non steroidal anti inflammatory drugs . there were no adverse effects like infection of the injury or cutting of the cord or vas deferens and the wound completely healed in a period of 3 weeks . for next ten days , we slowly tapered off the chlordiazepoxide and on the 10 day of admission , patient was discharged on tablet disulfiram . the patient is on regular follow - up now for last 8 months and completely abstinent from alcohol . some of the unusual features of the present case are as follows : commonly , amputation of penis and scrotum has been reported but here penis was spared.severe self injuries have been reported mostly in schizophrenia and other psychotic episodes but not in delirium.this case has no apparent sexual or religious connotation.because of his problems at work and home , there may be a sub - clinical depressive episode in the patient and the act was done as a suicidal attempt . commonly , amputation of penis and scrotum has been reported but here penis was spared . severe self injuries have been reported mostly in schizophrenia and other psychotic episodes but not in delirium . this case has no apparent sexual or religious connotation . because of his problems at work and home , there may be a sub - clinical depressive episode in the patient and the act was done as a suicidal attempt . psychoanalytically , self - injurious behavior has been linked to castration and explained as a process of failure to resolve oedipal complex , repressed impulses , self punishment , focal suicide , and aggression turned inward model , especially in depression cases . liebowitz and klein have postulated interpersonal loss preceding self - injurious behavior and linked it to rejection sensitivity . biologically , serotonergic depletion preceding genital self mutilation has been linked to lack of impulse control and depression . schweitzer and bhargava et al . claimed a strong moral and delusional component . in the present case , this was evident in the present case where he inflicted such a serious injury without seeking help . as has been communicated by rao and begam , we are in agreement that genital self mutilation , like any self - injurious behavior is not a single clinical entity and can occur in any psychiatric condition with corresponding psychopathology . it is suggested that there is no difference in the severity of self - inflicted injury between psychotic and non - psychotic group of patients , and sometimes it could be a rational suicidal act . thus , in this case , genital self mutilation was a psychotic solution to a conflict on the individual plane . in summary , genital self mutilation may be a pathway out of diverse psychological disorders or behavior and may be influenced by cultural factors .
genital self mutilation is a rare and a severe form of self - injurious behavior usually described in psychotic disorders , with delusions and hallucinations . it has been ascribed to sexual conflicts , body image distortions , internalized aggression , and suicidal intent . this phenomenon has been described in schizophrenia , affective psychosis , alcohol intoxication , and personality disorders . the present case genital self mutilation in a case of alcohol withdrawal state complicated by delirium is reported .
malaria is a major public health problem with 300500 million new infections each year and an estimated 584,000 deaths from malaria were reported worldwide , with most ( 90% ) of the deaths occurring in africa . the decline of susceptibility of plasmodium falciparum to chloroquine and sulfadoxine - pyrimethamine in many high - transmission areas resulted in the change of drug use policy in africa . in democratic republic of congo ( drc ) , current recommendation therapy for severe malaria in drc is artemisinin - based combination therapy ( act ) ; mainly , artesunate - amodiaquine has been recommended formally as a first - line antimalarial regimen since 2005 . however , this policy is not controlled and the use of antimalarials without a prescription can have adverse effects on chemosensitivity in kinshasa , the drc . the aim of the present study was to assess the self - medication against malaria infection in population of congolese students living in kinshasa , drc . this cross - sectional study was carried out in university of kinshasa , kinshasa , drc . medical records of all students with malaria admitted to centre de sant universitaire ( csu ) of university of kinshasa from january 1 , 2008 , to april 30 , 2008 , were reviewed retrospectively . csu is a primary level centre located in the city of university of kinshasa , kinshasa , drc , and serving an estimated 30,000 students . students who experienced an episode of malaria and presented the following criteria , axillary temperature > 38.0c with positive plasmodium falciparum parasites in thick blood smears and absence of febrile conditions caused by diseases other than malaria , were included in this study . students who took an antimalarial malaria parasitaemia was confirmed with thick blood smears stained giemsa and examined for trophozoites of plasmodium falciparum . the following information was collected and analyzed : ( 1 ) age and gender and ( 2 ) nature , dosage , and duration of antimalarial treatment . statistical analysis was performed using the statistics software spss for windows ( 15.0 spss , chicago ) . data are represented as means sd when the distribution was normal and median with range when the distribution was not normal . frequencies of different parameters of self - medication are expressed as proportions ( % ) . during the study period , 458 students had been admitted in our centre and 133 ( 29.0% ) had a diagnostic of malaria infection . the median age of the patients was 25.4 years ( range : from 18 to 36 years ) . self - medication was associated with the ingestion of artemisinin - based combination treatments ( acts ) in 14 cases ( 50% ) , sulfadoxine / pyrimethamine ( sp ) in 9 cases ( 32.1% ) , and quinine in 5 cases ( 19.9% ) . to our knowledge , the present study is the first attempt to examine the notion of self - medication with antimalarials drugs in a university of central africa . in kinshasa , malaria is highly endemic , stable with a perennial transmission . university of kinshasa is located in one of four districts of kinshasa , district of mont amba in the west region of this urban area . prevalence of anopheles gambiense was higher than that in other districts of the town [ 69 ] . in this report this high figure shows the existence of a gap in the health care system including the freedom to buy drugs without prescription in pharmacies . recent study in drc reported parasite genotyping which showed high frequencies of dihydrofolate reductase ( dhfr ) and dihydropteroate synthase ( dhps ) molecular sp - resistance markers , with 57% of the samples showing more than three mutations linked to sp resistance . self - medication with quinine was found in 20% of students . in national health care policy this rate is high for a molecule reserved for severe disease in congolese health care policy in management of malaria . a recent study supports our concerns and shows the appearance of an intermediate level of resistant forms to amino alcohols in the city of kinshasa compared with other areas of africa . in the drc however , with an important self - medication , surveillance of efficacy of these drugs becomes a necessity since here there are recent concerns that the efficacy of such therapies has declined on the thai - cambodian border . in our series , 50% of students used acts in self - medication . in comparison with our general population , fever treatment with acts another crucial health problem is counterfeit antimalarials , mainly act and sulfadoxine - pyrimethamine in developing countries as democratic republic of congo . this situation increases the risk of artemisinin and sp resistance developed by the use of subtherapeutic dosage of these drugs . self - medication remains an important public health problem in high school students of kinshasa . the recommendations of the national program against malaria are not sufficiently known in these communities . this should draw the attention of all partners involved in the fight against malaria in drc . the academic community should be a priority target for the information campaign about the disease and the dangers of self - medication and risk - taking counterfeit drugs . training and information actions must be reinforced for a better care of malaria and to preserve efficacy and safety of acts and quinine in drc .
aim . to assess the self - medication against malaria infection in population of congolese students in kinshasa , democratic republic of congo ( drc ) . methods . a cross - sectional study was carried out in university of kinshasa , kinshasa , democratic republic of congo . medical records of all students with malaria admitted to centre de sant universitaire of university of kinshasa from january 1 , 2008 , to april 30 , 2008 , were reviewed retrospectively . results . the median age of the patients was 25.4 years ( range : from 18 to 36 years ) . the majority of them were male ( 67.9% ) . artemisinin - based combination treatments ( acts ) was the most used self - prescribed antimalarial drugs . however , self - medication was associated with the ingestion of quinine in 19.9% of cases . no case of ingestion of artesunate / artemether in monotherapy was found . all the medicines taken were registered in drc . in this series , self - prescribed antimalarial was very irrational in terms of dose and duration of treatment . conclusion . this paper highlights self - medication by a group who should be aware of malaria treatment protocols . the level of self - prescribing quinine is relatively high among students and is disturbing for a molecule reserved for severe disease in congolese health care policy in management of malaria .
salmonellosis is a common foodborne disease caused by salmonella spp . and is of global significance . it is a significant pathogen of food producing animals and remains a primary source of salmonellosis 1 . salmonella spp . has been frequently reported in environmental samples since they find their way to the environment through the excreta of humans and animals . in 2004 , the european union ( eu ) alone recorded 192,703 human cases of salmonellosis 2 . the majority of seafood associated illness is due to the consumption of shellfish harvested from sewage polluted waters . it is well known that fish / shellfish normally do not harbour microorganism like salmonella but acquire it from contaminated water from which they have been harvested 3 . the work of koonse et al . ( 2005 ) 4 showed s. weltevreden to be the most frequent serovar at 21 % prevalence of the total serovars reported from aquaculture shrimp farms in three different countries . ( 2008 ) 5 who confirmed s. weltevreden as the most important serovar among the 64 different serovars isolated from seafood . it has been the predominant serovar in seafood in the asian region reported in several studies 6 - 8 . in this report the availability of draft genome sequence of s. weltevreden ( sw9 ) isolated from seafood in india has been announced . s. weltevreden ( sw9 ) was isolated from seafood ( fish ) obtained from the fish landing centre in mangalore , located in the southwest coast of india . culture based technique for the isolation and phenotypic identification of the isolate using a battery of biochemical tests was performed as per fda bacteriological analytical manual . the isolate was resistant to 6 antimicrobials including tetracycline , chloramphenicol , nalidixic acid , ampicillin , co - trimoxazole and erythromycin . genomic dna was extracted from s. weltevreden ( sw9 ) using a qiaamp dna minikit ( qiagen , germany ) . the raw sequence data was generated after library preparation on the ion torrent pgm platform and assembled using clc genomics workbench version 6 . structural gene prediction and functional annotation was performed using the rapid annotations subsystems technology ( rast ) server ( http://rast.nmpdr.org/ ) as it was shown to work exceedingly well in our previous studies 9 . a total of 2 , 64,365 reads with a mean read length of 150.2 bp for 200 bp fragmentation chemistry obtained from the ion pgm was assembled into 716 contigs . the draft genome had a length of 3,825,753 bp , with 6239 coding sequences and a gc content of 51.8% . the annotated genome had 109 genes responsible for resistance to antibiotics and toxic compounds , including 17 genes for multidrug resistance efflux pumps and 18 genes for mdtabcd multidrug resistance cluster and 8 fluoroquinolone resistant genes . the multidrug resistant isolate ( sw9 ) harbored the resistance genes to antibiotics like tetracycline , chloramphenicol , co - trimoxazole , florfenicol and presented point mutation in the quinolone resistance determining region ( qrdr ) responsible for resistance to nalidixic acid . the isolate was found to carry resistant genes such as tetb , cata1 for tetracycline and chloramphenicol respectively 10 . this whole - genome shotgun project has been deposited at ddbj / embl / genbank under the accession number jpio00000000.1 .
salmonella enterica subsp . enterica serovar weltevereden is the most frequent serovar isolated from asia . here , we report a draft genome sequence of multidrug resistant salmonella weltevreden 9 isolated from seafood . whole - genome of this isolate and annotation will help enhance the understanding of this pathogenic multidrug - resistant serovar .
spindle epithelial tumor with thymus - like differentiation ( settle ) is a malignant tumor of the thyroid gland , which shows thymic or related branchial pouch differentiation . this tumor is believed to be derived from the third or fourth branchial pouch and thymic remnants . settle is regarded as a low - grade malignant neoplasm because of its slow - growing nature and protracted clinical behavior . to the best of our knowledge , we present the cytologic , histologic , and immunohistochemical findings and the review of literature including its differential diagnosis . a 22-year - old woman complained of a bulging neck mass at the primary clinic . she was diagnosed with papillary carcinoma on fine - needle aspiration at a local pathology laboratory . the ultrasonography demonstrated a well - defined hypoechoic mass , measuring 3.9 3.4 cm in the left lobe of the thyroid . the frozen section slide showed mostly papillary epithelial configuration intermixed with focal spindle cell component . the touch preparation slides showed tight clusters of spindle or ovoid tumor cells with papillary configuration . on the histologic examination , the tumor was a highly cellular biphasic tumor characterized by fasciculated spindle cells with streaming pattern and tubulopapillary epithelial structures . the epithelial cells of the tubulopapillary structures showed abundant cytoplasm and round to ovoid nuclei . the tumor cells were positive for cytokeratin , vimentin , c - kit , epithelial membrane antigen ( ema ) , and thyroid transcription factor-1 ( ttf-1 ) . however , the tumor cells were negative for thyroglobulin , calcitonin , cd99 , s-100 protein , cd34 , smooth muscle actin , hbme-1 , and galectin-3 . the spindle cells showed elongated and cigar - shaped nuclei with fine chromatin and inconspicuous nucleoli [ figure 1 ] . no intranuclear cytoplasmic inclusions or nuclear grooves were seen . after the operation , the patient is doing well without any evidence of recurrence or metastasis for 12 months . ( a and b ) the smears were highly cellular and showed a biphasic pattern composed of dense groups of spindle cells and intermixed epithelial clusters . the spindle cells revealed scanty cytoplasm and uniform , elongated , or cigar - shaped nuclei . ( c ) the epithelial cells showed abundant cytoplasm and variable sized oval nuclei with indistinct nucleoli . ( d ) the tumor showed a biphasic histologic pattern composed of a spindle cell component and a tubulopapillary epithelial cell component . ( a : 100 , b : 200 , c : 400 , d : h and e , 100 ) we experienced a touch preparation cytology of settle during the frozen section diagnosis and described the characteristic cytologic features . we searched reports including the cytologic findings of settle in pubmed and found only eight cases in the english literature . most reports have described the cytologic findings of settle as highly cellular smears composed of spindle cells and/or epithelial cells . the previously reported cases , along with their characteristic cytologic findings , are summarized in table 1 . the cytologic findings of settle described in the literature recently , recondo et al . reported a case of settle with a comprehensive review of the literature . grossly , settle usually presents as an encapsulated or partially circumscribed mass with grayish to tan cut surface . microscopically , settle shows a highly cellular biphasic pattern and is composed of cellular sheets , short fascicles , interlacing bundles or attenuated storiform arrangement of spindle cells , and glandular epithelial component . the nuclei of spindle cells are oval to elongated and characterized by distinct nuclear membrane , inconspicuous nucleoli , and evenly distributed chromatin . the glandular tumor cells show narrow tubular , tubulopapillary , trabecular , or pseudopapillary structures . immunohistochemically , the tumor cells show immunoreactivity for pan - cytokeratin , smooth muscle actin , c - kit and vimentin , and no immunoreactivity for thyroglobulin , calcitonin , s-100 protein , chromogranin , synaptophysin , cd34 , cd99 , and ttf-1 . because settle is a rare tumor and not often considered , only eight reports have described the cytologic features of settle in fine - needle aspiration cytology samples of settle . the cytology shows a highly cellular smear and a biphasic pattern composed of spindle cells and intermixed epithelial cells . as summarized in table 1 , the main differential diagnoses in cytologic smears of settle are a spindle cell variant of medullary carcinoma ( mc ) and synovial sarcoma ( ss ) . the smear of mc shows a mixed population of spindle , plasmacytoid , and epithelioid neoplastic cells . the neoplastic cells of mc have an abundant or moderate amount of fine granular cytoplasm . the nuclei show the neuroendocrine features with eccentric location in both epithelioid and spindle tumor cells . however , the cytologic features of ss reveal severe cytological atypia , many mitotic figures , apoptotic bodies , and necrosis . we described a case of settle of the thyroid gland with touch preparation cytologic findings . although the incidence is very rare , setlle should be included in the differential diagnosis when a spindle cell neoplasm is encountered in touch preparation cytology in young patients with a thyroid mass . the touch preparation cytology during the frozen section diagnosis may be helpful to confirm the diagnosis of thyroid cancer .
we experienced a case of spindle epithelial tumor with thymus - like differentiation ( settle ) with touch preparation cytology performed during the intraoperative frozen section diagnosis in a 22-year - old woman . the tumor was partially encapsulated by fibrous capsule . it was a highly cellular biphasic tumor characterized by fasciculated spindle cells with streaming pattern and tubulopapillary epithelial component . the tumor cells were positive for cytokeratin , vimentin , c - kit , epithelial membrane antigen ( ema ) , and thyroid transcription factor-1 ( ttf-1 ) . however , the tumor cells were negative for thyroglobulin , calcitonin , cd99 , s-100 protein , cd34 , smooth muscle actin , hbme-1 , and galectin-3 . the reviewed touch smears showed tight clusters with high cellularity . most cellular clusters showed papillary configuration . however , some clusters showed spindle cells with streaming pattern . the spindle tumor cells showed elongated and cigar - shaped nuclei . although the incidence is very rare , setlle should be included in the differential diagnosis when a spindle cell neoplasm is encountered in touch preparation cytology in young patients with a thyroid mass .
