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congenital adrenal hyperplasia ( cah ) refers to a group of autosomal recessive disorders caused by an enzyme deficiency which leads to defects in biosynthesis of steroid precursors . depending on the severity and degree of 21 hydroxylase deficiency , the clinical spectrum may vary from mild form of non classical cah to classic cah . however , the non classical cah variant is more common with a prevalence rate of 1 in 1000 . it also helps in maintaining normal levels of precursors by suppressing adreno cortico trophic hormone ( acth ) . during childhood , the management is largely focused on achieving normal growth and attaining appropriate final adult height . johns medical college hospital , bangalore by the department of endocrinology on patients diagnosed to have cah and seen in the outpatient clinic between january 2012 and october 2012 . during this period data regarding demography , clinical presentation at time of diagnosis , treatment details , height sds and bmi were collected . all patients underwent biochemical testing for 17 hydroxy progesterone ( 17 ohp ) levels for assessment of adequacy of therapy . bone age assessment with left hand and wrist bmi was calculated for all patients and obesity was defined using who charts as values above 95th percentile . 17 ohp levels between 1 ng / ml and 12 ng / ml were considered appropriate ; values below 1 ng / ml suggested suppression and over treatment and values above 12 ng / ml suggested under treatment . 29 patients were included in the study of which 22 were females ( 76% ) and 7 were males ( 24% ) . based on the cross sectional data collected , 11 patients were adults ( age > 18 ) and 18 patients were children ( 62% ) . among the males , one child was identified at birth via a neonatal screening program , one child presented with early pubarche , the other 5 infants presented between their 3rd and 6th week of life with features suggestive of adrenal crisis - poor feeding , vomiting and failure to gain weight . one of the male patients incidentally also had a penoscrotal hypospadias which was surgically corrected . among the females , 9 infants were identified at birth due to presence of genital ambiguity ( 40% ) , 1 presented with symptoms of adrenal crisis at 4 weeks of life , 4 patients presented in the pre pubertal period due to early onset adrenarche ( 18% ) , 5 patients presented in the late adolescent period with marked virilization ( 23% ) and 3 patients presented with features of poly cystic ovarian disease ( pcos ) . all the five patients who presented in the late adolescent period had obvious genital ambiguity from birth , however , they sought medical attention only much later due to marked virilization and failure to attain menarche . one among them had actually been evaluated and even underwent a clitoroplasty at a young age , but unfortunately , the diagnosis of cah was missed as she was mistakenly categorized as probable ovo testicular dsd due to presence of mullerian structures with a phallic length of around 6 cm . all these 5 women had a masculine built at presentation with poor breast development , severe hirsutism , muscular body habitus , temporal balding and varying degrees of deepening of voice . the non classical cah ( nccah ) patients presented in adulthood with complaints of irregular cycles and hirsutism ; they had no features of virilisation . simple virilizing was the most common sub class seen among the women ( 81% ) whereas the salt wasting type was predominant among boys ( 85% ) . around 35% of patients had short stature as defined by height sds < -2.0 ; 2 patients who presented with adrenarche had increase in height sds , however , their bone age was also correspondingly advanced . all the children were treated with hydrocortisone ; almost all adult patients were on dexamethasone . none of the nccah patients were treated with glucocorticoids ; they were on anti - androgen therapy along with oral contraceptive pills . most adult classic cah patients with complaints of hirsutism were treated with either spironolactone or finasteride . pubertal induction with ethinyl estradiol was initiated for three of the adult patients who sought medical care late . analysis of 17 ohp levels revealed that 32% were suppressed with levels less than 1ng / ml ; appropriate in 47% and inadequate in 21% of patients . we report the phenotypic features of a cohort of patients with cah being evaluated and managed in a tertiary centre in southern india . in this series , the typical presentation of male babies were with adrenal crisis between the 3 and 6 week of life . around 20% of female patients with classic cah were identified and appropriately treated only after late adolescence even when genital ambiguity was present since birth . short stature was seen in one third of patients on therapy and the average final adult height among patients with classic cah was 142.37 , which was significantly lower than the mean adult height for females in our population . the mean final adult height of 157.5 cm for those with nccah was better than their classic cah counterpart . obesity and hypertension were not found to be significantly higher in this cohort of cah patients . one third of patients had suppressed 17 ohp levels suggestive of over replacement therapy which may also contribute to the reduction in the final adult stature .

congenital adrenal hyperplasia is a group of autosomal recessive disorders caused by enzyme deficiency which leads to defects in biosynthesis of steroid precursors . most common is 21 hydroxylase deficiency . clinical spectrum varies from non - classical cah to classic cah , and it may be simple virilising form or salt - wastinfg type . 29 patients were included in our study from january 2012 to october 2012 . 76% were females . male babies typically presented with adrenal crisis between 3rd to 6th week of life . around 20% of females were identified and appropriately treated only after late adolescence . short stature was seen in 1/3rd of patients . 1/3rd of patients had suppressed 17 ohp levels suggestive of over - replacement therapy which may contribute to final reduction in adult height .

the femoral head often leads to healing complications , while the more predictable prosthetic replacements are associated with poorer function and significant complications . the treatment of these fractures depends on the age of the patient , fracture displacement , bone quality , timing of surgery and activity level of the patient . displaced fractures in healthy , active patients are best treated by reduction and internal fixation . there are however , complications unique to femoral neck fractures which are almost impossible to predict . non - union and avascular necrosis non - union usually can definitely diagnosed within a year of fracture fixation with the same being achieved within 3 months at times . after non - union has been established , intervention is inevitable . the decision to proceed in the management of failed fixation is based on the careful consideration of various factors . in young patients revision internal fixation with cancellous or muscle pedicle bone grafting ( vascularised bone graft ) or an osteotomy results in useful outcome . we report a case of non - union of the fracture of the neck of the femur , who refused additional procedures after his non - union had been established . the study conforms to the declaration of helsinki and was approved by the institutional ethical board . a 38-year old male businessman reported to the out door department of our hospital with a history of a fall from height . clinical and radiological examination revealed a displaced fracture of the neck of the femur which was graded as garden type 4 ( fig . the patient was operated within 24 h. intraoperatively the garden alignment index was used as a yardstick of acceptable reduction . the fracture was fixed with 3 ao 6.5 mm cannulated screws in an inverted triangle . onward the patient complained of persistent but mild groin pain on ambulation which was assisted . the patients conservative line was continued till 1 year when his symptoms continued and radiologically there was no progress . at this point twenty - six months into the post operative followup the patients pain subsided and radiographs started showing signs of union . a radiograph taken 32 months after the fixation showed full union . non - union after femoral neck fracture can be defined as a lack of radiographic evidence of union 6 months after the fracture . delayed or non - union often manifests as continued pain with weight bearing beyond 3 months post fixation . incidence of non - union of femoral neck fractures has been reported to be between 2 and 22% and generally becomes apparent within 1 year [ 69 ] . 3radiograph at 32 months depicting union garden 4 type fracture of the neck of the femur showing persistent non - union at 2 years radiograph at 32 months depicting union anatomical reduction and rigid internal fixation are the two important surgeon controlled factors that may contribute to outcome . the preferred treatment for symptomatic non - unions in the elderly is prosthetic replacement . in the young active patients , if failure is due to technical errors , revision open reduction and internal fixation may be adequate . our case demonstrates that as long as the implant is holding and the patient is regularly followed up good results might be expected in cases as far as 32 months into the post fixation period .

fracture of the femoral neck continues to be a vexing clinical and therapeutic challenge for the orthopedic surgeon . the fracture has a propensity for non - union and avascular necrosis . it is a challenge for the orthopedic surgeon to decide when to intervene in a case with non - union where the implant continues to be in place . we present a case with persistent clinical and radiological non - union signs where the fracture eventually united after 32 months . the case bolsters the view that a continued conservative regime might entail good results in such situations .

granuloma faciale ( gf ) is an uncommon , benign , inflammatory skin disorder of unknown etiology . it is characterized by single or multiple , grey - brown or violaceous nodules or plaques primarily occurring on the face and occasionally at extra - facial sites . the disease is notoriously resistant to therapies and often tends to relapse when treatment is discontinued . we present a patient with multiple lesions of gf and its response to topical tacrolimus . a 35-year - old female presented to our department with a 5 year history of single , asymptomatic , grey - brown pigmented , nodule over the left cheek [ figure 1 ] . it started as a pin head sized papule which gradually increased to 2.5 cm 1.5 cm in size . two years later similar lesions appeared on the forehead , both arms and upper back . past and personal history was unremarkable . before treatment single , grey - brown nodule with prominent follicular orifices over left cheek . after treatment residual lesion after three months of tacrolimus application general physical and systemic examination was normal . cutaneous examination revealed multiple , well - defined , grey - brown , indurated , non - tender plaques , varying in size from 0.5 cm 0.5 cm to 1.5 cm 2.5 cm , present on the left cheek , left forehead , both arms and upper back . multiple grey - brown plaques over upper back routine hematological and biochemical investigations were normal . skin biopsy ( 4 mm ) from plaque revealed normal epidermis with clear sub epidermal grenz zone and pan dermal dense infiltrate comprising of neutrophils , lymphocytes , histiocytes and plasma cells . small dermal vessels showed infiltration of neutrophils in the vessel wall along with peri - appendageal and peri - neural infiltrate in subcutaneous fat [ figure 3 ] . ( h and e , 100 ) skin biopsy with normal epidermis and dense , mixed inflammatoey infiltrate beneath a narrow grenz zone in the dermis . infiltrate is composed of mononuclear cells with neutrophils and eosinophils she was started on intralesional triamcilone acetonide 10 mg / ml injection monthly with tab . six sessions of cryotherapy were performed once monthly after which she developed erythema and itching over the plaques and discontinued treatment . the lesions showed 40 - 50% improvement after 3 months of therapy [ figure 1 ] . classically , red - brown or violaceous nodules or plaques with associated telangiectasia and follicular accentuation are seen on the face over sun - exposed sites . differential diagnosis includes lupus pernio , lupus vulgaris , lymphoma , discoid lupus erythematosus and deep mycotic infection . skin biopsy is characterized by a mixed inflammatory infiltrate with a predominance of neutrophils and eosinophils in the dermis , in conjunction with small vessel vasculitis . there is a grenz zone that separates the infiltrate from the epidermis and pilosebaceous units . the disease is notoriously resistant to therapies and often tends to relapse once the treatment is discontinued . several medical and surgical modalities like topical and intralesional corticosteroids , cryotherapy , pulsed dye laser , puva , systemic corticosteroids , dapsone and antimalarials have been tried with variable success rates . carbon dioxide laser has also been used in a case of recurrent gf . surgical excision has been performed with often unsatisfactory results . ablative procedures may leave residual pigmentation and scarring , whereas long - term application of corticosteroids is associated with skin atrophy , telangiectasia and other possible adverse effects . in recent years successes with topical calcineurin inhibitors has been reported . several authors have reported complete or near - complete resolution of lesions after application of topical tacrolimus 0.1% ointment.[48 ] treatment regimens , duration and time to resolution of lesions have varied in these case reports [ table 1 ] . others have found time to resolution to be between 4 and 6 months . in our patient , treatment with tacrolimus 0.1% ointment twice daily for 3 months has resulted in improvement . reported cases of successful treatment of granuloma faciale ( gf ) with topical tacrolimus tacrolimus inhibits t - cell proliferation , production and release of several pro - inflammatory cytokines like interleukin-2 ( il-2 ) , il-4 , tumor necrosis factor - alpha , and interferon - gamma ( ifn - gamma ) . although the pathogenesis is still unknown , it has been suggested that gf may be an ifn - gamma mediated disease . in addition , an increased production of il-5 , probably induced by the clonal expansion of a locally recruited t - cell population may enhance the attraction of eosinophils into the lesions of gf . therefore , a possible mechanism of action of topical tacrolimus in this condition may be the inhibition of ifn - gamma and il-5 production and release , induced by the down - regulation of the t - cell activity , primarily involving the calcineurin binding and inactivation . however , we did observe eosinophils in skin biopsy in our case , probably since the biopsy was taken after one year of oral dapsone . our patient experienced a relevant improvement within 3 months of treatment with tacrolimus ointment after no response with intra lesional steroids , dapsone and cryo therapy . in conclusion , the previous reports and our observation suggest that topical tacrolimus may be a well - tolerated , efficacious therapy for gf .

granuloma faciale ( gf ) is a chronic condition characterized by red - brown plaques with follicular accentuation present usually on the face . we present a case of 35-year - old female with 5 year history of plaques over cheek and extra facial sites consistent with gf and its response to topical tacrolimus . this case supports previous reports of successful treatment of gf with topical tacrolimus .

chronic myelomonocytic leukaemia ( cmmol ) is a rare disease with an incidence of 0.370.72 per 100 000 population . the median age at diagnosis varies between 65 years and 75 years [ 14 ] . the radical treatment is allogeneic stem cell transplantation , although this treatment is unsuitable for most patients because of their advanced age [ 6 , 7 ] . the median survival of patients with cmmol is 1520 months [ 5 , 6 , 8 ] . several case reports have indicated that excess surgical stress causes an acute blastic crisis , which contributes to a poor outcome [ 9 , 10 ] . a 68-year - old man complained of having a reduced appetite for approximately 5 months and weight loss during the previous 4 months . on 12 may 2012 , he visited a nearby general internal medicine clinic for the evaluation of left lower quadrant abdominal pain . he was referred to the department of internal medicine at asahi general hospital ( asahi , japan ) for investigation and treatment . he began taking oral hydroxyurea ( hydrea ; bristol - myers squibb , princeton , nj , usa ) . he was unable to stop the gingival bleeding that had been ongoing for 5 hours . he then visited the emergency department of asahi general hospital and was referred to the department of dentistry and oral surgery . at the initial visit the right molar teeth ( 47 and 48 ) showed grade iii mobility , and their gingivae were painful with continuous bleeding . 1a ) . the right molar teeth ( 46 and 47 ) showed class iii alveolar ridge deficiency on panoramic radiographs and were mobile teeth ( fig . we packed an absorbable haemostat ( surgicel ; ethicon , somerville , nj , usa ) into the gingivae and sutured 30 silk threads above it . finally , we used surgical dressing packs ( coe - pak ; g.c . figure 1:(a ) the oral photograph at the initial visit , immediately after the gauze with the astringent has been removed . ( b ) the oral photograph after the extraction of teeth 47 and 48 . ( a ) the oral photograph at the initial visit , immediately after the gauze with the astringent has been removed . the internal medicine department assigned a clinical diagnosis of cmmol , based on the blood and bone marrow examinations conducted on 13 june 2012 . computed tomography on june 25 indicated that bone absorption had nearly reached the right inferior alveolar canal , and a ring of sequestrum was present under tooth 47 ( fig . we diagnosed right mandibular osteomyelitis , based on these clinical features . on 11 july 2012 , the patient was hospitalized in the department of haematology ward because of significant anaemia and decreased platelet numbers . on the same day , the internal medicine specialist placed him on intravenous piperacillin tazobactam ( zosyn ; pfizer , new york , ny , usa ) . he afterwards complained of severe pain in the socket ( 47 and 48 ) , despite receiving the potent opioid analgesic dihydrohydroxycodeinone ( fentanyl ; daiichi - sankyo , chiyoda - ku , tokyo , japan ) . hence , we decided to apply a surgical anti - inflammatory treatment in consideration of an acute blastic crisis . a bone marrow examination on july 13 showed no signs of acute blastic crisis of his cmmol . after we consulted an internal medicine specialist , we planned a surgical procedure to clean the inflamed region in his mouth . on the day before the surgery , he was administered a transfusion of platelet concentrate ( 20 units ) and piperacillin tazobactam to prevent bleeding and further infection . on july 20 , with the patient under local anaesthesia , we surgically resected the right lower sequestrum and extracted molar 37 , which displayed severe caries . the blood examination indicated improvement in the platelet count after the transfusion . after performing an inferior alveolar block , we removed the necrotic bone with ultrasonic bone surgery ( variosurg ; nsk , ashikaga , tochigi , japan ) until there was bleeding from the bone surface ( fig . we placed gauze with tetracycline ointment in the socket and sutured 30 silk thread to compress it ( fig . ( b ) we removed necrotic bone with ultrasonic bone surgery until there was bleeding from the bone surface . ( b ) we removed necrotic bone with ultrasonic bone surgery until there was bleeding from the bone surface . ( d ) we used 30 silk thread sutures to compress the gauze . on august 11 the formation of granulation tissue had nearly covered the surface of the bone , and all oral discomfort had disappeared ( fig . we later placed a bridge in the upper anterior teeth and added a partial denture ( fig . we placed a bridge for upper anterior teeth 12 , 11 , 22 and 23 , and a partial denture . we placed a bridge for upper anterior teeth 12 , 11 , 22 and 23 , and a partial denture . on 3 january 2013 , he admitted himself to the hospital in the haematology ward because of exertional dyspnoea . the internal medicine specialists diagnosed cmmol that was undergoing acute leukaemic transformation by bone marrow examination . he died on 10 february 2013 because of cardiac arrest , following haemorrhagic shock and cardiovascular failure due to cmmol . in all patients with cmmol , every effort must be made to use the least invasive procedure possible . in the present patient , our deepest concerns were to have a proper balance between invasiveness and healing , and to avoid evoking an acute blastic crisis [ 9 , 10 ] . our most important goal was to alleviate the patient 's discomfort by using a minimally invasive procedure to eliminate infection . based on haematology results , the oral surgery did not trigger the acute blastic crisis of cmmol in this patient . the surgery was necessary to improve his oral environment and to prevent further infection and more pain . because cmmol is associated with a risk of acute blastic crisis and a poor prognosis , any surgical procedure in these patients must be considered on a case - by - case basis .

we report a case of chronic myelomonocytic leukaemia ( cmmol ) in a 68-year - old man who developed osteomyelitis of the mandible . at the initial visit , he reported uncontrolled gingival bleeding , despite self - administered haemostasis . he complained of severe pain in the socket , despite potent opioid analgesia . after consultation with the internal medicine specialists , we undertook a surgical anti - inflammatory approach that included sequestrectomy with massive blood transfusion . his physical condition was ameliorated after the surgical procedure , and he was discharged from the hospital . however , 3 months later , he died because of cardiac arrest after haemorrhagic shock and cardiovascular failure because his cmmol had developed to an acute blastic crisis . this experience demonstrates that the most important goal in such cases is to alleviate a patient 's discomfort by applying minimally invasive actions to eliminate infection and improve the quality of life without causing deterioration in the cmmol status .

in a recent issue of critical care , we presented a series of 13 patients undergoing post - cardiotomy extracorporeal membrane oxygenation ( ecmo ) with bivalirudin - based anticoagulation . after this series , we satisfactorily continued this experience , reaching about 20 patients treated . however , we think that a word of caution should be added to our recently published experience . owing to its pharmacological profile , bivalirudin is rapidly cleaved by proteolytic enzymes , and its half - life is about 25 minutes when the renal function is normal . the use of bivalirudin for cardiopulmonary bypass ( cpb ) and ecmo is a feasible option . however , it is recommended that , during cpb , blood stagnation in the circuit be avoided because the rapid cleavage of bivalirudin may result in thrombosis . the ecmo circuit is devoid of a reservoir , and therefore blood stagnation is usually not a circuit- related problem . conversely , under some circumstances , the cardiac chambers may act as a ' natural reservoir ' , which entails blood stagnation and the risk for spontaneous intracardiac thrombosis . this is particularly true in the case of a very large right or left atrium with insufficient venous drainage or the case of very poor ventricular systolic function with intraventricular blood stagnation . to avoid this condition , we think that maintaining a partial ecmo support , leaving a minimal degree of intracardiac blood flow , may be a reasonable choice . this may be checked by echocardiographic view of heart valve movement or simply by the observation of some degree of pulsatile arterial blood pressure . we think that , in the event of echocardiographic evidence of a ' smoke effect ' within one or more cardiac chambers , bivalirudin should be replaced by standard heparin anticoagulation . intracardiac thrombus formation during ecmo with heparin anticoagulation has been described as well , but the pharmacokinetic properties of heparin may limit the risk of thrombus formation due to blood stagnation .

bivalirudin has been proposed as the sole anticoagulant in patients under extracorporeal membrane oxygenation ( ecmo ) or cardiopulmonary bypass . owing to the pharmacodynamic properties of bivalirudin , areas of blood stagnation should be carefully avoided in order to limit the risk of thrombosis . the ecmo circuit has no reservoir and is usually devoid of blood stagnation areas . conversely , under some circumstances , intracardiac blood stagnation areas may exist . in this case , there is a potential risk for the spontaneous formation of an intracardiac thrombus . we suggest that , under bivalirudin anticoagulation , a minimal degree of intracardiac blood flow with left heart valve movement is allowed .

ureterocele is a congenital urinary abnormality characterized by the presence of an intrabladder hernia or cystic ballooning of the lower end of a ureter lying between the mucosa and muscle of the bladder . the abnormality leads to urinary retention and recurrent urinary tract infection ( uti ) , which can cause irreversible damage to the kidney . this abnormality can be suspected in the fetus by antenatal ultrasonography ( usg ) and confirmed by other x - ray investigations after birth . the incidence of ureterocele is variable with the highest rate of 1:500 and it is generally found in females with duplex system association ( 95% ) . ureteroceles can have different clinical presentations , such as antenatal hydronephrosis , uti , vesicoureteral reflux ( vur ) , bladder outlet obstruction , prolapsed urethral mass , etc . usg and voiding cystourethrography ( vcug ) are essential initial procedures for a child suspected of having a ureteral anomaly . a 5-year - old female child was referred to our department for tc - diethylene triamine pentaacetic acid ( dtpa ) scan . she was a follow - up case of right - sided hydroureteronephrosis with pyonephrosis with percutaneous tube ( pcn ) insertion done on the right side . patient 's blood urea and serum creatinine were 29 and 1.3 mg / dl , respectively . usg of the abdomen revealed left - sided mild hydroureteronephrosis and gross enlargement of the right kidney , reaching up to lower abdomen with dilated pelvis and ureter . the child was referred for evaluation of glomerular filtration rate and excretory function of kidneys . tc - dtpa scan was done in our department with right pcn tube in situ . pcn tube was clamped during the acquisition of dynamic and prevoid static image and clamp released thereafter . on tc - dtpa scan , left kidney showed good perfusion and adequate cortical radiotracer concentration followed by good drainage into dilated ureter . right ureter and right pelvicalyceal system ( pcs ) were visualized in the postvoid image ( indirect evidence of vur ) [ figure 2b , thin arrow ] . retention of radiotracer was noted in dilated pcs and dilated ureter in delayed static images acquired till 4 h [ figure 2c and d ] . faint visualization of radiotracer was noted through the pcn tube after removal of clamp [ figure 2b , bold arrow ] . a large photopenia was noted in the suprapubic region in the urinary bladder ( ub ) during dynamic [ figure 1 ] as well as delayed static images acquired till 4 h [ figure 2a d ] . an usg of the abdomen was done in the radiology department of our hospital to find out the cause of this persistent photopenia in the ub . usg of the abdomen showed evidence of left - sided mild hydroureteronephrosis and grossly enlarged hydronephrotic right kidney with dilated right ureter and an intracystic ureterocele arising from the right side [ figure 3a and b ] . perfusion and dynamic images of 99mtc - diethylene triamine pentaacetic acid scan scintigraphy , posterior view with percutaneous clamp in situ , showing good perfusion and adequate cortical function and good drainage of left kidney . photopenia noted in the urinary bladder ( bold arrow ) ( a ) prevoid static image with right pcn clamp in situ . right ureter and right pelvicalyceal system visualization is noted suggesting indirect evidence of vesico - ureteric reflux ( thin arrow ) . ( c and d ) 3 and 4 h delayed static images showing persistent photopenia in the urinary bladder ( arrow marked ) ( a and b ) ultrasonography images depicting right ureterocele within the urinary bladder ( ub ) and dilated distal right ureter according to literature , 90% of the patients with ureterocele are diagnosed before the age of 3 years . most of the patients with ureterocele are classically diagnosed during the investigation for uti , asymptomatic hydronephrosis , and abdominal mass . although the age of diagnosis is decreasing , uti is still the most common clinical presentation of ureterocele in 50% of the patients promoting physician to make the thorough evaluation of the urinary system . the whole nephronourinary system could have already been negatively affected at the time of diagnosis . in our case , usg is an easy method to perform , noninvasive , and probably the best imaging modality for making the diagnosis . reflux can occur in the ipsilateral lower pole in almost half of the patients , but the contralateral system is also affected at a rate of 25% . the tc - dimercapto succinic acid ( dmsa ) scan should be undertaken routinely to assess the distribution of function in the duplex kidney and for detecting and follow - up of scarred tissue and nonfunctioning upper poles in cases of ureterocele . did a study on pediatric ureteroceles in 19 patients , on the diagnosis , management and treatment options . vcug was able to detect vur in 13 out of 17 patients ( 33% ) . dmsa scintigraphy showed ipsilateral renal scarring and nonfunctioning upper pole images in 7 out of 13 patients . computed tomography and mag3 scintigraphy did a study on the management of 36 patients with varied presentation of complicated ureteroceles . usg , micturating cystourethrogram , isotope renogram were done preoperatively in all the babies . in one of the cases , initial usg was unable to detect ureterocele in most of the cases in this study . they said that unless the usg is done in well - hydrated , cooperative patient , ureterocele is likely to be missed on usg . hence , an intravenous pyelogram , radioisotope renogram , and micturating cystourethrogram are invaluable in the complete understanding of ureterocele . in our patient also , the diagnosis of ureterocele was missed on initial usg . photopenia was noted in the ub on our scan , and this prompted us to investigate the patient further . possible causes for photopenic defects in the ub on nuclear scintigraphic studies include bladder papilloma , bladder polyp , carcinoma bladder , bladder calculus , foreign body in ub , intravesical ureterocele , etc . repeat usg revealed the cause of photopenia in ub in our case to be an intravesical ureterocele . on reviewing the literature we found that tc - dtpa scan is useful in the evaluation of renal function in patient with known ureteroceles . however , in our case on finding the photopenia in the ub on tc - dtpa scan , intravesical ureterocele was detected on usg .

ureterocele is a common ureteric anomaly detected in pediatric population . ureterocele diagnosis and evaluation need a variety of radiological methods . we report a case of 5-year - old female child sent for 99mtc - diethylene triamine pentaacetic acid scan for evaluation of glomerular filtration rate and excretory function of kidneys in view of right - sided hydroureteronephrosis and pyonephrosis with percutaneous tube in situ . incidental photopenia was noted in the urinary bladder . on ultrasonography of abdomen cause of this photopenia was found to be an intravesical ureterocele .

septic arthritis of the shoulder is uncommon in adults . it is a surgical emergency as joint destruction occurs rapidly and can lead to significant morbidity and mortality . a 43 years old male came to our outpatient department with complaints of pain and stiffness of his left shoulder . on examination , his shoulder movements were severely restricted . further evaluation with mri revealed septic arthritis of left gleno - humeral joint for which emergency arthroscopic debridement was done . septic arthritis of shoulder may not present with classical clinical features . hence , a through clinical and radiological evaluation will help us prognosticate and treat accordingly thereby preventing complications like septic shock , osteomyelitis . patients with septic arthritis usually develop moderate to severe joint pain , warmth , tenderness , effusion , restricted active and passive motion , and sometimes redness . we report an unusual presentation of shoulder septic arthritis in a 43 years old man with no other clinical signs and symptoms of classical septic arthritis . a 43-year - old man presented to the orthopaedic outpatient clinic with 2 months history of pain and limited range of motion in his left shoulder . his pain was insidious in onset , mild to moderate in intensity , aggravated by activities and associated with moderate rest and night pain . patient was diagnosed as frozen shoulder at an outside facility and had been given intraarticular depomedrol 40 mg injection for the same 40 days ago with no improvement in his symptoms . he is diabetic and has liver cirrhosis and is on treatment . on physical examination , the skin colour and temperature of the left shoulder were normal , but the shoulder was tender to touch over the anterior joint line . mri was obtained [ fig 2 ] which showed extraosseous soft tissue enhancements around left shoulder with soft tissue abscess in inter muscular planes of supraspinatous , infraspinatous , subscapularis . blood test revealed normal white cell count with normal differential count , crp-13.4 mg / l ( normal<5.0 mg the erythrocyte sedimentation rate was raised , at 88 mm / hour ( normal range , 0 - 20 mm / hour ) aspiration of shoulder joint was performed and fluid was sent for aerobic and anaerobic culture , afb staining , mycobacterium culture , and mycobacterium genetic testing . arthroscopic lavage of the joint was done and articular fluid sent for repeat aerobic and anaerobic culture . joint visualization was markedly limited because of severe inflammation and fibrinous changes of the joint . the articular cartilages of glenoid and humeral head were completely eroded down to the bone . susceptibility of the isolate was determined with the disk diffusion method and it was susceptible only to colistin . 3 ) fibrocollagenous and fibrovascular inflammed connective tissue containing proliferated blood vessels and perivascular mixed inflammatory cells and lymphoplasmacytoid cells . based on preoperative and intraoperative culture report , a definitive diagnosis of polymicrobial septic arthritis of the shoulder was established and patient was treated with combination antimicrobial treatment . our patient had muted inflammatory response probably due to immunocompromised state ( cirrhosis and diabetes ) which is not uncommon . most often it is hematogenous seeding of shoulder joint , however it can also happen after intraarticular steroid injections . septic arthritis of the shoulder is more common in immunocompromised patients and intravenous drug abusers . in this case report , the patient had atypical clinical symptoms and is immunocompromised and his primary source of infection seems to be hematogenous . our experience with this patient highlights the importance of maintaining high index of suspicion for septic arthritis in immunocompromised patients presenting with atypical shoulder pain associated with stiffness . in the setting of suspected septic arthritis , our initial pre - investigation diagnosis was atypical shoulder pain and stiffness of non specific etiology in an immunocompromised patient and infection was part of the differential diagnosis not the only diagnosis . esenwein et al in his report highlighted the importance of early intervention to prevent chondral damage , osteomyelitis and also to prevent systemic spread . various authors have highlighted the importance of early diagnosis and management of septic arthritis failing which could lead to osteomyelitis and septic shock . in our patient , the diagnosis was delayed due to atypical presentation and over reliance on clinical findings at an outside center . we strongly recommend early imaging studies in immunocompromised patients presenting with shoulder pain and practitioners should avoid loosely diagnosing as shoulder pain with associated stiffness as frozen shoulders . klinger et al . did a retrospective study on 21 patients who underwent surgical treatment for septic arthritis of the shoulder joint between 2000 and 2007 , and he concluded that patients with symptoms for less than 2 weeks did well with arthroscopic approach and early infection can be managed arthroscopically . our reports show that arthroscopic washout can give good result even after 2 weeks of clinical symptoms if there is no evidence of osteomyelitis . septic arthritis of the shoulder is very often due to hematogenous spread and diagnosis is often clinical . laboratory investigations and imaging studies like mri and usg may be useful in establishing the diagnosis but confirmation is usually by joint aspiration . patients who are immunocompromised and have insidious onset of moderate to severe pain and which fails to respond to trial of conservative treatment should be subjected to either ultrasound or mri instead of x - ray because very often in these group of patients it is primarily a soft tissue pathology like impingement , rc tear , calcific tendinitis or rarely infections and malignancies . we recommend that moderate pain of more than 4 weeks duration with severe stiffness in immunocompromised patient ( liver cirrhosis , renal failure , steroid treatment ) should be further evaluated with mri or ultrasound in the setting of normal x - ray and should not be loosely diagnosed as frozen shoulder . goldenberg in his report emphasized the role of local as well as systemic factors that predispose patients with cirrhosis to gram - negative bacterial joint infections . malnick also reported a case of spontaneous septic arthritis in a cirhottic patient that was due to e.coli . our study as well as other studies by goldenberg and malnick highlight the importance of including broad spectrum antibiotics with gram negative cover whilst waiting for final culture sensitivity . septic arthritis of shoulder may not present with classic clinical features . a through clinical and radiological evaluation should be executed and treatment initiated promptly to prevent complications like septic shock and osteomyelitis . the case highlights the importance of establishing anatomical and pathological diagnosis using mri in patients with shoulder pain instead of loosely diagnosing them as impingement or frozen shoulder . the case also challenges the practice of routine shoulder depomedrol steroid injection , in the setting of secondary frozen shoulder , atleast in immunocompromised individuals . primary idiopathic frozen shoulder is a rare condition and secondary frozen shoulder cases are often due to underlying shoulder pathology .

introduction : septic arthritis of the shoulder is uncommon in adults . it is a surgical emergency as joint destruction occurs rapidly and can lead to significant morbidity and mortality . accurate diagnosis can be particularly challenging in patients with underlying liver disease . mri is a useful adjunct in early detection of atypical causes of shoulder pain.case report : a 43 years old male came to our outpatient department with complaints of pain and stiffness of his left shoulder . on examination , his shoulder movements were severely restricted . further evaluation with mri revealed septic arthritis of left gleno - humeral joint for which emergency arthroscopic debridement was done.conclusion:septic arthritis of shoulder may not present with classical clinical features . hence , a through clinical and radiological evaluation will help us prognosticate and treat accordingly thereby preventing complications like septic shock , osteomyelitis .

tuberculosis ( tb ) is a major health issue in developing countries with over two billion people being infected with tuberculosis bacilli world wide ( 1 ) . although tb was recently regarded primarily as a disease of the developing countries it is now also on the increase in developed countries such as the united kingdom , mainly as a result of globalisation as well as an increase in immunosuppressed patients . figures by the health protection agency ( hpa ) show that the cases of tb in the uk increased by 5.5% in 2009 when compared to those in 2008 ( 2 ) . this caucasian patient presented to the emergency department with sudden pain localised to the ulnar aspect of her wrist . x - ray of the forearm was normal and she was treated conservatively but was lost to follow up . she presented again at the orthopaedic clinic 26 months later with continuing swelling and pain . ultrasound showed a synovial tumour on the dorso - medial aspect of the wrist ( figure 1 ) . ultrasound showing a synovial tumour on the dorsal medial aspect of the wrist however , special stains for fungi and acid fast bacilli were negative . subsequently the patient developed a sinus and continued to have a chronic discharge from her left wrist . x ray this time showed destruction of the ulnar styloid consistent with a bony infection ( figure 2 ) . mri showed destruction localised around the ulnar head with oedematous changes extending up the shaft of the ulna ( figure 3 ) . ap xray of the wrist showing destruction of the ulnar styloid consistent with a bony infection . soft tissue swelling can also be noted over the dorsum of the wrist mri showing localised destruction around the ulnar head . the mri also shows thickening around the extensor carpi ulnaris tendon in keeping with marked tenosynovitis at this site . the patient agreed to a repeat biopsy and excision of the necrotic bone ( figure 4 ) . microscopy of the specimen again showed a florid granulomatous inflammatory process with areas of fibrinoid necrosis . post - operative xray of the wrist showing excision of the ulnar styloid further enquiry revealed that our patient had been experiencing lethargy but no other features to suggest tuberculosis . following the diagnosis of tb , the patient recalled that in her former post as a nursing assistant , when she was much younger , she was exposed to patients with tb . although improvements in its management and treatment are continuously occurring an estimated 1.8 million people died of tb in 2008 ( 1 ) . extra - spinal tuberculous osteomyelitis is rare and comprises about 2 - 3% of all cases of osteoarticular tuberculosis , with the hip and knee joints being the most common following spinal involvement ( 3 ) . tuberculous involvement of the ulna is uncommon and tends to be more common in the diaphysis . a high index of suspicion of tb is important to obtain the correct diagnosis . in chronic soft tissue and bony infection the diagnosis of tb may not be evident from the history and examination and indeed no obvious risk factors may be present as in our case . in our patient the infection could have been contracted at a much younger age , when she was caring for patients infected with tuberculosis , and remained latent . however no other risk factors were evident that could explain why the disease was activated after such a long period of latency . it is also interesting to note that the actual distal radio - ulnar joint was not involved , most of the destruction being situated around the ulnar process . although localisation of the tuberculous infection in the bone without joint involvement can occur , this is rare ( 3 ) .

tuberculosis ( tb ) is a major health issue in both developing and developed countries such as the uk . healthy individuals who contract the infection have only 5 - 10% chance of converting to the active disease over their lifetime . we present the clinical history of a 76 year old female who attended the emergency department complaining of wrist pain , and was only diagnosed with tb after three years . this case report emphasises the importance of including tb in the differential diagnosis when treating patients with an infection resistant to conventional antibiotics , even though risk factors for tb may not be evident or special stains for tb are negative .

brachial plexus surgery using the da vinci surgical robot is a new procedure . to evaluate the advantages and the restrictions of the technique , a cadaveric study of supraclavicular and axillary approaches was conducted . we found that the axillary approach was useful and advantageous for lower roots , particularly for thoracic outlet syndrome ( tos ) . this report will focus on the evaluation of axillary robotic approach as the advantages and disadvantages of supraclavicular robotic intervention have been widely discussed in the literature . a human cadaver was subjected to this experiment in paris university ecole europenne de chirurgie anatomy laboratory and da vinci robot system was used . the left arm was tucked along the side and the right arm was placed in a semiflexed position extending toward the anesthesia location near the head , supported by foam and blankets ( figure 1 ) . a 6 cm long incision was made at the right axillar line , lateral to the edge of the pectoralis major muscle ( figure 2 ) . a self - retaining chung retractor was placed into the incision to elevate the pectoralis major muscle flap . the robot was docked as a camera ; right and left robotic arm were adapted in the incision area ( figure 3 ) . a 10 mm 0 downlooking scope , maryland forceps , and a curved scissors the working space was maintained with the self - retaining retractor , without co2 insufflation ( figure 4 ) . the subclavian artery was seen in front of the truncus and was positioned to the posterior of the working space . subclavian artery was dissected from the plexus and truncus of the lower plexus was exposed with blunt dissection . the plexus was exposed thoroughly from t1 to c7 levels . in this surgical setting , the operating surgeon , who has a wide experience in open brachial surgery of the brachial plexus , reported that lower brachial plexus exposure was easier from the axillary working area and a more wide range of motion was achieved to manipulate the robotic tools compared to the supraclavicular exposure for lower part of the brachial plexus . the development of robotic - assisted minimally invasive techniques began in urology , general surgery , and gynecology because of the generally large working spaces available in the abdomen for these types of surgeries [ 14 ] . since then , other surgeons have sought to use robotic devices in other areas , such as the brachial plexus [ 5 , 6 ] . brachial plexus dysfunction can be the result of shoulder trauma [ 7 , 8 ] . it can also occur with tos , which encompasses three separate disorders involving compression of the subclavian artery , subclavian vein , or brachial plexus in the triangular space bordered by the first rib , clavicle , and scalene muscles [ 9 , 10 ] . compression of the vessel - nerve package at the thoracic inlet has been treated with soft - tissue ( scalene muscle ) release and/or bone ( first rib ) resection . surgical approaches to first rib resection may be transthoracic , transaxillary , supraclavicular , infraclavicular , or thoracoscopic [ 9 , 10 ] . however , these approaches are typically associated with incomplete resection of the most medial portion of the first rib and neurovascular complications . theoretically , a minimally invasive transthoracic approach can obviate these problems , enabling complete resection of the offending portion of the first rib without neurovascular complication . , respectively , reported successful results of robotic en bloc first rib resection for tos treatment via transthoracic and transaxillary approaches [ 9 , 11 ] . 's techniques were only bony interventions and as being intrathoracic these need to be lung collapsed and lung complication can be waiting risk . although liverneaux et al . reported techniques and results of upper brachial plexus injury intervention via robotic surgery with a supraclavicular approach , they described the disadvantages as a narrow working space and difficulty to expose the c7 vertebra [ 1 , 5 , 12 ] . to our knowledge , this report is the first to objectively describe robotic axillar brachial plexus exposure . thus , we discuss the theoretical and clinical advantages and disadvantages of the axillary approach in the present report . the development of robot - assisted surgery has revealed new perspectives in peripheral nerve microsurgery . minimally invasive robot - assisted surgery could lead to modification of the classic algorithm for the treatment of traumatic brachial plexus lesions [ 6 , 8 ] . to date , exploration of these lesions has not been attempted less than 3 months after the traumatic event because clinical examination can not provide an accurate diagnosis or reliable prognosis in these first weeks . early intervention may enable initial assessment of the lesion and repair of potentially graftable nerve roots . several robotics properties are particularly adapted to microsurgery , such as high - resolution three - dimensional ( 3d ) visualization with up to 40 magnification , up to 10-fold magnification of surgical movements , elimination of physiological tremors , and the provision of ergonomic work conditions for otherwise uncomfortable surgery . robotic surgical systems allow high - definition magnified 3d visualization of the operative field , provide significant instrument maneuverability , even within a confined space , and may overcome the shortcomings of conventional approaches [ 2 , 5 ] . axillary ( infraclavicular ) brachial plexus intervention via robotic surgery has not been described previously . axillary intervention was previously performed as an open procedure to expose the plexus or resect the first rib for the treatment of tos . martinez et al . described first rib resection via robotic surgery but not to address plexus injury without transthoracic exposure , a novel minimally invasive approach to the first rib from inside of the chest . in addition , gharagozloo et al . reported first rib resection via transthoracic robotic surgery for paget - schroetter disease . 's techniques were considered more useful for lower brachial plexus viewing and assessing according to gharagozloo et al . the experience of the whole surgical team with robotic technology is important for the procedure . during learning curve period , two staff surgeons are required to participate in all procedures to ensure the safety of the program [ 5 , 9 ] . martinez et al . reported importance of the learning curve , not only for the surgeon but also for the entire surgical team and 180 minutes for the initial 10 cases . reported exclusion criteria include a history of previous incision in the same area and obesity , which present difficulties in robotic surgery initiation . other drawbacks of this new surgical approach are the increased cost of surgical equipment and longer operating time , especially during the learning curve period . however , we believe that the avoidance of a classic incision leads to significant patient satisfaction for cosmetic reasons and we believe that demand for this procedure from a select group of patients justifies the exploration of alternative ways to avoid classic brachial plexus exposure . this report presents our initial experience with robot - assisted axillary exposure of the brachial plexus region . in our opinion robotic surgery will be used routinely in the future for brachial plexus surgery and particularly for tos that is caused by bone and/or soft tissue . however , newer dedicated surgical instruments need to be developed and further studies should be conducted to evaluate in vivo application and results of this novel approach .

brachial plexus surgery using the da vinci surgical robot is a new procedure . although the supraclavicular approach is a well known described and used procedure for robotic surgery , axillary approach was unknown for brachial plexus surgery . a cadaveric study was planned to evaluate the robotic axillary approach for brachial plexus surgery . our results showed that robotic surgery is a very useful method and should be used routinely for brachial plexus surgery and particularly for thoracic outlet syndrome . however , we emphasize that new instruments should be designed and further studies are needed to evaluate in vivo results .

gossypiboma ( retained surgical sponge ) describes a mass of cotton or sponge that is left behind in the body cavity during a surgical operation . these foreign bodies can often mimic tumors or abscesses when detected clinically or with radiation . in fact , surgical sponges without opaque markers are the leading cause for the difficulty of a correct diagnosis . here , we present a case in which a foreign body , gauze without radiopaque markers , was left behind during a procedure that involved the removal of an intrapelvic tumor . after a computed tomography ( ct ) scan had revealed information leading to an inaccurate diagnosis , an accurate diagnosis of gossypiboma was successfully made using endoscopic ultrasound - fine - needle aspiration ( eus - fna ) . a 60-year - old female patient was referred with an unexpected detection of an intrapelvic tumor during a routine ct scan . the patient 's medical history included a hysterectomy and a left side ovariectomy at the age of 27 . the ct scan revealed a 45 mm tumor in the patient 's pelvis [ figure 1a and b ] , which was diagnosed as a gastrointestinal stromal tumor ( gist ) , a chronic expanding hematoma or leiomyoma . a forward - view eus ( tgf - uc260j , olympus optical co. , tokyo , japan ) identified a low echoic lesion when scanned from the sigmoid colon . fna was performed with a 22-gauge needle ( ezshot2 , olympus optical co. , tokyo , japan ) by using the forward - view eus [ figure 2 ] . examination of the fna specimen revealed a foreign body ( string of gauze ) displaying macrophage cells and necrotic tissue [ figure 3 ; hematoxylin and eosin , original magnification 40 ] . during the surgery , histological examination of the tissue adjacent to the foreign body revealed granuloma formations with fragmented silken threads [ figure 4 ; hematoxylin and eosin , original magnification , 100 and 200 ] . a : sagittal section ( black arrow ) ; b : horizontal section ( black arrow ) . endoscopic ultrasound ( eus)-fine - needle aspiration using forward - view eus a foreign body ( string of gauze ) obtained using the 22-gauge needle , with macrophage cells and necrotic tissue granuloma formations with fragmented silken threads gossypiboma describes a mass of cotton or a sponge that is retained in the body after a surgery . gossypiboma causes foreign body reactions of the surrounding tissue since they are inert and show no specific decomposition . in this case , radiopaque sponges were not used , and there were no adverse symptoms in the 30 years postsurgery . the typical appearance of gossypiboma shows a spongiform pattern with gas bubbles . in typical cases , however , it can be difficult to diagnose the disorder with ct scans in cases without gas bubbles . in the case described in this paper , radiologists using findings from a ct scan , initially and incorrectly diagnosed the lesion as gist with fibrosis . however , the disorder was correctly diagnosed by a team of gastroenterologists using eus - fna . the eus - fna procedure revealed a segment of string in a fixed foreign body in the pelvis . the application of eus using curved linear array ( cla ) endoscopes in the lower gi tract has been limited to the rectum and distal sigmoid colon because of the oblique - viewing optics . there were case reports using oblique - viewing cla echoendoscopes to evaluate lesions proximal to the sigmoid colon , but advancement of the cla echoendoscope beyond the sigmoid required the use of an overtube or a guide wire previously placed with a colonoscope . binmoeller et al . have reported the feasibility and safety of the front - view forward - array echoendoscope for evaluating right colon lesions . thus , we used a front - view forward - array echoendoscope to safely evaluate the intrapelvic tumor and sample the tissue . a correct diagnosis was obtained using this method . in summary , this is the first known case report diagnosing gossypiboma by eus - fna .

gossypiboma is a term used to describe a foreign mass within the body that is composed of a cotton matrix . gossypiboma can cause infection or abscess formation from an early stage , or it can remain clinically silent for many years . gossypiboma can be diagnosed using several methods including abdominal x - ray films , us of the abdomen , contrast - enhanced computed tomography of the abdomen , and during surgery . however , the variable appearance of gossypiboma can lead to diagnostic misinterpretations . here , we report a successful diagnosis of gossypiboma using a novel method , that is , endoscopic ultrasound - fine - needle aspiration .

to demonstrate the usefulness of enhanced depth imaging optical coherence tomography ( edi - oct ) in investigating choroidal lesions inaccessible to ultrasound sonography . in a 60-year - old woman with an asymptomatic choroidal nevus , normal oct was used to observe the macula and edi - oct to image the choroidal nevus that was inaccessible to ultrasound . the exact location of the lesion in the choroid and the dimensions of the nevus were measured . the lesion was located in the superior macula , and the nevus was homogeneous in its reflectivity . we observed a thickened choroid delineated by the shadow cone behind it , measuring 1,376 325 m in the larger vertical cut and 1,220 325 m in the larger horizontal cut in an image with a 1:1 pixel mapping and automatic zoom . edi - oct appears to be an excellent technique for measuring choroidal nevi and all choroidal lesions accessible to oct imaging by depicting their exact location in the choroid , their dimensions , and their demarcation from the surrounding healthy tissue , thus allowing for a more efficient and accurate follow - up . choroidal nevus is the most common tumor of the ocular fundus , present in 7% of the white population [ 1 , 2 ] . despite its benign nature , it presents a risk of visual loss and visual field loss , and can rarely transform into malignant melanoma . optical coherence tomography ( oct ) and ultrasonography have been used for imaging choroidal nevi . recently , enhanced depth imaging oct ( edi - oct ) has been used in order to measure choroidal thickness in normal and pathologic eyes [ 3 , 4 ] . here , we report a case of a flat choroidal nevus inaccessible to ultrasound sonography that was evaluated by edi - oct . a 60-year - old woman was referred to our department with an asymptomatic choroidal nevus in her left eye . her best - corrected visual acuity was 20/20 in both eyes . slit lamp and fundus examinations of the right eye were normal . in the left eye , the anterior segment was normal ; however , on fundoscopy a choroidal nevus located in the superior macula was observed ( fig . spectral - domain ( sd ) oct ( heidelberg engineering , heidelberg , germany ) revealed normal foveal thickness ( fig . 2 ) . the flat nevus was inaccessible to ultrasound sonography , and edi - oct was used to image its posterior surface ( fig . edi - oct is a new technique that consists of positioning a heidelberg sd - oct close enough to the eye in order to obtain an inverted image , averaged for 100 scans . the advantage of this technique is that the sensitivity of the imaging in deeper tissue layers is increased , and thus the obtained measurements are more accurate . we employed this technique to image the location of the nevus in the choroid and obtain its dimensions as a reference measure for the follow - up examination ( fig . 3 ) . heidelberg sd - oct provides an exact correlation between the oct image and the infrared photograph . as infrared light is absorbed by melanin , the nevus appears brighter . in the edi - oct image , the lesion appears homogeneous and hyperreflective at the level of the large choroidal vessels , masking the underlying choroidal vasculature . the choroidal thickness at the level of the nevus appears greater than the neighbor choroid ( fig . the diameter of the nevus was calculated based on the presence of a shadow cone created by the lesion 's lateral borders . the choroidal thickness was measured based on the hyperreflectivity between the bruch 's membrane and the beginning of the shadow cone ( fig . the choroid measured 1,220 325 m in the larger horizontal cut and 1,376 325 m in the larger vertical cut in an image with a 1:1 pixel mapping and automatic zoom ( fig . edi - oct appears to be an excellent technique for evaluating flat choroidal nevi that are inaccessible to ultrasound sonography and accessible to oct imaging , displaying simultaneously the lesion and the retinal changes . it is a reproducible examination that allows depiction of the exact location of the nevi in the choroid , their dimensions and their demarcation from the surrounding healthy tissue . to date , the software available for sd - oct does not allow for objective measures . the subjective measures obtained are useful upon comparison with the follow - up images . in order to limit the possibility of bias , these follow - up images must be obtained and compared with the same sd - oct machine . we believe that this technique is clinically useful and , together with color fundus photography , can provide a more efficient and accurate follow - up of choroidal nevi .

purposeto demonstrate the usefulness of enhanced depth imaging optical coherence tomography ( edi - oct ) in investigating choroidal lesions inaccessible to ultrasound sonography.methodsin a 60-year - old woman with an asymptomatic choroidal nevus , normal oct was used to observe the macula and edi - oct to image the choroidal nevus that was inaccessible to ultrasound . the exact location of the lesion in the choroid and the dimensions of the nevus were measured.resultsthe lesion was located in the superior macula , and the nevus was homogeneous in its reflectivity . we observed a thickened choroid delineated by the shadow cone behind it , measuring 1,376 325 m in the larger vertical cut and 1,220 325 m in the larger horizontal cut in an image with a 1:1 pixel mapping and automatic zoom . the macular profile and thickness were both normal.conclusionsedi-oct appears to be an excellent technique for measuring choroidal nevi and all choroidal lesions accessible to oct imaging by depicting their exact location in the choroid , their dimensions , and their demarcation from the surrounding healthy tissue , thus allowing for a more efficient and accurate follow - up .

a 3-year - old male , known to have isolated speech delay , presented to the emergency department with severe body aches and inability to walk . few days prior to his presentation , the child started to have flu - like symptoms in the form of rhinorrhea and low - grade fever . this was followed by irritableness , hypoactivity , and inability to move his legs and arms . he was seen in a private hospital where computed tomography of the brain and a lumber puncture were done that showed no abnormalities . in the next day , his condition continued to worsen . he developed difficulty in swallowing and dark - colored urine , so he was transferred to our institution . upon physical examination , the laboratory tests showed elevated creatine kinase level ( 1 778 856 u / l ; normal range 20 - 200 u / l ) , elevated serum transaminases ( serum glutamic - pyruvic transaminase [ sgpt ] = 1857 u / l [ normal range 0 - 50 u / l ] , serum glutamic - oxaloacetic transaminase [ sgot ] = 8626 u / l [ normal range 0 - 50 u / l ] ) , and creatinine 0.72 mg / dl ( 0.3 - 0.7 mg / dl ) . the child started on intravenous hydration and received 1 dose of intravenous immunoglobulin . on the second day , the creatine kinase and serum transaminase levels improved , with creatinine reaching 0.94 mg / dl , the highest level reached . the creatine kinase and serum transaminase levels continued to improve gradually over the following days , with slow improvement in the clinical condition . he received another 2 doses of intravenous immunoglobulin on days 13 and 14 of admission . muscle biopsy showed endomysial inflammatory infiltrates , mostly composed of cd8 and cd4 lymphocytes ; myofibers necrosis mostly ventral portion of myofibers ; residual granular microcalcifications ; and terminal complement complex deposits that outline necrotic myofibers , all features compatible with necrotic myopathy and early rhabdomyolysis ( figure 2 ) . the arrows showed the degenerated myofibers . upon discharge , the child had mild muscle aches , was able to sit , and move his arms and feet . he had persistent weakness in the lower limbs , but was unable to walk , with a follow - up examination 1 month after discharge , revealing that the child was able to walk normally with positive deep tendon reflex . two months after discharge , the creatine kinase level dropped to 193 iu / l . rhabdomyolysis results from skeletal muscle breakdown due to various causes . viral myositis , trauma , and inherited disorders are the most common causes in the pediatric age group . the breakdown releases the normal cell contents into the bloodstream , including creatine kinase , myoglobin , phosphorus , and potassium . rhabdomyolysis is suspected clinically when muscle aches , muscle weakness , and tea - colored urine are present . the diagnosis is based on the high creatine kinase level , which is the most sensitive marker . although there is no established cutoff levels , a concentration 5 to 10 times higher than the upper limit of normal reference range ( ie , 500 - 1000 other laboratory indicators include serum and urine myoglobin concentrations that may be useful but not essential for the diagnosis . the most common complication of rhabdomyolysis is kidney damage . in recent large pediatric studies reported by mannix et al and wu et al , it was shown that the rate of acute renal failure in the pediatric patients with rhabdomyolysis ranged from 5% to 8.7% . the risk factors that predispose a patient with rhabdomyolysis for developing acute renal injury are creatine kinase level concentration more than 5000 u / l , creatine kinase value upon admission , and slower decline in the serum creatine kinase level . other factors include high myoglobin level , persistent or abrupt increase in the potassium or calcium level , as well as persistent metabolic acidosis . on the other hand , a recent study by fernandez et al showed that the most reliable predictor of acute renal failure and the need for dialysis is the creatinine level above 1.7 , despite the peak creatine kinase . the mainstay for the prevention and treatment of acute kidney injury is early and aggressive volume resuscitation . other management options include alkalization of the urine , forced diuresis with mannitol , and loop diuretics . in severe cases or in our case , the child developed extremely high creatine kinase levels that were not reported in the literature . a thorough literature review was done searching for high creatine kinase levels in patients with acute rhabdomyolysis . in 2014 , a case report described a pediatric patient with mcardle disease who developed rhabdomyolysis with a high 6-digit creatine kinase level reaching 500 000 2 pediatric cases were reported . in both , the creatine kinase levels reached were 60 000 a case report of an adolescent with rhabdomyolysis due to undiagnosed hypothyroidism was reported with a creatine kinase level that reached around 34 000 a research article in 2013 examined the clinical spectrum of patients with rhabdomyolysis presenting to the pediatric emergency department , in which the peak serum level of creatine kinase was 9825.1 23 079.1 the only risk factor for developing acute renal injury that our patient had was a high peak creatine kinase level upon admission . on the other hand , he did not develop metabolic acidosis and had a normal albumin and electrolytes levels throughout his hospitalization . he responded very well to volume resuscitation with rapid decline in the creatine kinase level . although he received intravenous immunoglobulin for the fear of guillain - barre syndrome or other underlying immune - mediated diseases , neither the creatine kinase level decline nor the clinical status improvement was affected by the intravenous immunoglobulin administration . the sharp decline in the creatine kinase level there are no clues in the family history or the patient s personal history that may suggest an underlying inherited metabolic , muscular , or genetic disease . , this could be the first presentation of lipin-1 mutation that causes recurrent rhabdomyolysis in children . lipin-1 gene encodes the muscle - specific phosphatidic acid phosphatase , a key enzyme in triglyceride and membrane phospholipid biosynthesis . the episodes of rhabdomyolysis are mostly triggered by intercurrent infections and fever and to a lesser extent by fasting or exercise . the prognosis of lipin-1 deficiency is poor , with up to one - third of patients dying during an episode of rhabdomyolysis . in about 60% of patients with recurrent rhabdomyolysis ,

rhabdomyolysis is an acute life - threatening condition that can occur in childhood secondary to many causes . the authors report the case of a 3-year - old male child who presented with acute rhabdomyolysis . the peak plasma creatine kinase level was extremely high . the 2 main causes of rhabdomyolysis in childhood are viral myositis and trauma , which can sometimes lead to acute renal failure . the highest creatine kinase levels reported in the literature so far was a 6-digit level in 2014 case report . in this study , the authors report the case of a 7-digit creatine kinase level in a child secondary to viral myositis who did not require renal dialysis .