the reported incidence of vcd following abdominal hysterectomy is less than one percent . with the increased use of laparoscopic , including robotic - assisted approaches the rate of this complication is relatively higher after robotic - assisted hysterectomy reaching up to 4.1% . predisposing factors for vcd include poor wound healing , excessive pressure at the vaginal vault e.g. sexual intercourse [ 3 , 4 ] , and vaginal instrumentation . the use of adjuvant vaginal cuff brachytherapy for patients with endometrial cancer is effective in reducing vaginal vault recurrence , and there has been increased utilization of this adjuvant treatment in the united states . we report a case of vaginal cuff dehiscence after vaginal cuff brachytherapy with a review of relevant literature . although vcd is still very rare after vaginal cuff brachytherapy , the patients should be counseled about this rare treatment - related complication . in april 2012 , a 62 year old caucasian postmenopausal female elected to undergo robotic - assisted laparoscopic hysterectomy with bilateral salpingo - oophorectomy for her complex endometrial hyperplasia without atypia . final pathology revealed figo 2009 stage ib endometrioid adenocarcinoma , figo grade 1 with invasion of 6 mm of the 10 mm thick myometrium . after discussion of her case in gynecologic oncology multidisciplinary conference , she was recommended to receive adjuvant vaginal cuff brachytherapy . at consultation , she denied pain , vaginal bleeding or vaginal discharge . she presented for ct simulation for vaginal brachytherapy treatment planning approximately 9 weeks after her hysterectomy . during simulation , 1 ) . while there was no vaginal bleeding or discharge with the insertion of the vaginal cylinder , there was a small amount of blood on the vaginal cylinder upon removal . on speculum examination , there was evidence of complete vaginal cuff dehiscence with healthy appearing small bowel noted at the vaginal cuff opening without other abnormalities appreciated . on the same day , the patient underwent surgical closure of vaginal cuff via trans - vaginal approach using four sutures with # 0 vicryl in figure - of - eight fashion . reported rates for vcd after trans - abdominal hysterectomy have been reported as low as 0.03% , and are generally less than 1% [ 1 , 8 ] . however , with the increased use of laparoscopic , including robotic - assisted approaches due to a variety of benefits intra - operatively and post - operatively , there has been an increase in the rate of vcd . rates of vcd with robotic hysterectomy have been reported as 0.98% from the university of pennsylvania experience , and 4.1% from a retrospective review from mayo clinic in scottsdale , az . there are a variety of theories on the reason for this increase ; including the use of electrocautery for colpotomy , the suturing technique used , and the magnification view causing suture bites to be smaller than desired . proposed risk factors for vcd include poor surgical technique , smoking , use of valsalva maneuver , post - operative infection , hematoma , adjuvant radiation therapy , steroid use , connective tissue disease , vaginal trauma / rape , use of vaginal dilator , diabetes mellitus , chronic constipation [ 2 , 1012 ] . while the precipitating event may be known , as in the case of early resumption of sexual intercourse , often it is unknown . vaginal cuff dehiscence in post - menopausal patients has been associated with chronic pelvic prolapse or having a short , atrophic vagina . in pre - menopausal patients , the timing of this rare complication is very important . while most of it occurs in the first three months after hysterectomy [ 13 , 14 ] , some patients develop vcd more than four months after hysterectomy . there is a recent case report of two patients that experienced vcd after intracavitary vaginal hdr brachytherapy treatment . our patient is a post - menopausal , former smoker that underwent robotic - assisted laparoscopic hysterectomy approximately nine weeks prior to vaginal brachytherapy . she was noted to have a small amount of blood on the vaginal cylinder after ct simulation , and the cylinder was noted to project high on her images . there is debate in the literature concerning the best management of vcd , but early corrective intervention is necessary . the radiation oncologist should be aware of the very low risk of vcd , and take steps to aide in early detection and prevention of this rare complication . this very rare complication can be prevented by initiating vaginal brachytherapy no sooner than six weeks after hysterectomy to allow adequate healing time . however , delaying the start of adjuvant radiation treatment more than 9 weeks after hysterectomy may be associated with increased risk of tumor recurrence with worse survival endpoints in patients with uterine cancer . at the time of the brachytherapy simulation , careful speculum and manual examination of the vaginal cuff for findings of vcd the location of the vaginal cylinder should be radiologically verified with each application to rule out abnormal positioning of the cylinder . vaginal cuff deshiscence should be ruled out if the vaginal cylinder projects deep into the pelvis . to reduce the risk of dehiscence during insertion of the vaginal cylinder , it is helpful to avoid excessive pressure on the vaginal cuff . after the procedure , one should inspect the vaginal cylinder for any abnormal blood or clear vaginal fluid that could suggest dehiscence . vaginal cuff dehiscence is an unusual complication of hysterectomy . in preparation for vaginal cuff brachytherapy , the radiation oncologist should be aware of this very rare and under reported complication of vaginal brachytherapy . most of the time , vcd can be prevented by initiating vaginal brachytherapy no sooner than six weeks after hysterectomy . however , delaying the start of vaginal cuff brachytherapy more than 9 weeks after hysterectomy may be associated with increased risk of tumor recurrence in patients with uterine cancer .
vaginal cuff dehiscence is a rare , but potentially serious complication after total hysterectomy . we report a case of vaginal cuff dehiscence after vaginal cuff brachytherapy . a 62 year old female underwent a robotic - assisted laparoscopic hysterectomy with bilateral salpingo - oophorectomy , and was found to have international federation of gynecology and obstetrics ( figo ) 2009 stage ib endometrioid adenocarcinoma of the uterus . the patient was referred for adjuvant vaginal cuff brachytherapy . during the radiation treatment simulation , a computerized tomography ( ct ) of the pelvis showed abnormal position of the vaginal cylinder . she was found to have vaginal cuff dehiscence that required immediate surgical repair . vaginal cuff dehiscence triggered by vaginal cuff brachytherapy is very rare with only one case report in the literature .
a 76-year - old male with 160 cm in height and 58 kg in weight , respectively , was admitted for surgery with known aortic stenosis . preoperative echocardiography exams revealed the followings : aortic valve calcification and severe stenosis , a decreased left ventricular systolic function , trivial tricuspid regurgitation and ejection fraction of 40 - 45% . in a coronary artery angiography , 1 ) . however , compression of the coronary arteries by major vessels was not revealed . coronary computed tomography ( ct ) the patient was scheduled for aortic valve replacement . at the operating theatre , routine noninvasive and invasive blood pressure monitoring , electrocardiography ( ekg ) , anesthetic induction was done under target - controlled infusion ( tci ) of propofol and remifentanil . tee revealed severe calcified and stenotic aortic valve , trivial tricuspid regurgitation and a good regional wall motion . until the cpb initiation , vital signs were maintained well . at the time of the operation , aortic valve and annulus were severely calcified and both coronary arteries were originated from the left coronary sinus , but have different ostia . jude medical , minnesota , usa ) and the surgery were done without specific event . during cardiopulmonary bypass weaning , blood pressure and cardiac output were unstable . these situations were repeated for about 30 minutes , and dopamine and norepinephrine infusion were started . the patient was transferred to coronary care unit after surgery , and vital signs were also stable . anomalous origin of the right coronary was considered as the culprit for the difficult weaning process . iabp was removed the next day , and the patient was discharged without any special events . poor ventricle function , regional wall motion abnormalities , or significant unexplained arrhythmias after aortic valve surgery should suspect poor myocardial protection , coronary insufficiency due to mechanical cause , coronary spasm , coronary air / particle emboli , protamin / transfusion reactions , and anomalous coronary artery [ 1 - 4 ] . since the aortic valve has been replaced with a stentless tissue valve , a number of mechanical obstructions in the coronary arteries have been reported . during the surgery , distal suture line , near the coronary artery ostium , teflon pledget , vessel kinking or torsion , after coronary artery reimplantation , obstructs the coronary artery [ 4 - 8 ] . clinically significant air embolism occurs rarely and is usually self - limiting and resolved quickly . particulates emboli , such as a calcified aortic valve , papillary muscle , or aortic vegetation , immediately impair the hemodynamic at the beginning of coronary perfusion and change the ekg . sometimes , hemodynamic fluctuation should be suspected by the coronary artery spasm . intraoperative or postoperative coronary spasms are referable to the coronary artery trauma during manipulation , compression by the drains , electrolytes disturbance or releasing factors [ 9 - 11 ] . sometimes , spasm occurs during the cardiopulmonary bypass weaning , but mostly , resuscitation and intraaortic balloon pumps are needed in the postoperative period . although the right coronary artery was not compressed preoperatively , aortic valve replacement could have made anatomical change to the relationships between anomalous coronary artery and the major vessels . considering the recovery of vital signs postoperatively , blood volume changes in the heart and the major vessels , during the cpb weaning period , could have affected that relationship . the incidences of anomalous origin of the coronary artery is about 0.6 - 2% and the most common types are the anomalous circumflex origin from the right coronary artery or the right sinus of valsalva . anomalous right coronary origin from the left coronary sinus , anomalous left coronary origin from the right coronary artery , or sinus of valsalva and both coronary arteries from a single ostium were reported . the majorities of these anomalies are benign , but sometimes they result in severe myocardial ischemia , infarction , and sudden death . especially , they are vulnerable to compression , insufficiency and injury during valve surgery . considering that the coronary artery anomaly is accompanied more commonly in patients with aortic valve pathology , anesthesiologists should always be reminded about these complications . in the cases of mechanical obstruction when intraoperative coronary artery spasm is expected , intravenous or intracoronary injection of nitrates and calcium antagonists can relieve the spasm , and intracoronary injection of ergonovine can be used as a diagnostic tool . maintenance of high perfusion pressure can quickly resolve the coronary air embolism , but particulates embolus needs a rescue graft , but particulates embolus needs a rescue graft . authors did n't think about the possibilities of compression of the right coronary artery because there was no compression preoperative angiography . excluding other possible causes of unstable vital signs , aberrant coronary artery can be taken into consideration . conclusively , anesthesiologists should always take care for the coronary artery anatomy , even though it is a valve surgery , and especially since they have anomalous origins or pathologies .
we report a case of hemodynamic instability after aortic valve replacement , due to the anomalous origin of the right coronary artery . during the cardiopulmonary bypass weaning process , hemodynamic instability occurred . the cause was not identified at first , and compression of the anomalous right coronary artery was thought to be the culprit , thereafter .
although most patients are asymptomatic , occasionally , abdominal symptoms occur , ranging from vague pain , epigastric fullness , bleeding , or perforation . if symptoms persist despite the use of proton pump inhibitors , surgical resection is a treatment option . we present a patient with an unusual complaint of a gastric diverticulum , discuss the pitfalls in management and report a minimally invasive solution . a 45-year - old woman visited our outpatient clinic because of severe foetor ex ore . at first , she experienced a foul smelling breath only when burping , later it was a constant and invalidating complaint . at night , she would occasionally vomit . endoscopy showed a diverticulum 5 cm in length , high in the posterior corpus - fundic region of the stomach , with stasis of food residue . a barium study confirmed a posteriorly located gastric diverticulum 2 cm from the gastroesophageal junction ( goj ) ( figure 1 ) . a barium study reveals a posteriorly located gastric diverticulum 2 cm from the gastroesophageal junction ( goj ) . the food residue and contrast retention in the diverticulum were believed to explain the patients foetor ex ore , and a laparoscopic resection was decided on . with the patient under general anesthesia , laparoscopic access was obtained to the peritoneal cavity by an open subumbilical trocar placement . a liver retractor ( endo paddle retract , us surgical corp . , norwalk , ct ) was used to obtain a good upper abdominal view . because inspection of the stomach did not reveal a diverticulum at the anterior surface , the bursa omentalis was opened by dividing the gastrocolic omentum with a ligasure ( atlas , boulder , co ) . the stomach was retracted ventrally and to the right to inspect the posterior part of the greater curvature . dissection was continued up to the cardia , and the diverticulum was found 2 cm distally from the goj at the cranial boarder of the pancreas . dissection from its avascular adhesions to the retroperitoneum was performed until the saccular structure was clearly identified ( figure 2 ) . the diverticulum was resected at the neck with the endogia ( universal , us surgical corporation , norwalk , co ) . the neck of the diverticulum was retracted though the umbilical skin incision , a tip of the stapler line was cut open , the excrements were aspirated from the diverticulum , and the collapsed diverticulum could be extracted though the small umbilical incision ( figure 3 ) . the gastric diverticulum dissected from its vascular adhesions to the retro peritoneum and its resection at the neck with the endogia ( universal , surgical corporation , norwalk , co ) . histology showed a gastric diverticulum of 5.542.5 cm with normal mucosa , a thickened muscularis mucosae , and a thin and partly discontinuous muscularis propria . but because these diverticula are usually asymptomatic , it is now believed that the true prevalence of gastric diverticula is much higher . it is the most common gastric diverticulum typically located at the posterior wall just below the goj . the acquired variety lacks the muscular or serosal layer false diverticulum and is mostly located in the distal one third of the stomach , especially in the prepyloric region . either traction or pulsion diverticula may be present , often caused by other diseases , inflammatory processes , or tumors . most gastric diverticula are known to be asymptomatic . occasionally , a sensation of fullness in the upper abdomen immediately after meals occurs . only twice has an invasion with adenocarcinoma been reported . in about 10% of patients with a true diverticulum diverticula exceeding 4 cm are more prone to produce complications and tend to respond less favorably to medication . in case of chronic inflammation , ulceration , and hemorrhage , surgical treatment is a sound indication . in patients suffering from abdominal pain or a sensation of fullness and early satiety , indication for surgery essential for successful treatment with complete relief of symptoms is the association of the symptoms with the diverticulum . palmer1 found that , in 30 of 49 symptomatic patients with a gastric diverticulum , symptoms were attributable to other gastrointestinal diseases . even the open approach can be challenging because the diverticulum is often collapsed and hidden in the splenic bed . laparoscopically , access to the posterior gastric fundus is relatively easy by dividing the gastrocolic ligament . in our experience , the diverticulum was found by continuing the dissection up to the cardia by dividing the short gastric vessels . if even then the diverticulum is not found , the procedure can be combined with intraoperative gastroscopy or the stomach can be insufflated with 0.9% saline solution via a nasal tube while the antrum is closed by compression . laparoscopic resection has been reported to be feasible and produced an excellent outcome in previous cases . in one case , however , laparoscopic resection led to mixed results , reflecting the dilemma of ascribing nonacute symptoms to a gastric diverticulum . in our case , recovery was uneventful , and relief of the somewhat unusual symptoms was complete . if laparoscopic experience is available and familiarity with the diaphragmatic hiatus exists , we recommend laparoscopic resection , as a minimally invasive solution to a symptomatic gastric diverticulum .
gastric diverticula are rare and occasionally symptomatic . a sensation of fullness in the upper abdomen immediately after meals is the most common symptom . dyspepsia and vomiting are less common . ulceration with hemorrhage or perforation has been reported . if it is thought that complaints can be ascribed to the diverticulum and if proton pump inhibitors do not relieve symptoms , surgical resection is an option . knowledge of the pitfalls in diagnosis and treatment of a gastric diverticulum are essential for successful and complete relief of symptoms . we report a successful laparoscopic approach as a minimally invasive solution to a symptomatic gastric diverticulum .