malaligned implants often complicate the clinical laboratory procedures employed for fabrication of superstructures . due to improper load distribution , a stent is an appliance used for radiographic evaluation of height and width of the available bone during treatment planning for implant placement or during surgical procedures to provide site for optimum implant placement . the variable type of stents includes vaccuform or acrylic resin templates adapted over duplicated casts of diagnostic wax - up . these include plastic or metal tubes and channels in acrylic resin that dictate the position of implants . stents should be made of transparent material , stable and rigid when in position , should cover enough teeth to stabilize its position , and when no teeth are present , stents should extend onto unreflected soft tissue regions . with the use of computed tomography ( ct ) and computer added designing ( cad)/ computer added machining ( cam ) technology , it is now possible to construct a surgical implant stent that would allow the clinician to predetermine implant locations virtually and surgically place them without reflecting a tissue flap.[58 ] but the surgical stents prepared with ct and cad / cam technology are very much more cost effective than a conventional surgical stent and require enough time and special center and/or laboratories for their fabrication and later on their correction , if required . in this study , we used the conventional surgical stents to evaluate their efficacy in determining the position and diameter of implants . the aim of the study was to demonstrate the accuracy and clinical precision of conventional surgical stent protocol for optimum installation of dental implants in terms of position and diameter . the aim of the study was to demonstrate the accuracy and clinical precision of conventional surgical stent protocol for optimum installation of dental implants in terms of position and diameter . the study was conducted in the out - patient department of prosthodontics and dental material sciences in collaboration with department of oral and maxillofacial surgery , faculty of dental sciences , csmmu , lucknow . patients were treated in this study with the conventional surgical stents ; all the implants were placed to the desired depth as planned in virtual implant planning . all the patients ( 45 patients for whom 89 implants were placed at different sites ) [ figures 1 , 2 ] visiting the department of prosthodontics and oral and maxillofacial surgery for implant supported prosthesis placement and who followed the standard inclusion criteria for placement of implants were included in the study . first of all , diagnostic cast of the patients was made and acrylic teeth / tooth were waxed - up [ figure 3 ] . then , the impression of that waxed - up cast was taken and poured to reproduce another cast with the missing teeth / tooth replaced by dental stone . then , mesiodistal and buccolingual markings were drawn over these teeth / tooth to find a center point on the occlusal surface [ figure 4 ] . later on , after applying the separating media over these regions and filling the undercuts with wax , transparent self - curing material was applied at the teeth to be replaced along with some adjacent area antero - posteriorly , which confers the stability to the stent during implant placement [ figure 5 ] . the holes were drilled in the stent so formed at the center point for the assessment of the diameter and position of implant and filled with gutta percha [ figure 6 ] . this stent was placed in the mouth and radiograph was taken to evaluate the position of gutta percha . the efficacy of stents was then evaluated at the time of surgical exposure for implant placement and after the surgery , with the help of radiographs [ figures 7 , 8 ] . intraoral view of the patient radiograph of the patients waxed - up missing teeth missing teeth replaced by dental stone in the cast with markings stent made of transparent material stent in place with gutta percha filled in holes post - operative radiograph philip j. described a technique to construct a surgical guide on mounted diagnostic casts . he also used these mounted casts to determine whether sufficient space exists for a fixed cantilevered implant prosthesis . murat c cehreli suggested the fabrication of a bilaminar dual - purpose stent that facilitates implant placement with improved verification of implant positioning . the outer lamina is designed for use in the computed tomography ( ct ) evaluation using radiopaque markers . yen - chen ku presented a simple method of fabricating a vacuum - formed matrix filled with clear acrylic resin and a gutta - percha marker . the matrix can be used as not only a radiopaque marker for evaluation but also a surgical guide during the surgical stage for single implant therapy . roger a. solow showed that a radiographic - surgical template can facilitate consultation with a surgeon and patient , when implant - supported restorations are planned . a template that provides radiographic evaluation of the implant site and precise or modified surgical placement kivanc akea described a modified surgical stent that serves as a guide to proper mesiodistal paralleling of dental implants . he also used these mounted casts to determine whether sufficient space exists for a fixed cantilevered implant prosthesis . murat c cehreli suggested the fabrication of a bilaminar dual - purpose stent that facilitates implant placement with improved verification of implant positioning . the outer lamina is designed for use in the computed tomography ( ct ) evaluation using radiopaque markers . yen - chen ku presented a simple method of fabricating a vacuum - formed matrix filled with clear acrylic resin and a gutta - percha marker . the matrix can be used as not only a radiopaque marker for evaluation but also a surgical guide during the surgical stage for single implant therapy . roger a. solow showed that a radiographic - surgical template can facilitate consultation with a surgeon and patient , when implant - supported restorations are planned . a template that provides radiographic evaluation of the implant site and precise or modified surgical placement kivanc akea described a modified surgical stent that serves as a guide to proper mesiodistal paralleling of dental implants . the foremost advantage of the stent used in this study is its surgical ease , simplicity , precise accuracy and low cost . it can be fabricated with minimum laboratory procedures which are used in routine dental practice . on the other hand , the fixed type screw retained implant stents which are fabricated with the help of ct and cad / cam technology are very costly and require more time and laboratory procedures . the stents fabricated with the radiopaque markers provide radiographic as well as clinical ease for optimum implant installation . along with all these advantages the drill holes can directly be made through the stent , so the implant surgery may become less traumatic ( with the preservation of soft tissue including the gingival margins of adjacent teeth and the interdental papilla ) with decreased operative time which results in accelerated post - surgical healing , fewer post - operative complications and increased comfort and satisfaction for the patient . the possibility of displacement of stent during implant surgery was minimized by extension of the stent antero - posteriorly to cover enough area over the teeth / tooth present adjacent to edentulous area . in cases where no teeth are present , stabilization of stent was achieved by extending it over the unreflected areas like retromolar regions . this study shows the extreme accuracy of this conventional surgical stent . if each step of this protocol is followed precisely , it is possible to deliver an optimum implant installation in terms of position and diameter and later on function at a very much reduced rate and time , as taken in ct and cad / cam technology which are the most accepted methods

aim : demand from implant patients for quality and efficient treatment is increasing . fortunately dental implant treatment is evolving with patients expectations.materials and methods : the study comprised of 45 patients for whom 89 implants were placed at different sites . efficacy of the stents is evaluated in determining the position and diameter of the implants.conclusion:this study shows the extreme accuracy of this surgical stents in implant installation in terms of position and diameter .

neurofibromatosis ( nf ) is an autosomal dominant disease , discovered in 1882 , by the german pathologist friedrich daniel von recklinghausen , characterized by disordered growth of ectodermal tissues , and is part of a group of disorders called phakomatoses ( neurocutaneous syndrome ) . neurofibromatosis type - i ( nf-1 ) , also known as von recklinghausen syndrome , is caused by the mutation of a gene on the long arm of chromosome 17 , which encodes a protein known as neurofibromin . it is characterized by spots of increased skin pigmentation ( caf au lait spots ) , combined with peripheral nerve tumors and a variety of other dysplastic abnormalities of the skin , nervous system , bones , endocrine organs , and blood vessels . the localized form of neurofibromatosis type - i , first described by gammel in 1931 , is very rare . crowe et al . proposed the term sectorial neurofibromatosis for this localized form of neurofibromatosis , and miller and sparkes modified the nomenclature to segmental neurofibromatosis ( sn ) the current term for neurofibromas of segmental distribution . the commonly affected sites for sn are the thorax and abdomen ( 55% ) , upper extremities ( 20% ) , and lower limb and face ( 10% each ) . only few cases ( less than 10 ) of segmental neurofibromatosis over the face have been described so far . a 26-year - old female patient reported to the dental department complaining about the unesthetic appearance of her face , since eight years [ figure 1 ] . history revealed that eight years back , the patient underwent surgery in the neck region for a cyst and one to two days after surgery the patient got a swelling on the face . the swelling was of the same size since then and there was no pain associated with the swelling . the unesthetic appearance of the face on examination , the face of the patient appeared asymmetrical , bilaterally , with a growth on the right side of the face . the skin over the right side of her face was thickened and hyperpigmented , with a soft and loose hanging overgrowth . there were numerous small and large , sessile and pedunculated nodular growths localized on the right side of her neck , with dark - colored underlying skin . the skin over the right cheek and chin area showed coffee - colored pigmentation ( caf - au - lait spots ) [ figure 2 ] . nodular growths localized on the right side of the neck with dark colored underlying skin there was a soft , compressible , nontender growth on the lower lip . the right ear showed disfigurement and hypertrophy , with thickened cartilage and overlying hyperpigmented skin [ figure 3 ] . disfigurement of the ear and hypertrophy with thickened cartilage and overlying hyperpigmented skin intraorally there were numerous soft tissue growths with a sessile base seen on the right buccal mucosa [ figure 4 ] and tongue [ figure 5 ] . soft tissue growths with sessile bases on right buccal mucosa soft tissue growths with sessile bases on the tongue laboratory findings revealed a raised erythrocyte sedimentation rate ( esr ) ( 70 mm in the first hour ) , a decreased hemoglobin level ( 7.5 gm% ) , and the enzyme - linked immunosorbent assay ( elisa ) was found to be nonreactive . a panoramic radiograph disclosed asymmetric enlargement of the right side of the mandible , with loss of cortication of the lower border in the midline area , expansion of the body of the mandible causing displacement of the teeth , seen on right side , expansion of the right inferior alveolar nerve canal , enlarged mental foramen , resorption of the roots of the mandibular right teeth , and loss of the right antegonial notch . a deficiency was seen on the right side of the maxilla , with crowding of the posterior teeth [ figure 6 ] . a panoramic radiograph the right lateral oblique radiograph revealed a deep sigmoid notch , loss of the antegonial notch , an enlarged and rounded coronoid process , with resorption of the mandibular right teeth [ figure 7 ] . a right lateral oblique radiograph subsequently , biopsy of one of the nodules was taken and sent for histopathological examination . the histopathological slide revealed a non - encapsulated tumor of the dermis with a normal overlying epidermis . the tumor consisted of loosely spaced spindle cells and wavy collagenous strands in a clear matrix [ figure 8 ] . a non - encapsulated tumor of the dermis , with a normal overlying epidermis these tumors are composed of schwann cells , fibroblasts , mast cells , and vascular components . the caf au lait spots are irregularly - shaped , evenly pigmented , brown macules . most individuals with neurofibromatosis have six or more spots that are 1.5 cm or greater in diameter . in young children , five or more caf au lait macules , greater than 0.5 cm in diameter , are suggestive of neurofibromatosis . less than 1% of the healthy children have three or more such spots , although one or two caf au lait macules are commonly encountered in healthy individuals without disease . bone involvement includes pseudoarthrosis of the tibia , bowing of the long bones , and orbital defects . treatment of neurofibromatosis is predominantly surgical . when neurofibromas increase in size or cause pain , malignant transformation must be suspected , and excision or biopsy must be performed . acoustic neuromas and tumors that cause tinnitus and vertigo must be excised with great caution . plastic surgeons may be included in the correction of deformities , especially those of the face . considering the autosomal dominant inheritance pattern of neurofibromatosis , genetic counseling

segmental neurofibromatosis is a rare disorder , characterized by neurofibromas or caf-au - lait macules limited to one region of the body . its occurrence on the face is extremely rare and only few cases of segmental neurofibromatosis over the face have been described so far . we present a case of segmental neurofibromatosis involving the buccal mucosa , tongue , cheek , ear , and neck on the right side of the face .

patient aged 64 was admitted to the county hospital urology ward in september 2010 and diagnosed with tumor of the left kidney . ultrasonographic exam revealed the structure with a diameter of 45 mm in the central field of the left kidney . ct showed a solid focal lesion of the left kidney ( 30 x 40 x 32 mm ) located below the renal stalk and covering the renal pelvis and the paranephric section of the left ureter , apart from that no changes were observed ( figure 1 ) . radiological image of the chest did not reveal any pathological changes . in order to make the results of pre operational diagnosis sided ureterorenoscopy was conducted and a urine sample was taken for cytology to exclude epithelial nature of the tumor . after undergoing the above mentioned test , the patient was qualified for transperitoneal radical nephrectomy . with general anesthesia applied , the left kidney was removed through an incision below the left costal margin . during the operation , a significant peripheral infiltration was discovered which hindered access to the renal stalk of the left kidney . operative course passed without complications and the patient was discharged in good general condition 8 days after the surgery . left kidney tumor in ct exam . on pathological examination ( samples no 12399241239928 ) in all samples after detailed hematological work up which did not show any other tumors , the patient was qualified for radiation treatment and currently remains under permanent oncological supervision . medical records and literature mention several cases of malignant kidney lymphoma [ 1 , 2 ] . some authors claim that this entity accounts for 3% of all solid renal tumors among adults . lymphomatous renal involvement can have three different causes : most commonly it appears along with the generalized disease and enlarged lymph nodes . renal involvement develops without any symptoms then.it may be related to organ transplantation and infection with epstein such cases are rare and difficult to diagnose which may lead to mistakes in treatment . such cases are rare and difficult to diagnose which may lead to mistakes in treatment . the case presented in the report illustrates diagnostic and therapeutic difficulties that a urologist might encounter during treatment of patients with possible renal tumor . results provided by clinical imaging of focal lesions are inconsistent . what must always be taken into account is an atypical tumorous process , autoimmunological disorders as well as inflammatory condition . in this particular case control appointments in the department of hematology have been scheduled to take place every four months and the prognosis for the patient has been described as good . they develop from lymphocytes and , depending on the variety of b and t lymphocytes , those processes can have different clinical outcome . some of them are located solely within the lymph nodes or tissues and internal organs , while others are accompanied by leukemia . it might seem that when it comes to lymphomas , the pathogens initiating the course of the disease could be both , the epstein barr viruses which attack the b lymphocytes , and by htlv1 viruses dwelling in t lymphocytes and causing a subacute form of leukemia among adults . there can also be some co factors of environmental character : chloro organic compounds , ionization energy , benzene like substances , states of immunosuppression and autoimmunological diseases . the malignant lymphoma morbidity rate can also be increased by extensive exposition to herbicides , and is even 35 times higher among patients with a kidney transplant . it usually dwells within the lymphoid tissue of waldeyer 's ring , spleen and thymus . extranodal lymphomas can most often be found in the gastrointestinal tract , other types can be located in skin , bones , eye kidney might be at the stage of involvement which spreads through lymphatic system from the nodes in retro peritoneum . if the renal involvement occurs at an advanced stage of the disease , as yet another location of it , it is relatively easy to diagnose . once a patient is diagnosed with lymphoma of kidney , further examinations are required in order to rule out a systemic disease . the necessary test in such cases include : blood tests , bone marrow biopsy , radiological examination , pathomorphological evaluation and histo immunological evaluation of enlarged lymph nodes . the malignant types , however , build up rapidly , are resistant to treatment and show no signs of improvement within a short period of time . how long the patient will live depends on the histopathological diagnosis of the tumor and its stage of development . non hodgkin lymphomas are treated with chemotherapy , radiation , and , in some cases , surgeries . low malignant , originally extranodal , tumors are usually surgically removed and treated with radiotherapy . cell lymphoma associated with chronic inflammation arising in a renal pseudocyst with barely noticeable clinical symptoms . publications in english present several cases of spontaneous rupture of the ureter as primary symptom of malignant lymphoma . risk of their occurrence increases from 1.2% in 5 years after the operation , to 6.8% in 20 years after the transplantation , which is related to epstein barr virus infections . some authors also report on cases of co existence of inflammatory intestines disease , including the crohn disease , and malignant lymphomas but then changes can most frequently be located in the vicinity of intestines or other adjacent sections of the digestive tract . the presented case leads to a conclusion that each focal lesion of unclear etiology found on imagining studies should be treated as a possible malignant lymphoma and ought to be histopathologically verified as quickly as possible .

we present a 64year old patient suffering from extranodal malignant lymphoma . the patient was admitted to the county hospital urology ward with suspicion of the left kidney tumor . as part of pre operational diagnosis , ultrasonographic examination and computed tomography of kidneys were conducted . the results confirmed the initial diagnosis . after undergoing radical surgical treatment , the patient is currently under the care of hematology ward of oncology centre .

a young man was referred with a chief complaint of blurred vision and pain of the left eye 1 month after herpetic encephalitis in the left frontotemporal lobe . the patient had multiple foci of retinitis in the retinal periphery associated with vitritis , blot retinal hemorrhage and retinal arteriolitis . the impression of acute retinal necrosis was confirmed by polymerase chain reaction of aqueous humor by detecting herpes simplex virus type 2 ; therefore , the patient received intravenous acyclovir . herpetic encephalitis may be a risk factor for acute retinal necrosis . the virus may reach the eye by the trans - axonal route . acute retinal necrosis ( arn ) is one of the clinical presentations of herpes simplex virus ( hsv ) . this disease may present several years after a primary infection , or it may occur following systemic herpetic infection such as herpetic dermatitis . the prevalence of the disease is equal in both sexes and it occurs in the 5th-7th decade of life . american uveitis society criteria for the diagnosis of arn syndrome are : one or more foci of retinal necrosis with discrete borders located in the peripheral retina , rapid progression in the absence of antiviral therapy , circumferential spread , occlusive vasculopathy with arteriolar involvement , prominent vitritis , and anterior chamber inflammation . optic neuropathy , scleritis , and pain are supportive but not required . in most patients , the diagnosis is made clinically . polymerase chain reaction ( pcr ) analysis of the aqueous humor can detect the cause of arn specifically . pcr is very helpful in the detection of the varicella zoster , hsv type 1 and 2 . patients with arn due to hsv-1 and varicella zoster virus tend to be older , whereas those with arn due to hsv-2 tend to be younger [ 3 , 4 ] . in this article , we report one case of unilateral arn 1 month after the herpetic encephalitis . the patient is a 25-year - old man who was admitted with a chief complaint of decreased vision and pain in left eye for the previous 4 days . the patient also complained of perception of floating objects in the visual field of the left eye . the patient had no past medical history indicating an immunocompromised state ; he had been admitted to the neurology ward due to herpetic encephalitis 1 month previous to the occurrence of visual symptoms . herpetic encephalitis diagnosis was made by brain mri ( fig . 1 ) and pcr analysis of the cerebrospinal fluid . he did not report any underlying disease or drug consumption except oral acyclovir 800 mg every 8 h. at physical examination , the right eye was normal . in the left eye , visual acuity was 0.1 , papillary reflex was normal and marcus gunn sign was negative . slit lamp examination of the left eye revealed conjunctival hyperemia and ciliary injection , diffuse fine keratic precipitates in the corneal endothelium , 3 + cellular reaction in the anterior chamber , 3 + cellular reaction in the vitreous without snowball or snowbank opacities , or vitreous hemorrhage . in fundoscopic examination , multiple foci of retinitis and retinal necrosis 2 ) associated with arteriolar involvement ( arteriolitis ) , blot retinal hemorrhage and optic disc hyperemia . pcr analysis of aqueous humor revealed hsv-2 , so the patient was admitted with the diagnosis of arn and received intravenous acyclovir for 10 days ( 10 mg / kg / day ) . after 48 h of antiviral therapy , a systemic corticosteroid ( prednisolone 1 mg / kg / day ) was introduced and subsequently tapered over several weeks to treat active inflammation . the patient also received aspirin to treat an associated hypercoagulable state . with this treatment , no new lesion developed and opacity of the media decreased ; so prophylactic barrier laser photocoagulation in the areas of healthy retina at the posterior borders of the necrotic lesion prevented retinal detachment . intravitreal antiviral agents such as ganciclovir and foscarnet were not used in this patient because the disease was controlled by intravenous acyclovir . after discharge from hospital , oral acyclovir 400 mg every 5 h was started . during follow - up , the inflammation decreased and the patient had no vitreous traction or retinal detachment or other eye involvement . the brain and retinal involvement were on one side ; after 1 month of left frontotemporal lobe involvement of the brain , the retina was involved on the left side . some of the studies report arn several years after neonatal and infantile herpetic encephalitis [ 5 , 6 ] ; therefore , herpetic encephalitis may be a risk factor for arn development . other articles report that the virus accesses the retina from the brain by the trans - axonal route ; consequently , this virus can cause recurrent episodes of arn [ 8 , 9 ] . these reports may necessitate a prophylaxis with antiviral agents after herpetic encephalitis to prevent arn . in a study by pavsio et al . , the role of long - term prophylaxis with acyclovir in children who had herpetic encephalitis is an important issue . prophylaxis could be considered after hsv encephalitis to prevent arn or after arn to prevent second eye involvement . acyclovir used as prophylaxis for recurrent genital hsv infection in adults for 5 or more years has been associated with minimal toxicity and the selection of resistant strains has not been demonstrated , but there is little experience with the duration of prophylaxis that would have been necessary to prevent arn in our patient . with this report we reemphasize the correlation between these two diseases . on the other hand , arn may be an indication of possible central nervous system involvement and neuroimaging may be necessary in all cases of arn to rule out herpetic encephalitis .

purposeto report a case of unilateral acute retinal necrosis after herpetic encephalitiscase reporta young man was referred with a chief complaint of blurred vision and pain of the left eye 1 month after herpetic encephalitis in the left frontotemporal lobe . the patient had multiple foci of retinitis in the retinal periphery associated with vitritis , blot retinal hemorrhage and retinal arteriolitis . the impression of acute retinal necrosis was confirmed by polymerase chain reaction of aqueous humor by detecting herpes simplex virus type 2 ; therefore , the patient received intravenous acyclovir.conclusionherpetic encephalitis may be a risk factor for acute retinal necrosis . the virus may reach the eye by the trans - axonal route . prophylaxis with acyclovir may be necessary after herpetic encephalitis to prevent acute retinal necrosis .

an infliction in the life expectancy figure of patients with sickle cell disease ( scd ) occurred around the middle of the 1970s ( fig . this minimal increase in life expectancy coincided with my appointment at thomas jefferson university as the associate director of the newly created adult sickle cell center . the number of adults at that time was small and the transition from pediatrics to adult programs was at the age of 18 years . the trickle of patients increased gradually and we were faced with adolescent and young adult african american patients who were in a state of confusion . stripped from the protective sphere of the pediatric world and the empathy of their pediatric hematologists and the pediatric ancillary staff , they were in a state of fear , anxiety , depression and , worst of all , severe pain . the fact that most patients were barely educated , many without a high school degree , unemployed , mediocre health coverage , and dysfunctional family structure conferred a logarithmic dimension to the problem . the arrow indicates the infliction point where life expectancy of patients with sickle cell disease began to increase . the steady stream of admissions of patients with acute painful vaso - occlusive crises ( vocs ) to the emergency department ( ed ) and hospital were not welcome by most providers , hospital administration , the house and nursing staffs . there was subtle resentment of the patients that sometimes extended to the hematologists who showed compassion to the patients . soon labels such as drug addicts , drug - seeking behavior , and hospital hopping and frequent flyer emerged . listening to and believing the patients and keeping detailed records of ed and hospital admissions and the analgesics prescribed , revealed that most patients genuinely do not respond to a certain analgesic or a certain dose . increasing the doses of an analgesic or switching to another drug solved the problem in most patients . accordingly and with the approval of the institutional review board ( irb ) , i issued an identification wallet - sized , plasticized card that was carried by patients and presented to the provider treating their voc in the ed , hospital or any other medical facility . information printed on both sides of the card included : 1 ) demographic data and a recent photograph ; 2 ) hematological data including reticulocyte count ; 3 ) medical data including the type of scd , its complications and co - morbidities if present ; 4 ) all medications being taken by the patient and the recommended treatment of vocs including the name , dose , and the route of administration of the analgesics in question ; and 5 ) my name and contact information for answering questions if needed . it was not expensive to issue these cards . a polaroid camera available at that time and a laminator were the only equipments needed to issue these cards . later on , information on the card was computerized and a printed copy was given to the patient . the patients were very compliant in carrying it as faithfully as they carry their medical cards . some providers liked it very much because it facilitated having a concise history about the patients . while this controversy was brewing , interesting developments in basic science were in progress to understand the pharmacodynamics and pharmacokinetics of opioids . in the 1970s , it was hypothesized that opioids have receptors to bind to and activate in order to relieve pain by blocking or minimizing the transmission of painful stimuli and raising the pain threshold . it did not take long after that to identify opioids as ligands that bind to stereospecific and saturable receptors in the central nervous system and other tissues [ 3 , 4 ] . in addition , recent elegant studies [ 6 - 10 ] have revealed a helical structure of the opioid receptors , which forms pockets in which the corresponding opioid ( ligand ) fits snugly ( fig . recognition is highly specific , such that only l - isomers of certain opioids exert analgesic activity . physiologically , by binding to receptors , opioids initiate a series of biochemical events including activation of g proteins , inhibition of adenylate cyclase , and extrusion of potassium ions , resulting in hyperpolarization of cell membranes [ 14 - 16 ] ; this delays or prevents transmission of painful stimuli . thus , the riddle why some patients respond to one opioid but not another had a pathophysiologic explanation . ( a ) morphine - like molecule ( yellow ) in the deep pocket ( blue ) of the -opioid receptor . ( b ) -opioid receptors from an intimate pair when crystallized with a ligand ( yellow ) such as morphine . knowing how an opioid molecule ( yellow ) sticks in the pocket of its receptor ( blue ) parallel to the progress in the pharmacodynamics of opioids mentioned above , a concomitant advance in the pharmacokinetics of opioids was bubbling to the surface . phase i involves the cyp enzymes and phase ii metabolism conjugates the drug to hydrophilic substances , such as glucuronic acid , sulfate , glycine , or glutathione . morphine , hydromorphone and oxymorphone are metabolized by glucuronidation , whereas the majority of the other opioids are metabolized by the cytochrome p450 isoenzyme system . the net effect of an opioid depends on the availability of enzyme(s ) to convert it into metabolites that could be active or inactive . briefly , the cyp2d6 genotypes are categorized into phenotypes based on the activity of the variant enzymes . ultrarapid metabolizers ( ums ) have greater than normal activity due to duplication or triplication , of active alleles [ 20 - 23 ] , extensive metabolizers ( ems ) have normal enzyme activity , intermediate metabolizers ( ims ) have decreased enzyme activity , and poor metabolizers ( pms ) have absent or little enzyme activity . patients who are ums of fentanyl would rapidly convert it into inactive metabolites with minimal or absent analgesic effect requiring increasing the dose of fentanyl . on the other hand , patients who are pms of fentanyl would experience prompt relief with relatively small doses of fentanyl but higher doses could be toxic due to the accumulation of unmetabolized fentanyl . the cyp3a4 enzyme metabolizes more than 50% of all drugs ; consequently , opioids metabolized by this enzyme have a high risk of drug - drug interactions . together , current data on the pharmacodynamics and pharmacokinetics of opioids show great variability of genotypes among patients and extreme variability in individual responses to opioids . determining the pharmacogenetics profile of each patient facilitates the choice of drugs that would be efficacious for that patient and avoid those drugs associated with harmful drug - drug interaction . this approach in diagnostics and therapeutics ushers in the dawn of a new field for the management of individual patients based on their unique pharmacogenetics , phenotypic and biomarker characteristics . this future approach is referred to as personalized medicine or , more recently , precision medicine . we hope that this methodology would be approved and sponsored by the insurance companies for patients with scd . in the meantime , listening , believing and respecting the patient with sickle cell pain should be maintained for now as the approach to individualized therapy .

in the 1970s , sickle cell pain was treated with trial and error approach by increasing or decreasing the dose of an opioid or switching from one analgesic to another . this approach was controversial with criticism and doubt about its usefulness . since then , advances in determining the structure of opioid receptors and the role of the cyp450 enzymes in metabolizing opioids revealed that these anatomic and metabolic findings are not the same in all persons , thus explaining the variability in response to opioids among patients . thus , the trial and error approach has a scientific basis after all .

type iii dysbetalipoproteinaemia is a kind of lipid metabolism disorders , caused by apo - e deficiency , which leads to accumulation of chylomicrons and very low density lipoprotein remnants in the plasma.123 lipid metabolism disorders are mostly manifested by xanthomas , which are known as yellowish cholesterol - rich material in large foam cells accumulating in the skins and tendons.14 these yellowish lesions may appear all over the body , on the palm of the hands , sole of the foot , tendons , and even on the eyelids.5 these lesions firstly described with or without hyperlipidaemia in association with monoclonal immunoglobulin . based on this fact , there are three forms of xanthoma : hyperlipaemic xanthoma , normolipaemic xanthoma and necrobiotic xanthogranuloma.6 xanthomatosis is usually associated with hyperlipidaemia , and morbidity and mortality of this condition are related to atherosclerosis and pancreatitis.7 hyperlipaemic xanthoma lesions are more polymorphic and can include tuberous , tendinous , palmar or eruptive xanthoma . verruciform xanthoma usually presents as a hyperkeratotic , cauliflower like , verrucous or papillomatous lesion.6 it initially was described as a unique clinicopathologic lesion of the oral mucosa and was subsequently reported in the skin . the epidermal - mucosal changes of hyperparakeratosis , neutrophilic infiltrate and dermal - submucosal foam cell reaction are distinctive features of these xanthomas.8 hereby , we present a rare type of xanthomas calling cauliflower xanthoma in a 43-year - old man with dysbetalipoproteinaemia . a 43-year - old man was presented to outpatient endocrine clinic of tabriz university of medical sciences , tabriz , iran , by his sister for evaluation of skin lesions . he had multiple lesions on shoulders and back , lateral and medial part of dorsal surface of the foot , medial and lateral malleus of both feet , and dorsal and palmar surface of both hands . siblings of the patient have been followed up in that clinic for clinically diagnosed type - iii dysbetalipoproteinaemia . this diagnosis was based on characteristic palmar , eruptive , tuberous and trauma site xanthomas with typical high levels of both total cholesterol and triglycerides in the plasma . history of coronary artery or other atherosclerotic disorders were negative in index case and siblings . there were not any other complaint , and as a greengrocer , he had an active lifestyle . he had a pathology report of skin lesion biopsy that was performed by a dermatologist few months ago , with presence of lipid laden macrophages consistent with diagnosis of xanthoma . fasting serum lipid levels reported as : total cholesterol = 507 mg / dl , triglycerides = 470 mg / dl and high density lipoprotein ( hdl ) cholesterol = 41 mg / dl . he had normal fasting blood sugar , and normal thyroid , renal and liver function tests . physical examinations revealed numerous xanthomas resembling cauliflower on both knees [ figure 1 ] , unusual eruptive , tuberous xanthomas same as previous lesions , on the lateral malleus of both feet [ figure 2 ] . cauliflower xanthomas on the knees and lateral malleus of both lower extremities cauliflower xanthomas on the lateral malleus of lower extremities there were lots of smaller xanthomas on the right shoulder [ figure 3 ] , and cauliflower like xanthomas on the extensor side of the left upper extremity , especially on the elbow [ figure 4 ] . based on available information , the patient diagnosed as familial dysbetalipoproteinaemia ( fdl ) and the unique lesions on the lower limb called cauliflower xanthoma . although siblings of this case had the same disorder , they did nt develop such lesions . xanthomas on the shoulder and back of the neck xanthomas on the extensor side of the hand by searching in the medical data bases , we can not find a previous such a lesion . therefore , we decided to present this case without revealing patient 's name , after taking his consent . considering the fact that the treatment with high dose statin was effective in correction of lipid abnormality and regression of skin lesions in his three siblings , we wanted to try the treatment procedure in this patient but he refused any intervention . fdl , also known as hyperlipoproteinemia type - iii or broad beta disease , is a rare inherited disorder characterised by improper metabolism of certain lipids , specially plasma cholesterol , triglyceride rich chylomicron and very low density lipoproteins ( vldl ) remnants.1 presence or absence of the symptoms of this disease depends on two major risk factors : genetic and diet.910 mutations in the gene for apolipoprotein e ( apo e ) are the main cause of this disease . replacement of an arginine by a cysteine in position 158 of the 299-amino acid chain of apo e5 is responsible for the defective binding of chylomicron and vldl remnants to cell receptors . thereafter , slower plasma clearance of these particles occurs , and results in the abnormal accumulation of lipids in the body.9 on the other side , diet has an essential role in the development of the disease . this means that with standard cholesterol diet , symptoms of fdl will not appear , even in the genetically susceptible person.10 xanthomas can be a symptom of fdl . xanthomas may also be the symptoms of a generalised histiocytosis , or a local fat phagocytosing storage process.11 they are yellowish lesions on the skins and tendons , macroscopically . these macrophages are filled with lipid droplets , which are dissolved and removed from tissue during histologic processing.8 fdl is mostly diagnosed by combination of clinical and laboratory findings.12 most cases are inherited as autosomal recessive trait . men are more susceptible for fdl probably because of protective impact of estrogen in women . most of the diagnosed patients were typically young males , with strong family history , characteristic skin lesions , high serum levels of cholesterol and triglycerides and confirming skin histology.113 most of these cases have premature atherosclerosis and other signs of ischaemic disorders.9 the patient in this article was a middle aged man with family history of dysbetalipoproteinaemia and high levels of serum lipids as follows : total cholesterol = 507 mg / dl and triglycerides = 470 mg / dl . he had a rare form of xanthoma all over his body , causing social problems for him . nicotinic acid , clofibrate , statins or gemfibrozil properly reduce cholesterol and triglycerides in people affected with dysbetalipoproteinaemia.1 the patient mentioned in this article refused receiving any medical intervention . he has just been advised to have low lipid diet , fish oil and regular moderate exercise in order to reducing serum lipid levels . there should be distinctive monitoring of fdl patients including regular checking of their serum lipids . controlling underlying disorders , and reducing excess calories , saturated fat and cholesterol , is the main aim of treating these patients . according to this article , cauliflower xanthomas could be a symptom of fdl , and it should be considered as a differential diagnosis while approaching to these lesions .