anorectal malignancies are commonly adenocarcinoma or squamous cell carcinoma , with melanoma having a relative incidence of 0.54% of all malignancies in this region [ 1 , 2 ] . this site is the third most common primary location for melanoma after skin and retina and yet only 0.41.6% of all primary melanomas arise here [ 2 , 3 ] about 1% of all anorectal carcinomas are melanomas , typically presenting in the fifth or sixth decade of life and predominantly in women . prognosis is very poor with a median survival of 24 months and a 5-year survival of 10% . due to rarity of this entity there is no consensus on which surgical approach is favourable . the surgical procedure of choice ranges from an abdominoperineal resection ( apr ) to wide local excision ( wle ) with or without adjuvant radiotherapy . we report a case of parm in a 50-year - old caucasian man presenting intermittent prolapse of an anorectal mass during 6 months with occasional bleeding . the patient was in good condition with no weight loss and past medical and family history unremarkable . proctoscopy confirmed the physical examination and showed a 3 cm bleeding villous polyp that originated above the dentate line . endorectal ultrasound showed a fusion of all layers , infiltration of the sphincters and no suspect lymph nodes . magnetic resonance imaging of the pelvis highlighted a high suspicion of internal sphincter infiltration and no inguinal or pelvic lymph nodes . computed tomography ( pet ct ) showed an intensely hypermetabolic lesion centred on the lower rectum ( standardized uptake value ( suv ) = 35.1 ) and no evidence of disease extension loco - regionally or at distance ( figs 1 and 2 ) . the histopathology of the biopsy concluded to a primitive malignant melanoma due to the tumour profile in immunohistochemistry : human melanoma black ( hmb-45 ) + + + , s100 protein + + + , melan a + , wide spectrum cytokeratins negative . the oncologic multidisciplinary council validate a surgical treatment without neoadjuvant radio or chemotherapy . due to high suspicion of internal sphincter involvement an apr the histopathology reported a pedicle , mostly exophytic tumour of the anorectal junction , measuring 2.5 2.5 1.5 cm . this tumour is associated to a tubulo - villous polyp in low grade dysplasia coexisting on the tumour pedicle . follow up , the patient presented a single hypermetabolic lymphadenopathy localized in the left obturator region , measuring 13 mm long axis ( suv = 9.5 ) and several spots of increased glycolytic activity in the hepatic parenchyma , which only one was corresponding to a small hypodense lesion ( suv = 4.5 ) . the patient was included in a study protocol comparing a new treatment anti - pd1 ( nivolumab ) with ipilimumab ( monoclonal antibody anti - ctla-4 ) or both . about 1% of all anorectal carcinomas are melanomas , typically presenting in the fifth or sixth decade of life and predominantly in women . it may be difficult to identify , and may be misdiagnosed as a haemorrhoid , rectal polyp , or as an ulcerative lesion following prolapse through the anal orifice . macroscopically , the tumours are polypoidal and pigmented while microscopically , the cells are arranged in nests with characteristic immunostaining specific for melanosome protein . however the histologic markers of s-100 , hmb-45 and vimentin are frequently identified and help clarify the diagnosis . if biopsy shows a specimen suspicious for sarcoma ( e.g. leiomyosarcoma ) , s-100 staining should be performed and if positive the tumour is most likely to be a melanoma . tumour histology includes epitheliod or spindle cells but as in our case can be a mixed tumour . tumours with pure epithelioid histology were less likely to recur [ 1 , 2 , 4 , 5 ] . figure 1:(a ) t2-weighted axial , coronal and transverse mri images , demonstrating the polypoid 3 cm anorectal mass . ( b ) pet ct showed an intensely hypermetabolic lesion centered on the lower rectum.figure 2:histologic illustration of the collision tumour . ( a ) t2-weighted axial , coronal and transverse mri images , demonstrating the polypoid 3 cm anorectal mass . ( b ) pet ct showed an intensely hypermetabolic lesion centered on the lower rectum . the rarity of this case is based on the observation of a tubulo - vilous polype on the tumour pedicle . actually there was a small in situ melanoma on the anal side that turn into a polypoid and invasive melanoma upwards to the rectal mucosa . collision tumour. we do not know to this date if there is any association of those two tumours on the anorectal melanoma pathogenesis . to our knowledge , there is no other similar observation reported in the exception of case reporting synchronous anorectal melanoma and rectal adenocarcinoma in a distance of 3 - 4 cm between them . in the absence of metastasis surgical therapy however , several recent studies suggest that , if possible , sphincter - sparing local excision and adjuvant radiation is well tolerated and can effectively control loco - regional disease while avoiding the functional morbidity of the apr [ 35 , 8 , 9 ] . on the other hand , mesorectal lymph nodes are involved in preference to inguinal lymph nodes in contrast to squamous cell carcinoma of the anus . if an apr is performed , the mesorectal lymph node resection may contribute to a better staging of the disease . there is no value of prophylactic inguinal lymph node resection [ 2 , 3 , 5 ] . patients without lymph node metastasis have a survival advantage with a 5-year survival rate of 20 versus 0% in patients with metastasis . a worse prognosis was also associated with tumour thickness , tumour necrosis ( important histologic feature , representing a biologically more aggressive tumour ) and perineural invasion . this reported survival is not up to date due to the small number of cases and does not take into account new medical treatments ( nivolumab and ipilimumab ) .
abstractanorectal melanoma is a rare disorder . there have only been a few cases reported and there is no consensus of treatment . we report a case of 50-year - old caucasian man presenting intermittent prolapse of an anorectal mass during 6 months with occasional bleeding . biopsies came up with the diagnosis of malignant melanoma . no distant metastases were found . he underwent an abdominoperineal resection due to internal sphincter invasion . a second tumour was diagnosed in final histologic examination : a dysplastic rectal polyp invaded by the melanoma ( collision tumour ) . at 12 months of follow up he presented loco - regional recurrence ( a single pelvic lymph node ) and hepatic metastases . he was included in a study protocol comparing new medical treatments ( nivolumab versus ipilimumab or both ) .
a 6yearold male was brought to our emergency department having sustained an accidental penetrating injury to zone 1 of the neck with a sickle . on examination , there was a foreign body sickle ( fig . 1 ) with its wooden handle as an entry point at the right submandibular region with no active bleeding and without evidence of vascular or neurological injuries . the sickle had serrated part on its concave surface and blunt part on its curvature . 2 . what is the superior limit of foreign body piercing the neck in this patient ? penetrating neck injury can be life threatening because of the increased risk of injury to the vital structures such as blood vessels , airway , cervical spines , and nerves which are present in such a small confined area 1 . the foreign body may be providing tamponade effect on a major blood vessel so it should not be blindly pulled out until radiological evaluation 2 . our patient initially has undergone plain xray of the neck as a means of costeffective investigation . but from xray alone , it was not possible to find the accurate information regarding the relation of foreign body with the adjacent vital structures . computed tomography scan can be helpful to know the extent of the injury sustained by the patient with penetration of neck with sharp objects such as sickle , knife , broken glasses , metallic and wooden foreign bodies . in this case , the computed tomography showed the metallic foreign body penetrating the soft tissue of neck on right side reaching the opposite side orbit . intraoperatively , the foreign body was found to be penetrated to floor of mouth , involving dorsum of tongue and soft palate , then entered into left nasal cavity , piercing the middle turbinate , and finally entered into left orbital cavity lying posterior , that is , very close to the orbital apex ( fig . 2 , 3 , 4 ) . coronal view of ct scan showing the entry point and the extent of foreign body . axial view of ct scan showing metallic foreign body in left orbit . correlating the radiological and clinical finding , although xray plain film is a costeffective investigation method in the case of metallic foreign body penetrating the neck , one should not rely alone on it , as it can not reveal status of neurovascular structures lying adjacent to foreign body in an anatomically important area such as head and neck region . ct scan is a definitive imaging technique which yields important information and makes interpretation easy . so , it must be taken before planning the removal of foreign body in operation theater .
key clinical messagect scan is the most important investigation in patients with penetrating neck injury in which it can show the extent of internal injury which may be overlooked . without ct scan being performed , one should not try to remove foreign body by just pulling blindly , as it can injure vital structures .
laparoscopic posterior mesh rectopexy ( lpmr ) is a good surgical option for rectal prolapse with low morbidity and mortality . specific complication like mesh erosion into the rectum following lpmr is very rare with only one such case , in which mesh got expelled via rectum , reported in literature till date . we report a case of mesh erosion into the rectum after lpmr which was managed with laparoscopic mesh removal and review the published literature . a 40-year - old man presented with occasional bleeding per rectum since 10 months with low grade intermittent fever for 1 week . he had history of lpmr for complete rectal prolapse 2 years back , carried out elsewhere . sigmoidoscopy revealed hyperemic mucosa with ulceration 15 cm from anal verge , with a part of the polypropylene mesh projecting into the rectum [ figure 1 ] . computed tomography ( ct ) scan of abdomen showed asymmetric anterior wall thickening of rectosigmoid junction with fat stranding and minimal fluid collection . review of his surgical notes revealed that a 15 cm 10 cm polypropylene mesh was used for rectopexy . since it was a large mesh fixed with multiple tacks and possible dense adhesions , transanal approach was not attempted . under general anesthesia , in lithotomy position , diagnostic laparoscopy was done . appendix was inflamed with its tip densely adherent to the mesh [ figure 2 ] . there was a small rectal perforation at the site of erosion , which was confirmed by air leak test . sigmoidoscopic appearance of mesh erosion into rectum intra - operative images of ( a ) tip of appendix adherent to the mesh and ( b ) mesh eroding into rectum laparoscopic rectopexy is presently the preferred management approach for rectal prolapse as it has better results especially in terms of less post - operative pain , shorter hospital stay , and similar recurrence rate as open rectopexy . it may present with discharge of pus or blood per rectum , even several months or years after surgery . it can be evaluated by doing a flexible sigmoidoscopy , which will usually reveal an ulcerated lesion at the site of erosion with visualization of mesh . ct scan of abdomen with contrast helps in localizing the mesh in relation to rectum as well as pelvic collection , if any . it is less common with type i mesh when compared to type ii , iii , and iv meshes due to bigger pore size with lesser chance of infection . other factors that are found significant are poorly controlled diabetes mellitus , tobacco use , prior history of pelvic irradiation , and repeat procedures , which contribute to poor wound healing and subsequent infection , erosion or extrusion . same causes can be considered as possible etiologies for mesh erosion after lpmr as well . certain surgical technical errors like unrecognized rectal injury and deeper stitches through the rectum may also contribute to mesh erosion . bigger sized mesh which can produce more folding after fixation , as in our case , may be a possible cause for erosion . another possible cause in our case could be infection of mesh due to adhesion of inflamed appendix to it . since , the incidence is very rare , no definite protocol regarding management is available in literature . we preferred laparoscopic approach in our case , as it was too big mesh to remove through transanal approach . moreover , only a part of mesh was protruding into the rectal lumen and it was fixed with multiple tacks to the sacrum . severe inflammation and friable tissues precludes primary repair of the rectal defect where diverting ileostomy or colostomy plays an important role . in summary , the predisposing factors could be mesh infection , larger mesh , and unrecognized rectal injuries . laparoscopic excision of mesh is a feasible and promising approach to manage such an unusual complication following lpmr .
laparoscopic posterior mesh rectopexy ( lpmr ) is now an accepted surgical treatment for complete rectal prolapse . it is associated with complications such as partial mucosal prolapse , fecal impaction , constipation , and rarely recurrence . erosion of the mesh into the rectum after lpmr is very rare . we report herein the case of 40-year - old man who presented with mesh erosion into the rectum and managed successfully by the laparoscopic excision of mesh . this is probably the first such case managed by the laparoscopic approach .
clinical management of infertility among organ transplant recipients is a challenge to the treating physician with hitherto limited information in literature . in particular , artificial reproductive techniques ( art ) have been seldom attempted among these patients due to the possibility of life - threatening complications . the tremendous progress in the fields of art and transplantation medicine offers the opportunity to safely explore various options for pregnancy . the authors present a case of a young postrenal transplant female with secondary infertility who successfully delivered a healthy baby following a single blastocyst transfer . a 30-year - old woman , married for 8 years was referred with a desire to conceive . at the age of 23 she underwent renal transplant for immunoglobulin a nephropathy at the age of 26 . at the time of presentation to the authors , she was on tacrolimus , azathioprine , and prednisolone . her serum blood urea nitrogen and creatinine were within normal limits ( 14 mg / dl and 0.8 mg / dl , respectively ) . the patient 's basal hormone profile worked on day three of menstrual cycle as follows : luteinizing hormone ( lh ) - 8.28 mlu / ml , follicle - stimulating hormone ( fsh ) - 4.04 mlu / ml , prolactin - 19.03 ng / ml , and anti - mullerian hormone - 4.1 ng / ml . antral follicular count was more than 24 follicles indicative of polycystic ovarian morphology . all the cycles were ovulatory with appropriate endometrial lining . however , she failed to conceive . the option of in vitro fertilization ( ivf ) was opted by the couple after being fully counseled of the potential risks associated with art and pregnancy . following a discussion with the nephrologists and high - risk pregnancy specialist , ivf cycle was planned and initiated without precycle suppression with oral contraceptive pills . a mild stimulation protocol with recombinant fsh 150 iu / day ( injection gonal f , merck kgaa ) was started on day 3 and continued for 5 days . recombinant fsh dose was reduced to 75 iu / day and continued till day 11 . gonadotropin - releasing hormone ( gnrh ) antagonist , injection cetrorelix acetate 250 mcg ( injection cetrotide , merck kgaa ) was administered subcutaneously on menstrual cycle day 7 and continued until ovulation trigger . ovulation was induced with injection triptorelin 0.2 mg ( injection decapeptyl 0.2 mg , ferring pharmaceuticals ) , when more than 3 follicles measured > 17 mm . injection human chorionic gonadotropin ( hcg ) 1500u was administered immediately after oocyte retrieval to rescue the luteal phase . then , 10 mature oocytes were retrieved and all the 10 oocytes were subjected to icsi . on day 3 , eight excellent quality embryos developed and extended culture was done till day 5 . endometrial thickness was 8 mm and a single expanded blastocyst was transferred with labotect catheter . luteal phase support was started 24 h after oocyte retrieval and continued until 8 weeks with 90 mg progesterone gel ( crinone 8% gel , merck kgaa ) once a day . the patient did not develop any signs or symptoms of ovarian hyperstimulation syndrome ( ohss ) . serum beta hcg levels were estimated to be 800 iu on day 14 of embryo transfer . transvaginal ultrasound was performed 2 weeks after embryo transfer and single viable fetus corresponding to 6 weeks was observed . throughout the treatment cycle and during pregnancy , the patient 's anti - rejection drugs ( prednisolone , tacrolimus , and azathioprine ) were continued at maintenance doses . the patient was monitored vigilantly and the entire course of pregnancy was uneventful with normal blood pressure and renal function . there was no proteinuria observed during weekly urine monitoring and the patient did not develop any signs of preeclampsia . cesarean section was performed electively at 37 weeks and the patient delivered a healthy male baby weighing 3.2 kg . in 1995 , the first successful ivf in a renal transplant recipient was performed by lockwood et al . the first successful blastocyst transfer in a renal transplant recipient was reported in 2008 by fichez et al . to the best of the authors ' knowledge , there have been only 7 cases reported till the year 2015 , on successful pregnancies after ivf in renal transplant recipients . renal insufficiency disrupts the normal gonadal function due to dysfunction of hypothalamo - pituitary - gonadal axis resulting in high fsh , lh , and prolactin levels . after successful transplantation , subsequent spontaneous pregnancies have been reported in 12% of the women in reproductive age . many studies have claimed that the fertility is restored in 1 to 3 years following transplantation . the risk of graft rejection is high during the 1 year and therefore it is not advised to plan ivf within that period . this was the only case to have safely delivered a 37 weeks term baby without any maternal and neonatal complications . maternal complications during pregnancy are pregnancy - induced hypertension , preeclampsia , deep vein thrombosis , infections , and graft rejection while the fetal complications include preterm birth , very low birth weight , intrauterine growth restriction , or small for gestational age babies . this can be attributed to the altered metabolism of the recombinant fsh which in turn is due to the impaired renal function . further , enlarged ovaries can rarely obstruct the transplanted kidney during induction resulting in deterioration of renal function . the present case report did not develop ohss despite having polycystic ovaries because of the mild stimulation protocol with vigilant monitoring . the risk of ohss was reduced to a significant extent by triggering final oocyte maturation with gnrh agonist trigger and using small dose of hcg after oocyte retrieval . single embryo transfer is recommended while the remaining embryos could be cryopreserved to avoid complications that arise from multiple pregnancies . it is also recommended that transplant recipients satisfy certain criteria before contemplating pregnancy to avoid complications [ figure 1 ] . although pregnancy causes an increase in glomerular filtration rate , hyperfilteration is compensated by the normal intraglomerular pressure . therefore , no glomerular damage occurs if allograft function is normal prior to pregnancy . according to alston et al . , the graft rejection rate among posttransplant pregnancies is 6% , which is similar to that of a nonpregnant population . with more such successful cases , with increasing cases of renal transplantation , women approaching for infertility treatment are expected to increase and more complicated cases have to be dealt with . it is safe to plan pregnancy through art for renal transplant recipients under vigilant multi - specialty care . it is prudent to initiate mild stimulation protocol and trigger final oocyte maturation with a gnrh agonist to avoid complications such as ohss .
numerous spontaneous pregnancies have been reported in renal transplant recipients ; however , only a few pregnancies after the use of assisted reproductive techniques . the authors report a case of renal transplant recipient with secondary infertility who delivered a healthy baby without any complications . the report highlights the importance of minimal stimulation protocol during ovarian stimulation , single embryo transfer , and the need for multispecialty care for these patients . to the best of the authors ' knowledge , the present report is the first such case from india and also the second in the world to report a blastocyst transfer among renal transplant recipients .
collapsing glomerulopathy ( cg ) is being increasingly recognized as a cause of end - stage renal disease . it is currently classified as one of the pathological variants of focal segmental glomerulosclerosis ( fsgs ) . the exact cause of this lesion is still not known , but the list of associated genetic and acquired diseases has been growing . cg is characterized by focal to diffuse , segmental to global , implosive collapse of the glomerular capillary tufts associated with marked proliferation and swelling of overlying podocytes resulting in the formation of pseudo crescents on light microscopy . immunofluorescence study is usually negative or shows only focal segmental positivity of immunoglobulin m ( igm ) , c3 and occasionally c1q . electron microscopy shows collapse and wrinkling of glomerular basement membrane ( gbm ) and greatly hypertrophied overlying podocytes with diffuse foot process effacement . cg associated with certain infections such as human immunodeficiency virus ( hiv)-1 infection , parvovirus b19 , cytomegalovirus ( cmv ) infection , human t - cell lymphotropic virus-1 , hepatitis c virus ( hcv ) , leishmaniasis and febrile illness has been documented . a 40-year - old man with diabetes and hypertension for 5 years presented with prolonged fever , cough and hemoptysis for 6 weeks duration and anasarca with breathlessness and oliguria ( urine output < 200 ml / day ) of 2 weeks duration . he denied smoking , alcohol consumption and intravenous ( iv ) drug abuse . on clinical examination , he was febrile , pale , edematous , tachypneic ( respiration rate 26/min ) , tachycardic ( pulse rate 104 bpm ) and hypertensive ( blood pressure 160/100 mmhg ) with absent diabetic retinopathy . he had few crepitations and cavernous type of bronchial breath sounds in the infraclavicular and axillary region of the right lung . urine analysis showed protein 3 + , blood 3 + and pus cells 1 - 2/hpf . complete blood count revealed a total white cell count of 10400/mm ( polymorphs 58% , lymphocytes 36% , others 6% ) , hemoglobin of 8.3 g / dl and a platelet count of 3.69 lakhs / mm . serum urea was 124 mg / dl , and creatinine was 6.4 mg / dl . his fasting lipid profile was deranged with elevated total cholesterol ( 369 mg / dl ) and triglyceride ( 542 mg / dl ) . liver tests showed normal bilirubin and transaminase levels with low total protein ( 4.9 g / dl ) and albumin ( 2.0 g / dl ) levels . anti - nuclear antibodies , anti - neutrophil cytoplasm antibodies , anti - gbm antibody tests were negative . after stabilizing him with three hemodialysis sessions , renal biopsy was performed . on light microscopy , 6 out of 9 glomeruli showed the collapse of capillary tuft with florid hyperplasia of the overlying podocytes [ figure 1 ] . tubular epithelial cells showed signs of acute injury , interstitial edema with inflammatory cell infiltrate . immunofluorescence microscopy was negative for antisera to igg , igm , iga , c3 , c1q , kappa , and lambda light chains . some podocytes contain protein resorption droplets , a common finding in collapsing lesion ( pas , 400 ) occasional microcystically dilated tubules containing proteinaceous casts were seen in the edematous interstitium ( pas , 200 ) our patient presented with nephrotic proteinuria , severe renal failure , and sputum positive pulmonary tb . the absence of lambda and kappa light chains in the immunofluorescence of renal biopsy and normal serum protein electrophoretic pattern ruled out multiple myeloma . hence , after excluding all other possible causes , we surmise that the most probable cause of cg in our patient could be pulmonary tb . at present disturbance in the immune system , coupled with genetic susceptibility , likely contribute to the changes in glomerulus . coventry and shoemaker reported a case of cg in a 16-year - old girl who presented with steroid - resistant nephrotic syndrome and pulmonary tb . in the absence of the usual associations ( adult age group , african - american race , or history of iv drug abuse ) , this lends support to the hypothesis that immune dysregulation due to infection per se , rather than infection by specific viral agents , may lead to cg in susceptible individuals . rodrigues et al . reported an hiv - negative patient with tb - related cg who needed dialysis for 5 months but presented full renal recovery after tb treatment and corticotherapy . light microscopy picture of our patient showed podocyte hyperplasia with the collapse of glomerular tuft . the therapeutic approaches empirical and analogous to those used for noncollapsing fsgs , i.e. , use of steroids or immunosuppressive agents . our patient was started on category i anti - tuberculous treatment ( att ) as per directly observed treatment strategy and oral prednisolone 1 mg / kg / day . we stopped oral steroids after tapering at the end of 2 months as his blood sugars became uncontrollable with insulin . he became sputum negative for mycobacterium tb and dialysis - independent at the end of 2 months . his serum creatinine and 24 h urine protein were 3.4 mg / dl and 2 g / day during the last follow - up . long - term follow - up is needed to assess the progression of renal disease in this patient .
collapsing glomerulopathy ( cg ) usually presents with reduced glomerular filtration rate , heavy proteinuria and has unfavorable prognosis . numerous associations with cg are found . we encountered a case of cg associated with pulmonary tuberculosis presenting with proteinuria and dialysis - requiring severe renal failure . our patient made partial recovery of his renal function and became dialysis - independent after antituberculous therapy and oral steroids . long - term follow - up is needed to assess the progression of the disease .
purely extragenital ls contributes to 20% of the total cases.morphoea may coexist with ls.ls lesions may follow lines of blaschko . purely extragenital ls contributes to 20% of the total cases . lichen sclerosus ( ls ) is an inflammatory dermatitis of unknown etiology affecting primarily vulvar , perianal and perineal skin of prepubertal , perimenopausal and postmenopausal women . extra genital ls is most common on the face , neck , shoulders , and upper portion of the trunk . libow and coots first described extragenital ls developing in a pattern corresponding to blaschko 's lines in 1998 . we report a case of extra genital ls et atrophicus along with morphoea presenting in a blaschkoid pattern in an indian patient . a 40-year - old female presented with slightly pruritic eruption that initially started over the left shoulder 2 years back and then gradually enlarged to involve the left arm , left side of her abdomen , and the left lower limb . an examination revealed discrete and confluent , porcelain white , sharply circumscribed , slightly atrophic , papuloplaques over the left arm and left leg and left side of abdomen with a sharp demarcation at the midline [ figures 1 and 2 ] . the genital , perianal , and the mucosal areas were normal . the skin over the posterolateral aspect of abdomen on the left side and over the lower part of thigh was slightly hyperpigmented and indurated . over this affected area the epidermal changes were missing and the overlying skin was smooth . hypo and hyperpigmented macules over the trunk with a sharp demarcation at the midline near the umbilicus lichen sclerosus lesions on the left lower limb routine hemogram and urine examination were within normal limits . biopsy done from the area with porcelain - white discoloration of epidermis showed follicular plugging overlying a thinned out epidermis devoid of rete ridges . histopathology done from the smooth , hyperpigmented , and indurated lesion on the left flank showed a relatively normal epidermis without follicular plugging . there was patchy periappendageal inflammation and subcutaneous fat layer showed patchy septal inflammation with lymphocytic infiltrates . follicular plugging overlying a thinned out epidermis devoid of rete ridges with vacuolar alteration of basal cell layer . moderate papillary dermal edema along with focal homogenization and periadnexal lymphohistiocytic infiltrate was evident in the dermis ( h and e , original magnification 40 ) thinned out epidermis with collagenization of dermis along with patchy dermal inflammatory infiltrate ( h and e , original magnification 40 ) the patient was started on twice daily topical tacrolimus 0.1% ointment . the lesions were less obvious after 1 month of starting the therapy though there was no change in the cutaneous atrophy component . ls predominantly affects the genitals in females though the female preponderance could be due to a reporting bias . balanitis xerotica obliterans is believed to be the male counterpart of the same disease involving the glans penis and/or the prepuce . meffert et al . , found that purely extragenital ls accounted for approximately 20% of the total cases . the neck , shoulders , and upper portion of the trunk were the most frequent sites of involvement . the first case of ls following blaschko 's lines , described by libow and coots had a zosteriform involvement of the trunk . our index case differs from their case in having involvement of the left half of the body with a definite demarcation at the midline near the umbilicus . in the index case we also had coexistence of ls along with morphoea . the coexistence of morphea and ls in the same patient suggests that these lesions represent a spectrum which may reflect similar etiologic events or closely related pathologic processes in these two diseases . isolated reports of ls involving the scalp , palms , soles , or even widespread exist but ls following blaschko 's lines are rare . the lines of blaschko were first described in 1901 and represent a pattern that is observed in many skin disorders . these lines , which are invisible under normal conditions , indicate the normal embryonic movements of the skin that occur during embryogenesis . disorders that occur along the lines of blaschko are believed to result from two different clones of cells that evolve early in embryogenesis . lyonization or random inactivations in x - linked disorders , postzygotic somatic mutations in somatic conditions , and gametic half - chromatid mutations have been proposed as mechanisms . to our knowledge this is the first case of ls with morphoea in an indian patient corresponding to the lines of blaschko .
lichen sclerosus ( ls ) is an inflammatory dermatitis of unknown etiology that mostly affects the genital region in both the sexes . in active cases the histopathologic changes differentiate between ls and morphoea though in chronic cases it is very difficult to diagnose with certainty . coexistence of both the conditions in a single patient indicates that these lesions represent a spectrum of similar pathologic process . coexistence of both the conditions along blaschko 's lines is so far not described in literature . we report an indian patient with both ls and morphoea occurring along blaschko 's lines .
a 49-year - old korean woman presented intermittent swallowing difficulty , especially when eating solid foods , for the duration of 3 years . other complaints were palpitation and chest discomfort for a year before admission . physical examination revealed that the vital signs were stable except the body temperature which was 38.8c . esophagography revealed massive dilatation of the esophagus with a huge sausage - shaped filling defect along almost the entire length of the esophagus ( fig . 1 ) . on esophagoscopy , a huge smooth elongated mass , filling almost the entire lumen of the esophagus , was found . the proximal end of the mass was lobulated and there was bluish discoloration at its distal end . fatty component was suggested to be the main constituent of its upper portion ( fig . the mass was 20 cm in length , 7 cm in diameter and had a stalk measuring 3 cm in length and 1 cm in diameter just below the pharyngoesophageal junction ( fig . it was mostly covered by grossly unremarkable smooth mucosa with ulceration and necrosis at the distal end . the pathological examination disclosed lipoma with sarcomatous change at the distal end with interstitial infiltration of the well differentiated liposarcoma cells into the adjacent lipomatous area ( fig . 4 ) . there was no evidence of invasion at the stalk or metastasis to other sites . except for a leakage from the proximal anastomosis site , which required another operation on the 8th postoperative day she remained well and was on a regular diet for the following 7 months until now . benign tumors of the esophagus usually arise from the lower portion of the esophagus . but the pedunculated tumors commonly arise from the upper fourth of the esophagus . the wall of the upper portion of the esophagus is thinner and is habitually approximated by tonic muscular contractions . the peristaltic action of the esophagus tends to elongate and mold the tumor and gives rise to a pedicle . the reported cases were lipomas , pedunculated lipomas , fibrolipomas , fibrovascular polyps , and combinations of these terms . squamous cell carcinoma , adenocarcinoma and leiomyosarcoma were also reported in the pedunculated tumors of the esophagus . but it has not been proved whether they are malignant tranformations from benign tumors or malignacy de novo . as expected , there are several reports of esophageal lipomas and only one report of liposarcoma of the esophagus . the symptoms of the pedunculated tumors are sometimes so minimal that leads to a delay in the diagnosis until they became large enough to produce serious symptoms . the symptoms of all neoplasms of the esophagus are practically the same and one can not make a diagnosis from the symptoms . neoplasms , when large enough , may produce stenosis with severe dysphagia , regurgitation of food and marked dilatation of the esophagus . a pedunculated tumor itself may be regurgitated into the oral cavity or into the larynx causing cough , hoarseness , dyspnea or even death . in the esophagogram , the dilatation of the esophagus can incorrectly suggest achalasia if the tumor itself is overlooked . even endoscopy can miss the tumor , and a biopsy may miss the exact nature of the tumor as it is covered with normal epithelium . once the pedunculated tumor of the esophagus is diagnosed , resection is indicated because of progressive dysphagia , possible fatal regurgitation , possible bleeding and possible chance of malignancy . surgical removal through cervical esophagotomy for the larger tumors or endoscopic removal for the smaller ones is usually known to be sufficient . but because of the frequent presence of large vessels in the stalk , the direct surgical approach is more prudent and is the treatment of choice for most pedunculated esophageal polyps except for smaller ones . there is frequent local recurrence of liposarcomas of other organs and meticulous follow - up should be done .
a case of liposarcoma in a giant pedunculated lipoma of the esophagus in a 49-year - old korean woman who presented intermittent swallowing difficulty for 3 years is reported . endoscopy and esophagography revealed that a giant smooth longitudinal tumor mass almost entirely occupied the esophageal lumen . a total esohagectomy was done . the tumor was 20 cm in length and 7 cm in average diameter with an obvious stalk measuring 3 cm in length and 1 cm in diameter . microscopic examination disclosed a lipoma with focal ulceration and liposarcomatous change infiltrating into interstitial fibrous tissue at its distal end .
the rate of hair growth is usually normal but the anagen phase is truncated , resulting in shorter hair . there are mainly two types of woolly hair ; the autosomal dominant woolly hair , also called hereditary woolly hair , and the less commoner autosomal recessive familial woolly hair . we herein describe a case of woolly hair with associated keratosis pilaris , periodontal changes , canaliform dystrophy of nails and recurrent bullous impetigo a 4-year - old boy , product of third degree consanguineous marriage , presented with history of light colored curly hair on the scalp and skin - colored pinhead type of raised lesions over the body since birth . he also gave a history of recurrent pus - filled lesions over the body since the age of 3 months . multiple skin colored , spiny follicular papules were present over his scalp , back and arms [ figures 2 and 3 ] . multiple bullae were present over his extremities , some of which were filled with yellow fluid with a positive hypopyon sign [ figure 4 ] . woolly hair on the scalp with sparse eyebrow hair keratosis pilaris lesions on the scalp keratosis pilaris lesions on the trunk bullous impetigo lesions on the forearms canaliform dystrophy of bilateral toe nails dental caries with increased interdental spaces investigations revealed normal hematological and biochemical parameters . skin biopsy of the keratotic papules revealed features consistent with keratosis pilaris [ figure 7 ] . gram stain of pus from the bullous lesion revealed gram positive cocci , and the culture grew staphylococcus aureus . microscopy of hair revealed an elliptical cross - section , axis rotation and calibre variation . woolly hair is extremely curly , with the average diameter of hair recorded up to a maximum of 0.5 cm . it is different from the curly hair of black people , in that the curled hair of black people lies separately while the curls of woolly hair usually merge . woolly hair can appear as a part of systemic disease ( woolly hair syndrome ) or without systemic findings ( non - syndromic woolly hair ) . non syndromic wooly hair can be inherited as either an autosomal dominant or recessive disorder . an earlier classification has described four types of woolly hair : hereditary woolly hair , familial woolly hair , symmetrical circumscribed allotrichia and woolly hair nevus . our patient , in addition to woolly hair , had widespread keratosis pilaris ( involving the scalp , trunk and upper extremities ) , canaliform dystrophy of bilateral toe nails , dental changes in the form of increased interdental spaces and dental caries and recurrent bullous impetigo . a dominant form of woolly hair has also been reported in an english family with associated ichthyosis and deafness . additional features in a dutch family with woolly hair included dental caries , acral keratoderma and facial abnormalities . a case of woolly hair with keratosis follicularis spinulosa decalvans , teeth abnormalities , scarring alopecia and ophthalmological abnormalities has also been reported . another case of woolly hair associated with pachyonychia congenita , nail changes in the form of yellowish brown hypertrophy and wedge - shaped subungual hyperkeratosis of all 20 nails with natal teeth has been reported . a case similar to ours , but without the nail and teeth findings , has been described earlier by devinder et al . naxos disease is characterised by woolly hair , palmoplantar keratoderma and dilated cardiomyopathy with right ventricular dysplasia . it is an autosomal - recessive disorder occurring due to mutation in the plakoglobin gene . carvajal disease is similar clinically to naxos disease , except for left ventricular involvement and presentation at a younger age , and it is due to mutation in the desmoplakin gene . acquired woolly hair occurs most commonly in the context of patterned hair loss . our patient did not have any cardiac involvement and there was no history of any drug intake prior to onset of lesions . to the best of our knowledge , woolly hair with a combination of associated findings in the form of keratosis pilaris , canaliform dystrophy of nails , dental changes and recurrent bullous impetigo occurring together has not been reported earlier in literature .