familial dysbetalipoproteinaemia ( fdl ) is an inherited disorder in which both cholesterol and triglycerides are elevated in the plasma , pre - disposing the people to coronary artery disease and peripheral vascular disease . the disease is mostly manifested by xanthomas , which have variable forms according to lipid amounts in the plasma of the blood . hereby , we report a 43-year - old man with fdl , presenting with a rare form of xanthomas calling cauliflower xanthoma all over the body .

craniofacial defects are rare , disfiguring facial anomalies , with an incidence of about 1.4 to 4.9 cases per 100,000 live births . craniofacial clefts may be caused by failure of the maxillary processes to fuse , external pressure , amniotic bands , oligohydramnios , central disorganization of the neural crest , and hematomas . in 1973 , the median cleft of the upper lip , or tessier 0 class , has a variety of presentations , ranging from a minimal notching of the lip , vermilion , and nose to a wide cleft that divides craniofacial structures . tessier 0 clefts result from failure of the two medial nasal processes to fuse at midline . median clefts are broadly classified into true and false ; false clefts are due to agenesis of the medial nasal process , while true clefts are due to failure of the medial nasal process to fuse . treatment of median clefts depends on the clinical presentation and may vary from simple alignment of orbicularis oris and vermilion , to reconstruction of the cupid 's bow and philtrum for true median clefts , and complex craniofacial procedures in case of false median clefts . various techniques have been described to repair mild or moderate true median cleft lips . here we report using a pfeifer incision to correct a moderate true median cleft of the upper lip . pfeifer incision includes wavy lines , which elongate the incised tissues as the waves are straightened to close in a straight line , and also provide extra tissue for a tension free closure . a five - year - old boy with a facial cleft presented to our clinic . on examination , there was a median cleft of the upper lip involving the white roll , with no bony involvements . the highest- ( points a , a ) , and the deepest - points ( points b , b ) of the white roll were marked on both sides . subsequently , a wavy incision line was made starting from the deepest point and extending over the philtrum just above the cleft ( fig . a diamond incision was made over the vermilion and the labial mucosa , intra - orally extending just beyond the cleft margin . incision was made and the mucosal tissue covering the area medial to the incision site ( sterile zone ) was removed . undermining of the orbicularis oris muscle was performed and it was approximated using 4 - 0 vicryl . the cupid 's bow was properly aligned , with equal height on both sides ( fig . vermilion form was satisfactory and the fullness and continuity of the orbicularis oris were maintained . incision marking for pfeifer technique ( a , a : highest points on white roll ; b , b : deepest points on white roll ) postoperative result after suture removal a median cleft can also be called midline cleft or vertical cleft through the centre of the upper lip , and is a rare anomaly , the exact developmental origin of which is not clear . tessier 0 clefts occur during the third week of gestation due to failure of the two medial nasal processes to close in midline . it can occur in isolation or be a part of a syndrome such as orofacial - digital syndrome . false clefts are associated with abnormalities of the forebrain and are categorized under the category of holoprosencephaly . in 1937 , veau categorized median clefts to notch of the lip , median cleft extending to the columella , and defects due to atrophy of the midline facial structures . median cleft face syndrome , frontonasal dysplasia , and tessier 0 clefts are various terms used to describe abnormalities associated with true median clefts that are not accompanied by forebrain abnormalities . the tessier 0 anomaly may present as a small notch in the soft tissue , or in association with hypertelorism , midline craniofacial osseous defects , and hairline abnormalities . various treatment options are present for mild deformities which do not involve the white roll . when developing the treatment plan , reconstruction of the cupid 's bow , labial philtrum , vermillion , and buccal mucosa should be kept in mind . urata and kawamoto described using a v - y flap , while weimer et al , used a diamond incision to repair these anomalies . da silva frietas and colleagues described a mucosal z transposition technique to treat mild cases . for moderate defects involving the philtrum , these waves are subsequently approximated in a straight line , which help expanding the length and width of the tissue . this incision has previously been used to correct other tessier clefts with a high success rate . the above - mentioned studies prove the versatility of the pfeifer incision . in our case we were able to achieve adequate symmetry of the philtrum and the cupid 's bow , which is important in cases like this . the postoperative results were excellent and proper approximation of the orbicularis oris muscle and the vermilion was achieved with a symmetrical cupid 's bow and philtrum . the only disadvantage of this technique is that the final closure line is placed directly over the philtrum . since the presentation of midline cleft deformities varies widely , each case should be individually considered and treated . pfeifer incisions can successfully provide mucosal length , vermilion fullness , and lip symmetry in patients with moderate median cleft lip .

median cleft is the midline cleft of the lip . it develops due to incomplete or failed fusion of the median nasal prominence . it can present with minimal deformities such as involvement of the vermilion border , or complex clefting of the midline structures and brain . median clefts are broadly classified as true and false clefts . this case report describes a rare case of median cleft of the upper lip involving the white roll , which was not associated with any other deformities . treatment included reconstruction of the philtrum and the cupid 's bow while maintaining vermilion fullness and continuity , and minimizing scar formation . various techniques have been advocated for treatment of this type of median upper lip cleft . here we describe a technique using pfeifer incision to correct our patient 's defect . pfeifer incision consists of wavy lines and its use has been advocated for correction of various craniofacial abnormalities .

spontaneous pneumothorax ( sp ) comprises the largest group and is classified into primary spontaneous ( psp ) and secondary spontaneous pneumothorax . psp usually occurs in young , tall , thin men , especially smokers , in the absence of an underlying lung disease . although , sp is a relatively common condition , bilateral primary spontaneous pneumothorax ( bpsp ) is a very rare clinical condition with an occurrence ranging from 1.3 to 1.9% of all cases of sp . herein , we present a case of bpsp in an overweight young male patient , which is an exceptionally rare event . an otherwise - healthy , non - smoker , 20-year - old , overweight ( bmi 29.8 kg / m ) greek male patient was presented to the emergency department complaining of low - intensity chest pain and breathlessness of progressive worsening over the preceding 7 days . on admission , blood pressure was 125/70 mmhg , heart rate 100 beats / min , sao2 97% on air and axillary temperature 36.8c . initial plain chest x - ray demonstrated bilateral pneumothoraces ( more prominent on the left side ) and no deviation of the trachea ( fig . 1 ) . plain chest x - rays on admission revealing bilateral pneumothorax . on emergency basis , the patient was managed with bilateral tube thoracostomy through the fifth intercostal spaces . during hospitalization , both lungs were gradually expanded ; the use of continuous aspiration was necessary for a couple of days , in order to achieve complete expansion of the left lung ( fig . 2 ) . next day the plain x - ray revealed recurrence of pneumothorax on the left side ( fig . the tube was removed after 5 days ; no recurrence took place at this time . during the next 3 days the patient remained under observation ; serial physical examinations and chest x - rays were normal and the patient was discharged home . recurrence of pneumothorax on the left side after removing the chest tube . during hospitalization a chest computed tomography ( ct ) was performed and revealed the presence of blebs at the apices of both upper lungs ( fig . the patient was referred to a tertiary center for an elective video - assisted thoracoscopic surgery intervention ( vats ) . three years after , no recurrence of pneumothorax took place and the patient remains healthy . psp usually occurs in the absence of any obvious underlying lung disease ; however , blebs and bullae apparently play a role in the pathogenesis of psp , as they are found on ct or during thoracoscopy or thoracotomy in 4879% of these patients . diagnosis of bilateral pneumothorax presents difficulties ; while unilateral pneumothorax is relatively easily suspected from the patient 's medical history , physical and radiological findings , the diagnosis of bilateral pneumothorax is usually delayed . the presence of equally diminished breath sounds in both sides and the presence of a not deviated trachea often mislead clinicians and delay chest drain insertion which is usually performed after the transaction of chest ct . although sp is a relatively common condition , bpsp is a very rare clinical condition . lee et al . , in their study of the 616 patients with 807 episodes of psp , found that the incidence of bpsp was 1.6% ( 13 patients ) . in univariate regression analysis , patients with psp compared with patients with bpsp had significantly lower body weight , bmi , higher body height / body weight ratio and higher incidence of bilateral blebs / bullae seen in hrct of the lung . however , in multiple regression analysis , only bmi and the presence of bilateral blebs / bullae retained statistically significant importance . huang et al . found that only lower bmi and smoking were significantly associated with the formation of bpsp . in their study , the proportion of bilateral blebs / bullae seen in hrct was higher in the bpsp group than that in the psp group ( 63 vs 53% , respectively ) , but the difference was not statistically significant ( p = 0.724 ) . the present report describes a case of bpsp in an overweight patient showing that psp is an existing condition in patients with bmi value higher than the normal . generally , if an sp affects < 20% of one lung , observation is efficient ; the absorption rate of the air is 1.25% ( 5075 ml / day ) . if pneumothorax affects > 20% of the lung or if it increases during observation , chest tube drainage may be required . air leakage can be diminished in 5 h and in 48 h in 52 and 82% of patients with tube thoracostomies , respectively . in patients with bpsp , one side should always be drained regardless of the extent of pneumothorax , whereas the other side can be managed by simple observation depending on the extent of the air in the pleura space . prolonged air leakage is the most common indication for operation in the first episode of pneumothorax . today compared with open thoracotomy , this procedure provides visualization of the entire thoracic cavity by video and causes less postoperative pain . single - stage bilateral vats procedure for bpsp has been advocated as an elective procedure to avoid subsequent anesthetic and operative procedures and longer hospital stays . if any underlying pulmonary disease is detected during surgical treatment , the existence of pleural communications should be investigated and mediastinal pleura should be examined carefully . in addition , apical pleurodesis further reduces the risk of recurrence . in our case , the patient was successfully managed initially with a bilateral tube thoracostomy and finally with an elective bilateral vats procedure . in conclusion , bpsp is a rare clinical condition and usually develops in patients with low bmi and bilateral blebs / bullae . bpsp in overweight - obese patients is an existing condition and its early diagnosis requires high suspicion index . bpsp needs urgent assessment and management , followed by bilateral single - stage vats treatment as a safe and effective procedure .

bilateral primary spontaneous pneumothorax ( bpsp ) is a rare condition , accounting for 1.6% of spontaneous pneumothoraces . patients with primary spontaneous pneumothorax have typically low body weight . bpsp in overweight / obese patients is an exceptionally rare event . the present report describes a case of an otherwise - healthy 20-year - old male patient with bmi 29.8 kg / m2 presented to our hospital owing to low - intensity chest pain and shortness of breath 7 days after the onset of symptoms . plain chest x - ray revealed the presence of bilateral pneumothorax . chest tubes were inserted on both sides . during hospitalization , a computed tomography demonstrated multiple blebs on the surface of the upper lobes of both lungs . hereupon , the patient referred to a tertiary center and underwent elective single - stage minimally invasive bilateral video - assisted thoracoscopic surgery . the present case report shows that bpsp in overweight / obese patients is an existing condition .

the prevalence of syphilis in the normal population has decreased significantly since 1977 in korea ( 1 , 2 ) . however , in western countries the incidence of syphilis has risen , mainly in the 20 - 24 age group and its clinical manifestations have been various ( 3 ) . sexual activity has become more acceptable in our culture recently , initiating various forms of sex , and as a result , many different clinical conditions of sexually transmitted disease ( std ) have appeared . it is usually asymptomatic and less frequently presents as proctitis , ulcer and pseudotumors ( 3 , 4 ) . therefore , it is difficult to diagnose and physicians occasionally might prescribe inappropriate treatments ( 5 , 6 ) . in this paper we describe a case of primary rectal syphilis which was suspected to be rectal cancer . he complained of bowel habit change with tenesmus , mucous discharge , anal pain and intermittent presence of blood in stool . he had a 2 cm sized , firm , non - tender and indurated nodule on the left side of the inguinal area and multiple condyloma acuminatum around the anus . sigmoidoscopy in our gastrointestinal endoscopy unit showed two indurated masses of 1.5 to 2 cm on the posterior wall of the middle and lower rectum ( fig . the masses had a coin like appearance , and each of them had a slightly depressed and ulcerated surface . the vdrl quantitative test was 1:64 and the fta - abs igm and igg test was positive . a human immunodeficiency virus ( hiv ) the patient was treated with intramuscular penicillin g benzathine ( 2,400,000 iu im/1 week , thrice ) . follow - up sigmoidoscopy after 1 month showed nearly complete regression of the chancre ( fig . a repeat biopsy was done and the histologic result showed focal lymphoid hyperplasia . a vdrl quantitative test after 3 months was 1:1 . most physicians have a tendency to consider rectal ulceration as a neoplasm ( 7 , 8) . similarly in our case symptoms and signs suggested rectal neoplasia and the clinical history did not reveal homosexuality . however , after the vdrl test the patient confessed that he had been raped by a homosexual male . therefore , when rectal syphilis is suspected ( i.e. , perianal condyloma acuminatum associated with inguinal lymphadenopathy ) the history of sexual intercourse , especially rectal intercourse , has to be taken . a dark - field examination is recommended by many studies ( 3 ) . in our case , however , a dark - field examination was not performed , because rectal chancre was highly suggested by the clinical history , anorectal lesions , pathologic findings and positive serologic test . additionally , like other syphilis cases benzathine penicillin therapy induced a rapid regression of the rectal lesions . we believe when a positive serologic syphilis test is associated with ulcerative lesion , additional laboratory tests are not necessary . inguinal lymphadenopathy need not require an initial biopsy when evidence of regional infection is present . usually after antibiotic therapy of at least 4 weeks , most physicians might decide whether a biopsy is necessary . in this case , however , the physician did not have experience recognizing rectal syphilis , so he immediately obtained a biopsy specimen from the inguinal lesion . . in western countries the incidence of rectal syphilis is rising , especially in groups of active homosexual males . bassi et al . emphasized in his case report that endoscopists should keep in mind the rising incidence of syphilis ( 3 ) . therefore an initial physician should encourage his / her patient to inform him / her of the patient 's sexual history .

a 30-yr - old man was referred for suspicious rectal cancer because of ulcerated lesions in the rectum and a palpable mass in left inguinal area . sigmoidoscopy showed two indurated masses and histologic evaluation of biopsy revealed obliterative endarteritis with heavy plasma cell infiltration . both venereal disease research laboratories ( vdrl ) and fluorescent treponemal antibody absorption ( fta - abs ) tests were positive . after injection of penicillin g benzathine for 3 weeks , the rectal chancre and the palpable mass disappeared .

dysphagia is the subjective awareness of difficulty in the passage of solids or liquids from the oropharynx to the stomach . dysphagia can be classified into an oropharyngeal or an esophageal location , and it is caused by neuromuscular motility disorders and mechanical obstruction.1 ) mechanical dysphagia is associated with intrinsic or extrinsic compression , resulting in progressive intolerance to solids . the term , dysphagia aortica , has been used to describe difficulty in swallowing caused by external compression from an ectatic , tortuous , or aneurysmal aorta as a result of age - related degeneration.2 ) dysphagia aortica is classically seen in elderly women with short stature who have hypertension and kyphosis.3 ) we report herein a patient with dysphagia associated with an aortic aneurysm . an 86-year - old woman presented with worsening nausea and vomiting . because of her progressive dysphagia to solids for the last 6 months , she had ingested only semisolids and liquids . three days before seeking evaluation at our hospital , she had difficulty in swallowing liquids , along with nausea and vomiting . the medical history revealed that she had been diagnosed with primary hypertension , an ascending aortic aneurysm , congestive heart failure , moderate aortic regurgitation , and moderate mitral regurgitation 6 years previously . because of old age she had undergone vertebroplasty due to multiple compression fractures of the thoracic and lumbar vertebrae 5 years ago . on admission to the hospital the blood pressure was 130/90 mmhg , the pulse rate was 64 beats / min , the respiratory rate was 28 breath / min , the body temperature was 36.0 , the height was 1.43 m , and the body weight was 37 kg . the physical examination showed a diastolic murmur at the right upper sternal border and a pansystolic ejection murmur at the left lower sternal border . the laboratory findings were as follows : the white blood cell ( wbc ) count was 5,100/mm , the hemoglobin was 11.8 g / dl , the platelet count was 151,000/mm , the blood urea nitrogen ( bun ) was 39.8 mg / dl , the creatinine was 1.5 mg / dl , the total protein was 5.8 g / dl , the albumin was 3.2 g / dl , the lactate dehydrogenase ( ldh ) was 546 her chest radiograph revealed blunting signs at both costophrenic angles , cardiomegaly with a cardio - thoracic ratio of 0.8 , and an enlarged , tortuous aorta ( fig . 1 ) . computed tomography ( ct ) of the chest demonstrated an enlarged , tortuous aorta ( fig . the diameters of the ascending thoracic aorta , the descending thoracic aorta , and the proximal abdominal aorta were 7 cm , 6 cm , and 5.3 cm , respectively . the upper gastrointestinal barium study revealed marked extrinsic compression of the distal esophagus just above the esophagogastric junction ( fig . we concluded that the symptoms and the results of the imaging studies were consistent with dysphagia aortica . although we recommended surgical correction of the aortic aneurysm or percutaneous endoscopic gastrostomy , the patient declined any invasive procedures and she was transferred to a nursing home on the 12 hospital day . the esophagus normally begins on the right side of the thoracic aorta and then descends . then , the esophagus lies on the left side of the aorta and penetrates the diaphragm through the diaphragmatic histus.4 ) the aging process and the accompanying degenerative changes with the loss of elasticity causes a dilated , elongated , and distorted aorta , which may result in a so - called reverse c- or reverse s - shaped aorta.3 ) as a result , the esophagus is pushed and compressed by the aorta against the cardiac chambers , which are anterior in location.5 ) there is no gold standard diagnostic procedure for dysphagia aortica . the association of suggestive symptoms , such as progressive intolerance to solids with concomitant weight loss along with the results of imaging and other diagnostic studies provide a high index of suspicion.6 ) the diagnostic work - up includes radiologic , endoscopic , and manometric studies . on a standard chest radiography and ct scan , the enlargement of the aortic arch and the tortuous dilated aorta can be observed . a barium swallow test may show partial esophageal obstruction and pulsatile movement of the barium synchronous with aortic pulsation.7 ) endoscopy reveals pulsatile extrinsic compression and stenosis of the lower esophagus with proximal dilatation . esophageal manometry may demonstrate a localized high pressure band with superimposed pounding that is synchronous with the cardiac pulsation.8 ) however , the typical findings of dysphagia aortica can be inconsistent . the radiographic findings are often inconclusive because a dilated , tortuous thoracic aorta is frequently encountered in elderly patients with dysphagia . although a ct scan is useful for evaluating not only the aortic lumen , but also the aortic wall,9 ) occasionally this is of no value in assessing compression of the esophagus by the aorta.2 ) false negative results are common for barium swallow studies . in addition , the classical manometric features suggestive of dysphagia aortica also occur in normal subjects.8 ) the differential diagnosis of dysphagia aortica includes various common structural and neuromuscular abnormalities.1 ) gastroesophageal reflux disease and motility disorders are common causes of dysphagia . the co - existence of these conditions and the lack of sensitivity and specificity of the usual diagnostic tests make it difficult to diagnose dysphagia aortica with certainty . wilkinson et al.2 ) demonstrated that a video solid bolus swallow test could be useful in determining the manometric findings that are suspicious for dysphagia aortica when the standard evaluation fails . mild cases may be treated conservatively , such as avoiding sticky solids and feeding on a liquid diet . the surgical procedures include transposition of the distal esophagus , separation of the distal esophagus from the aorta , esophagomyotomy , division of the right crus of the diaphragm , aortic resection , and repair of an aneurysm . for patients who are not candidates for surgery , insertion of a feeding tube via percutaneous endoscopic gastrostomy ( peg ) although we did not perform esophageal manometry , the patient 's symptoms and imaging studies were consistent with the classic findings of dysphagia aortica . although the patient declined any invasive procedures , a peg for a feeding tube might have been helpful for nutritional support . dysphagia aortica is an uncommon type of dysphagia that is caused by extrinsic mechanical compression . it should be differentiated from other causes of dysphagia , such as gastroesophageal reflux disease or motility disorders , because dysphagia aortica often requires surgical intervention that can significantly reduce the morbidity and these interventions can be curative in some situations .

dysphagia aortica is difficulty in swallowing caused by extrinsic compression of the esophagus due to an ectatic , tortuous , or aneurysmatic atherosclerotic thoracic aorta . this condition is very uncommon , and it is usually associated with old age , women with short stature , hypertension , and kyphosis . we report herein a case involving a patient with dysphagia who had an aortic aneurysm .

kcm is itself very rare and distinct type of keratoacanthoma which usually occurs over extremities and scalp is unusual site for development of lesion . treatment is difficult as lesion of kcm present with large raised , rolled borders with peripheral extension . keratoacanthoma ( ka ) is a rapidly evolving tumor , composed of keratinizing squamous cells originating in pilosebaceous follicles and resolving spontaneously if left untreated . it is relatively common , especially in whites occurring in middle age while being uncommon in dark - skinned . . it presents as firm , rounded , flesh - colored or reddish papule ; with a rapid growth phase becoming 10 - 20 mm and then spontaneous healing taking place over three months . there are three rare clinical variants of solitary ka , namely giant ka , keratoacanthoma centrifugum marginatum ( kcm ) and subungual ka . in kcm , lesions are large , reaching upto 20 cms . the most common locations are dorsa of hands and legs , lesions on scalp being rare . we report a rare case of kcm occurring on the scalp which is an unusual site . a 62-year - old male , watchman by occupation presented with asymptomatic raised lesion on scalp since one year . lesion had developed de novo , as a pea sized lesion with gradual increase to cover entire vertex . cutaneous examination revealed a single , irregular , 12 15 cm , yellowish plaque on vertex of scalp with nodular surface and a central crater [ figure 1 ] . on palpation , the plaque was firm , non - tender , non - indurated and not attached to underlying structure . ultrasonography of lesion showed ill defined hypo echoic mass involving epidermis , dermis and subcutaneous tissue . a single , irregular , 12 15 cm , yellowish plaque on vertex of scalp with nodular surface and a central crater . deep punch biopsy revealed exoendophytic , globular , well circumscribed central cup shaped crater , epidermal invagination with marked hyperkeratosis and horn cyst with rich keratin filled crater imparting glassy appearance and well demarcated regular base . . marked epidermal proliferation with crater formation with horn pearls and inflammatory infiltrate at the base of the lesion was suggestive of a fully developed lesion of kcm . complete surgical excision with grafting was done successfully with dramatic improvement with no recurrence at 9 months [ figure 5 ] . exoendophytic , globular , well circumscribed central cup shaped crater , epidermal invagination with marked hyperkeratosis and horn cyst with rich keratin filled crater imparting glassy appearance and well demarcated regular base epidermis forming buttress over the pseudhorn cyst dense nodular lymphocytic infiltrate in the upper dermis surgical excision with grafting the etiology is multifactorial that includes chronic ultraviolet ray exposure , smoking , contact with chemical carcinogens like pitch , mineral oil , tar , trauma and vaccination . the role of human papilloma virus remains inconclusive but in one study , hpv type 6 and 11 were detected within the lesion . it can be localized to any region of the body but is more frequently seen on dorsum of hands and legs . progressive peripheral extension with a raised rolled - out margin and atrophy at the centre is a characteristic feature of kcm . the margin of the lesion showing multiple comedonal orifices giving rise to a cribriform pattern may represent a unique phenomenon of kcm . it is hypothesized that this typical appearance may arise as a result of sequential involvement of multiple adjacent hair follicles in a centrifugal fashion . kcm does not show tendency for spontaneous regression , a feature also seen in giant ka , which grows rapidly reaching a size of 5 cm or more and occurring commonly on nose and eyelids . kcm is differentiated from giant ka by absence of downward vertical spread and destruction of underlying tissue . nevertheless , in giant ka spontaneous involution takes place after several months , often accompanied by detachment of a large keratotic plaque . the other differential diagnoses include squamous cell carcinoma , lupus vulgaris , botryomycosis , blastomycosis - like pyoderma and pseudoepitheliomatous hyperplasia , hypertrophic lupus erythematosus , atypical mycobacterial infections , or deep fungal infections . there are reports that kcm has been treated successfully with oral retinoids ( acitretin , etretinate , or isotretinoin 0.5 - 1 mg / kg / day ) which should be given until complete clearance of lesion . surgical intervention is a preferred mode of therapy keeping in mind that a wide excision is performed to prevent subsequent recurrence . other treatment modalities successfully used include topical 5-fluorouracil , intralesional injections of interferon alpha , methotrexate , or bleomycin , or mohs micrographic surgery . in kcm , lesions are usually too large for excisional biopsy for diagnosis , but certain characteristic features like clinical behavior , appearance of lesions , histological features ( hyperkeratosis and hyperplasia ) , and cytological features ( tumor cells with eosinophilic and glassy cytoplasm , few mitoses ) help clinch the correct diagnosis . first time from india we are reporting case of kcm presenting over scalp which is successfully treated with surgical excision without any recurrence .

keratoacanthoma ( ka ) is a rapidly evolving tumor , composed of keratinizing squamous cells originating in pilosebaceous follicles and resolving spontaneously if left untreated . it is relatively uncommon in dark - skinned and occurs in middle aged individuals . males are three times more affected than females . it presents as firm , rounded , flesh - colored or reddish papule ; with a rapid growth phase followed by spontaneous healing over three months . two types of ka exist i.e. , solitary and multiple . there are three rare clinical variants of solitary ka , namely giant ka , keratoacanthoma centrifugum marginatum ( kcm ) and subungual ka . in kcm , lesions are large , reaching upto 20cms . there is peripheral extension with raised , rolled border and atrophy in the center . there is no tendency toward spontaneous involution . the most common locations are dorsa of hands and legs , lesions on scalp being rare . a rare case of kcm occurring on scalp which is an unusual site is reported .

they are found most commonly in the cranial and peripheral nerves , and occurrence in the omentum is very rare . however , there have been some cases reported to develop serious complications and , if there was malignancy , to cause metastasis or recurrence . we presented a case of schwannoma originating from the great omentum , including histological and immunohistological studies . a 55-year - old man was referred to our department for the treatment of a tumor detected close to the stomach by ultrasound . medical examination including ultrasound had been regularly performed to follow up his gallbladder stone for the past 6 years . although he had no complaints and symptoms , the tumor had been increasing in size over one year . abdominal and endoscopic ultrasound showed a 2.0 1.3 cm cystic mass lateral to the wall of the stomach . its component included an 11.8 5.7 mm elevated lesion ( fig . 1 , fig computed tomography scan of the abdomen and pelvis showed a 2.6 1.9 cm cystic mass , which was slightly enhanced , and a gallbladder stone ( fig . 3 ) . magnetic resonance imaging demonstrated a hyperintense mass on t2-weighted image . a distance from the stomach wall laboratory tests , including tumor makers , were normal . under a perioperative diagnosis of cystic tumor in the abdomen and cholelithiasis , we performed laparoscopic resection with vessel sealing system , which revealed a 2.0 cm mass arising from the great omentum , not adherent to other organs . grossly , the tumor was configured by a well - encapsulated round mass measuring 30 18 15 mm in diameter ( fig . histologically , the spindle - shaped cells were arranged in interlacing bundles and fascicles , together with varying numbers of tumor cells containing various amounts of light brown or grayish pigment ( fig . these alternate with looser antoni b tissue , which is comprised of cells showing clear , vacuolated cytoplasm due to lipid accumulation . immunohistochemically , most of the neoplastic cells reacted moderately to nse , ncam and s-100 protein ( fig . schwannomas are classified as one kind of the peripheral nerve sheath tumors , of which schwannnoma and neurofibroma are the most frequent . currently , the most precise determination of the tumor 's cell type is established by its immunohistochemical profile , ultrastructural features , or both . a tumor composed of cells with distinctly schwannian characteristics schwannomas are known to mainly arise from the peripheral and caranial nerves , the extremities and the retroperitoneum . they are seldom found in the abdomen , especially the extragastrointestinal tract , of which solitaly schwannoma of the great omentum is an extremely rare tumor . in 303 schwannomas reported by das gupta , one case originated from the retroperitoneum , others from the central nervous system and peripheral nerve . in another review of the literature , stout and of these , 37 were in the stomach , 3 in the small bowel , and none in the omentum and the abdominal cavity . previously published cases of schwannoma in the omentum were collected from a computerized medical literature search ( pubmed ) . only 6 cases of schwannoma from the great omentum have been observed ( table 1 ) . we can see that more cases of schwannoma from the lesser omentum have been recorded than from the great omentum . it is mentioned that the lesser omentum contains a small amount of nerves in almost equal distribution , the great omentum has a paucity of nervous tissue and anatomically consists of fat and lymphatic tissue . we found a case of malignant schwannoma arising from the omentum which demonstrated peritoneal metastasis , and another reported case of the small intestine emphasized poor prognosis since only 2 of 24 patients survived for more than 5 years . benign schwannomas are also reported to increase in size and to eventually cause complications by compressing other organs or by causing bleeding in or outside the gastrointestinal tract . pigmented schwannoma is another type of schwannoma , usually arising from the sympathetic nervous system . additionally abdominal schwannomas cause diagnostic problems because clinical symptoms are uncharacteristic or misleading even if the tumor is large . ct imaging typically showed a low attenuation mass , peripheral enhancement and cystic degeneration pattern . mri disclosed schwannoma of low signal intensity on t1-weighted image and high signal intensity on t2-weighted image . in spite of these characteristic , it is difficult to exclude other abdominal tumors , leiomyomas , lymphomas and unspecified sarcomas etc . while previous cases confirm that the tumor may attain a considerable size , bigger than 5 cm or producing symptoms , our case of a schwannoma which was small and asymptomatic is hardly diagnosed . however this tumor showing progression in size is thought to have potential to behave very aggressively despite benign histological features , which stresses the need of more information on this type of tumor and diagnosis . consequently , histological analysis of the surgical specimen is necessary for a correct diagnosis , and common treatment for schwannoma is surgery . therefore laparoscopic resection seemed to be the most adequate method to diagnose and rule out malignant tumor as a minimally invasive surgery . the majority of tumors can be safely resected , the surgeon being careful of the dividing feeder vessels because schwannomas are well known as hypervascular tumors . to our knowledge there are no case reports documenting other treatments , including chemotherapy for schwannomas in the abdominal cavity .