woolly hair is a congenital abnormality of scalp hair manifesting as short , kinked hair , which may also involve the hair over the other parts of the body . keratosis pilaris has been a well known association of woolly hair , and can also be a part of the naxos or carvajal syndromes . we herein present a case of woolly hair with associated keratosis pilaris , canaliform dystrophy of nails , increased interdental spaces and recurrent bullous impetigo . although keratosis pilaris and teeth abnormalities have been reported as isolated associations with woolly hair , such a combination of findings as seen in our patient has not been reported before .
a patient was immediately referred to our department after epidural endoscopy with severe bilateral visual impairment and underwent complete ophthalmologic examination . a 45-year old man suffered from low back pain unresponsive to pharmacologic treatment , radiating to the left hip and left lower extremity , due to a back injury 4 years ago . other medical history was noncontributory and he was otherwise healthy without hypertension , diabetes mellitus , cardiovascular disease , or blood clotting abnormalities . the patient underwent an epidural endoscopy and endoscopic adhesiolysis in the level of l4l5 and o5s1 by infusion of 120 cc of normal saline . at the end of the procedure the patient received an epidural injection of 80 mg methylprednisolone . after the operation the general condition of the patient was good without clinical signs of elevated cerebrospinal pressure and blood pressure and heart rate remained stable . however , a few hours later , he became aware of a major decrease of vision in both eyes and was immediately admitted to the department of ophthalmology . at presentation , best corrected visual acuity ( bcva ) was 20/400 re and counting fingers le . the ophthalmic examination revealed the presence of extensive scattered preretinal , subhyaloid , and subretinal hemorrhages in both eyes and especially in the le ( figure 1 ) . ocular history before the operation did not reveal any ocular disease and bcva was 20/20 in both eyes . examination of the fundus showed mild vitreous hemorrhage with extensive intraretinal and subretinal hemorrhages involving the macula especially of the le . four months after the operation , bcva was 20/30 in both eyes . despite the improved vision fundus examination revealed a remarkable resolution of the retinal and subretinal hemorrhages bilaterally with mild pigmentary changes of the macula especially of the le ( figure 2 ) . intraocular hemorrhages often occur in association with acute subarachnoid hemorrhage and secondary aneurysms of the anterior communicating and internal carotid arteries ( terson 1900 ) . the present case represents an extremely rare incidence of intraocular hemorrhage following epiduroscopy and endoscopic adhesiolysis ( amirikia et al 2000 ; brian et al 2005 ) . several mechanisms have been proposed to explain the occurrence of intraocular hemorrhage ( purdy et al 1998 ) . it seems that a sudden increase of the epidural pressure after the epiduroscopy causes cerebrospinal fluid to effuse through the communication of the subarachnoid space within the optic nerve sheath and subsequently compresses the optic nerve and its vasculature . specifically , the retinochoroidal anastomosis and the central retinal vein are occluded resulting in venous stasis allowing extravasation of blood through the vessels causing subhyaloid , retinal , and subretinal hemorrhages . the different types of hemorrhages suggest an acute generalized extravasation of blood within the various layers of the posterior segment opposed to the direct tracking of blood from within the optic nerve sheath . more particularly , intracranial pressure can be directly affected if the dura is punctured and fluid is added to the subarachnoid space . the visual prognosis is considered to be good , with recovery occurring within 6 months ( tabandeh 2000 ) . the present case is rare and it represents a rare complication of a rather common operation . however , as endoscopic spinal procedures become widely established , it is anticipated that similar cases will be recognized more frequently in the future . therefore , surgeons should be aware of the possibility of possible vision loss after this procedure .
purposeto report a case of acute visual loss after endoscopic spinal surgery.methodsa patient was immediately referred to our department after epidural endoscopy with severe bilateral visual impairment and underwent complete ophthalmologic examination.resultsvisual acuity was decreased in both eyes . fundus examination revealed the presence of retinal and vitreous hemorrhages bilaterally . four months later , visual acuity increased and the hemorrhages were remarkably resolved.conclusionthe present case represents an extremely rare incidence of intraocular hemorrhage following epiduroscopy .
late vkdb may be fatal with high risk of intra .- cranial hemorrhage . though petechiae , purpura and/or ecchymoses may be seen in less than 30% of infants with vkdb , they are not the sole presenting features per se . vitamin k deficiency bleeding ( vkdb ) is a rare but serious bleeding disorder , which occurs due to inadequate activity of vitamin . k - dependant coagulation factors ( factor ii , vi , ix , x ) . this en tity is likely to be missed or overlooked because it may mimic relatively commoner diseases such as immune thrombocytopenic purpura , leukemia , and hemophilia . though a few case series have described the fatal intra - cranial manifestations of vkdb , very little is mentioned about its dermatological manifestations in literature . late vkdb is an uncommon entity with incidence of 4 to 10 per 1 lakh live births and occurs mostly in exclusively breastfed infants . late vkdb is common in southeast asia with incidence increasing to 35 per 1 lakh live births in thailand . an 8-month - old exclusively breastfed male infant presented in casualty with multiple skincolored to bluish , raised swellings on trunk and upper extremities of 4 days duration . there was no history of fever , excessive cry , jaundice , bleeding diathesis , vomiting , breathlessness , or any drug intake in recent past . the infant was a product of non - consanguineous marriage and was born at term in a primary health center . cutaneous examination revealed multiple firm to soft , non - collapsible , subcutaneous , non - tender , skin colored to bluish nodules of 1 - 6.5 cm in diameter on the chest , elbow , and knees [ figures 1 and 2 ] . general examination revealed a grade iii malnourished child ( as per classification of indian academy of pediatrics ) with normal developmental milestones . fibrinogen level , platelet count , routine urine microscopy , and other hematological and biochemical parameters were within normal range . blood culture was sterile , and skin biopsy was not performed as fnac from a large purpuric nodule revealed frank blood . multiple nodular lesions on right elbow , right knee , and lateral side of chest ( highlighted by black arrows ) skincolored to purplish nodules on chest wall and right elbow ( highlighted by black arrows ) a provisional diagnosis of late idiopathic vitamin k deficiency bleeding ( vkdb ) disorder was made , and the child was given 7.5 mg vitamin k1 intra - muscularly once - daily for 3 days . all the lesions subsided by 6 day . the infant was put on proper complementary feeding , and no recurrence was noted during follow - up even 6 months after the initial episode . vkbd has been classified based on age of onset as early ( occurring within 24 hours of birth ) ; classical ( day 1 - 7 of birth ) , and late vkdb ( 1 week to 12 months after birth ) . idiopathic vkdb was described in 1966 as acquired prothrombin complex deficiency ( apcd ) , and majority of cases have been reported from southeast asia . exclusive breastfeeding , in absence of any other cause of vitamin k deficiency , is the main cause implicated in idiopathic variant . further , studies have shown a key role of low vitamin k level in the mother 's breast milk in pathogenesis of apcd syndrome . late vkdb may be secondary to either diseases associated with mal - absorption of vitamin k ( viz - diarrheas , celiac disease , cystic fibrosis ) or hepatobiliary diseases associated with decreased synthesis of vitamin k ( viz - biliary atresia , 1-anti - trypsin deficiency , hepatitis ) . vkdb may also result due to antagonism of vitamin k by drugs such as carbamazepine , phenytoin , barbiturates , cephalosporin , rifampicin , isoniazid . clinical presentation of late vkdb ranges from life - threatening intra - cranial hemorrhage to skin bleeds with nodular purpura . neurological signs and symptoms with / without intra - cranial hemorrhage are the most common presenting feature in 50 - 80% of patients . nodular purpura is not a common presenting feature . though skin bleeding in the form of petechiae , purpura , or ecchymoses may occur in 10 - 30% of patients , it is a presenting feature in only half of such infants . nasal , gastrointestinal , umbilical , or urogenital bleeding may also occur along with / without seizures , vomiting , poor feeding , altered sensorium . late vkdb may be fatal , and risk of intra - cranial hemorrhage may be as high as 80% . nodular purpura. it occurs on the lower extremities , back , chest abdomen , buttocks , upper extremities face , and neck in descending order of frequency . these lesions are bluish - violet in color with raised infiltrated purplish centers , and their diameter may vary from 1.5 cm to 7.5 cm . a clearly prolonged pt ( inr > 3.5 ) or low quick value ( < 20% ) along with normal fibrinogen concentration and platelet count is highly suggestive of vkdb . circulating acarboxy proteins are present . skin biopsy is inconclusive and showed only extravasations of rbcs without vasculitis or intravascular thrombosis in a case series . children with vkdb due to mal - absorption require chronic administration of high doses of oral vitamin k ( 2.5 mg twice / week to 5 mg / day ) . a single intra - muscular infection of vitamin k ( 1 mg ) is recommended prophylactically to prevent vkdb . purpura fulminans , extramedullary erythropoeisis ( blueberry muffin baby ) , and protein c or protein s deficiency should be excluded in early and classical vkdb . extremely rare hereditary deficiencies of factors v , vii or x may need to be excluded by analysis of single factors . to conclude , nodular purpura in infancy should be taken seriously in a breastfed infant as these may denote warning bleeds , which may precede potentially fatal intra - cranial bleeding . it has much more clinical relevance in a developing nation like india , where exclusive breastfeeding is continued for very long periods . further vitamin k prophylaxis is not practiced uniformly in india . as a rare presentation of vkdb , this case report and review of literature is an attempt to make all the dermatologists aware of this potentially life - threatening disease that is easily treatable . nodular purpura may precede potentially fatal intra - cranial hemorrhage and hence its presence should prompt treating physician to take adequate remedial steps as soon as possible .
vitamin k deficiency bleeding ( vkdb ) disorder is an uncommon entity , which occurs due to inadequate activity of vitamin k - dependant coagulation factors . an 8-months - old exclusively breast - fed male infant presented with multiple , purpuric and nodular non - collapsible swellings on trunk of 4 days duration . investigations revealed raised activated partial thromboplastin time and prothrombintime . fibrinogen level and platelet counts were normal . late vkdb usually presents as intra - cranial or mucosal hemorrhages.[1 ] though skin and mucosal bleeding may occur in 1/3rd of infants with vkdb , nodular purpura is not the common presenting feature . earlier recognition of vkdb and immediate investigation / treatment helps prevent the potentially fatal outcome of the disease . very little is mentioned about this entity in dermatology literature .
psychiatric disorders are a major cause of disability after traumatic brain injury ( tbi ) . substantial psychological and neurobehavioral evidence is available to support the hypothesis that tbi is a risk factor for subsequent psychiatric disorders . the organic bipolar disorder is a rare entity when compared with organic mania or depression . due to its rarity , a 49-year - old man sustained head injury 1 years back which resulted in right temporal , frontal hemorrhagic contusions , and subarachnoid hemorrhage . ten days after discharge , behavioral changes such as excessive speech , irritability , increased anger outburst , abusing others verbally , increased psychomotor activity , lability of affect , decreased sleep , and appetite were observed for a period of 1-month . this was followed by decreased activity , reduced social interaction , monosyllable reply to questions asked , reduced sleep , and appetite for a period of 2 months . after 8 months , the patient presented in emergency psychiatry department with excessive speech , authoritative behavior , increased anger outburst , predominant irritability , wandering behavior , dancing and singing songs , hyper - religiosity , grandiosity , and disturbed sleep . the examination revealed irritable mood , flight of ideas , pressured speech , increased self - esteem , over familiarity , and grandiose ideas . computed tomography scan of the brain showed gliotic changes in the right temporal lobe and right frontal lobe . with this presentation , we made a diagnosis of organic bipolar affective disorder current episode mania without psychotic symptoms . he was treated with tablet divalproex sodium 2 g ( weight of patient = 98 kg , valproate dose = 20 - 30 mg / kg ) and tablet haloperidol 20 mg . the patient had severe akathisia within a week of starting tablet haloperidol , which was treated with tablet trihexyphenidyl 4 mg . so the dose of tablet divalproex sodium was reduced to 1.5 g . at present , the patient is maintaining in the euthymic state on follow - up . our patient was diagnosed with organic bipolar affective disorder according to international classification of diseases-10 criteria ( f06.31 ) secondary to tbi . this diagnosis was considered because of temporal correlation of onset of affective symptoms and tbi . bipolar and related disorders are relatively uncommon consequences of tbi . among other psychiatric disorders , past , personal and family history were not significant in our patient , unlike previous study which shows that genetic vulnerability and previous psychiatry history as one of the factors that could trigger a mood disorder following a tbi . in our case report , episodes of mania and depression were short lasting when compared with the usual course of the functional bipolar affective disorder which correlated with the previous studies . similarly , the patient had right side injury which was associated with more frequent manic episodes but on the contrary he also had one depressive episode . literature shows tbi - related affect disturbances shows frequent brief episodes of irritability and impulsive behaviors which was evident in our patient . studies show valproate may exacerbate cognitive impairment in some patients with tbi , but it appears less likely to do so than lithium . even after starting tablet divalproex sodium in a minimal therapeutic dose with respect to body weight in our patient , he developed toxicity and behavioral disorientation within a short period of time ( blood levels of valproate-127 mcg / ml ) . so the dose of divalproex sodium was decreased which resulted in remission of disorientation . also with anti - psychotics tablet haloperidol 20 mg , he developed severe akathisia and extrapyramidal side effects which resolved with trihexyphenidyl and gradual tapering of haloperidol . this reflects the need for a gradual increase in dose and careful monitoring of adverse effects of drugs in patients with tbi , as head injury makes them more prone to side effects of the drugs even with minimal dosage . in particular , unopposed selective serotonin reuptake inhibitors are prescribed routinely for the treatment of posttraumatic disturbances of affect and behavioral dyscontrol syndromes , which is generally inadvisable among persons with secondary mania or mixed states . as it raises , the possibility of the sensitizing role of brain injury for anti - depressant - induced mania . further research on long - term follow - up of patients with the organic bipolar disorder may lead to better understanding of clinical features , prognosis , and treatment .
psychiatric disorders are common consequences of traumatic brain injury ( tbi ) . but organic bipolar disorder is a rare entity when compared with other disorders . here , we report this 49 year old patient with bipolar affective disorder following traumatic brain injury , its presentation and management . though the pathophysiology of this disorder involves the interaction of factors that precede trauma ( eg , genetic vulnerability and previous psychiatric history ) , factors that pertain to the traumatic injury itself ( eg , type , extent , and location of brain damage ) , in our patient it showed an atypical presentation .
a 40-year - old female visited our clinic for agradual visual disturbance in her right eye in january 2007 . her best - corrected visual acuity ( bcva ) was 20/160 od and 20/20 os . a few barely detectable creamy - yellow retinal lesions were seen in the right eye . eighteen months prior , she had been diagnosed with pcnsl in the right temporo - parietal lobe which had been pathologically confirmed as malignant large b - cells . complete remission was achieved by whole brain irradiation . a granular pattern of mixed hyperfluorescent and hypofluorescent spots , along with leakage from the retinal vessels and the optic disc , her visual field was considerably constricted and oct ( stratus oct model 3000 , zeiss instruments inc . san leandro , ca ) showed small irregular rpe detachments in the right eye ( fig . p1 amplitude and latency of the right eye were markedly decreased compared with those of the left eye , as determined by pattern visual evoked potentials ( vep ) . prominent suppression of the central signal was noted in multifocal erg of the right eye compared with that of the left eye ( fig . 2 ) . after three months of failure to follow - up , the patient 's bcva decreased to no light perception ( nlp ) od and 20/200 os , and active panuveitis was observed in the right eye it showed a granular pattern and leakage in angiography , small rpe humps in oct , and considerable visual field constriction ( fig . 3 ) . a thorough systemic evaluation was performed , but there was no evidence of intracranial or visceral involvement , and the hematologic exam was nonspecific except for an elevated erythrocyte sedimentation rate . mega - dose intravenous steroid therapy was carried out , followed by diagnostic vitrectomy with retinal biopsy of the right eye . diffuse large malignant b - cells with strong immunoreactivities in cd20 immunostaining were seen in the preretinal membrane biopsy specimen otherwise , no abnormal cells were obtained from the vitreous biopsy ( fig . intravitreal injections of mtx ( 800 g/0.1 ml in the right eye , 400 g/0.05 ml in the left eye ) were performed twice weekly for one month , once weekly for the next month , once every two weeks for the following month , followed by nine monthly injections . during the treatment , punctuate epithelial erosions and vortex keratopathy developed , but they subsided completely with topical instillation of 0.003% leucovorin eye drops and lubricants ( fig . 5 ) . the final bcva was nlp od , 20/20 os , and both eyes were free from malignant cells in vitreous biopsy six months later . the granular pattern on fluorescein angiography was persistent but there was no dye leakage ( fig . the visual field constriction was slightly improved , and the small rpe humps in oct had disappeared . although p1 latency was markedly delayed in the right eye compared with that of the left eye , the p1 amplitudes showed a minimal difference in flash vep . the amplitude of b waves was markedly decreased in a scotopic erg of the left eye the photopic erg response , oscillatory potentials and 30 hz flicker response were relatively well preserved , with erg waves being flat in the right eye . no ocular complications except minimal opacities of the crystalline lenses were noted in both eyes . of ocular masquerade syndromes , piol is reported to be the most common.14 piol typically presents as a posterior uveitis4 non - specific findings of vitreous debris , chorioretinal thickening , widening of the optic nerve , elevated chorioretinal lesions , and retinal detachment can be seen in ultrasonography.15 subretinal and sub - rpe infiltrations of piol are reported to be common , and creamy - yellow lesions on a fundal exam might be associated with ped,16 which was demonstrated in our oct images . on fluorescein angiography , small sub - rpe infiltrations may appear as blocked hypofluorescence , and as the tumor regresses , it can become an rpe window defect.16 in our case , vasculitis and disc leakage disappeared as the tumor regressed ; the granular pattern which might have been due to clumps of pigment epithelial cells , rpe atrophy and subretinal fibrosis , were persistent . vitreous biopsy can be a useful tool to diagnose piol , but prior steroid therapy might suppress the number of vitreous cells , including lymphoma cells , which may result in a negative vitreous cytology.17 electrophysiological findings obtained from eyes with piol were commented on in a brief report by wang et al.18 which showed subnormal rod and cone responses . in our case , the b wave amplitude of the scotopic erg of the left eye was markedly decreased ; other erg waves might be subnormal but were relatively well preserved . the eog arden ratio was significantly reduced in both eyes and could represent abnormal outer retinal and rpe functions . intravitreal chemotherapy has been reported to have few complications,11,12 and because mtx is well tolerated by intraocular tissues , repeated injections are feasible without retinal toxicity.19 toxic keratopathy can also be easily managed with topical leucovorin eye drops and lubricants . we modified the chemotherapy protocol of a previous report12 by adding two additional once - every - two - week injections for consolidation because the patient had useful vision in only one eye and any other treatment choice was not readily available . we also used a double dose regimen in the right eye where tumor involvement of the vitreous and retina was massive , no useful vision remained , and , being a vitrectomized eye , a shorter half - life of mtx was expected . intravitreal chemotherapy was effective even though the patient had a history of systemic chemotherapy failure for pcnsl . in summary , primary intravitreal chemotherapy can be effectively used to treat piol and had minimal ocular complications in this patient .
a 40-year - old female visited our clinic for visual disturbance of the right eye , in which a few creamy - yellow retinal lesions and visual field constrictions were noted . she had been treated for primary cns lymphoma and was in complete remission . after failure to follow - up for three months , she lost vision in the right eye , at which time active panuveitis was seen . decreased vision and field constriction was observed in the left eye . her left eye showed a granular pattern and dye leakage from the vessels and disc on fluorescein angiography and small rpe humps were seen in optical coherence tomography ( oct ) . diffuse large malignant b - cells with strong immunoreactivities with cd20 immunostaining were seen in the epiretinal membrane biopsy specimen . intravitreal injections of methotrexate ( mtx ) ( 800 g/0.1 ml in the right eye , 400 g/0.05 ml in the left eye ) were performed twice weekly for one month , once weekly for the following month , once every two weeks for the next month , followed by nine monthly injections . both eyes were free from malignant cells on vitreous biopsy six months later . there was no leakage seen by angiography , but the granular pattern persisted . visual field constriction was slightly improved , and the small rpe humpsdetachments seen in oct disappeared . eog arden ratio was decreased in both eyes , and b wave amplitude of scotopic erg was decreased in the left eye . she was free from recurrence until six months later . no ocular complications except minimal opacity of the crystalline lenses were noted in both eyes .
riedel 's thyroiditis is a chronic inflammatory disease of the thyroid gland characterized by invasive fibrosis that partially destroys the thyroid gland and extends into adjacent neck structures . it was recognized in 1986 by riedel , who described two patients with hard goiters and tracheal compression symptoms . the diagnosis of riedel 's thyroiditis is clinically difficult because this form of thyroiditis can mimic malignant neoplasm or the fibrous variant of hashimoto thyroiditis during preoperative physical , radiologic , and pathologic examination [ 4 - 6 ] . we describe here a rare case of riedel 's thyroiditis in an elderly patient and review the literature for its radiologic characteristics . a 77-year - old female patient presented with general weakness , dysphagia and dyspnea . on physical examination , there was a firm , painless , large mass in the anterior neck and wheezing sounds were noted on auscultation . a thyroid function test was abnormal , which showed hypothyroidism : t3 of 62.4 ng / dl ( 71 to 161 ng / dl ) , free t4 < 0.1 ng / dl ( 0.8 to 1.7 ng / dl ) , thyroid stimulating hormone ( tsh ) > 100 iu / ml ( 0.86 to 4.69 iu / ml ) , tsh receptor antibody ( ab ) of 0.70 u / l ( 0 to 1.5 u / l ) , thyroglobulin antigen of 0.9 ng / ml ( 0.1 to 32.5 ng / ml ) , anti - thyroglobulin ab of 1,359.0 iu / ml ( 10 to 124.2 iu / ml ) , and anti - thyroid peroxidase ab > 600 iu / ml ( 5 to 13.5 iu / ml ) . imaging modalities including ultrasonography ( us ) , computed tomography ( ct ) , magnetic resonance imaging ( mri ) , and positron emission tomography ( pet ) scan were performed ( fig . 1 ) . all showed a diffusely enlarged mass covering both thyroid lobes , extending to the infra - hyoid level and encircling the trachea and thyroid cartilage . this mass caused tracheal stenosis , but there was no evidence of tracheal invasion on mri . despite the combination of these imaging modalities , the thyroid mass was not differentiated from a malignant neoplasm such as anaplastic thyroid carcinoma , or severe thyroiditis . preoperative pathologic diagnostic procedure including fine - needle aspiration cytology ( fnac ) was not performed because we have thought that fnac might not be helpful for differentiating anaplastic thyroid cancer from severe thyroiditis and operation should be performed for relieving the compressive symptoms . first , the pyramidal lobe and isthmus were resected , and an intraoperative frozen section was performed due to the suspicion of anaplastic thyroid carcinoma . the results of the frozen section demonstrated that there were no malignant epithelial cells , and the possibility of riedel 's thyroiditis was suggested . even though the benign result of the frozen section , bilateral total thyroidectomy was performed clearly without complications due to the purpose of relieving the compressive symptoms and tiny possibility of malignancy ( total weight , 361 g ) . immunohistochemical stainings with various markers were performed for differential diagnosis with the following results : presence of apple green birefringence in congo red staining , negative for acid - fast bacilli , and negative for calcitonin and carcinoembryonic antigen . the patient was discharged from the hospital 4 days after surgery without any complications such as bleeding or hypocalcemia . the patient has received routine check - ups and thyroid function tests along with thyroid hormone replacement ( l - thyroxine 0.05 mg / day ) . although the etiologic mechanisms underlying riedel 's thyroiditis are unclear , the prevailing view is that it is part of a generalized fibro - inflammatory process that also involves other organs . the main characteristics of riedel 's thyroiditis is invasive fibrosis that partially destroys the thyroid gland and extends into adjacent neck structures . it is difficult characteristics for physicians to distinguish riedel 's thyroiditis from malignant neoplasms of the thyroid clinically because both clinical examination and imaging of riedel 's thyroiditis suggests malignancy . us of riedel 's thyroiditis shows a hypo - echoic and hypo - vascular mass with extension into adjacent soft tissues however , this appearance is nonspecific and can be seen in other disease processes that present with diffuse fibrotic involvement , such as hashimoto thyroiditis , lymphoma , and thyroid carcinoma . thus , it is hard to distinguish riedel 's thyroiditis from other forms of thyroiditis . although the most important diagnostic tool for thyroid disease is fnac under us guidance , riedel 's thyroiditis usually can not be diagnosed accurately by preoperative cytology . various imaging modalities including ct , mri , and pet can also be performed for diagnosis of riedel 's thyroiditis . the extent of fibrosis and compression of the trachea and/or esophagus is easily defined by ct , in which the thyroid appears hypo - dense to normal , and invasion of nearby tissues might be observed . ct reveals a hypo - dense , infiltrative mass that might suggest a malignant process , although malignant neoplasms usually appear heterogeneous . several reports indicated that such lesions are homogeneously hypo - intense on both t1- and t2-weighted images , and are enhanced homogeneously after administration of contrast material . a hypo - intense appearance of riedel 's thyroiditis is due to fibrous tissue that replaces the gland and reduces mri signal intensity in all pulse sequences because of an extremely short t2 value and long t1 values . mri images in normal and abnormal thyroids reveal homogeneous or heterogeneous hyper - intensity compared with non - involved parenchyma ( particularly on t2-weighted images ) . the mri signal intensity may change with the degree of inflammation in riedel 's thyroiditis . mature fibrous tissue is usually hypo - intense on both t1-and t2-weighted images , which is related to hypocellularity and abundant collagen stroma . on the other hand , immature fibrosis containing few collagen fibers , numerous fibroblasts , and vascular endothelial cells can have heterogeneous variable signal intensity on t2-weighted images and can be enhanced to a variable degree in relation to its vascularity . pet features of riedel 's thyroiditis and retroperitoneal fibrosis in patients with multifocal fibro - sclerosis were defined recently . the pet images showed an intense , hyper - metabolic abdominal mass surrounding the aorta and increased glucose metabolism in the thyroid . the authors explained these aspects as a result of active inflammation involving lymphocyte , plasma cell , and fibroblast proliferation . pet might be helpful for evaluating disease activity and patients ' response to corticosteroid therapy . in conclusion , various imaging modalities , including us , ct , mri , and pet , can be performed for the diagnosis of riedel 's thyroiditis , but may not be helpful for the definite diagnosis of riedel 's thyroiditis and differentiation from thyroid malignancy . diagnostic thyroidectomy should be performed for the accurate diagnosis for an accurate diagnosis for riedel 's thyroiditis .
riedel 's thyroiditis is an extremely rare disease that is difficult to diagnose correctly using preoperative diagnostic tools because it can mimic malignant neoplasm or the fibrous variant of hashimoto thyroiditis during preoperative physical , radiologic , and pathologic examination . we describe here a rare case of riedel 's thyroiditis in an elderly patient and its radiologic characteristics .
lymphoepithelioma is a term used to designate an undifferentiated malignant epithelial tumor of the nasopharynx that is histologically distinctive because of a markedly prominent lymphoid infiltrate ( 1 ) . carcinomas with similar histological features arising outside the nasopharynx are called lymphoepithelioma - like carcinoma ( lelca ) . lelca occurs in organs such as salivary glands , the uterine cervix , the thymus , the lung , the skin , the stomach , the bladder , the prostate , and the breast ( 2 ) . it is very rare that lelca occurs in the urinary system , there is one case where it occurred in the renal pelvis , and one case where it occurred in the ureter and the kidney simultaneously , but has not been reported so far in urinary bladder in korea . the author et al . have lately experienced lelca that occurred in the urinary bladder , and thus have reported the case along with philological considerations . a 78-yr - old woman presented with gross hematuria for 2 weeks on november 23 , 2009 . the patient was a non - smoker and had not been exposed to carcinogen . physical examination and vital sign were normal at the time when the patient was admitted to this hospital , numerous rbc were observed on urinalysis . there were no notable findings on blood test and chest radiography . on cystoscopy , a frond - like mass was observed at the bladder trigone , which measured about 1 cm ( fig . 1 ) . since hematuria continued , foley catheterization was performed . on computerized tomography ( ct ) of the abdomen and pelvis , the findings of enhancement and a small - sized mass were observed on the inner surface between posterior walls in the bladder trigone . the findings of perivesical infiltration and lymph node metastasis were not observed . on the authority of above findings , the authors suspected the case to be carcinoma in situ of the urinary bladder or transitional cell carcinoma , and thus transurethral resection of bladder tumor ( turbt ) on histopathological examination , it was found that 90% of lesions were lelca and a few lesions were non - invasive transitional cell carcinoma . on microscopy , syncytial growth pattern and indistinct cytoplasmic borders were observed with the severe infiltration of lymphoid cells . tumor cells were positive for cytokeratin 7 but were negative for cytokeratin 20 ( fig . the infiltrated lymphocytes were composed of abundant cd3 positive t cells and cd20 positive b cells . for leukocyte common antigen ( lca ) , the patient has been without recurrence and metastasis for 3 months after the operation , and currently is on a follow - up . ( 3 ) in 1991 , is uncommon with a reported incidence between 0.4 and 1.3% of all bladder carcinoma ( 4 ) . in the urinary tract , they typically arise in the urinary bladder , although isolated cases have been reported in the renal pelvis , ureter , and urethra ( 5 , 6 ) . as suggested by amin et al . ( 7 ) , lelca was categorized as pure ( 100% ) , predominantly ( more than 50% ) , or focal ( less than 50% ) . if other classification is applied , lelca was classified as pure when 100% of the tumor showed lymphoepithelioma - like carcinoma pattern , and mixed when associated with usual infiltrating urothelial carcinoma , adenocarcinoma , or squamous carcinoma ( 2 ) . it has been suggested that pure / predominant lelca responds to chemotherapy and may best be treated with bladder preservation therapy ( 1 , 13 ) . according to previous reports , pure and predominant lelca is more favorable than focal lelca in prognosis ( 2 , 3 , 7 ) . this case was the predominant type that lelca accounted for over 90% of lesions and was fractionally accompanied with non - invasive transitional cell carcinoma . the differential diagnosis is usually malignant lymphoma , invasive transitional cell carcinoma , squamous cell carcinoma and small cell carcinoma . ( 5 ) note that it is imperative to distinguish between lelca and malignant lymphoma , as primary bladder lymphoma is extremely rare . therefore , immunochemical staining , such as lca and keratin , may be used for differentiation . ( 8) maintained that immunochemical staining techniques were helpful to distinguish bladder lymphoma from undifferentiated carcinoma . pooly differentiated transitional cell carcinoma ( tcc ) with a lymphoid linfiltrate should be distinguishable from lelca in that the latter is characterizied by syncytia of tumor cell , vesicular nuclei and prominent nuclei ( 7 ) . the microscopic findings of lelca are characterized by the indistinct cytoplasmic border and the syncytial growth pattern with the prominent lymphocytic infiltrate . it is important to check cytokeratin in tumor cells on immunochemical staining , in order to ascertain that cells are originated from epithelial cells ( 1 ) . the principal symptoms of lelca were mostly gross hematuria , solitary mass , and tumors measured 1 to 5 cm ( 10 , 12 ) . the epstein - barr virus is regarded as one of factors of the lelca that occurred in the thymus gland ( 9 ) . however , it has not been elucidated that lelca of the urinary system is related with ebv ( 10 ) , gulley et al . ( 11 ) reported that ebv , detected from nasophayneal carcinoma , was not detected in 9 out of 11 cases of lelca . also in 9 cases of lelca reported by holmang et al . for lelca treatment , surgical therapy and chemotherapy can be applied . in the case of small tumors that measure 5 cm and less , turbt is applied , but in the case of big or invasive tumors , radical cystectomy may be applied . as nasopharyngeal lymphoepithelioma is well reacted to chemotherapy , methotrexate , vinblastine , doxorubicin and cisplatin may be applied to chemotherapy ( 7 ) . 13 ) reported that primary chemotherapy was performed on 3 patients with muscle invasive lymphoepithelioma of the bladder and as a result their bladder functions were salvaged . it is presumed that bladder lymphoepithelioma may be well reacted to radiotherapy , like nasopharyngeal lymphoepithelioma ( 14 ) . ( 12 ) applied radiotherapy to 4 patients , but could not evaluate whether the patients got cured successfully . lelca is a rare tumor , and it is important to differentiate it from other tumors .