schwannoma in the abdomen is an uncommon neoplasm that occurs most frequently in the cranial and peripheral nerves ; it is extremely rare in the great omentum and only 6 cases of schwannoma of the great omentum have been observed previously . we report the case of a schwannoma found in the great omentum of a 55-year - old man who was treated with laparoscopic surgery . though it was difficult to diagnose preoperatively , the tumor showed malignant potential by rapidly increasing in size . histologically it was configured by a well - encapsulated round mass measuring 30 18 15 mm in diameter . immunohistochemically most of the neoplastic cells reacted moderately to nse , ncam and s-100 protein . we document the clinicopathological study of a schwannoma of the great omentum , followed by a review of the literature .

sweet 's syndrome ( ss ) presenting in a localized and symmetric pattern is a very rare phenomenon . there have , however , been , a few case reports for the same . ss per se was first reported in 1964 by robert douglas sweet . though classically presenting as tender pseudovesicles , ss may demonstrate numerous topographic variations , which could be quite confusing for the diagnosing dermatologist . a 45-year - old farmer from nepal presented to the department of dermatology with complaints of an abrupt onset of an asymptomatic cutaneous eruption involving the neck and both the forearms since the past 5 days . the lesions were smaller earlier which had progressively increased in size over the past 5 days to reach the current status . there were no associated constitutional symptoms and no history of contact with cattle or sheep . , papules and plaques of varying sizes with a striking central umbilication were identified involving the forearms and neck that gave an illusion of vesiculation [ figures 13 ] . some of the lesions over the neck had a crust overlying the depressed center [ figures 4 and 5 ] . gram staining of the tissue obtained from one of the papules after needle extirpation was inconclusive . a skin biopsy from these lesions showed absence of epidermal changes with a dense inflammatory neutrophilic dermal infiltrate along with dermal edema as the hallmark finding , without evidence of vasculitis [ figures 68 ] . laboratory parameters revealed a hemoglobin level of 14 g / dl , a total white blood cell count of 13,000 cells / mm with 89% neutrophils , and a normal peripheral smear . his renal and hepatic profiles were normal , and imaging studies of the abdomen and pelvis were insignificant . the patient was diagnosed as ss and started on 20 mg of prednisolone once daily for 2 weeks and 100 mg of dapsone once at bedtime for 2 weeks . fleshy pseudovesicles with central umbilication seen on the right forearm of our patient over the left forearm , a plaque with the characteristic central umbilication seen , closely mimicking the lesions encountered in molluscum contagiosum . furthermore , to note here is the symmetric pattern of lesion arrangement molluscoid pseudovesicles over the nape of the neck juicy plaques with a central depression and an overlying crust seen on the right side of the neck similar lesions on the left side of the neck as seen in figure 4 . to note here again , is the striking symmetric distribution of the nuchal lesions section of the plaque on the right forearm demonstrating dermal edema and a dense dermal inflammatory infiltrate ( h and e , 10 ) same section showing a dense inflammatory infiltrate composed of neutrophils around the blood vessels and also diffusely distributed ( h and e , 20 ) absence of vasculitis on microscopy ( h and e , 40 ) sweet 's syndrome is the prototypical neutrophilic dermatosis , generally characterized by fever , an increase in the neutrophilic count along with tender pseudovesicles distributed over the body in an asymmetric manner . etiologically , three types of ss have been described , namely the idiopathic , paraneoplastic , and the drug - induced type of ss . as mentioned earlier , there have , however , been reports of the occurrence of ss localized to specific sites of the body though , some of which have presented with very unusual morphologic patterns , as shown by sommer et al . who described palmoplantar pustulosis as the sole finding in a ss patient , verma et al . demonstrating the occurrence of ss confined to the photo exposed sites alone , verma who elucidated a rare recurrent bullous eruption localized to the flexural aspect of both the forearms in a ss patient and brechtel et al . who reported a localized facial presentation of ss manifesting as an inflammatory lesion with a central depression superimposed with several pustules on the surface of the plaque . a variant of ss referred to as neutrophilic dermatosis of the dorsal hands ( nddh ) is a specific entity confined only to the dorsa of the hands and has been extensively reviewed in literature by galaria et al . , takahama and kanbe , cook et al . nddh usually presents as edematous and violaceous plaques and papulonodules on the radial aspect of the dorsal surface of both hands . our patient presented with molluscoid pseudovesicles distributed over the neck and forearms only , which is a very rare morphologic pattern witnessed in ss and to the best of our knowledge has not been previously described . second , our patient lacked the usual constitutional symptoms that herald ss , which made the diagnosis a challenge . third , as no systemic association with ss could be identified in our patient , he was further sub - classified as a case of idiopathic ss , which again is a rarity in male patients , who usually present with the paraneoplastic or the drug - induced variant of ss . the hallmark finding in our patient was the central depression in all the pseudovesicles examined . the other possibilities , we kept in mind after clinically examining our patient , were , atypical erythema multiforme , histioid leprosy , milker 's nodule , cowpox , and ecthyma contagiosum , but these were eventually ruled out . hence , to conclude , we see that ss is a dermatosis that could be a great mimicker just like leprosy and syphilis and therefore when faced with diagnostic dilemmas in these kinds of atypical presentations , it would be worthwhile considering ss as a differential diagnosis . idiopathic sweet 's syndrome ( ss ) may even occur in males though highly uncommonthe absence of constitutional symptoms does not necessarily rule out the diagnosis of ssthe plaques encountered in ss could even be asymptomatic.molluscoid lesions seen in ss is a very rare morphologic pattern encountered and to the best of our knowledge is being reported for the 1 time in literaturelocalized patterns of ss are not so rare as previously thought . idiopathic sweet 's syndrome ( ss ) may even occur in males though highly uncommon the absence of constitutional symptoms does not necessarily rule out the diagnosis of ss the plaques encountered in ss could even be asymptomatic . molluscoid lesions seen in ss is a very rare morphologic pattern encountered and to the best of our knowledge is being reported for the 1 time in literature localized patterns of ss are not so rare as previously thought .

sweet 's syndrome is a well - known entity in the field of dermatology . it has presented itself in myriad forms , well - recorded in literature . our patient presented with a sudden eruption of nontender molluscoid pseudovesicles distributed over the neck and forearms alone . a complete diagnostic work up for the same did not give any clue regarding an underlying systemic ailment , responsible for the dermatosis . this case is being presented because of this extremely rare morphology of gomm - button disease .

we used monthly reports of slide - confirmed malaria and annual census based population data from 434 counties ( municpios ) in the brazilian amazon region for 19961999 , during which no coordinated national malaria interventions occurred ( 12 ) . to study the relationship of reported malaria cases to climate , we used monthly precipitation and temperature from the cru ts 2.1 gridded climate data set for selected states ( 13 ) ( figure 1 ) . to consider how the precipitation malaria relationship depends on surface water conditions , including the extent of open water and wetlands , we used 100 m 100 m maps from the jers-1 synthetic aperture radar satellite and calculated the percentage of maximum inundatable open water and wetland coverage for each county ( figure 2 , panel a ) ( 14 ) . in this region , monthly temperatures were between 24.6c and 29.4c ( well within the range for optimal malaria transmission ) for 95% of the observations ( 18,416 of 19,364 ) included in the analysis ( temperature relationships not shown ) . malaria incidence per 1,000 population ( black lines ) and mean monthly precipitation ( blue lines ) during la nia ( orange bars ) and el nio ( red bars ) events for the states of a ) amazonas , b ) mato grosso , and c ) roraima . a ) percentage of wetlands in amazon basin counties ( shades of blue ) , counties without wetlands data ( yellow ) , and counties with < 80 total malaria cases ( gray ) . wetland colors correspond to percentage wetland values in panel d. b ) risk ratios for malaria incidence for 1 sd ( 14 cm ) change in monthly precipitation ( january 1996december 1999 ) , plotted at each county seat of government ; c ) spatially smoothed risk ratios for 14-cm changes in monthly precipitation . in both panels , red shaded squares show reduced risk for 14-cm increase in monthly precipitation ; blue shaded squares show increased risk for malaria with increased precipitation . d ) boxplot of risk ratios for malaria incidence for 14-cm changes in monthly precipitation , by percentage wetland cover . if the lag and the rainfall coefficient vary across regions , meaningful geographic comparisons would be difficult to achieve because neither the lag nor coefficient have consistent meanings across models . to interpret results , either the coefficient must be fixed and the lags varied ( difficult to do ) or the lags must be fixed and the coefficients varied ( relatively easy to do ) . the aim is to describe the variable patterns of malaria incidence and precipitation , not create a highly predictive model . we chose to fix the lag and vary the coefficients . to assess the association between malaria incidence and precipitation data , we estimated the rate ratio of malaria incidence associated with 1 sd - increase in monthly precipitation ( 14 cm ) for each county by using the following poisson regression model , which includes a flexible temporal trend represented as a natural cubic spline with 6 degrees of freedom ( figure 2 , panel b ) : malariait poisson(it ) log it = log popit + i + iprecipit + fi(t ) i normal(g(lati , loni ) , ) the ( estimated ) regression coefficients from the county - specific models were then modeled as a spatially smooth surface , a thin - plate spline . degrees of freedom for the thin - plate spline were selected using generalized cross - validation ( figure 2 , panel c ) . the relationships between precipitation and malaria incidence in the amazon basin are spatially varied and change signs , depending on the region . positive correlations between monthly precipitation and malaria incidence ( rate ratios > 1 ) occur in the upland regions of the southwest and central amazon basin , whereas negative correlations between precipitation and incidence ( rate ratios < 1 ) occur in the north , largely along the main waterways of the amazon river and the major wetland regions of the basin ( figure 2 ) . for a 14-cm increase in monthly rainfall , the malaria rate can double in the upland area , yet decrease by up to 80% along the main amazon channel . the p values of the precipitation coefficient are 0.00020.0009 along the main waterways and 0.0040.10 in uplands areas . we hypothesize that this reversal of the malaria precipitation relationship from positive to negative is related to the extent of open water and wetlands in the basin . mosquito habitats in wetlands or along large rivers may wash out or become too deep during months with high precipitation , but in areas with fewer wetlands , mosquito habitats are limited by precipitation . to test this hypothesis , we compared the malaria precipitation association for 338 counties that reported > 80 cases of malaria over the 48 months against the estimated percentage of open water and wetland cover for each county ( figure 2 , panel d ) . as expected , the precipitation - linked risk for malaria fell as the percentage of wetland in each county increased , but the risk for malaria varied in counties with low percentages of wetlands . the central - east region had the lowest level of malaria incidence , which may explain why this region also lacked a malaria - precipitation relationship . studies have proposed that flooding created new pools of water suitable for mosquito larvae as the water levels slowly receded from alluvial forests along the rio branco river in roraima and the maroni river on the frontiers of suriname and french guiana ( 6,15 ) . our results suggest that monthly precipitation along the amazon basin can have both strong positive and negative associations with malaria incidence . further research is needed to address the limitations of our study , including its short time frame and the crude countywide approximation of percentage wetlands as an exposure . the quality and reliability of the health data were concerns , but we verified that the distribution of null reporting was unbiased temporally and spatially . also , our study did not quantify increasing malaria incidence in response to increasing or decreasing precipitation or the impact of lag factors . instead , we focused on the seasonality of these patterns until longer data series of malaria incidence and climate data are available . our evidence suggests that precipitation drives malaria risk in the amazon basin , but the relationship varies in the uplands ( more precipitation , more / less malaria ) and is negative in areas dominated by wetlands and large rivers ( more precipitation , less malaria ) . our findings show the need to account for local landscape characteristics , especially the extent of wetlands and open water , in regional to global projections of the effects of climate change on malaria . better understanding the impact of climate and landscape on malaria will improve our ability to assess health risks .

climate changes are altering patterns of temperature and precipitation , potentially affecting regions of malaria transmission . we show that areas of the amazon basin with few wetlands show a variable relationship between precipitation and malaria , while areas with extensive wetlands show a negative relationship with malaria incidence .

the clavicle is a rare site for bone tumours . according to the world health organisation , the giant cell tumor is an aggressive potentially malignant lesion which means that its evolution based on histological features is unpredictable . sites commonly affected by giant cell tumour are proximal tibia , distal femur and distal radius . the oncologic patterns of clavicle resemble that of flat bones and not other long bones . among tumors of clavicle , a 60 year old man presented to our department with pain and swelling over lateral end of left clavicle ( fig . 1 , fig . the pain was insidious in onset , non radiating and had no diurnal variations and was aggravated on shoulder movements and relieved on taking medications . we got a plain radiograph which revealed which an expansile radiolucent lesion arising from lateral end of left clavicle ( fig . 3 ) . the swelling demonstrated geographic type destruction without any soft tissue component or periosteal reaction . 4 ) . to aid in the diagnosis fine needle aspiration cytology was done which revealed a predominantly cellular lesion having sheets of plump , oval mononuclear cells with mild pleomorphism . the cells had moderate cytoplasm , oval to elongated nucleus with moderate anisokaryocytosis with irregular nuclear membrane . amongst these cells , many multinucleated giant cells were also which were distributed evenly . the differential diagnosis which were kept in mind are aneurysmal bone cyst , non ossifying fibroma , eosinophilic granuloma and tuberculous osteomyelitis . since the clavicle does not necessary require reconstruction and the patient was a retired school teacher , not engaged in any physical work so surgical resection of the tumor was planned . after proper investigations and pre anaesthetic clearance , a wide excision of the mass along with 3 cm of the healthy tissue was done ( fig . the excised mass was sent for histopathological examination which also confirmed it to be a giant cell tumor . the range of motion of the left shoulder was normal and post operatively there was no neurovascular deficict . the patient was happy with the surgical outcome and at 1 year follow up there was no evidence of recurrence or metastasis . . primary bone tumors of the clavicle are more likely to be malignant than benign , and amongst these tumors which occur in clavicle , giant cell tumor is a rare entity . the differential diagnosis of giant cell tumor of clavicle which pose a diagnostic challenge both for the surgeon and the histopathologist are primary aneurysmal bone cyst , non ossifying fibroma.eosiniphilic granuloma and tuberculous osteomyelitis . giant cell tumor is basically a cellular lesion made up of sheets of plump mononuclear cells with mild pleomorphism . the cells have moderate amount of cytoplasm , oval to elongated nuceuswith moderate anisokaryosis with irregular nuclear membrane and 01 nucleolus . amongst these cells are multinucleated giant cells distributed in a regular fashion.no collagen formation , no new bone formation or no necrosis is usually seen . giant cells in abc are smaller as compared to giant cell tumor and their arrangement is loose with collagen formation . histologically it has histiocytes loaded with lipid and hemosiderin and spindle cells arranged in storiform or whorled pattern and there is presence of collagen fibres . eosinophilic granuloma has diagnostic langerhans cells and also it has large number of leucocytes , fibroblasts , plasma cells and lymphocytes . curettage remains the main stay of treatment for giant cell tumors but for giant cell tumors occurring in expendable bones like distal ulna , iliac wing or proximal fibula , en bloc resection is performed without any reconstruction . after extensive search of literature it was found that there are very few case reports describing giant cell tumors of clavicle , , . due to the paucity of the available literature no definite treatment guidelines are available on the management of giant cell tumor of clavicle . some authors reported not so favourable outcomes after total claviculectomy due to pain , loss of muscle strength and dropping of shoulder . while some authors established that total or subtotal excision of clavicle was rarely associated with loss of function . based on their reports we also performed partial claviculectomy and at one year follow up , patient was well satisfied with the clavicle is a rare site for bone tumors and shares its oncological behavior with that of flat bones rather than long bones . we have reported this case to emphasize the fact any expansile lytic lesion occurring in the lateral end of clavicle should be taken seriously and the diagnosis can be easily missed both clinically and radiographically if the clinician is not aware of the wide array of differential diagnosis which range from an infectious etiology like tuberulous osteomyelitis to a neoplastic etiology like giant cell tumour . there seems to be a difference in opinion regarding functional outcome after claviculectomy for tumour like lesions of clavicle and our case report further highlights the fact the claviculectomy without any reconstruction seems to be a good option with no disability noted in long term . since it is a single case report involving a single subject and we are not reporting the first case of this type in the literature , hence no approval was taken from the relevant ethics committee but written informed consent was taken from the patient to publish his details and clinical photographs . written informed consent was obtained from the patient for publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request . ak , kk and js contributed to the development of protocol and edited the manuscript .

highlightsgiant cell tumour of clavicle is a rare entity.the differntial diagnosis of giant cell tumour of clavicle which pose a diagnostic challenege gor both surgeon and histopathologist are aneurysmal bone cyst , non ossifying fibroma , tubercular osteomyelitis and eosniophilic granuloma.claviculecomy is a good option with no disability noted at long term .

the pylorus , duodenal c - loop , and ileocecal valve are the three physiological narrowings in the gastrointestinal tract , and most of the swallowed indigestible foreign bodies pass through it without complications . however , foreign bodies such as a toothbrush can not pass out of the stomach , and the gastrointestinal tract should get rid of these objects as soon as possible to avoid pressure necrosis and gastrointestinal perforation . although these objects are extracted either by endoscopy or laparoscopic gastrostomy , we devised an innovative technique by using pneumatic gastric insufflation and extracted the toothbrush by a tiny gastrotomy under local anesthesia . a 35-year - old male presented in our hospital at m.m . institute of medical sciences and research ( mmimsr ) , mullana , ambala , haryana , india in may 2013 ; he had accidentally swallowed a toothbrush two months back and there was a history of epigastric discomfort especially after meals . however , the vital signs were within normal limits and the abdomen was soft and nontender . x - ray of the abdomen suggested the presence of a foreign body and a computed tomography ( ct ) scan was done which confirmed a toothbrush lying in the stomach [ figures 1 , 2 ] . an upper gastrointestinal endoscopy was done which revealed the toothbrush in the stomach with its head toward the gastroduodenal junction . biopsy forceps were used to deliver the toothbrush by holding its bristles [ figure 3 ] . the endoscope was kept inside to insufflate and distend the stomach and a minilaparotomy with gastrotomy of 1.5 - 2 cm was performed through the midline under local anesthesia and the toothbrush was successfully removed . x - ray of the abdomen suggesting the presence of the toothbrush in the abdomen computed tomography ( ct ) scan of the abdomen showing the presence of the toothbrush in the tomach endoscopic picture of toothbrush with biopsy forceps in situ to retrieve it from the stomach in the stomach , 80 - 90% of foreign bodies pass uneventfully through the gastrointestinal tract without complications . however , objects longer than 10 cm like a toothbrush can not negotiate the duodenal c - loop due to its fixed position in the retroperitoneum , and these must be removed as soon as possible to avoid pressure necrosis and gastric perforation . more than 40 cases of toothbrush ingestion have been reported in the literature till date . however , extreme caution and experience of the endoscopist is required for such procedures . in failed cases of endoscopic removal , however , we devised a simple technique of minilaparotomy and gastrotomy under local anesthesia for removing such foreign bodies . in this technique , the stomach is distended with the help of air insufflation through the endoscope and a small incision is made in the midepigasrium under local anesthesia and the foreign object can be removed directly under the vision of the endoscope . a swallowed toothbrush is a rare occurrence and it never passes through the gastrointestinal tract spontaneously . early removal of the toothbrush is critical for reducing morbidity and mortality . in cases where endoscopic removal fails , endoscopy still remains an aid in performing surgical gastrotomy for delivering such complex foreign bodies under local anesthesia .

most of the ingested foreign bodies pass uneventfully through the gastrointestinal tract . however , long and rigid foreign bodies are associated with an increased risk of gastrointestinal impaction , perforation , and bleeding . spontaneous passage of a toothbrush has not been reported till date and the technique of its removal is a curiosity for surgeons . endoscopy is a recommended technique for the removal of such complex foreign bodies . however , if it fails , the foreign body can be removed successfully with a laparoscopic gastrotomy . we devised an innovative technique by using pneumatic gastric insufflation and extracted the toothbrush by a tiny gastrotomy under local anesthesia .

para - aortic and pelvic lymphadenectomy is often performed in the treatment of ovarian or testicular cancer . internal herniation of a small bowel behind the external iliac artery after lymphadenectomy is a very rare complication to this procedure and to our knowledge only reported twice in the literature . in the first paper published in 1978 the author described how they managed their patient with laparotomy and resection of a perforated small bowel . the hernia orifice was closed by using a free peritoneal graft harvested from the under surface of the anterior abdominal wall . this patient had to undergo several surgical procedures to re - establish blood flow to the extremity because of trombosis of the iliac artery . as there are few similar cases reported worldwide , there is no consensus or guidelines available . we did not close the orifice . because of the skeletonized vessel we were afraid of compromising the vessel if we tried to close the defect . at follow - up after 10 months a 56-year - old woman underwent in 2008 prophylactic bilateral laparoscopic salpingoophrectomy because of a mutation in brca1 gene . the histological result revealed a serous papillary adenocarcinoma in the right ovary and on its surface . therefore a restaging operation was performed . she underwent laparotomy with total abdominal hysterectomy , omentectomy , appendectomy , with a radical retroperitoneal lymphadenectomy which involved en bloc dissection and removal of the para - aortic and iliac lymphe nodes . there was no metastasis in the biopsies taken from the rectum wall and the laparoscopy trocar ports as well as the 46 harvested lymphe nodes . the patient did not receive any radiation therapy . in 2012 , 4 years later , she was admitted to hospital with a 2 days history of severe abdominal pain , vomiting and the inability to pass gas or stools for the last few days . the abdomen was distended , diffused tender and tympanitic , without guarding or rebound tenderness . her groin examination was normal without any sign of herniation through the femoral or inguinal canals . blood pressure 140/90 mmhg , pulse 71 beats / minute and a temperature of 37.9 c . our patient received 90 ml of iomeprol 350 mgl / ml i.v . and this was sufficient to see the vessels . the ct scan demonstrated transition zone both where the loop entered behind the external iliac vessel and where the intestine passed out behind the vessel as a closed loop . the small bowel was dilated both proximal to , as well as inside the closed loop ( figs . 2 and 3 ) . the patient received a prophylactic antibiotics composed of metronidazole 1.5 g and doxycycline 400 mg i.v . we established pneumoperitoneum with a pressure of 12 mmhg and inserted two other working ports of 12 mm and one of 5 mm . we examined the non dilated bowel from the ileocoecal junction , approximately 200 cm from the ilecoecal junction the small bowel made a closed loop underneath the left external iliac artery ( picture 1 ) . the small bowel was strangulated by the external iliac vessel . by gentle manipulation we reduced the herniated small bowel ( picture 2 ) that soon recovered . the blue color and venous congestion were fading out and we noticed peristaltic movement of the previously herniated bowel we therefore did not perform any bowel resection . the defect underneath the artery was about 23 cm ( picture 3 ) in diameter . we found it risky to try to close the orifice and decided to leave it unrepaired . we noticed pulsation on the iliac vessel after the small bowel reduction and we evaluated the pulsation of the dorsalis pedis artery and the posterior tibial artery during the operation . the abdomen was exsufflated and the fascia defects in the 12 mm ports were sutured and the skin closed with stapler . the patient was observed in the intensive care unit until the next morning , with focus on her leg pulse and color . dalteparin 5000 ie was given subcutaneously 6 h after surgery and continued for the first 24 h after surgery . the postoperative course was uneventful and she was discharged from hospital the day following operation . due to limited peritoneum in the area of the hernia defect and the skeletonized external iliac artery , we did not do any direct suture of the orifice as this might have compromised the artery and/or the vein . due to previous omentectomy , an alternative might have been to harvest a free peritoneum graft to cover the defect as described in the two other cases . another option was to use a mesh covered with an oxidized regenerated cellulose or expanded polytetrafluoroethylene ( eptfe ) that minimize bowel adhesions . a longer follow - up of the patient is needed to clearly conclude if this simple procedure has been sufficient . if the patient later experience any sign of recurrence and need another operation we will have to close the defect . a challenge would have been if the bowel could not be reduced due to long standing obstruction . in such a case a bowel resection and/or a vascular surgical procedure might have been necessary . the small bowel in ileus might be fragile , gangrenous or necrotic and there is probably a higher risk of bowel perforation performing a laparoscopic approach . we do not routinely use verres needle in laparoscopy , we prefer to use visiport as an optical trocar or an open hasson approach that is another safe alternative to minimize the chance of bowel perforation on entrance into the abdominal cavity . strangulated internal hernia behind the iliac vessel is a rare entity only reported twice in the literature . this is the first case in the literature where laparoscopically reduction of the internal herniated bowel underneath the iliac vessel is performed . a quick recovery with no complications , short hospital stay and minimal discomfort postoperatively made our approach an acceptable surgical option for this patient . written informed consent was obtained from the patient for publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request .

introductioninternal herniation of a small bowel behind pelvic vessels is a rare complication seen after pelvic lymphadenectomy.presentation of casea 56-year - old woman was operated due to a gynecological cancer . 4 years thereafter she presented with a 2 days history of abdominal pain and vomiting . clinical and radiological findings indicated a small bowel obstruction . a loop of small bowel had herniated behind the left external iliac artery . using laparoscopic technique the herniated bowel was reduced . due to limited peritoneum around the area and skeletonized vessel , we decided not to do any repair of the hernia orifice . the postoperative recovery was uneventful , bowel activity returned to normal and she was discharged the next day.follow-up was done at 1 month and the latest at 10 months . she did nt experience pain or discomfort after the operation.discussiondue to limited peritoneum around the skeletonized vessel , we decided to leave the hernia orifice unrepaired . we found it hazardous to do any direct suture of the orifice or use a free peritoneal graft to repair the defect as the fibrosis and inflammatory process might have compromised the artery or the vein . a longer follow - up of the patient is needed to clearly conclude if this simple procedure has been sufficient . we agreed that if the patient would experience any sign of recurrence and need another operation we would close the defect at that time.conclusion4 years after pelvic lymphadenectomy a small bowel herniation behind an external iliac artery occurred . the patient was successfully treated with reduction of the small bowel using laparoscopic technique . a quick recovery with minimal discomfort and no sign of recurrence after 10 months made our approach an acceptable surgical option .

fibro - osseous lesions of the jaws represent a diverse group of entities which include developmental ( hamartomatous ) lesions , reactive or dysplastic processes and neoplasms . benign fibro - osseous lesions of the head and neck region are uncommon and represent a wide range of tumors sharing some histopathological features ; which comprise fibrous dysplasia , ossifying fibroma ( of ) , and cement - osseous dysplasia . of can be further divided into conventional and juvenile forms ( juvenile ossifying fibroma ( jof ) ) . jof has to be distinguished from a larger group of ofs on the basis of the age of the patient , site of involvement and clinical behaviour . according to the world health organization ( who ) classification of odontogenic tumors 2005 , jof is further subdivided into psammomatoid jof ( psjof ) and trabecular jof ( trjof ) . benjamins in 1938 first reported psjof as an osteoid fibroma with atypical ossification of the frontal sinus , golgi in 1949 called it as psammomatoid ossifying fibroma , and johnson in 1952 coined the term juvenile active ossifying fibroma . according to who classification of odontogenic tumors 2005 , it was named as the term juvenile psammomatoid ossifying fibroma is now used to designate the neoplasm of the craniofacial skeleton of young age with well - defined clinicopathological features . the pathogenesis of these jaw lesions are related to the abnormal development of basal generative mechanism that is essential for root formation . this paper highlights a rare entity of psjof involving the maxillary sinus and nasal cavity on the left side . a 20-year - old female patient reported with a painless progressive swelling in the left cheek region and difficulty in breathing since 1 year . past medical history revealed that the patient underwent surgery in a private dental clinic 5 years back for the impacted tooth 23 which was associated with pathology . the swelling was 6 4 cm in size approximately extending anteroposteriorly from ala of the nose to 5 cm from tragus of the ear on the left side . nasal polyp was seen in the left nostril with obliteration of left ala of the nose [ figure 1 ] . nasal polyp seen in left nostril with obliteration of left ala of nose intraoral examination presented a swelling extending anteroposteriorly from the distal aspect of 21 to mesial aspect of 26 ; medially till the mid palatine raphe and laterally the buccal vestibule was obliterated and the swelling extended from 21 to 26 . paranasal sinus ( pns ) x - ray revealed haziness in the left maxillary sinus . computed tomography ( ct ) scan confirmed well - defined mixed radiolucent and radiopaque areas with calcifications extending superoinferiorly from infraorbital rim to alveolus and anteroposteriorly from the nasal septum to post zygomatic buttress on left side [ figure 2 ] . axial computed tomography revealing well - defined radiolucent and radiopaque areas with calcifications histopathological examination of incisional biopsy revealed connective tissue stroma with numerous spherical / irregular ossifications interspersed with cellular fibrous tissue [ figure 3 ] . the ossifications showed peripheral brush border surrounded by an eosinophilic rimming [ figure 4 ] . haemorrhagic areas were also seen . the constellation of clinical , radiological , and histopathological features of this lesion supported an interpretation of psammomatoid variant of jof . photomicrograph of hematoxylin and eosin ( h&e ) stained section ( 100 ) showing numerous spherical and irregular calcifications interspersed with fibrous tissue photomicrograph of h&e stained section ( 400 ) showing peripheral brush border surrounded by eosinophilic rimming under general anesthesia , a weber - ferguson incision was given to expose the complete lesion . subtotal maxillectomy was performed and the tumor mass along with nasal and ethmoidal polyps were removed with the help of a chisel and mallet . after complete removal of the mass , borders were carefully osteotomized to avoid the chances of recurrence [ figure 5 ] . the excised specimen was sent for histopathological examination [ figure 6 ] and the diagnosis of psjof was confirmed . jof are benign , potentially aggressive fibro - osseous lesions of the craniofacial bones . the word psjof is a unique variant of jof that has a predilection for orbit and pnss accounting for about 72% followed by calvarium 11% , maxilla 10% , and mandible 7% . the ethmoidal sinuses are most commonly involved , followed by the frontal sinuses , the maxillary sinuses , and the sphenoid sinus . both the variants of jof show a predilection for males . the swelling in this case is associated with maxilla involving maxillary sinus and nasal cavity in a 20-year - old female patient . table 1 summarizes the clinical presentation of case series of psjof reviewed in the literature . the patients may develop exopthalmoses , bulbar displacement , and nasal obstruction . in the present case because of the extension of the tumor into the nasal cavity , nasal obstruction was present on the left side . radiographically , psjof can be radiolucent , radiopaque , or mixed depending upon cystic changes and the degree of calcification . sclerotic changes are evident in the lesion which may show a ground - glass appearance . histologically , psjof shows highly cellular fibrous stroma often with whorled pattern containing closely packed spherical ossicles resembling psammoma bodies . these ossicles are round to oval in shape which have a basophilic center and peripheral pink rim showing some radiating fibers which corroborates with the microscopic features provided in the present case . psjof should be differentiated from extracranial meningioma with psammoma bodies , which demonstrates epithelial membrane antigen ( ema ) positivity and even the psammomatoid ossicles in psjof are clearly different from spherical true psammoma bodies . other differential diagnosis include fibrous dysplasia , osteoma , cementoblastoma , well - differentiated osteosarcoma , psammomatous extracranial meningioma [ table 2 ] . differential diagnosis the clinical management of smaller lesions is simple excision with surrounding marginal bone , whereas larger lesions warrant more aggressive surgical management . prognosis is good with a recurrence rate of about 30 - 58% . no malignant transformation has been documented . even though these lesions tend to locally invade , there were no cases of metastasis being reported . therefore , it is very important to correlate the clinical , radiographical , and histopathological findings for proper treatment . early detection and complete surgical excision of the lesion followed by long - term follow - up is necessary for proper clinical management .

juvenile ossifying fibroma ( jof ) is an uncommon , benign , bone - forming neoplasm with an aggressive local growth that is distinguished from other fibro - osseous lesions primarily by its age of onset , clinical presentation and aggressive behaviour . jof is considered as a variant of the ossifying fibroma ( of ) and the former includes psammomatoid jof ( psjof ) and trabecular jof ( trjof ) . both variants involve the craniofacial bones with the trabecular variant being more common in the jaws and the psammomatoid variant being more common in the craniofacial skeleton . psjof is an unique variant of jof that has a predilection for the sinonasal tract and the orbit particularly centered on the periorbital , frontal , and ethmoid bones . we report a rare case of massive psjof involving the maxillary sinus in a 20-year - old female .

endoscopic methods are among the most important and frequently employed means of treating pancreatic pseudocysts . when possible an endoscopic approach should generally be attempted in all patients with pseudocysts , and for pancreatic fluid collections endoscopic transmural drainage is the treatment of choice . if the cyst contains more than 2/3 solid material a self - expandable stent should be selected to ensure effective drainage . an additional advantage of the self - expandable metal stent is that the necrotic tissue contained within the cyst can be actively removed using an endoscope . this article discusses the unusual case of a stent that became dislodged and migrated into the omental bursa . following this migration some time was allowed to elapse , and the gastrotomy closed completely . since a simple endoscopic retrieval was no longer possible , a combined laparoscopic - endoscopic technique ( rendezvous technique ) was used instead , and the stent was successfully retrieved . in 2013 , a 49-year - old male smoker with a history of diabetes mellitus and arterial hypertension developed severe necrotizing pancreatitis . imaging revealed paralytic ileus along with an expanding cystic formation near the liver hilus and pancreatic head with compression of the duodenum . a pseudocyst near the left abdominal wall with enormous but stable proportions was also found to be compressing the left colon transversum ( 14 cm ) . due to the extreme thinness of the wall areas of necrosis in the corpus area developed , necessitating transgastral cystotomy and placement of a wall stent ( niti - s nagi stent - taewoong - medical co , seoul , south korea , 14 mm wide , 22 mm long ) . twenty - four hours later , however , the stent became dislodged and migrated into the omental bursa . an attempt at endoscopic removal the following day was unsuccessful , making the insertion of a second stent ( nagi fully covered taewoong,16 mm wide , 22 mm long ) necessary . endoscopic retrograde cholangiopancreatography ( ercp ) was then performed , along with papilotomy and insertion of an 8.5 fr . two weeks later , after significant clinical improvement and drainage of necrotic tissue , the second transgastric stent was removed with no complications . for technical reasons , retrieval of the migrated stent was not possible . at follow - up gastroscopy 4 weeks later the gastric wall had completely closed and hence that endoscopic localization of the stent was no longer possible . in november 2013 the pseudocysts had regressed significantly , and a computed tomography of the abdomen showed the migrated stent in the omental bursa with no direct contact to the gastric wall . in february 2014 , we decided to attempt a laparoscopic - endoscopic retrieval of the displaced wall stent . in the first step , intraoperative endoscopy demonstrated normal gastric mucosa so that a purely macroscopic localization of the stent was not possible [ figure 1 ] . after localization of the stent via image converter we made a targeted 3 cm incision into the posterior gastric antrum using an endo knife ( erbe , germany ) . due to the location of the stent deep within the omental bursa neither optical nor palpatory contact could be made , and consequently , endoscopic retrieval was not an option . in the next step , first , electrocoagulation was used to make three incisions in the anterior wall of the greater gastric curvature ( two in antrum , one in fundus ) . then , 3 ( applied medical kii , usa ) 5 mm trocars were inserted [ figure 1 ] . dissection within the omental bursa was performed using two dissectors , inserted through an incision that had been made endoscopically . after localization of the stent and some blunt dissection it was possible to carefully dislodge the stent into the gastric lumen [ figure 2 ] . after further inspection , the transgastral trocars were removed , and the gastric wall incisions were closed with lahodny stitches ( ethicon , usa ) . the postoperative clinical course was without complications , and the patient was discharged on the 6 postoperative day . at follow - up 4 weeks later , no complications were found . the gastric wall has closed , and the stent can not be localized ( a ) . three applied kii 5 mm trocars in the stomach ( b ) blunt dissection near the omental bursa after incision and retrieval of the stent ( a ) . currently , endoscopic ultrasound - guided drainage is a safe and effective means of treating pancreatic fluid collections . compared with surgical or percutaneous drainage it is less invasive and results in lower mortality , costs , and hospitalization times . drainage - related complications either may be related directly to the procedure or can occur in relation to the stents themselves . the stent should not , however , be removed before complete involution of the pseudocyst or before the passage of at least 2 months . on average , 15% of stents will become accidentally dislocated , but efforts to develop technically superior stents continue . tllez - vila et al . , for example , report on fully covered self - expandable metal stents with an innovative anti - migration system . describe a stent - in - stent combination which employs the mechanical advantages of both plastic stents and fully covered self - expandable metal stents . although these new solutions will help reduce the rate of migration , they will not eliminate it completely . in most cases of stent migration endoscopic retrieval can be performed without difficulty . in cases of extraluminal loss , however , a surgical approach is often necessary , and a minimally invasive exploration of the abdominal cavity is the approach of choice . retrieval from the retroperitoneal space may pose a more significant challenge due to the proximity of the large vessels . when endoscopic retrieval fails a combined laparoscopic - endoscopic approach offers an alternative to open surgery . the choice of technique is likely best predicated by individual patient presentation and local expertise . with this endoscopy - laparoscopy combination ( rendezvous technique )

endoscopic drainage is a widely used treatment for pancreatic pseudocysts . drainage - related complications may be related directly to the procedure or may occur later as stents migrate or erode into adjacent structures . migration of a self - expanding metal stent into peritoneal cavity and incorporation in the omental bursa is rare . when endoscopic retrieval fails a combined laparoscopic - endoscopic ( rendezvous technique ) approach offers an alternative to open surgery . we report a case of successful gastroscopic - transgastric laparoscopic removal of a stent that was dislocated into the omental bursa after a year observation period .

adenomyoepithelioma has been described as a rare benign neoplasm that occurs almost exclusively in the breast . in the breast it is defined as a neoplasm composed of two structures , namely , tubules limited by an inner epithelial layer of duct - like cells and an outer layer with mostly clear myoepithelial cells . apart from the breast , adenomyoepitheliomas have been described in the salivary glands and in the lung . in the skin , these neoplasms seem to be exceedingly rare , with only few reports of adenomyoepitheliomas published to date [ 47 ] . we present the case of a 53-year - old woman with a clinically benign nodular cutaneous lesion that revealed histopathologic and immunohistochemical features of adenomyoepithelioma . a 53-year - old woman presented to her dermatologist with a 3 cm asymptomatic nodule on the left forearm . the biopsy specimen consisted of a 2.5 1.9 1.5 cm tan ellipse of skin and contained a hard yellow nodule measuring 1.5 cm in greatest dimension . examination of the sections at scanning magnification revealed beneath an intact epidermis a zone of fibrosis within the upper part of the dermis ( figure 1 a , b ) . beneath the scar , there was a large , lobulated neoplasm , which for the most part was surrounded by a compressed fibrous pseudocapsule . each of the aggregates of neoplastic cells varied in sizes and shapes and some were large and nodular ( figure 1a c ) . at higher magnification , most of the cells that constituted the lesion displayed myoepithelial differentiation with polygonal and plasmacytoid features ( figure 2 ) . the cells presented sometimes as solid sheets , but also as cords and solitary units . in places glandular and ductal structures were evident ; in some areas apocrine - type secretion within glandular structures was found ( figure 2 a c ) . f ) . in some areas cells were present within a myxoid stroma ( figure 2 g foci of cells with pleomorphic nuclei and mitotic figures were identified ( figure 2 k the myoepithelial cellular component of the neoplasm stained for s100 protein and was negative for cytokeratin and carcinoembryonic antigen ( cea ) expression . we describe a cutaneous neoplasm composed of myoepithelial cells and a focal epithelial and glandular component . the s100 positivity indicates myoepithelial cells , while epithelial - glandular cells are negative for s100 protein expression . based on findings by conventional microscopy and immunohistochemistry , this neoplasm shows myoepithelial differentiation and focal epithelial lined tubules with features of apocrine secretion , findings that are consistent with the diagnosis of primary adenomyoepithelioma of the skin . while various adnexal neoplasms with a myoepithelial cellular component have been described in the skin , adenomyoepithelioma of the skin seems to be extremely rare . what are the criteria that distinguish adenomyoepithelioma from other benign cutaneous neoplasms with myoepithelial differentiation ? while myoepithelioma is defined as a benign neoplasm consisting exclusively of myoepithelial cells embedded in a myxoid stroma , adenomyoepithelioma shows in addition to myoepithelial cells a second component displaying various degrees of epithelial - ductal differentiation . chondroid syringoma , at the other end of the spectrum is a benign adnexal neoplasm that , in addition to apocrine epithelium , manifests various degrees of follicular and/or sebaceous differentiation . clinically , myoepitheliomas occur usually in children and young adults and are located on the extremities , while mixed apocrine tumors affect older individuals and are usually found on the face . the few cases of cutaneous adenomyoepitheliomas that have been described to date , including the present case , were described in older patients and were located on the extremities and on the trunk . pleomorphic adenoma in the breast is regarded as the analogue to mixed apocrine tumor in the skin , while adenomyoepithelioma of the breast is defined as a neoplasm with nodular aggregations of clear myoepithelial cells that surround epithelial lined tubules . occasionally , the myoepithelial component predominates and loses the close association with epithelial structures . in the skin , the predominance of myoepithelial cells in relation to ductal epithelial structures lacking features of follicular and/or sebaceous differentiation separates this neoplasm from mixed apocrine tumor . therefore , myoepithelioma , adenomyoepithelioma and mixed apocrine tumor lie in the spectrum of neoplasms with pure myoepithelial differentiation at one end and apocrine - sebaceous - follicular differentiation at the other . the most important question for the patient is the biologic potential of such a lesion . while the chronic course in our patient and the majority of the histopathologic features suggest a benign neoplasm , foci of cells having pleomorphic nuclei and mitotic figures were present . both findings could indicate the potential for locally aggressive behavior and/or metastasis , as has been rarely described in adenomyoepitheliomas of the breast . therefore , adenomyoepitheliomas of the skin should be completely excised , as has been recommended in the reported case .

analogous to adenomyoepitheliomas of the breast , cutaneous adenomyoepithelioma is composed of two components , one being myoepithelial , the other ductal epithelial , and it lies in the spectrum of neoplasms having a pure myoepithelioma at one end and a mixed apocrine tumor at the other . we present the case of a 53-year - old woman with a 3 cm nodular lesion on her left forearm that had been present for many years . histopathologic examination revealed a large lobulated neoplasm surrounded by a compressed fibrous pseudocapsule . most of the cells that constituted the lesion displayed myoepithelial differentiation arranged in solid sheets , cords , and solitary units . glandular and ductal structures with features of apocrine differentiation composed the second part of the neoplasm . the myoepithelial cellular component of the neoplasm stained for s100 protein and was negative for cytokeratin and carcinoembryonic antigen ( cea ) . based on findings by conventional microscopy and immunohistochemistry , the neoplasm was classified as primary adenomyoepithelioma of the skin .

beyond its role of cellular energy currency and phosphate donor , atp plays a potent signaling role through its extracellular release and activation of cell surface purinergic receptors . fast responses to atp are mediated through activation of p2x receptors , a family ( p2x1p2x7 ) of atp - activated ligand - gated ion channels and metabotropic effects through the activation of p2y receptors ( p2y12 , p2y4 , p2y6 , p2y1114 ) which couple to heterotrimeric g proteins . human leukocytes including monocytes , mast cells , neutrophils and central microglia express a diverse repertoire of p2y receptors though p2x1 , p2x4 and p2x7 are the dominant p2x subtypes present . activation of purinergic receptors in leukocytes is coupled to the production and secretion of cytokines and other pro - inflammatory molecules including prostaglandin e2 . purinergic receptors are associated with inflammation , with some receptors inhibited either directly ( p2y12 ) or indirectly through the activity of anti - thrombotic , in the case of platelet p2y12 , or anti - inflammatory agents , in the case of the action of statins on monocyte p2x4 . atp can act as a non - peptide damage - associated molecular pattern ( damp ) release from injured cells and tissues . in this fashion the release of cellular atp is unregulated and released due to cell lysis or puncture . cell surface purinergic receptors are activated by this atp damp signal which serves to initiate an inflammatory response and promote wound healing . however , atp can be released from cells physiologically and act as a critical signal for painful , inflammatory processes . do release other nucleotides including utp and udp - sugars but our focus here is atp release . mechanisms of atp release during physiological processes remain diverse and controversial . investigation into how cellular stress stimulates atp release in non - leukocytes suggests roles for connexin and pannexin hemichannels , maxi- and volume - regulated anion channels and efflux through the p2x7 receptor , though release routes and signal transduction mechanism underlying atp release in leukocytes remain poorly defined . in neutrophils atp is released in response to activation of fmlp receptor by bacterially derived n - formylmethionine . the precise release mechanism is unclear but occurs at the leading edge of migrating neutrophils . released atp and its subsequent metabolism to adenosine at the cell surface activate p2y2 and a3 receptors serving to direct cell orientation and promote migration in response to chemotactic signals . bacterially derived lipopolysaccharide can stimulate central microglia to secrete atp which in - turn activates neighboring astrocytes and modulates excitatory neurotransmission . this raises the possibility that atp can act in a feed forward loop possibly amplifying responses to itself or other external cues which couple to atp secretion . furthermore , such constitutive secretion leads to activation of cell surface gq - coupled p2y receptors which elevate intracellular calcium levels through release of calcium . this mode of constitutive secretion generates a constant pericellular atp cloud or halo which appears to be important in regulating intracellular calcium homeostasis following p2y receptor activation . in cells of hematopoietic lineage such as monocyte / macrophage , nk killer and mast cells , secretory lysosomes have evolved as bifunctional organelles which combine classical degradative properties with secretion . the mechanism of lysosomal atp transport remains undefined though a nucleotide transporter ( v - nut ) has been characterized for other atp containing vesicles . the signal transduction coupling external cues such as cytokines , chemokines and bacterially derived molecules to atp release is poorly defined . what are the release machines in leukocytes and do they differ from other cell types ? it is expected that therapeutic intervention in agonist stimulated atp release is a potentially novel route to pharmacological modulation of innate immune responses but also in chronic inflammatory disease where normal inflammatory responses are heighten and act deleteriously .

release and reception of extracellular atp by leukocytes plays a critical role in immune responses to infection , injury and cardiovascular disease . leukocytes of both the innate , adaptive immune and central nervous system express a repertoire of cell surface receptors for atp ( p2x and p2y receptors ) and its metabolites . atp acts as a damage - associated molecule pattern ( damp ) released by injured or dying cells . detection of released atp by neighboring leukocytes initiates inflammation and wound healing . however , recent evidence from our group and others suggests atp release by leukocytes themselves serves to regulate homeostatic mechanisms and coordinate responses to external pro - inflammatory cues . examples include the homeostatic control of intracellular calcium and regulation of migratory guidance during chemotactic response to external cues . though there has been some progress in elucidating atp release mechanisms of some mammalian cells types , release conduits and coupling signal transduction machinery remain larger elusive for leukocytes . our recent studies suggest a role for secretory lysosomes in releasing atp in monocytes . though poorly defined , targeting atp release mechanisms in leukocytes have great anti - inflammatory potential .

job syndrome is one of the hyper immunoglobulin e ( ige ) syndrome ( hies ) conditions characterized by signal transducer and activator of transcription 3 ( stat3 ) signaling mutations . patients with job syndrome have characteristic clinical features in association with mutations in stat3 signaling . these include marked elevation in serum ige and susceptibility to staphylococcal infections of the pulmonary tract and skin.1 one of the hallmark features of the disease includes the formation of abscesses . on the basis of the elevation of serum ige levels , it was hypothesized that there would be a lower incidence of clinical anaphylaxis among pediatric job syndrome patients . an electronic medical record database , including both inpatient and outpatient records , was utilized . the database from a large urban children s medical center was searched from january 1 , 2009 to december 31 , 2014 , using the international classification of diseases , ninth revision ( icd-9 ) codes assigned to each diagnosis , job syndrome , hies , and anaphylaxis . the icd-9 codes used corresponding to each diagnosis were as follows : asthma , 493.00493.99 ; job syndrome , 288.1 ; and anaphylactic shock , 995.61 ( peanut ) and 995.67 ( milk ) . the anonymous data generated for anaphylactic shock , asthma with anaphylactic shock , and job syndrome / hies were used to identify patients . the total number of patients was identified with 1 ) job syndrome / hies ; 2 ) asthma with anaphylactic shock ; 3 ) anaphylactic shock with and without peanut allergy , and with and without milk allergy ; and 4 ) job syndrome / hies with anaphylactic shock . the database search was conducted with the medical center health information support and did not involve personal health information analysis . the study was exempt from irb review because there was no use of personal health information , as discussed with the university of california irb office . the total number of patients with job syndrome / hies identified in the database was 18 . among the 22,890 patients evaluated with asthma of the 614 total anaphylactic patient cases identified , 286 ( 46.5% ) had anaphylaxis with no determined specific cause . there were 250 cases ( 40.7% ) of peanut allergen - induced anaphylaxis and 78 cases ( 12.7% ) were because of milk products . some patients may have been coded for one or more causes of shock . despite the elevation of ige associated with hies / job syndrome a one- and two - tailed t - test showed that there was no statistically significant difference when comparing asthma and anaphylaxis and hies with anaphylaxis . a one- and two - tailed t - test showed that there was no statistically significant difference when comparing asthma and anaphylaxis and hies with anaphylaxis . this study shows that there were no identified cases of anaphy - laxis among the job syndrome / hies population studied . this low incidence is in contrast to established higher rates of ana - phylaxis among atopic conditions associated with high levels of serum ige . the incidence of anaphylaxis among children and adolescents has been reported as between 0.05% and 2%.2 this study identified > 600 cases of anaphylaxis , during the same time period at the same medical center , among the non - hies pediatric population . the association between allergic disease and autosomal - dominant ( ad ) hies was studied in a murine model . these mutations result in a very elevated serum ige level , usually without any evidence of atopic disease . the authors found that in a murine model of mast cell degranulation , mast cell - induced anaphylaxis was blunted in the mutant mice . the animals treated with a c188 - 9 stat3 inhibitor demonstrated altered physiologic responses . the responses measured included body temperature and survival . those treated with the inhibitor had longer survival and less change in body temperature.3 in a cellular model described in the same citation , human umbilical vein cells from ad hies patients or control cells treated with a stat3 inhibitor demonstrated aberrant responses to mast cell mediators , such as histamine or platelet - activating factor . the cells from two newborns with hies were less permeable than wild - type cells when exposed to mast cell mediators.3 these findings suggest that stat3 mutations confer resistance to mast cell degranulation . a clinical study of food allergies and adult hies conducted a clinical evaluation of food allergies in a cohort of 71 patients with ad hies compared with healthy control subjects ( n=41 ) and atopic subjects ( n=65 ) . the results indicated that fewer patients with ad hies developed food allergies and anaphy - laxis than atopic patients with marked serum ige elevation , eczema , and no stat3 mutations . the study reported that fewer patients with ad hies had anaphylaxis to a food allergen than atopic controls ( 8.5% vs. 33.3% ) . the ad hies patients did demonstrate that they had specific ige to food allergens . when comparing peanut anaphylaxis between the groups , there were no cases of peanut - induced anaphylaxis found in the patients with hies.4 the authors concluded that stat3 mutations may play a role in protecting the individual from food allergies as a trigger for anaphylaxis . in studying the activation of a key regulatory cell , basophils from hies patients were less sensitive to ige cross - linking than basophils from non - allergic subjects.4 this study demonstrates that among the pediatric cases identified , there have been no cases of anaphylaxis among the hies patients . this is in contrast to a large number of children without hies who did have anaphylaxis due to peanut or milk allergens . this study finds support for a potential protective effect of stat3 mutations in preventing anaphylaxis and demonstrates this finding in a pediatric population . further studies are warranted to identify the molecular and cellular pathways involved in reducing vascular permeability during mast cell degranulation among children with hies .

patients with autosomal - dominant ( ad ) hyper immunoglobulin e ( ige ) syndrome ( hies ) or job syndrome develop frequent dermatologic and pulmonary infections . as patients have an extreme elevation of ige levels , this database analysis study sought to study the association between ad hies , job syndrome , and anaphylaxis . hies is a heterogeneous group of immune disorders characterized by extremely elevated levels of serum ige . although the molecular defects and clinical phenotypes found in association with job syndrome are well characterized , the association with severe allergic reactions and anaphylaxis is a subject of ongoing investigation .

the most often reported reasons have been dehydration of dentin , removal of tooth structure during root canal treatment , prolonged use of high concentrations of irrigation solutions , and excessive pressure during obturation . in the literature there are several studies in which the fracture resistance of endodontically treated teeth were evaluated or the techniques for reinforcing of these teeth were described . it is important to examine systems in an in vitro model prior to in vivo use in order to identify treatment or materials that might improve clinical performances . extracted human teeth are also widely used for in vitro studies in fracture resistance tests . however standardization among the extracted teeth should be performed in order not to affect the study 's results . standardization of the roots is one of the important steps in the study in which fracture resistance is evaluated . if roots were not distributed among the groups equally , these variables could have affected the results of the studies . in many studies , the mesiodistal ( md ) and buccolingual ( bl ) dimensions and the lengths of the roots researchers also attempt to choose the same type of teeth in order to standardize the specimens . in spite of these standardization attempts , it has been discussed that the standard deviations within the groups were rather high , rendering the results meaningless , and prompting studies using a larger number of specimens . the aim of this study was to determine how physical ( weight , volume , and density ) and morphological ( md and bl dimensions ) properties affect the fracture resistance of roots , and which criteria are important for standardization in fracture resistance evaluated studies . the null hypothesis was that different physical properties of roots would not affect the fracture resistance of endodontically treated roots . seventy - five human canine teeth extracted for periodontal reasons with completed apices and similar lengths were used in this study . mesiodistal ( md ) and buccolingual ( bl ) radiographs were taken of the specimens to evaluate the anatomical structures of the teeth . the teeth with internal or external resorption , those which had two or more root canals , and those with calcification were discarded . the teeth were examined under a stereomicroscope to discard specimens with cracks and craze lines . soft tissues and calculus were removed mechanically from the root surfaces using a periodontal scaler . specimens were decoronated with a diamond disc under a water coolant to obtain a standardized root length of 16 mm . was carried out , weight , volume [ figure 1 ] , and density [ figure 1 ] were calculated with precisa xb 220a precision balance ( precisa , gravimetrics ag , dietikon , switzerland ) which had a capacity of 220 g , and a readability of 0.0001 g. weight change in distilled water is equal to root volume , because density of distilled water is 1 g / cm . md and bl dimensions at 16 mm ( the coronal end of the root ) from the apex of the each root were recorded with root number . a size 10 k - file ( dentsply , maillefer , ballaigues , switzerland ) was inserted into the canal until it was visible at the apical foramen , and the working length was determined to be 1 mm short of this position . root canal shaping procedures were performed with protaper universal rotary files ( dentsply maillefer , ballaigues , switzerland ) , and apical region was prepared to size # 30 ( f3 ) in all specimens . the canal was irrigated with 2 ml freshly prepared 5% naocl solution with a 27-gauge needle after each file . a final rinse with 5 ml 17% edta for 1 min , followed by rinsing with 5 ml 5% naocl for 1 min was applied for smear layer removal . the specimens were dried with paper points and filled with gutta - percha and ah plus sealer ( dentsply detrey , kontanz , germany ) using cold lateral compaction . calculation of root volume and density using a precise balance proper wax stencils were molded and the roots were mounted into acyclic resin at an angle of 45 degrees to its long axis , leaving 6 mm of each root exposed [ figure 2 ] . a universal testing machine ( instron corp . vertical force was loaded with a speed of 1 mm / min until fracture occurred . the results indicated that volume ( r = 0.427 , p < 0.001 ) and weight ( r = 0.394 , p < 0.001 ) of the roots had a more significant effect than bl ( r = 0.197 , p = 0.085 ) , md ( r = 0.239 , p = 0.037 ) dimension which implying that volume or weight had greater importance regarding the prediction of fracture strength [ table 1 ] . physical and morphological properties of the samples along with pearson correlation coefficients and p values . when extracted human teeth are used for evaluating the fracture resistance of roots , there is a potential for large uncontrollable variations to affect strength . for that reason , all controllable factors should be standardized as much as possible . many researchers who have carried out this type of study - performed standardization , according to root length , md and bl dimensions . however , many of these studies admitted very high standard deviations as normal . thus , this study aimed to control factors such as weight , volume , and density , which can affect the fracture resistance of teeth in order to contribute to the standardization process . in the current study , the volumes or weights of root specimens have more statistically significant value than density , md , and bl dimension . in previous studies , however , these variations did not take into account when the standardization of groups was performed . future studies should be standardized to inhibit high standard deviations . during the study design , standardization is generally achieved based on the lengths , and md and bl dimensions of roots with referrals to other studies in the literature . however , this study has shown that the physical properties of teeth can affect the resistance of roots . thus , it is important to determine the alternative properties of roots which could be useful for the standardization process in future investigations . this is the first study in the literature to conclude that the volume or weight of root as the most important determining factors in root fracture. within the limitations of this study , it can be concluded that when forming groups to evaluate fracture resistance , after the root length is standardized , the roots should be equally distributed according to their volumes or weights , rather than their md and bl dimensions , as these dimensions can not closely simulate the entire strength of the root as much as the volume or weight .

objective : the aim of this study was to determine how physical and morphological properties affect the fracture resistance of roots , and which criteria are important for root specimen standardization in fracture resistance studies.materials and methods : seventy - five freshly extracted human canine teeth were selected . crowns were sectioned from the cement enamel junction and the root lengths were set at 16 mm . then they were prepared up to protaper f3 file . each root was numbered and data were obtained by measuring mesiodistal and buccolingual dimensions , volume , weight , and density . tests for fracture strength were performed using an instron testing machine ( instron corp . ma , usa ) . the force was applied axially , angled at 45 degrees with a constant speed of 1 mm / min . for each sample , the force at the time of fracture was recorded in newtons . results were evaluated statistically using linear regression analysis.results:volume and weight of the roots had more effect than mesiodistal or buccolingual dimensions on root fracture resistance.conclusions:in root fracture resistance studies , volume or weight of the roots must be standardized when distributing roots to groups .