a 78-yr - old woman presented with gross hematuria for 2 weeks . on cystoscopy , a frond - like mass was observed at the bladder trigone . transurethral resection of bladder tumor was performed for the mass . histopathological findings showed that 90% of lesions were lymphoepithelioma - like carcinoma ( lelca ) and a few lesions were non - invasive transitional cell carcinoma . on microscopy , syncytial growth pattern and indistinct cytoplasmic borders were observed with the severe infiltration of lymphoid cells . the case was followed - up for 8 months without recurrence . this is the first report of a lelca case in korea .
vector - borne diseases constitute a large percentage of emerging infectious diseases in both human and animal populations.1 lyme disease , a spirochetal infection transmitted by the blacklegged ixodes tick , is the most common tick - borne disease in the united states . in recent years , a growing number of additional pathogens , both viral and bacterial , have been found to be present in the tick population.2 anaplasma phagocytophilum , an intracellular rickettsial pathogen , also transmitted by ixodid ticks , has been shown to have increasing prevalence in the tick population worldwide.3 many studies to date have analyzed the prevalence rate of anaplasmosis and lyme disease in the tick population of dogs , humans , and ticks themselves.4,5 some studies have even sought to analyze the percentage of dogs infected with borrelia burgdorferi and anaplasma in a specific community in an attempt to project a potential infectivity risk for humans living within that community.6 in any static environment , dogs and humans are exposed to the same tick population and therefore have similar risks of infection for the pathogens which those ticks may carry . the purpose of this study was to compare the prevalence rates of both lyme disease and anaplasmosis in dogs and humans in the same community over a given time period . comparative analysis of these results would subsequently be beneficial to both medical and veterinary personnel in establishing a relative risk of infection for other members of that community subsequent to a tick bite , and aid those practitioners in selecting proper tests and treatment for their respective patient populations . blood samples from 202 dogs and 202 humans were tested for both lyme disease and anaplasmosis from february through october , 2013 . the 202 dogs were tested as part of a routine wellness screening at bernardsville animal hospital ( somerset county , nj , usa ) using the snap 4dx plus test ( idexx laboratories , inc . , the snap 4dx plus test simultaneously screens dogs for dirofilaria immitis ( the causative agent of heartworms ) , and for antibodies to b. burgdorferi , a. phagocytophilum , anaplasma platys , ehrlichia canis , and ehrlichia ewingii . for the purpose of this study , test results for a. phagocytophilum and b. burgdorferi were used . the 202 human patients were screened for both b. burgdorferi and a. phagocytophilum as part of an evaluation for lyme disease at a private medical practice ( somerset county , nj , usa ) . lyme disease antibodies were tested via lyme western blot analysis ( igm / igg ) and lyme c6 peptide enzyme - linked immunosorbent assay ( elisa ) , and tests for a. phagocytophilum by igg / igm elisa . infectivity rates were categorized as either negative for both infections , positive for lyme disease only , positive for anaplasmosis only , or positive for both lyme disease and anaplasmosis based on antibody testing ( table 1 ) . the majority of dogs and humans tested were negative for antibodies for both infections ( figure 1 ) . among those dogs and humans which were antibody positive , antibodies to anaplasmosis alone were the most common in both groups ; 9.40% in dogs and 16.33% in humans . the presence of antibodies for lyme disease was the second most common finding in both groups , 4.95% in dogs and 15.34% in humans . antibody positivity for both lyme disease and anaplasmosis was the least common finding in both groups , 3.96% in dogs and 10.39% in humans . both humans and dogs were more likely to show antibodies to a. phagocytophilum than antibodies to b. burgdorferi , the causative agent of lyme disease . there is increasing concern regarding the incidence of coinfections in areas highly endemic for lyme disease . many studies have quantified the prevalence rate of various tick - borne pathogens separately in humans and animals within various communities . this is the first study to date to simultaneously examine the incidence rate of two tick - borne diseases , a. phagocytophilum and b. burgdorferi in dogs and humans in the same community during a single tick season . of clinical significance , the concordance rates for anaplasmosis , lyme disease and coinfections for both diseases were remarkably similar in both groups . antibodies to a. phagocytophilum had a higher incidence than antibodies to b. burgdorferi in both the dogs and humans tested . other studies comparing the prevalence of a. phagocytophilum and b. burgdorferi in dogs in russia and ticks in belgium showed similar higher incidences for anaplasma compared to borrelia.7,8 this observational study has important veterinary and medical implications for the public health of those members , both animal and human , living within the community . the availability of the snap 4dx plus test affords dogs a convenient and reliable assay for diagnosis . conversely , many human patients reporting a tick bite in endemic areas are only screened for lyme disease . since antibodies to anaplasmosis were higher in both dogs and humans in this observational study , routine screening for anaplasmosis in humans would be highly advisable and would aid physicians in diagnosis and treatment recommendations .
ticks are important vectors of disease and transmit an extensive array of bacterial , viral and protozoan diseases to both humans and dogs within a community . borrelia burgdorferi , the causative agent of lyme disease , has been extensively studied within both the human and veterinary population . anaplasma phagocytophilum , an intracellular rickettsial pathogen also transmitted by ixodid ticks , has emerged as an important zoonotic infection with significant veterinary and medical implications , and is responsible for both canine granulocytic anaplasmosis and human granulocytic anaplasmosis . multiple surveys exist in the international literature referencing infectivity rates of both of these diseases separately in both the dog and human populations . this is the first study to simultaneously examine the infectivity rate of both anaplasmosis and lyme disease in humans and dogs in a community endemic for tick - borne diseases .
rayer first reported renal vein thrombosis associated with nephrotic syndrome in 1837 , thrombosis is known to be one of the main complications of nephrotic syndrome with the renal vein being the most frequent site.[15 ] awareness of pathogenesis and assessment of the risk factors for arterial thrombosis are required to allow appropriate management of these patients . there are no reliable risk factors to suggest anticoagulation therapy in patients with nephrotic syndrome and prophylactic anticoagulants remain controversial . our case once again highlights the dilemma in managing these patients ; however it also throws some light on management . a 68-year - old retired office manager ( male ) was admitted to the hospital with worsening epigastric pain , clay colored stools , nausea , and ankle swelling . he also complained of weakness and tingling in left arm , slurred speech and difficulty getting words out . his risk factor for cerebro - vascular disease included only hypertension that was well - controlled with felodipine . on examination , there was non - pitting pedal edema , right - sided cerebellar signs ( past pointing , dysdiadochokinesia ) , left hemiparesis , and expressive dysphasia . urine dipstick had 2 + blood , 4 + protein with urinary protein creatinine ratio of 1401 ( approx . computed tomography ( ct ) and magnetic resonance imaging ( mri ) of the brain showed widespread cerebral infarcts in the middle cerebral and posterior cerebral artery territory [ figures 1 and 2 ] . protein c and s levels were at lower end of normal range that was considered secondary to renal loss rather than primary deficiency . ct of brain showing infarcts in middle cerebral artery infarcts mri of brain showing cerebellar infarct haematoxylin and eosin stain : ( a ) shows normal glomerulus . ( b ) shows vacuolated renal tubular cells suggesting heavy proteinuria and uptake of albumin by cells siver stain of glomerulus showing normal basement membrane electron microscopy of glomerulus showing flattening of podocyte foot processes other investigations for hypercoagulable state such as anti - cardiolipin antibodies , factor v leiden mutation were within normal limits . contrast ct of chest / abdomen / pelvis looking for malignancy was within normal limits . infarcts were considered secondary to arterial thombosis as a result of hypercoagulable state seen in nephrotic syndrome . patient did not receive any prophylactic or therapeutic anticoagulation with heparin or low molecular weight heparin during his hospital stay . nephrotic syndrome was treated with three doses of intravenous methyl prednisone and was started on oral prednisone at 1 mg / kg that was tapered down over a period of 3 months . there was good response to treatment with resolution of proteinuria and improvement in renal function . nephrotic syndrome is one of the secondary causes of hypercoaguable state.[68 ] hypercoagulable state can be either primary or secondary . primary causes are due to deficiencies of anti - thrombin iii , protein c or protein s. other secondary causes include : malignancy , pregnancy , and oral contraceptive use . thrombotic complications in patients with nephrotic syndrome occur in both venous and arterial systems , with the most common being renal vein thrombosis . in adults , the majority of thromboses are venous and arterial thromboses are more common in children . review of literature shows that hypercoagulable state is greater in nephrotic syndrome secondary to membranous nephropathy . in our case , there are no data on the absolute risk of development of thromboembolism , but an observational study shows the risk of vascular thrombosis is greatest within the first 6 months of diagnosis , with an annual incidence rate of 1.48% for arterial thromboembolism . cerebral infarction in nephrotic syndrome is rare and has been previously reported in very few cases . described two adult patients , 34 and 36 years of age , who presented with acute cerebral infarction and were found to have a hypercoagulable state due to nephrotic syndrome . although it is not clear why nephrotic syndrome causes a hypercoagulable state , there have been many hypothesis for its mechanism , such as urinary loss of antithrombin iii , factor xii deficiency , protein c , and protein s deficiency , increased platelet aggregation , and increased hepatic production clotting factors.[1316 ] most authors report increased fibrinogen levels . treatment of arterial thrombosis involves anticoagulation with either conventional heparin or low molecular weight heparin or warfarin . prophylactic anticoagulation in patients with nephrotic syndrome and cerebral infarcts is unclear , and we did not give any prophylaxis in our patient . treatment of nephrotic syndrome involves general measures such as treating hypertension , hyperlipidaemia , proteinuria with angiotensin inhibiters , and edema with salt restriction and diuretics . specific treatment of nephrotic syndrome involves immunosuppression with either steroids and antimetabolites or biological agents .
we report a case of 68-year - old caucasian man who presented with cerebral infarcts secondary to arterial thrombosis associated with nephrotic syndrome . his initial presentation included edema of legs , left hemiparesis , and right - sided cerebellar signs . investigations with computed tomography and magnetic resonance imaging of brain showed multiple cerebral infarcts in middle cerebral and posterior cerebral artery territory . blood and urine investigations also showed impaired renal function , hypercholesterolemia , hypoalbuminaemia , and nephrotic range proteinuria . renal biopsy showed minimal change disease . cerebral infarcts were treated with antiplatelet agents and nephrotic syndrome was treated with high dose steroids . patient responded well to the treatment and is all well till date .
acute kidney injury ( aki ) is a clinical syndrome characterized by a sudden reduction in glomerular filtration rate sufficient to cause accumulation of toxic nitrogenous waste products . morbidity and mortality in aki are high despite enormous research on the patho - physiology and technological advances in its management . the contributory factors to high mortality rate in aki include late presentation , inadequate dialysis , and overwhelming sepsis . studies have demonstrated that 5% of all hospital admissions and 30% of patients admitted into intensive care unit are attributed to aki . although the incidence of pregnancy related aki ( praki ) has significantly reduced in developed countries as a result of increase in awareness programs on etiological factors and prompt treatment of volume responsive cases , it is still responsible for 15 - 30% of aki in developing nations . praki due to ante / postpartum hemorrhage , septic abortion , and toxemia of pregnancy remains a major challenge to health care givers . intermittent hemodialysis ( hd ) is the most freqent form of renal replacement therapy for aki in developing countries including nigeria . in recent times , there has been increased in awareness program on praki , improvement in gynecological and obstetric care , and upgrading of our hd facilities . it is , however , not clear if the foregoing has translated into better outcome of praki . we , therefore , undertook an 8-year retrospective study of praki in usmanu danfodiyo university teaching hospital ( uduth ) , sokoto , north - western nigeria in a bid to document our current status . an audit was undertaken of praki cases managed with intermittent hd between may 2007 and april 2015 at the renal care centre of uduth , sokoto , the only tertiary and referral centre for sokoto , kebbi , and zamfara states with a population of over 3 million people . patient demographics , causes of aki , clinical features , duration of pregnancy at diagnosis , number of dialysis sessions , and outcome were obtained from the patients records . the diagnosis of praki was based on sudden onset of oliguria / anuria and/or serum creatinine elevation of > 2.5 mg / dl in a setting of pregnancy . preeclampsia , eclampsia , postpartum of aki and rifle were defined as in a previous study . the data were analyzed using spss version 20 ( statistical package for the social sciences , a proprietary software by ibm corporation . quantitative variables were expressed as percentages and qualitative variables as median or mean . a p < 0.05 was taken as the level of statistical significance . a total of 38 cases of praki with an age range between 15 and 30 years were dialyzed during the period under review . oliguria , body swelling , clinical pallor , fever , hypertension , and hypotension were the observed clinical features as shown in table 1 . clinical characteristics of the patients laboratory parameters at the first session of dialysis duration of pregnancy at the diagnosis of aki revealed that the majority was in the third trimester accounting for 55.3% followed by postpartum period ( 26.3% ) , first trimester ( 13% ) , and second trimester ( 5% ) , respectively . the hd sessions ranged from 1 to 6 times and majority ( 61% ) required 3 sessions before full recovery of renal function . all patients had dialysis as at when due because the unit has a policy of commencing dialysis before payment is made for all cases of aki . it was only 5 of the 21 patients who survived that came for follow - up and in fact , one of them was pregnant with normal renal function at follow - up visit [ figure 1 ] . praki is a disease of young women ; the youngest in our study was 15-year - old , while the oldest was 30-year - old . this finding is in accord with report by najar et al . and also similar to observations by arrayhani et al . who reported the age ranged from 18 to 40 years a major contributory factor is early marriage , which is a very common practice in this part of the country and , is closely linked with islamic practices . the majority of the patients were un - booked and primigravidas , which was not surprising because of the general low level of education and poor health care facilities in sub - saharan africa including nigeria . the level of education in this part of nigeria is very low , in particular , among females and is associated with low health care seeking behavior . oliguria was common in our study and is related to blood loss as ante / postpartum hemorrhage . the previous studies have alluded to the fact that oliguria is a frequent feature in aki of varied etiologies . prerenal aki is the leading form of aki worldwide and oliguria is attributable to poor renal perfusion . praki was very frequent in the third trimester ( 55% ) , and similar result was observed in pakistan ( 86% ) , but our findings contrast with report from india where majority were in postpartum period ( 75.6% ) . the major causes of praki in the third trimester included eclampsia , ruptured uterus , and ante - partum hemorrhage . two cases of praki that occurred in the second trimester in our study were attributable to sepsis and pregnancy - related hypertension . septicemia as a major cause of aki in developing countries has been reported in previous studies . it is not surprising that prerenal aki features dominated the biochemical parameters of our patients which are in accord with findings from an indian study . the mortality was higher among patients that presented in the third trimester and postpartum period . studies have shown that postpartum eclampsia is associated with unfavorable outcomes ; hence , the poor clinical outcome among our patients is not surprising . praki is a very serious complication of pregnancy with associated high morbidity and mortality . in conclusion , praki is a disease of young women in their reproductive age group with a poor outcome in a setting of predominantly preventable etiologies . a more intensive health education and improvement in gynecological and obstetric care has the potential of impacting positively on the outcome of praki .
pregnancy related acute kidney injury ( praki ) patients that underwent hemodialysis ( hd ) between may 2007 and april 2015 were studied with specific reference to clinical features , laboratory values , duration of pregnancy at the diagnosis of acute kidney injury and outcome . it involved 38 patients aged between 15 and 30 years . the main clinical features were fever , edema and oliguria . the leading etiological factors included ante / postpartum hemorrhage , septic abortion , and toxemia of pregnancy . the majority of cases occurred during the third trimester . praki is a dreaded complication of pregnancy with high morbidity and mortality . hd improved patient survival in our study .