renal clear cell carcinomas represent about 3% of all visceral cancers and account for 85% of renal cancers in adults . the tumours occur most often in older individuals , usually in the sixth and seventh decades of life , and are often diagnosed at incurable stages . in western countries the frequency of renal cancer remains relatively high , there being approximately 30,000 new cases and 12,000 deaths per year from the disease . the causes of kidney cancer are believed to be environmental ( such as cigarette smoking , asbestos , petroleum products , heavy metals , unopposed oestrogen therapy , hypertension and obesity ) , genetic or a mixture of both . to date there are 19 hereditary syndromes described in which renal cell cancer may occur ( table 1 ) . genetic syndromes characterised by an increased risk of renal cancer ( familial cancer database - facd , http://facd.uicc.org ) the identification of genetic predispositions to renal cell cancer remains a priority since knowledge about the underlying molecular genetic basis of the disease will allow for a better understanding of the mechanisms giving rise to the disease and , perhaps more importantly , allow for the identification of individuals who are at risk of disease development . there are two aspects of these criteria that can be problematic in the clinical setting with respect to the identification of familial renal cell cancer patients . the first is the difficulty in fulfilling criteria in countries where large families and extensive pedigrees are impossible to identify , for whatever reason , even though the incidence of hereditary renal cell cancer may be quite high . second , the criteria do not take into consideration the existence of family cancer syndromes where renal cell cancer may occur in association with an extra - gastric malignancy . from a clinical perspective , there is a necessity to be able to identify hereditary renal cell cancer families with a minimum set of criteria that will provide a high likelihood of ascertainment . the aim of this study was to determine whether a minimum set of criteria could be established to identify suspected hereditary renal cell cancer patients when there is restricted information about the familial occurrence of disease . a total of 146 clear cell renal carcinoma ( ccrc ) patients comprising 3 groups were enrolled in the study . group a ( familial renal cancer ) : comprising 46 patients affected by ccrc from 22 randomly selected families with at least two renal cancers among first or second degree relatives , independent of age at diagnosis of tumours . group a1 ( nuclear pedigree ) : comprising 25 patients affected by ccrc from group a. none of the parents of these patients have been diagnosed as affected with renal cell cancer . group b : a total of 100 individuals diagnosed with ccrc between the years 1993 and 1997 irrespective of family history were collected from the city of szczecin ( total population 400,000 ) . the following inclusion features ( if ) for the identification of suspected hereditary forms of clear cell renal cancer were used and compared against one another for their sensitivity and specificity : if 1 : at least one of the parents of the patient with ccrc was affected by lung cancer if 2 : at least one of the parents of the patient with ccrc was affected by gastric cancer if 3 : ccrc diagnosed at the age of 45 years or younger if 4 : ccrc diagnosed at the age of 50 years or younger if 5 : ccrc diagnosed at the age of 55 years or younger univariate statistical analysis ( chi - squared , odds ratio ( or ) , and sensitivity and specificity of selection were performed using the sas and logit programs . univariate statistical analysis ( chi - squared , odds ratio ( or ) , and sensitivity and specificity of selection were performed using the sas and logit programs . the comparison of the five ifs was undertaken to identify the most consistent criteria that can be employed in a clinical setting for the identification of suspected hereditary renal cell cancer , based on nuclear pedigree data . the first comparison was between group a ( associated with a genetic predisposition to disease ) compared to unselected cases from group b ( table 2 ) . the results indicate that all inclusion features are more frequent in group a ( or 1.62 - 4.88 ) . the second comparison was performed between group a1 and group b ( table 3 ) . the results indicate that there is a very strong correlation between hereditary ( familial ) predisposition to ccrc and occurrence of at least one of the following ifs : if1 , if2 or if5 - or 13.4 ; p < 0.00001 . the recognition of features that can be used for the identification of familial predispositions to ccrc in situations where extensive pedigree analysis is unknown or impossible to ascertain but the prevalence of the disease is relatively high in the population will aid in the identification of individuals at increased risk of developing ccrc . by using the criteria described herein and the consequent recognition of significant odds ratios for some of the inclusion features to identify ccrc families , we believe that the identification of additional genes associated with this malignancy will be expedited . of particular interest are the odds ratio values for the inclusion features if5 between groups a1 and b and if 1 between groups a1 and b , which were relatively high ( 6.21 and 6.09 , respectively ) . since these inclusion features are significant we have a relatively high degree of confidence that the reported observations are not biased and are an accurate reflection of the validity of our approach for the identification of hereditary ccrc families . indeed , these criteria have been tested in our outpatient clinics to successfully identify hereditary ccrc . therefore if we have families matching if1 or if2 or if5 , we are confident that a diagnosis of familial ccrc can be made . at present it seems reasonable to offer the option of ultrasonography examination to all individuals identified by the use of our inclusion features beginning at the age of 5 to 10 years before the youngest ccrc identified within the patient 's family . such surveillance should only be an option and not a recommendation because the efficiency of such management procedures has not been rigorously determined to reduce morbidity and/or mortality . with respect to surveillance the real value of this will have to be established by studies on large cohorts of individuals from families matching pedigree and clinical criteria of suspected hereditary ccrc with identified constitutional dna variants associated with genetic predispositions . so far , the list of genetic changes associated with ccrc is somewhat limited but should be extended in the near future as more knowledge is gained about the genetic factors associated with altered ccrc predisposition . in summary , we advocate the use of our criteria for suspected hereditary clear cell renal cancer identified in this report in order to : a. offer an ultrasound examination option , b. create repositories of nuclear clear cell renal cancer families for future studies on the efficiency of surveillance for individuals with genetic predispositions to renal cancer , c. perform further studies to aid in the identification of genetic factors associated with ccrc .

renal clear cell carcinomas represent about 3% of all visceral cancers and account for approximately 85% of renal cancers in adults . environmental and genetic factors are involved in the development of renal cancer . although to date there are 19 hereditary syndromes described in which renal cell cancer may occur , only four syndromes with an unequivocal genetic predisposition to renal cell carcinoma have been identified : vhl syndrome ( mutations in the vhl gene ) , hereditary clear cell carcinoma ( translocations t(3:8 ) , t(2:3 ) ) , hereditary papillary carcinoma ( mutations in the met protooncogene ) and tuberous sclerosis ( mutations in the tsc1 and tsc2 genes ) . little is known genetically about the other forms of familial renal cell cancer . since there is a growing awareness about the necessity of early intervention , clinical criteria have been developed that aid in the identification of hereditary forms of renal cancer . the aim of the current study was to identify minimal inclusion criteria so that nuclear pedigree families can be ascertained for risk assessment and/or kidney tumour screening . the results reveal that inclusion features described herein , such as ( a ) renal clear cell cancer diagnosed before 55 years of age , and ( b ) renal clear cell cancer and gastric cancer or lung cancer among first degree relatives , are useful in identifying suspected hereditary clear cell renal cancer patients .

intracranial hypotension syndrome ( ihs ) is characterized by a postural headache that is aggravated in the erect or sitting position and relieved in the supine position . it is often associated with one or more of the following symptoms : nausea , vomiting , dizziness , diplopia , photophobia , hearing impairment , neck stiffness , and blurred vision6 ) . acquired or secondary intracranial hypotension is less common but has been reported after spinal procedures such as lumbar discectomy and dural puncture for myelogram , spinal anesthesia . we report a case of a 54-year - old man who was successfully treated with epidural blood patches for intracranial hypotension due to csf leakage into the lumbosacral area after l4 - 5 discectomy . a 54-year - old male admitted to the er complaining of headache from 13 days ago . it did not occur when lying down but became intensely squeezing around the occipital area within 10 seconds after being seated or standing . past medical history was insignificant other than undergoing l4 - 5 laminectomy and discectomy at a local hospital 15 days ago . physical examination did not display any neurologic findings such as neck stiffness and laboratory results as well as brain mri findings were not remarkable . under the strong impression of intracranial hypotension , he admitted to neurology department and underwent conservative therapy including absolute bed rest ( abr ) and hydration . a mr myelogram was performed , showing a pseudomeningocele accompanied by large csf leakage in the lumbosacral area ( fig . because resolution of symptoms was unsatisfactory , an epidural blood patch was placed on the sixth admission day . after placing the patient in a prone position , a 22 gage touhy needle was inserted through the l5-s1 epidural space through a paraspinal method . after confirming the epidural space by the loss - of - resistance technique , a dye was injected to confirm the epidural space with the c - arm . after three days of abr following the procedure , the patient practiced changing positions by sitting . five days after the procedure , the patient did not complain of any symptoms ( vas 0 ) . 2 ) . we decided to perform one more procedure of epidural patch placement because of the residual csf leakage 6 days after the first intervention . a total of 10 ml autologous blood was injected , and the patient was discharged the next day . follow up for six months confirmed no recurrence of symptoms ( fig . intracranial hypotension syndrome can occur spontaneously . acquired or secondary intracranial hypotension is less common but has been reported after spinal procedures . reported that the incidence of incidental durotomy during spine surgery was 3.84% in patients who underwent spinal surgery at a single spine unit5 ) . in one study looking into medicolegal aspects of spine surgery , 146 malpractice cases were reviewed and incidental durotomy was second most frequent complication in such cases4 ) . the study has shown that potentially serious problems such as pseudomeningocele , csf fistula formation , meningitis and arachnoiditis with subsequent chronic pain are all related to dural tears and csf leakage after spinal surgery . good long - term clinical results were noted in all patients with durotomy repaired at identification and are comparable to long - term results of patients undergoing similar surgical procedures but without durotomy . however , cammisa et al.1 ) reported the incidence of clinically significant durotomy occurred during surgery but not indentified at the time was 0.28% , and patients had subsequent surgical repair of dural defects because of failure of conservative therapy . in one study which investigated incidental durotomy during spine surgery and its treatment , in addition to primary repair , drain , bed rest , hydration , antibiotics had been used for treatment by spine surgeon7 ) . however to our knowledge , there have been no previous studies which compared the effects of epidural blood patch for the treatment of incidental durotomy with other treatments . epidural blood patch has been employed over the last five decades in the treatment of postdural puncture headache ( pdph ) and has proven to be beneficial . the mechanism by which an epidural blood patch ( ebp ) relieves pdph is not known . the " plug " theory for symptom resolution proposes that the blood injected during ebp forms a gelatinous plug , sealing the dural hole and preventing further csf leak into the epidural space . in the absence of continued loss , regeneration of csf restores csf pressure and alleviates the headache3 ) . the " pressure patch " hypothesis emphasizes the impact of injected blood or other fluid ( crystalloid or colloid ) on the pressure dynamics of the cns . the injected fluid increases epidural pressure which , in turn , elevates subarachnoid csf pressure by compressing the dura8 ) . the optimal volume of blood that must be injected is controversial but recommended volumes have increased over time . crawford 's initial experience using 6 - 15 ml produced a 30% failure rate while later experience with 20 ml produced a 96% success rate , so 20 ml became the recommended volume and has since become commonly cited as a " target volume " to enhance patch efficacy2 ) . however , the effects of ebp in intracranial hypotension are mostly focused on post - dural puncture during csf tapping or spontaneous intracranial hypotension . even patients with rather large amount of csf leakage and patients with severe complications such as intracranial hematomas have been effectively treated with ebp , so we considered epb for postoperative incidental durotomy due to such evidence . the effects of ebp in postoperative intracranial hypotension can not be discussed after one successful clinical trial , but additional trials and studies can provide the basis of epb treatment in such cases . when incidental durotomy was recognized postoperatively , conservative therapy or surgical correction has been the therapeutic choice . however , because there has been no report on indication for ebp according to the degree of dural injury or the size of peudomeningocele , additional research is needed . in our case , the patient underwent l4 - 5 spine surgery at a local orthopedic , but dural injury was not recognized at that time . after admitting to our hospital , there was no significant resolution of symptoms after conservative therapy including the five days of bed rest and hydration . epidural blood patch can be used as an effective treatment modality for csf leakage after lumbar spine surgery .

intracranial hypotension syndrome typically occurs spontaneously or iatrogenically . it can be associated with headache , drowsy mentality and intracranial heamorrhage . iatrogenic intracranial hypotension can occur due to dural pucture , trauma and spine surgery . treatment may include conservative therapy and operation . we report a case of a 54-year - old man who was successfully treated with epidural blood patches for intracranial hypotension due to cerebrospinal fluid ( csf ) leakage into the lumbosacral area after spine surgery .

most pituitary adenomas are clinically inactive . in patients with long - standing compression of the optic chiasm , ganglion cells may undergo axonal degeneration . spectral domain optical coherence tomography ( sd - oct ) is able to identify retinal nerve fiber layer ( rnfl ) and ganglion cell loss in the retina . we present a case in which sd - oct was used to diagnose an asymptomatic pituitary macroadenoma . sd - oct identified atrophy of the ganglion cell and nerve layers , with preservation of outer layers bilaterally . as macroadenomas enlarge , they can induce uncrossed axon loss , resulting in nasal field defects and reduced visual acuity . in these cases , there is atrophy of the nasal and temporal portions of the optic disc , thus occupying a horizontal band across the disc . sd - oct is able to identify rnfl loss in eyes with band atrophy of the optic nerve , which correlates with visual field defects found in perimetry . sd - oct is a useful tool to assess the structural and functional damage of ganglion cells . in our case the sd - oct demonstrated a symmetrical loss of the rnfl and the ganglion cell layer in both eyes , indicating important optic nerve damage . pituitary adenoma is the most common cause of the chiasmal syndrome.1 the tumor is classified based on size as microadenoma , smaller than 10 mm , or as macroadenoma when it exceeds 10 mm in diameter.2 some tumors secrete one or more hormones in excess , so - called secretory pituitary adenomas , but most are clinically inactive.3 classically , the nonsecretory tumors present with vision loss,4 whereas patients with secretory tumors are usually referred to ophthalmologists for evaluation due to hormonal imbalances that affect bodily functions.5 in patients with long - standing compression of the optic chiasm , ganglion cells may undergo axonal degeneration4 . optical coherence tomography ( oct ) is able to identify retinal nerve fiber layer ( rnfl ) and ganglion cell loss in retina.6,7 we present a case in which the oct ( 3d oct-2000 spectral domain oct , topcon corporation , tokyo , japan ) was used to diagnose an asymptomatic pituitary macroadenoma . the authors adhere to the international standards developed in the 2 conference on research integrity in singapore ( 2010 ) , while performing this study . the patient agreed to have their case published , and all information presented is non - identifiable.8 a 48-year - old , caucasian female presented with progressive vision loss in both eyes for several months . her past ocular , medical , and family history was non - contributory with no fatigue , loss of libido , no mood change , and no weight change . on examination , best - corrected visual acuity was hand motion in the right eye and 2/3 in the left eye . spectral domain optical coherence tomography ( sd - oct ) demonstrated diffuse atrophy of the ganglion cell and nerve layers , with preservation of outer layers bilaterally ( figure 1 and figure 2 ) . magnetic resonance imaging of the brain was performed and a pituitary macroadenoma with suprasellar extension was observed ( figure 3 ) . pituitary adenomas are common benign tumors of the pituitary gland that account for 12% of all intracranial tumors.9 people can develop pituitary adenomas at any age . some adenomas secrete hormones in excess . of the secretory tumors , the most common are prolactinomas.5 they may be relatively small when detected . the most common symptoms include headaches , menstrual changes in females , sexual dysfunction in males , vision problems , and behavioral changes.5 diagnostic imaging may include a high - resolution , t1 weighted , gadolinium enhanced magnetic resonance imaging.10 other secretory tumors may secrete corticotropin , growth hormone , gonadotropins , or thyroid - stimulating hormone.11 adenomas remain confined to the pituitary gland , but when a pituitary adenoma is 10 mm it is called a macroadenoma . macroadenomas can compress the rest of the pituitary gland and surrounding structures , such as the crossing retinal ganglion cell axons in the optic chiasm , resulting in bitemporal visual field loss.4 as the tumor enlarges , it has been shown to induce uncrossed axons loss , resulting in nasal field defects and reduced visual acuity . in these cases , there is atrophy of the nasal and temporal portions of the optic disc with relative sparing of the superior and inferior parts where the majority of temporal fibers enter . the optic atrophy occupies a horizontal band across the disc , called bowtie or band atrophy.12,13 oct is a noninvasive technique that allows cross - sectional imaging of the retina and quantifies the thickness of the rnfl around the optic nerve head . a number of studies have demonstrated that oct is able to identify rnfl loss in eyes with band atrophy of the optic nerve.6,7 the degree of rnfl thickness reduction has been shown to correlate with that of visual field defects using goldmann perimetry4 and automated static perimetry.14,15 this fact gives oct prognostic value regarding the visual outcome , as demonstrated in different studies.16,17 oct is a useful tool to assess the structural and functional damage of ganglion cells , objectively and quantitatively.4 in our case the sd - oct demonstrates a symmetrical loss of the rnfl and the ganglion cell layer in both eyes , indicating important optic nerve damage . sd - oct can help in the diagnosis of adenomas in equivocal situations in which there is only subtle optic nerve pallor in the setting of nonspecific visual complaints or visual field defects , or when patients are referred to screen for subclinical compressive optic neuropathies .

introductionmost pituitary adenomas are clinically inactive . in patients with long - standing compression of the optic chiasm , ganglion cells may undergo axonal degeneration . spectral domain optical coherence tomography ( sd - oct ) is able to identify retinal nerve fiber layer ( rnfl ) and ganglion cell loss in the retina . we present a case in which sd - oct was used to diagnose an asymptomatic pituitary macroadenoma.clinical casea 48-year - old female presented with progressive vision loss in both eyes . sd - oct identified atrophy of the ganglion cell and nerve layers , with preservation of outer layers bilaterally . magnetic resonance imaging of the brain showed a pituitary macroadenoma . the pathological diagnosis was nonfunctioning adenoma.discussionas macroadenomas enlarge , they can induce uncrossed axon loss , resulting in nasal field defects and reduced visual acuity . in these cases , there is atrophy of the nasal and temporal portions of the optic disc , thus occupying a horizontal band across the disc . sd - oct is able to identify rnfl loss in eyes with band atrophy of the optic nerve , which correlates with visual field defects found in perimetry . sd - oct is a useful tool to assess the structural and functional damage of ganglion cells . in our case the sd - oct demonstrated a symmetrical loss of the rnfl and the ganglion cell layer in both eyes , indicating important optic nerve damage .

percutaneous nephrolithotomy ( pcnl ) is a widely accepted treatment for urinary calculi > 2 cm or resistant to other modalities . complications of this procedure are well reported ; however , to our knowledge no mention has occurred in the literature of a retained nephrostomy tube after pcnl . thus , we will present the management of a retained council tip catheter after pcnl . a 76-year - old gentleman underwent a pcnl for a 2.2x1.4-cm lower pole calculus via an uneventful upper pole puncture . incomplete stone fragmentation was obtained using the ultrasonic lithotripter ; therefore , a 24-french council tip nephrostomy tube was placed , the balloon was inflated with 3 ml of sterile water , and the patient was scheduled for a second look nephroscopy when a holmium laser was available . one week later at the second look nephroscopy , attempts to deflate the catheter balloon were unsuccessful . after transection of the balloon port valve , attempted passage of a 0.038 movable core bentson guidewire down the balloon port was unsuccessful . an antegrade nephrostogram outlined the catheter balloon within the renal pelvis ( figure 1 ) . with the c - arm in a vertical orientation , an 18-gauge chiba needle ( cook , bloomington in ) was advanced through a puncture site directly over the balloon under fluoroscopic guidance . the balloon was successfully punctured by the chiba needle with clear efflux through the needle . contrast was then instilled through the chiba needle to evaluate the integrity of the balloon and exclude the possibility of retained balloon fragments from a ruptured balloon ( figure 2b ) . flexible nephroscopy was performed to evaluate for balloon fragments , which were not identified , and to remove the lower pole stone . ( a ) chiba needle ( white arrow ) has punctured and drained the catheter balloon . ( b ) contrast injected via chiba needle has filled the catheter balloon ( dark arrow ) and confirmed its integrity . a retained urethral catheter due to balloon malfunction is a well - recognized urologic complication . many solutions to this problem have been attempted , including overinflation until rupture , puncture of the balloon via a wire through the balloon port , and puncture of the balloon under ultrasound guidance via a suprapubic or transvaginal route . given this infrequent but known complication of balloon - type cystostomy catheters , it is not surprising that a similar complication occurred in our patient . our familiarity with the use of the chiba needle and 3-dimensional fluoroscopy for percutaneous renal access facilitated percutaneous puncture of the catheter balloon . if equipment or experience limits the urologist 's ability to perform this technique , collaboration with an interventional radiologist using conventional c - arm or ultrasound for guidance should be considered . prevention of a retained catheter due to a balloon - port malfunction requires use of sterile water instead of saline and limiting the time of catheter placement . malecot tubes have been utilized for nephrostomy drainage , but these tubes are not without difficulties . tissue bridge formation over flanges of the tube has occurred , requiring endoscopic removal . tubeless pcnl would avoid these issues entirely ; however , the tubeless technique is contraindicated in the face of significant postoperative bleeding , urinary extravasation , ureteral obstruction , or anticipated need for a second - look nephroscopy . pigtail catheters following pcnl may represent a good alternative for drainage and access , while minimizing the risk of catheter malfunction or retention .

percutaneous nephrolithotomy is a widely accepted treatment for urinary calculi , but it is not without complications . we present the case of a 76-year - old male with a retained council tip catheter after percutaneous nephrolithotomy . fluoroscopic guidance was used to perform percutaneous puncture of the catheter balloon , and the catheter was removed without complication . advantages of various nephrostomy tube designs and additional measures to prevent this type of complication are discussed .

a seventy five year old libyan man was seen in the urology department of tripoli medical centre , tripoli , libya with six month history of left loin pain . the patient noted a mass in the left loin two days before he was assessed in the hospital . the urine analysis revealed pus cells , protein , and no sugar , but urine culture yielded no bacterial growth . liver function tests showed slightly elevated alkaline phosphatase at 160 iu / l ( 44 to 147 iu / l ) , and lactate dehydrogenase at 152 iu / l ( 105 - 333 full blood count revealed haemoglobin of 8.3g% , ( 1315g% ) , white cell count of 14.7103/mm3 ( 411103/ mm3 ) , and platelets of 3.4 105/ mm3 ( 1.54.5 105/ mm3 ) . the patient 's vomiting subsided with proton pump inhibitors , but the loin pain showed partial response to analgesic . abdominal ct - scan showed an irregular mass in the upper pole of left kidney . the patient was posted for partial nephrectomy with the provisional diagnosis of renal tuberculosis or renal infarction involving upper pole . pathologically , the kidney was noted to have greyish white soft tissue mass , firm in consistency measuring 6x4x2 cms , with adjacent dilated calyces . the renal resection margin and external surface appeared free of tumour ( figure 2 ) . microscopically , the tumour consisted of pleomorphic squamoid cells arranged in nests and sheets with few foci of epithelial pearl formation and keratinisation . areas of necrosis and frequent mitosis were noted . the malignant cells nore were seen to infiltrate the adjacent renal parenchyma as nests of cells and also focally renal capsule ( figures 3 & 4 ) . the dilated calysis showed areas of squamous metaplasia and severe dysplasia with focal invasion of basement membrane into renal parenchyma to form the tumour ( figure 5 ) . photomicrograph revealing calyseal subepithelial invasion of malignant cells ( left ) and invasion of renal parenchyma adjacent to glomerulus with epithelial pearl formation ( right ) . postoperative chest x - ray ( pre - operative chest x - ray was normal ) revealed massive pleural effusion in the left lower lobe ( figure 6 ) . however , no histological typing is available as tissue showed only necrotic material with scanty malignant cells . he was put on cisplatin and sunitinib on the suspicion of primary lung tumour and showed no response . the patient was suspected clinically to have renal tuberculosis and was later found to have renal squamous cell carcinoma with possible lung secondaries . the common renal malignancy in adults is of clear cell type , followed by papillary carcinoma and chromophobe cell carcinoma [ 1 , 2 ] . primary neoplasms of the renal collecting system are rare , accounting for less than 5% of urothelial tumours in urinary system [ 4 , 5 ] . the transitional cell type is the most frequent ( 85%95% ) , followed by squamous cell carcinoma ( 6%15% ) and adenocarcinoma ( 7% ) . usually , renal squamous carcinoma is highly aggressive and of a high grade at presentation . haematuria , the classical presenting complaint of renal cell carcinoma , is not common in this entity as in this case . the incidence of co - existing stone was reported in a wide range of 18% to 100% . the present case demonstrates the transition from calyseal urothelium to squamous metaplasia , dysplasia and invasive squamous malignancy . there is a well known association between chronic pyelonephritis , renal pelvic stones , phanecetin ingestion and radiotherapy with squamous cell carcinoma . the case was clinically suspected as renal tuberculosis because of sterile pyuria and past history of cough with fever . the renal malignancy was not considered due to loin pain and absence of haematuria as the renal cell carcinoma classically presents as painless haematuria . the tumour can be difficult to diagnose by imaging modalities as the usual features are the presence of calculi and hydronephrotic changes with ureteral obstruction . filling defects or obstructive lesions in the renal pelvis by intravenous / retrograde urography or detection of a solid mass by ultrasonography can be the signs of the tumour . central renal squamous cell carcinoma presents more with intraluminal components and is usually associated with lymph node metastasis whereas peripheral renal squamous cell carcinoma presents with prominent renal parenchymal thickening and might invade the perirenal fat tissue before lymph node or distant metastasis could be identified . the survival of patients with central renal squamous cell carcinoma was reported to be significantly shorter than those with peripheral renal squamous cell carcinoma . nativ and colleagues reported that patients with locally invasive renal squamous cell carcinomas had 1 and 2 yearsurvival rates of 33% and 22% , respectively . if metastasis develops , adjuvant chemotherapy or irradiation has little effect on the unfavourable prognosis . our patient underwent partial nephrectomy because of suspicion of renal tuberculosis and was later found have pulmonary metastasis . in conclusion , this patient presented with lion pain and a mass without haematuria and was found to have renal squamous cell carcinoma with lung metastasis . as renal calyseal tumours could present with atypical features , a proper preoperative workup may help in better management though outcome is short lived .

a seventy five year old gentleman with the clinical diagnosis of renal tuberculosis was found to have renal squamous cell carcinoma . the clinical presentation and management are being discussed .

a 55-year - old woman visited the emergency clinic of hanyang university seoul hospital with chest pain that had persisted for one month and had become aggravated two days earlier . ten years before this admission , the patient had undergone double valve replacement with aortic and mitral mechanical valves due to aortic regurgitation and mitral steno - insufficiency . echocardiography revealed normal motion and function of the mitral and aortic valve prostheses , and coronary angiography findings were also normal . however , coronary computed tomographic angiography revealed three large lobulated aneurysms with calcified walls bulging from the base of the left ventricle ( fig . the leak in the left ventricle was located in the sub - mitral valve prosthesis area . based on this finding , we diagnosed the patient with a pseudoaneurysm of the left ventricle , and surgical treatment was planned . arterial cannulation via the left femoral artery and venous cannulation via the left femoral vein and left pulmonary artery were performed . three communicating aneurysmal sacs were observed in a single plane , identical to that observed in coronary computed tomographic angiography . the neck of the first sac was located at the base of the left ventricle . under cardiopulmonary bypass and fibrillation , a defect measuring 15 mm in diameter was identified in the sub - mitral left ventricular wall with calcification but with no infection . closure of the defect was performed with a supple peri - guard pericardium patch with apex processing ( synovis , st . the walls of the aneurysmal sacs were closed , and the patient was weaned from cardiopulmonary bypass without difficulty . the vital signs of the patient remained stable , and she was extubated six hours after the operation . follow - up echocardiography was performed on postoperative day eight , and no abnormal findings , including cardiac wall motion , were noted . no leakage at the closed aneurysmal neck was observed , and there was no evidence of recurrence of the pseudoaneurysm ( fig . she has remained disease - free for six years postoperatively , with regular coumadin anticoagulation therapy due to the presence of the prosthetic aortic and mitral valves . a left ventricular ( lv ) pseudoaneurysm , or false aneurysm , is defined as a contained rupture or perforation of the myocardium . the rupture of the myocardium occurs rarely in clinical practice ; it is most often associated with myocardial infarction or cardiac surgery , such as mitral valve replacement ( mvr ) , and is usually fatal [ 24 ] . in 1980 , cobb et al . roberts and morrow suggested that inadvertent invasion into the lv free wall is possible during excision of the mitral valve if there is poor visualization of the operating field when using the tips of the scissors . other causes of complete or incomplete rupture of the lv free wall include an oversized prosthetic valve , excessive extirpation of calcium in the mitral annulus , myocardial erosion caused by the struts of the prosthetic valve , the untethering of the fibrous structures of the left ventricle during resection of mitral leaflets , an increase in lv contractility after aortic cross- clamping , enhanced lv wall stress with the support of inotropic agents , and other mechanical trauma between the free wall and the papillary muscles , such as rubber catheter wedging or metal pump suction during valve replacement . the wall of a false aneurysm comprises the fibrous obliteration of the pericardial sac , resulting from adhesion between the parietal and visceral layer of the pericardium . while a true aneurysm has nonrestrictive continuity with the lv cavity , a pseudoaneurysm has a defect in the myocardial continuity and a well - defined neck , representing a history of lv wall perforation . thus , such pseudoaneurysms are more likely to undergo rapid enlargement and rupture than true aneurysms . therefore , surgical correction , including the resection of the aneurysmal sac and patch repair or primary closure of the aneurysmal neck , is indicated and recommended for pseudoaneurysms . in the present case , we diagnosed a lv pseudoaneurysm using coronary computed tomographic angiography . myocardial infarction was excluded as a cause of the lv pseudoaneurysm because coronary angiography revealed normal findings and cardiac markers were in the normal range , although electrocardiography revealed myocardial ischemia in the inferior wall . the cause of lv pseudoaneurysm in our patient was a late - term complication of mvr . echocardiography revealed normal function of the mitral valve prosthesis ; therefore , mitral reoperation was not indicated . in such patients , if a repeat sternotomy for entering the operative field is considered , it may be difficult to approach the heart due to massive pericardial adhesions , and the possibility of injury or rupture of the pseudoaneurysm during dissection may be higher . a direct approach to the operative field via a left lateral thoracotomy can avoid these complications and preserve the previously implanted prosthetic valves . further , it has other advantages , such as making it easier to establish cardiopulmonary bypass and obviating the need to cross - clamp the aorta , in contrast to the sternotomy approach . in our operation , a fibrillator was used and the exposure of the operative field was excellent . in the operative field , we found three large lobulated pseudoaneurysms with calcified walls in a line , bulging from the base of the left ventricle . the configuration of the pseudoaneurysms suggested a previous episode of lv rupture , which had developed over time . in patients with lv pseudoaneurysms , there is a well - defined approach to surgical correction . the neck of the pseudoaneurysm is dissected , and the perforated site can be closed by patch repair or primarily . the mortality rate of this surgery is approximately 10% . , we have reported a case of lv pseudoaneurysm that occurred as a late complication after mvr . we performed a left lateral thoracotomy via the fifth intercostal space to approach the operative field directly and successfully repaired the sub - mitral lv pseudoaneurysm using a prosthetic patch . a few cases of lv pseudoaneurysms have been previously reported ; however , no case reports have previously been presented of korean patients with an lv pseudoaneurysm following mvr who were treated with an operative technique employing a left lateral thoracotomy .