systemic sclerosis ( ssc ) is a connective tissue disorder characterized by internal organ involvement , dermal thickening and fibrosis . ssc is categorized into two groups : limited cutaneous ( lcssc ) and diffuse cutaneous ( dcssc ) forms . the former ( the crest syndrome ) is characterized by calcinosis , raynaud 's phenomenon , sclerodactyly , esophageal dysmotility , and telangiectasia . although joint involvement is not so common in lcssc , arthritis is reported to occur at a rate of 15% . hand joints are usually affected , followed by wrist , elbow , and knee joints . however , the sternoclavicular joint ( scj ) is rarely involved in limited cutaneous ssc , and there is insufficient information about this involvement . the primary treatment of such involvement is conservative , involving nonsteroidal anti - inflammatory drugs , rest , and cold application . in patients unresponsive to conservative treatment although ultrasonography ( us ) has been increasingly used for interventional pain management in recent years , other imaging techniques are also used for intra - articular injection . one of these techniques , fluoroscopy - guided scj injection has been scarcely covered in the literature . in this report , we present the technique of fluoroscopy - guided intra - articular steroid injection and its outcomes in scj arthritis resistant to conservative management in a patient with limited cutaneous ssc . a 50-year - old woman diagnosed with limited cutaneous ssc two years earlier presented to the rheumatology outpatient clinic with swelling and pain on the right side of her neck and the ventral surface of her chest for 3 months . she had been using hydroxychloroquine 200 mg bid , nifedipine 30 mg / day , and acetylsalicylic acid 100 mg / day . laboratory tests revealed antinuclear antibody 1/1000 + , anticentromere antibody + + + , and crp : 9,49 mg / dl , while other parameters were normal . mri revealed signs of inflammation consistent with right - sided sternoclavicular joint arthritis ( fig . 1 ) . diagnosed with scleroderma - associated sternoclavicular joint arthritis , the patient was started on prednisolone 10 mg / day . having no satisfactory relief of her pain despite a 10-day trial of medical therapy , the patient was referred to our pain medicine outpatient clinic . on physical examination she had tenderness , swelling , and minimally increased temperature on her right scj upon palpation . her pain was rated 8 on the nrs ( numeric rating scale ) . a fluoroscopy - guided intra - scj steroid injection was planned . after informed written consent was given for the scj injection , the patient was placed in a supine position on the fluoroscopy table and monitored . skin overlying the area of interest was cleansed with povidine iodine and covered with a sterile drape . the right scj was palpated and then imaged with the aid of fluoroscopy in the anteroposterior view . under fluoroscopic guidance , a 5-cm , 21 g needle was entered into the right scj . the contrast material was injected to verify an intra - articular location of the needle ( fig . 2 ) . then , a mixture of 20 mg methylprednisolone and 0.5 cc bupivacaine 5% was injected . the nrs level was 0 and there was no tenderness in joint palpation at control examinations at the 1st hour , 1st month , and 3rd month . in this case , steroid injection into scj was done under fluoroscopy guidance for scj arthritis secondary to limited cutaneous scleroderma . pain level dropped to a level of nrs 0 at the control examinations at the 1st hour , 1st month , and 3rd month from a preprocedural level of nrs 8 , indicating 100% pain relief and improvement in examination findings . there are only a few reports about steroid injection into the scj under fluoroscopy guidance . in which 40 mg methyl prednisolone and 1 ml bupivacaine were injected into the scj under fluoroscopy guidance in a patient with osteitis condensans of the clavicle . that patient experienced an immediate relief of pain following the procedure , and the outcomes of the procedure were consistent with that of our patient , whose pain had regressed to nrs 0 at the 1st hour . that case had some differences from ours , including the lack of reporting of long - term outcomes ; using 40 mg methylprednisolone ; and applying an scj injection for osteitis condensans of the clavicle . in a retrospective analysis , peterson et al . explored the 20 - 30 minute results of the ct - guided injection of triamcinolone 40 mg into a total of 83 scjs of 50 patients with osteoarthritis or scj subluxation . pain relief was achieved in a short period in a majority of patients , as was the case in our study . that study , however , differs from our study with regard to reporting a ct - guided procedure ; including patients with different etiological causes ; and providing long - term follow - up data . our technique has the significant advantage of using a lower radiation dose with fluoroscopy - guided technique compared to the ct - guided technique . furthermore , taking into account the small size of scj and the possible risks of steroid injection , we preferred using a lower steroid dose compared to the cases of galla et al . and peterson et al . this case is an important contribution to the literature due to its reporting the rare scj scj involvement in a patient with limited cutaneous scleroderma , obtaining an easy access to the target area under fluoroscopy - guidance , showing a lower complication risk , and providing long - term treatment outcomes . however , it also has some limitations , including the lack of reporting long - term treatment outcomes , exposing the patient to radiation unlike the us - guided procedure , and requiring special hardware and equipment unlike the blind procedure . ultrasound provides the advantages of injection accuracy , reliability , and the ability to perform real - time image - guided procedures . in this case , fluoroscopy - guided injection was preferred , as we had much more experience with fluoroscopy - guided injections than with ultrasound - guided injections . in conclusion , scj arthritis should be considered among the etiological causes of chest pain in patients with limited cutaneous systemic sclerosis . for these patients , intra - scj steroid injection is an effective treatment option when pain does not respond to conservative treatment methods . among injection options , fluoroscopy - guided intra - sternoclavicular joint steroid injection should be regarded as a safe alternative method .
we report a case of fluoroscopy - guided intraarticular steroid injection for sternoclavicular joint ( scj ) arthritis caused by limited cutaneous systemic sclerosis ( ssc ) . a 50-year - old woman diagnosed with limited cutaneous ssc presented with swelling and pain in the right scj . mri revealed signs of inflammation consistent with right - sided sternoclavicular joint arthritis . after the failure of oral medications , we performed fluoroscopy - guided injection in this region . she reported complete resolution of her symptoms at 4 and 12-week follow - ups . this outcome suggests that a fluoroscopy - guided scj injection might be a safe and successful treatment option for sternoclavicular joint arthritis .
relapsing polychondritis ( rp ) is an uncommon autoimmune inflammatory disease that may affect cartilage throughout the body . we report on a case of fever of unknown origin in which 18f - fluorodeoxyglucose positron emission tomography / computed tomography ( 18f - fdg pet / ct ) was performed to make a diagnosis of rp . our case demonstrates that the use of 18f - fdg pet / ct is a useful diagnostic tool to accurately determine the extent of inflammation throughout the body which can be identified by an increased 18f - fdg uptake . relapsing polychondritis ( rp ) is an uncommon autoimmune disorder that is characterized by recurrent inflammation and destruction of the cartilaginous tissue in various sites of the body with high risk of misdiagnosis . auricular and nasal cartilage is usually the first to be affected at the onset of the disease . airway involvement is present in up to 50% of patients with rp and is a major cause of morbidity and mortality . a 67-year - old man was admitted to hospital for presented with a 2-month history of persistent cough with sputum and 10-day history of low - grade fever . additionally , the patient 's auricles swelled rapidly before admission , and the left one is markedly enlarged . diagnostic antibacterial and antituberculous therapy did not cause any significant improvement in the symptoms and he had a persistent fever of 39 c or higher . the patient then underwent an 18f - fdg pet / ct examination as a systemic search to explain fever of unknown origin . pet images and pet / ct fusion images demonstrated intense symmetric fdg uptake in auricle , larynx , tracheobronchial tree and all intercostal cartilages ( fig . fdg was also accumulated in hilar , mediastinal , and axillary lymph nodes ( fig . 1c , d ) . maximum intensity projection ( mip ) also showed intense fdg uptake in these tissue and nasal cartilages ( fig . the patient underwent a tragus cartilage biopsy , and pathological results showed cartilaginous lymphocytic infiltration and degeneration , in line with the performance of osteochondritis . in addition , the computed tomographic ( ct ) scan showed the corresponding parachondral soft tissue around intercostal cartilages was swollen and the wall of tracheobronchial tree was thicken . the lymph nodes mentioned above were enlarged ( please confirm it on ct ) ( fig . two months after starting therapy , the laboratory data , such as whole blood count and esr , had returned to normal levels . pet images ( left ) , unenhanced ct images ( middle ) , and the pet / ct fusion imagings ( right ) of auricle , larynx , trachea , and all costal cartilages and the axillary , hilum , and mediastinal lymph nodes , respectively . coronal ( a ) , sagital ( b ) , and mip pet images showed moderate fdg accumulation in the nasal cartilages , laryngeal cartilages , all costal cartilages , tracheobronchial tree and the axillary , hilum and mediastinal lymph nodes . rp is an uncommon autoimmune disease without clear process , and often occur with connective tissue disease simultaneously . it can affect multiple organs including nose , ears , peripheral joints , tracheobronchial cartilage and eyes , heart and skin . its diagnosis is mostly based on clinical manifestations and symptom - driven diagnostic testing multidetector . the corresponding clinical manifestations are ear cartilage inflammation , nasal cartilage inflammation , peripheral non - erosive polyarthritis , episcleritis , keratitis , and other multisystem diseases which involve respiratory system , cardiovascular system , and nervous system . as the disease progresses , respiratory tract involvement usually perform affect larynx , trachea , bronchus stenosis , bronchiectasis , pneumonia , atelectasis , etc , in which the main airway and the left and/or right bronchus stenosis are most common . davis et al reported that not just the main airway and the left and right bronchial stenosis but the surrounding small bronchi may also be involved . the diagnosis bases on clinical manifestations , and no specific laboratory methods or specific histologic findings are considered pathognomonic for rp . awareness should be raised about systemic multiple cartilage damage associated with unexplained chronic cough , sputum , hoarseness , wheezing , and even dyspnea . multidetector ct can clearly define the location and extent of the fixed airway narrowing and wall thickening . bronchoscopy can visually observe the edema , thickening of bronchial wall , and disappeared cartilage ring as an invasive operation . tc-99 m methylene diphosphonate ( mdp ) bone scintigraphy has also been used to assess rp . some case reports demonstrated that scintigraphic findings were improved after prednisolone therapy , therefore , mdp scanning may be a valuable method in the follow - up of rp . our case demonstrates that although the diagnosis is mainly established clinically , the use of 18f - fdg - pet / ct has been proven to be a useful diagnostic tool to accurately determine the extent of inflammation throughout the body . several studies have reported clinical value of fdg pet / ct imaging for the diagnosis of rp . most of them well depicted tracheobronchial tree and intercostal cartilages involvement , but the nasal cartilages , larynx , and reactive lymphadenopathy were rarely mentioned entirely , as was noted in our case . currently , there is no ideal treatment of this disease . primary therapy includes corticosteroids , dapsone , and other immunosuppressants , which are partly useful for acute episode . for the tracheobronchial stenosis and/or softening , intratracheal stent implants can significantly improve respiratory symptoms , which is an effective treatment . in conclusion , fdg pet was found to have a growing role in the diagnosis and follow - up of relapsing polychondritis .
abstractbackground : relapsing polychondritis ( rp ) is an uncommon autoimmune inflammatory disease that may affect cartilage throughout the body.case report : we report on a case of fever of unknown origin in which 18f - fluorodeoxyglucose positron emission tomography / computed tomography ( 18f - fdg pet / ct ) was performed to make a diagnosis of rp.conclusion:our case demonstrates that the use of 18f - fdg pet / ct is a useful diagnostic tool to accurately determine the extent of inflammation throughout the body which can be identified by an increased 18f - fdg uptake .
cystic changes of the spleen are very rare . based on the presence or absence of cellular lining of the cystic wall , splenic cysts are classified as primary ( true ) and secondary ( pseudo ) although carbohydrate antigen 199 ( ca 199 ) is a well - known marker for pancreatic adenocarcinoma , high levels of ca 199 may be seen in patients with benign gastrointestinal , hepatobiliary , and splenic diseases . so far , elevated ca 199 serum levels accompanying splenic cysts have been reported in approximately 30 cases , most of which are of epithelial origin . because the spleen has an important immunologic role , splenic conservation techniques , such as partial splenectomy , cystectomy , marsupialization , and cyst decompression , for the treatment of benign splenic cysts are more favorable . with all the advances in laparoscopic surgery , all conservative approaches can be done laparoscopically . among these techniques , laparoscopic cystectomy is less invasive and has the lowest morbidity . it also has the benefit of reducing postoperative patient discomfort and offers a shorter hospital stay than other surgical laparoscopic techniques . so far , only one case of laparoscopic cystectomy for splenic epidermoid cyst with elevated ca 199 cyst has been published . this is the second known report of this procedure being used to remove a splenic epidermoid cyst in a patient with elevated ca 199 . a 28-year - old woman , in otherwise excellent health , was admitted to our general surgery department with a chief complaint of left upper quadrant pain . biochemical investigations showed an elevated ca 199 level ( 56.9 u / ml ; normal : 0 - 27 u / ml ) . the indirect hemagglutination test and computerized tomography ( ct ) and magnetic resonance imagining ( mri ) of the abdomen demonstrated a cyst , 7156 mm in diameter , between the upper pole of the spleen and the stomach ( figure 1 ) . the aspirated cystic contents showed epithelial fragments with no evidence of malignancy ; however , the ca 199 level of the content was high . this finding confirmed the diagnosis of a splenic epidermoid cyst ( figures 2 and 3 ) . the patient was placed in the right decubitus position under general anesthesia . on exploration , a 7060-mm cystic lesion at the upper pole of the spleen , which was pushing the stomach to the left , the short gastric vessels and tissues between the spleen and the cyst were divided using the ligasure ( valleylab , co , usa ) . to complete the laparoscopic cystectomy , follow - up ct of the abdomen after 6 months showed no recurrence of the cyst . the patient is doing well with normal ca 199 levels and no recurrence for 16 months . splenic cysts may be primary ( true ) or secondary ( pseudocyst ) , depending on the presence or absence of an inner cellular lining . most of the true splenic cysts are epithelial in origin and accepted as having embryonic inclusion of epithelial cells from adjacent structures . although ca 199 is a reliable marker for pancreatic adenocarcinoma , both normal and tumoral human epithelial cells may produce it . high serum levels of ca 199 may accompany benign gastrointestinal diseases , such as liver cirrhosis , acute cholangitis , obstructive jaundice , and benign pancreatic conditions . a patient with splenic cysts can present with abdominal pain , splenomegaly , atelectasis , and left shoulder pain , and occasionally infection or abscess formation even rupture or hemorrhage . the first case of a splenic cyst producing elevated serum ca 199 was reported by terada et al21 in 1994 . since then , elevated ca 199 serum levels have been reported in about 30 cases of splenic cysts , most of epithelial origin . complete removal of the cyst is needed to prevent recurrence . because the spleen has an important immunologic role , especially in the body 's defense against encapsulated bacteria , splenic conservation techniques for the treatment of benign splenic cysts is the aim of surgical treatment . thus , treatment options for splenic epidermoid cysts , especially in young patients , because it marks the beginning of a disease , may include partial splenectomy , total cystectomy or fenestration , accessed either by laparotomy or laparoscopy . conservative treatment is not possible when the spleen is almost totally occupied by the cyst , and total splenectomy is required for eradication . different laparoscopic approaches , such as splenectomy , partial splenectomy , cystectomy , marsupialization , and cyst decompression , have been reported for splenic true and pseudo cysts . robert et al reported the first case of successful laparoscopic complete excision of a splenic epidermoid cyst , and sardi et al reported in 1998 the first case of laparoscopic cystectomy of a splenic epidermoid cyst producing high levels of ca 199 . complete removal of the splenic cyst is curative and remains a good choice for safe treatment in cases with epidermoid splenic cyst . laparoscopic splenic cystectomy is the surgical approach of choice for the splenic epidermoid cyst due to its minimal invasiveness , low morbidity , reduced postoperative pain and discomfort and shortened hospitalization while achieving the same technical success as an open approach . this is the second reported case of laparoscopic cystectomy of a splenic epidermoid cyst in a patient with elevated ca 199 . the use of ligasure made the laparoscopic cyst excision safe , expeditious , with minimal blood loss .
true nonparasitic splenic cysts are rare . reports of approximately 30 cases of benign true splenic cysts producing elevated ca 199 serum levels have been published . the traditional treatment of splenic epidermoid cysts is splenectomy . however , with all the advances in laparoscopic surgery , conservative laparoscopic approaches are accepted as the most preferred techniques for this benign disease . laparoscopic cystectomy with its minimal invasiveness and low morbidity is the ideal technique for properly selected patients . so far , only one case report of laparoscopic cystectomy for splenic epidermoid cyst with elevated ca 199 levels has been published . this is the second known report of this procedure being performed to remove a splenic epidermoid cyst in a patient with elevated ca 19 - 9 levels .