we present a case of left ventricular pseudoaneurysm , which is a very rare and fatal complication of cardiac procedures such as mitral valve replacement . a 55-year - old woman presented to the department of thoracic and cardiovascular surgery at hanyang university seoul hospital with chest pain . ten years prior , the patient had undergone double valve replacement due to aortic regurgitation and mitral steno - insufficiency . surgical repair was successfully performed using a prosthetic pericardial patch via a left lateral thoracotomy .

sarcoidosis - related granulomatous reaction of the immune system could be attributed to environmental factors . however , data supporting this hypothesis remain controversial . clinical features of this disease include the following nonspecific symptoms : cough , dyspnea , erythema nodosum , febrile arthritis , uveitis , and parotitis sarcoidosis commonly targets hilar and mediastinal lymph nodes , which are found in more than 90% of the patients . the diagnosis is established on the basis of compatible clinical and radiological findings and supported by histological evidence in one or more organs of noncaseating epithelioid cell granulomas in the absence of organisms or particles . the correlation between sarcoidosis and malignancy remains unclear , despite that this topic has been increasingly investigated . in this article , we report a case of non - luminal her-2/neu - positive breast cancer in a patient without history of sarcoidosis and initially suspected to have metastatic disease . a 52-year - old woman was presented to our hospital . she noted a lump in her left breast during self - examination . palpation revealed a nodular lump of tight , elastic consistency in the upper inner quadrant of the left breast . mammography scan demonstrated a 19 mm 18 mm mass on the border of the inner quadrants on the left breast ( figure 1 ) . ultrasound examination revealed enlarged lymph nodes in the left axilla ( 10 mm in diameter ) , left supraclavicular lymph node ( 18 mm 10 mm ) , and multiple right enlarged supraclavicular lymph nodes , with a maximum size of 16 mm 7 mm . plain chest x - rays showed no abnormal findings ( figure 2 ) . the suspected diagnosis was breast cancer at t 1n 3 cm 0 . excisional biopsy of the left supraclavicular lymph node was performed to verify the diagnosis and differentiate the nature of the lesion . histological examination of the obtained material revealed no cancer but multiple epithelioid cell granulomas . based on these results , lumpectomy with urgent histology of resection margins urgent histodiagnosis revealed clear margins and demonstrated a lump in the breast , which was identified as infiltrative carcinoma . routine histological examination revealed infiltrative , moderately differentiated ( g 2 ) breast carcinoma with microcalcifications ( figure 3 ) . noncaseating epithelioid cell granulomas of sarcoidosis without tumor growth were found in 6 of 15 lymph nodes ( figure 4 ) . the molecular type of breast cancer was identified as non - luminal her-2/neu - positive through immunohistochemistry . therefore , the post - operative diagnosis of the patient was left breast cancer ( t 1n 0 m 0 ) , with sarcoidosis of left axillary and right supraclavicular lymph nodes . at the time of writing this article , the patient had been undergoing radiation therapy and directed to immunologist for sarcoidosis management and follow - up . mammography scan demonstrating a 19 mm 18 mm mass on the border of the inner quadrants of the left breast ( white arrow ) . ( a ) craniocaudal . ( b ) mediolateral oblique view . plain chest x - ray revealed no abnormal findings . infiltrative moderately differentiated ( g 2 ) breast carcinoma with microcalcifications ( h&e staining , 200 ) . lymph nodes with noncaseating epithelioid cell granulomas of sarcoidosis without tumor growth ( h&amp;e staining , 200 ) . the correlation between sarcoidosis and carcinogenesis remains unproven , although such relation has been described in numerous studies . brincker and wilbek first found this link in their study on 2544 sarcoidosis cases ; the incidence rates of lymphomas and lung cancer were 11 and 3 fold higher , respectively , in patients with sarcoidosis than those in the population . reported 21 cases of sarcoidosis developing after primary malignancies , including 10 cases after breast cancer . blank et al . defined breast cancer , cervical cancer , and b - cell lymphoma as the most common malignancies in patients with sarcoidosis . positron emission tomography ( fdg - pet / ct scan ) is one of the most advanced and precise diagnostic tools for such diseases . however , the application of this method in the assessment of regional and distant metastasis spread is limited . in cases of simultaneous sarcoidosis and malignancies , the functions of fdg - pet / identified that the maximal standardized uptake value ( suv ) in patients with maligna- ncies is significantly higher than in patients with benign diseases . nevertheless , in patients with granulomatous process , the maximal suv is similar to that in patients with malignant diseases . therefore , fdg - pet / ct scan may only be an additional diagnostic tool used to assess the extent of disease spread without differentiation between malignant and granulomatous disorders ; this tool could also create diagnos- tic difficulties and misunderstandings in patients with such simultaneous comorbidities 9 , 10 . this study reports a case of non - luminal her-2/neu - positive breast cancer in a patient presenting non - caseating epithelioid cell granulomas of sarcoidosis after treatment and initially suspected to have metastatic disease . a very recent retrospective study described the clinical biological and radiological characteristics of 12 patients presenting sarcoidosis associated with solid tumor ; sarcoidosis frequently affects patients with breast cancer ( 32.3% ) and thus must be considered in the differential diagnosis of the disease . in the current study , a review of literature is also presented and included 61 other patients showing similar association between cancer and sarcoidosis . this case report emphasizes the importance of differential diagnosis of lymph node involvement in cancer patients . diagnosis of sarcoidosis denied that the presence of metastasis could lead to changes in the post - operative management of the patient . although several epidemiological studies reported the association between cancer and sarcoidosis , further studies , including case reports , may represent an opportunity to obtain additional biological and clinical information to clarify the mechanisms underlying the association of cancer and sarcoidosis . furthermore , the present case report highlights the importance of histological determination in lymph nodes for distinguishing metastasis from sarcoidosis .

sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies . however , granulomatous and metastatic lymph node lesions are difficult to distinguish even when using precise and modern diagnostic methods , such as positron emission tomography . thus , histological verification is the only method that can be used to accurately describe the nature of this disease . in this article , we report a case of non - luminal her-2/neu - positive breast cancer in a patient without history of sarcoidosis and suspected to have metastatic disease .

this complication usually occurs during abdominal aortic surgery and is an unusual complication following live donor nephrectomy . live kidney donors are healthy individuals who willingly undergo major surgery to improve the well - being of another individual . therefore , it is of utmost importance to minimize the risks of this procedure and thus maximize donor safety . a 39-year - old mediterranean female with a bmi of 26 , underwent an elective , living - related , transperitoneal left - sided totally robot - assisted donor nephrectomy using the da vinci surgical system . donor nephrectomy was performed by a transplant surgeon and assisted by a second transplant surgeon , as described elsewhere [ 2 , 3 ] . briefly , the procedure was performed with the da vinci surgical system s using three arms with a fenestrated bipolar forceps ( endowrist bipolar cautery instrument ; intuitive surgical , sunnydale , usa ) in arm 1 , a monopolar cautery hook ( endowrist monopolar cautery instrument ; intuitive surgical , sunnydale , usa ) to dissect structures in arm 2 and the camera ( 3d vision system 12 mm 30 endoscope ; intuitive surgical , sunnydale , usa ) in arm 3 . after full exposure of the kidney and vascular structures , a pfannenstiel incision was made and an endo - bag ( endocatch ; us surgical , norwalk , usa ) was introduced into the abdomen . subsequently , the ureter was clipped and divided with scissors , and the renal artery and the vein were transected using an endoscopic stapler ( endogia ; us surgical , norwalk , usa ) . the kidney was then placed in the endobag and extracted through the pfannenstiel incision . before removing all trocars , the renal bed and vascular stumps were inspected using the da vinci , and intra - abdominal hemostasis was performed with the fenestrated bipolar forceps . the operation field was found to be dry after which all incisions were closed intracutaneously . postoperatively , the patient was placed on dipidolor patient - controlled analgesia combined with paracetamol . during the postoperative recovery she was discharged at postoperative day 3 with a normal appetite and no wound problems . on postoperative day 11 , she visited the outpatient clinic for a follow - up visit and the nausea was completely gone . however , she noted abdominal pain located on the right side near the iliac crest and reported two episodes of hematuria . laboratory results showed a decreased c - reactive protein ( crp ) level and normal leukocyte count . on postoperative day 19 , the patient noted bright red fluid leakage from the pfannenstiel incision without any further symptoms and contacted the nurse practioner . it was decided that she would visit the outpatient clinic if the leakage would worsen or other complaints would emerge . on postoperative day 29 , the patient visited the outpatient clinic for her scheduled 1-month postoperative follow - up visit . she still experienced pain in her abdomen which was mildly controlled with anti - analgesia . the pain progressed when she laid on her right or left side and , at night , her abdomen was more distended than usual . on physical examination , she had a distended abdomen , with tenderness on palpation on the right and the left side , and below the umbilicus , with a shifting dullness on percussion during movement . an abdominal ultrasonography showed intraperitoneal fluid in all four quadrants of the abdomen and a diagnostic paracentesis demonstrated yellow fluid with a lipid aspect , suspected of being chylous ascites and confirmed by biochemical analysis showing positive triglycerides of 57.98 mmol / l . she was admitted to the hospital for drainage and a medium - chain triglyceride - based diet was started . on day 4 the medium - chain triglyceride - based diet was continued for a total of 4 weeks after which a second abdominal ultrasonography was done , which showed no fluid in the abdomen . in this case report , we describe the first reported complication of chylous ascites after total robot - assisted donor nephrectomy . this complication is more common in major oncological surgical procedures , where lymph nodes are often resected . therefore , from an oncological point of view these patients often casually accept this complication . nevertheless , healthy live kidney donors are different from patients who undergo extensive dissection damaging lymphatic vessels necessitating postoperative treatment . in over three decades , > 1500 live donor nephrectomies have been performed at our centre and never before have we encountered a case of postoperative chylous ascites after a donor nephrectomy . a review of the literature resulted in a total of 56 reported cases of chylous ascites after donor nephrectomy ( supplement 1 ) . a huge contrast has been compared with the thousands of live donor nephrectomies that have been published over the years . the occurrence of chylous ascites is low and the expectation is that this is an underestimation . there is no clear treatment pathway ; however , a median chain triglyceride diet combined with a therapeutic drain or octreotide seems to be the first step . we could not identify any other reported cases of chylous ascites after robot - assisted donor nephrectomy [ 2 , 47 ] . during a robot - assisted donor nephrectomy , it is not necessary to dissect the lymfatic tissues around the aorta and the origo of the real artery . larger lymphatics around the renal vessels are either clipped or sealed by modern energy devices . in our case , a fenestrated bipolar forceps and a monopolar cautery hook were used for dissection and sealing . however , with monopolar energy , although control the lymphatics for a short time , necrosis can occur later on leading to leakage of large amount of chyle . the complication is often diagnosed after a few weeks , causing a delay in treatment . fortunately , the complication can be successfully treated with conservative measures within a few weeks as demonstrated in this case .

we present the first case report of chylous ascites following total robot - assisted donor nephrectomy . a 39-year - old female underwent a transperitoneal left - sided total robot - assisted donor nephrectomy . the procedure was uneventful and the patient was discharged without any symptoms . at postoperative day 29 , the patient presented with abdominal pain , nausea and a distended , painful abdomen with shifting dullness . she was diagnosed with chylous ascites by ultrasonography and puncture analysis , and treated with therapeutic drainage and dietary restriction . after 4 weeks , she was free of symptoms . the occurrence of this complication is rare after donor nephrectomy . fortunately , the complication can be successfully treated within a few weeks with minimal discomfort for the patient as demonstrated in this case . it is of utmost importance to minimize the risks and limit discomfort for live kidney donors who willingly undergo major surgery to improve the well - being of another individual .

it is one of the most commonly used medicines in the treatment of rheumatoid arthritis or psoriatic arthritis . we report the case of an adult woman with psoriatic arthritis who developed acute severe hepatitis following long - term treatment with mtx . the periodic biological monitoring of liver enzymes : alanine aminotransferase ( alat ) , aspartate aminotransferase ( asat ) , gamma - glutamyl transpeptidase ( gamma - gt ) performed every 6 months did not reveal any abnormalities . mtx was stopped in the year 2005 due to remission of the disease . in 2009 , it was reintroduced at a dose of 15 mg / week due to decompensation of the disease . monitoring of liver enzymes performed every 6 months showed an elevation < 2 times the normal levels on average of asat and alat since 2010 . assessing the risk - benefit ratio and given the severe decompensation of the disease , mtx was continued . in may 2013 , patient presented with jaundice following the development of asthenia and weight loss . laboratory investigation showed the following results : prothrombin time ( 71% ) , asat 580 u / l , alat 620 u / l , serum gamma - gt 146 iu / l , serum alkaline phosphatase 170 iu / l and total serum bilirubin 12 mg / dl . antimitochondrial antibodies ( titer > 1/40 ) and antinuclear antibodies ( titer of 1/640 ) were detected in serum . routine laboratory investigations for infection with hepatitis a , b , and c viruses and serology for cytomegalovirus and epstein - barr virus were negative . methotrexate was stopped , and therapy with oral methylprednisolone ( 80 mg / day ) was initiated . a significant improvement in the following parameters was observed a week later : prothrombin time ( 88% ) , asat 270 u / l , alat 340 u / l , s. gamma - gt 122 iu / l , and s. alkaline phosphatase 173 iu / l . after 4 months , all laboratory parameters had normalized , and serum was negative for antimitochondrial and antinuclear antibodies . patients taking mtx are more likely to discontinue therapy because of adverse drug effects rather than because of lack of efficacy . adverse effects of mtx are , usually , mild and self - limiting , but may include serious adverse drug reactions such as hematopoietic suppression , pulmonary , and hepatotoxicity . hepatic fibrosis is a usual adverse reaction reported with long - term use of mtx therapy in psoriatic patients . causality assessment of the adverse drug event ( ade ) was carried out using who - umc criteria , naranjo 's scale , and roussel uclaf causality assessment method ( rucam ) scale . in this case , patient improved on withdrawal of the drug , and there were no other confounding factors that could have caused this adverse effect . hence , the ade was probably caused by mtx ( who - umc criteria : probable ; naranjo 's score : 7 , probable ; and rucam scale : 5 ) . in addition , sufficient and continuous exposure to the drug , lack of previous evidence of autoimmune disease and complete resolution of the condition within 4 months of discontinuation of the suspected drug confirmed the diagnostic criteria of drug - induced autoimmunity . the first case of autoimmune hepatitis associated with mtx was described in 2011 in a 57-year - old man after 11 years of treatment with mtx . a complete normalization of laboratory parameters was obtained 5 months after stopping this drug . to the best of our knowledge , it , usually , occurs at higher doses and also positively correlates with the cumulative dose of drugs . other risk factors for the development of elevated liver enzymes and changes in liver biopsy include obesity , higher alcohol intake prior to commencement of mtx and persistent hepatitis b or c infection . in the present case , appearance of autoantibodies and transitory autoimmune disease associated with infliximab in a patient with psoriatic arthritis has also been documented and the preexistent serological signs of autoimmunity are believed to be a risk factor for the development of similar autoimmune reactions . this report confirms the need to monitor liver enzymes carefully in patients with psoriatic arthritis using long - term treatment with mtx . clinicians must collaborate with the pharmacovigilance center to detect and notify serious adverse effects of mtx . further research is needed to identify the exact mechanism and establish , if possible , primary means of prevention of this serious adverse effect .

methotrexate ( mtx ) is one of the most commonly used medicines in the treatment of psoriatic arthritis . the drug can produce steatosis and cirrhosis . autoimmune hepatitis is a rare and serious adverse effect . we describe the case of a 53-year - old woman who developed autoimmune hepatitis after a long - term use of mtx for psoriatic arthritis . hepatitis was completely resolved 4 months after stopping this drug . the pathophysiologic mechanisms of a drug - induced autoimmunity are unclear and complex . this report confirms the need to monitor liver enzymes carefully in patients using long - term treatment with mtx for psoriasis or rheumatoid arthritis .

blunt traumatic infrarenal aortic injuries are rare , with a few case reports in the literature . a more common occurrence is an intimal flap which may form after blunt injury to the aorta , and most of these will resolve with anticoagulation alone [ 1 , 2 ] . blunt aortic injury which initially presents as an intimal flap ( grade i ) may progress and evolve into an intramural hematoma ( grade ii ) or a pseudoaneurysm ( grade iii ) . aortic intimal flap progressing to dissection or pseudoaneurysm is an occurrence identified in 2% or less of the patients , and the majority of these occur in the thoracic aorta [ 1 , 2 ] . this article presents the case of a young woman found to have an intimal flap in the infrarenal abdominal aorta after a car accident , and the injury progressed to a pseudoaneurysm over 3 months . a 47-year - old woman with a history of hypertension and smoking was involved in a high - speed motor vehicle accident and presented to the emergency department with back pain . computed tomographic ( ct ) scans were obtained of her abdomen and pelvis . on a single image from the abdominal scan , what appeared to be either an intimal flap or a small amount of contrast extravasation outside of the infrarenal aorta could be seen . her care providers had differing opinions as to what was being seen on the image . since the abnormality was only seen on a single image , the exact etiology could not be delineated ( fig . the patient was observed in the hospital overnight , had minimal complaints the next day , and was discharged on aspirin . figure 1:initial ct scan shows enhancing vascular abnormality on the right side of the aorta , shown by the black arrow . initial ct scan shows enhancing vascular abnormality on the right side of the aorta , shown by the black arrow . a follow - up ct scan was obtained 3 months later . at the location of the previous aortic abnormality , there was a 3 1 cm pseudoaneurysm that had formed and could now be clearly seen ( figs 2 and 3 ) . figure 2:pseudoaneurysm now clearly seen 3 months later . figure 3:black arrow pointing to pseudoaneurysm with enhancing flow outside of the aortic lumen . operative repair was recommended , but the patient s aorta measured only 18 mm at the aortic bifurcation and was too small for even the smallest endovascular stent graft available at our institution . therefore , an open replacement of her infrarenal aorta was performed using a dacron 22 mm graft . in the operating room , after the aorta was clamped and opened , a 4 mm tear in the intima had formed a mature channel into the pseudoaneurysm . the patient recovered very well and was discharged from the hospital without complications . at her 2-year follow - up , she was in good health and had no further problems , but continues to smoke . blunt abdominal aortic trauma occurs in only 0.040.1% of all nonpenetrating traumas [ 3 , 4 ] . and the vast majority of these ( 92% ) are associated with multiple other injuries [ 3 , 5 ] . our patient had no other injuries , and there was no retroperitoneal hematoma or other signs of vascular injury in the area , as is usually seen on ct when the aorta is injured [ 35 ] . the lack of other radiographic traumatic findings added to the initial confusion as to whether there was a true injury to the aorta , or if the single image was showing artifact or an enlarged vertebral vessel . since the majority of intimal flaps heal with anticoagulation alone ( 55% ) or remain stable ( 40% ) , a conservative plan including daily aspirin and re - imaging in 3 months was chosen . when repeat imaging showed a 3 cm pseudoaneurysm , operative repair was necessary . endovascular repair was initially chosen for this case , but after measuring the patient s aorta , femoral vessels and the degree of taper at the bifurcation , there was not an appropriate endovascular prosthesis available in such a small size . only one study has compared conventional open repair with endovascular repair for blunt injuries of the abdominal aorta and found similar results in both groups . endovascular repair is generally favored since the injuries tend to be focal and discrete , making them quite amenable to a short graft . further , the patients usually have other significant injuries that would complicate a long , and potentially morbid , open operation on the aorta . although all blunt infrarenal aortic injuries are unusual , most of them consist of intimal flaps or dissections [ 3 , 58 ] . a post - traumatic pseudoaneurysm , as this patient had , is even more unusual after blunt trauma . one prior publication did document the delayed occurrence of a post - traumatic infrarenal aortic pseudoaneurysm that was diagnosed 3 years after injury . endovascular repair for pseudoaneurysms must include post - procedure angiography to document no evidence of a type i or type ii endoleak , which could allow filling of the aneurysm sac and later rupture . this case report highlights the need for delayed imaging for even minor intimal flaps or small aortic injuries after trauma , since progression does occur . while the vast majority of these injuries improve over time or remain stable , a small percentage will worsen and require intervention .

blunt traumatic infrarenal aortic injuries are unusual , and the formation of a delayed pseudoaneurysm of the aorta is even more rare . in this report , a young woman developed a small intimal flap of the infrarenal aorta after a motor vehicle accident which progressed into a 3 cm pseudoaneurysm after 3 months . operative repair was successful and the patient recovered . this case illustrates the importance of repeat imaging of small blunt aortic injuries since progression can occur .

strains carrying proa - tev - rpt1 were grown in 6 l of sd medium to od600 0.8 to 1.2 . cells were harvested , washed once with ice - cold water and drop - frozen in liquid nitrogen . frozen yeast samples were then ground using an mm301 grinding mill ( restch ) under liquid nitrogen following manufacturer s instructions , or using a mortar and a pestle as previously described24 . ground powder was hydrated in proteasome buffer ( 50 mm tris - hcl [ ph7.5 ] , 5 mm mgcl2 , 1 mm edta , and 10% glycerol ) supplemented with 2 mm atp , protease inhibitor tablets ( complete , roche ) , 2 mm pmsf , 1 mm benzamidine , 10 g / ml pepstatin a , and 1 g / ml antipain . cell extracts were cleared at 30,000 g for 30 min at 4c , and the supernatants were mixed with rabbit igg resin ( cappel , mp biomedicals ) for 90 min at 4c . resins were collected at 900 g for 2 min at 4c and washed with proteasome buffer containing 50 mm nacl three times , followed by a final wash with proteasome buffer alone . bp1 was then released from the resin by incubating with actev protease ( invitrogen ) at 2.5 unit / l culture in proteasome buffer containing 2 mm atp and protease inhibitors for 1 hr at 30c . eluates were concentrated using ultrafree-0.5 centrifugal filter device with 30 kda nmwl ( millipore ) . strains carrying proa - tev - rpt1 were grown in 6 l of sd medium to od600 0.8 to 1.2 . cells were harvested , washed once with ice - cold water and drop - frozen in liquid nitrogen . frozen yeast samples were then ground using an mm301 grinding mill ( restch ) under liquid nitrogen following manufacturer s instructions , or using a mortar and a pestle as previously described24 . ground powder was hydrated in proteasome buffer ( 50 mm tris - hcl [ ph7.5 ] , 5 mm mgcl2 , 1 mm edta , and 10% glycerol ) supplemented with 2 mm atp , protease inhibitor tablets ( complete , roche ) , 2 mm pmsf , 1 mm benzamidine , 10 g / ml pepstatin a , and 1 g / ml antipain . cell extracts were cleared at 30,000 g for 30 min at 4c , and the supernatants were mixed with rabbit igg resin ( cappel , mp biomedicals ) for 90 min at 4c . resins were collected at 900 g for 2 min at 4c and washed with proteasome buffer containing 50 mm nacl three times , followed by a final wash with proteasome buffer alone . bp1 was then released from the resin by incubating with actev protease ( invitrogen ) at 2.5 unit / l culture in proteasome buffer containing 2 mm atp and protease inhibitors for 1 hr at 30c . eluates were concentrated using ultrafree-0.5 centrifugal filter device with 30 kda nmwl ( millipore ) .

substrates of the proteasome are recognized and unfolded by the regulatory particle ( rp ) , then translocated into the core particle ( cp ) to be degraded1 . a hetero - hexameric atpase ring , containing subunits rpt1-rpt6 , is situated within the base subassembly of the rp1 . the atpase ring sits atop the cp , with the rpt c - termini inserted into pockets in the cp26 . we have identified a novel function of the rpt proteins in proteasome biogenesis through deleting the c - terminal residue from each rpt . our results indicate that assembly of the hexameric atpase ring is templated on the cp . we have also identified an apparent intermediate in base assembly , bp1 , which contains rpn1 , three rpts , and hsm3 , a chaperone for base assembly . the rpt proteins with the strongest assembly phenotypes , rpt4 and rpt6 , were absent from bp1 . we propose that rpt4 and rpt6 form a nucleating complex to initiate base assembly , and that this complex is subsequently joined by bp1 to complete the rpt ring . our studies show that assembly of the proteasome base is a rapid yet highly orchestrated process .

kyphosis is a progressive curvature of the thoracic spine , which results in rounding or bowing of the back . a curved spine accompanied by restricted neck motion and poor lung reserve present challenges for an ophthalmic surgeon , who is used to patients neck and head resting flat on the operating table and the eye in the horizontal position . intraoperative difficulties include poor view , inability to maneuver surgical instruments properly and constant threat of elevated vitreous pressure . several innovative solutions have been published , including the maximal reverse trendelenburg position and pillows under the head and neck , use of a donut and wedge - shaped head positioner , allowing patients to remain in the upright sitting position while their head is positioned horizontally in a modified waiting room chair , performing surgery while standing [ 5 , 6 ] , using a 4-section orthopedic operating table , or laying the patients on their sides . however a 49-year - old obese male with controlled secondary glaucoma needed cataract surgery in the right eye . the past ocular history was positive for recurrent uveitis and glaucoma shunt surgery in the right eye . cataract density was + 4 , and the best - corrected visual acuity was 20/200 . the anterior chamber was shallow and the glaucoma shunt tube was visible at the 11 o'clock position . the patient was planned for pupillary dilation using iris retraction hooks , lens capsule staining with trypan blue and phacoemulsification with intraocular implantation under local anesthesia . the anesthesiologist was reluctant to use general anesthesia because of the patient 's disability and poor health . in the operating room , the patient could only recline to about 40 from the vertical axis despite maximal reverse trendelenburg position and a number of pillows under his back , neck , head and legs ( fig . 2 ) . while trying to focus the microscope , it became evident that by grasping the eye at the inferior limbus with a fine forceps and pulling it upwards , it could be moved to a more desirable horizontal position . a single 7/0 vicryl ( polyglactin ) corneal retraction suture was placed at the limbus at the 6 o'clock position to achieve the desired view ( fig . the assistant surgeon was able to rotate the eye to the desired positions during the whole procedure by relaxing or pulling the suture . patients with kyphosis may present several unique challenges to an eye surgeon , the foremost being the inability to lay the head and neck in the ideal horizontal position . the resulting poor view makes focusing and manipulation of tissues and instruments difficult . tilting the head end of the operating table down and raising the patient 's legs may compromise venous return from the orbit , causing venous engorgement and elevated posterior vitreous pressure during surgery . in addition , the patient 's poor pulmonary reserve itself may further impede orbital / jugular venous return , resulting in increased posterior vitreous pressure . other features of kyphosis that may adversely affect intraocular surgery are tenderness and stiffness of the back and difficulty in breathing . several practical solutions have been described by very experienced surgeons but complications may still occur . a wedge - shaped pillow with a built - in donut for the positioning of the head has been used by some surgeons . livingston and mackool recommended using more than one of these positioners in severe cases . another approach suggested performing cataract surgery while standing , using loupes and making an inferotemporal scleral tunnel [ 5 , 6 ] . the surgeons were unable to remove the subincisional cortex because of a shallow anterior chamber secondary to elevated posterior vitreous pressure . prasad et al . recommended using a 4-section orthopedic operating table to enable an extreme reverse trendelenburg position for cataract and glaucoma surgery . however , because of a blurred vision , these authors were unable to perform curvilinear capsulorhexis and had to resort to can - opener capsulotomy . in short , there is no single solution for the different groups of difficult patients . we have found that a single retraction suture at the inferior limbus can rotate the eye to any desired position . this may allow both the patient and surgeon to be in a comfortable position without compromising good view . it may be tried practically in all patients with kyphosis or other conditions that prevent them from reclining such as torticollis , chronic obstructive pulmonary disease , chronic congestive heart failure or morbid obesity . the surgeon can still sit on the preferred temporal side of the patient and perform intraocular surgery of choice . with this maneuver , it may be unnecessary to resort to extreme and uncomfortable tilting of the patient and avoid complications such as those described above . the authors have no financial or proprietary interest in the materials described in the article .

we describe a 49-year - old man with advanced kyphosis and dense cataract , who could only recline to about 40 from the vertical axis despite a maximal reverse trendelenburg position and pillows under the head , neck , shoulders and knees . with a single corneal retraction suture at 6 o'clock , the eye could be rotated horizontally , which enabled the surgeon to perform a complex cataract surgery despite prior glaucoma shunt , posterior synechiae , a small pupil and the need to stain the capsule . as the eye can be brought into any desired position with a retraction suture , patients with kyphosis or other conditions that prevent them from assuming a supine position can still have safe intraocular procedures . this maneuver reduces the need to tilt patients to an uncomfortable position that may cause pain , increased breathing difficulty and elevated posterior vitreous pressure .

assisted / supported mechanical ventilation is designed to interact with patient muscle activity and ' share ' the work of breathing . if properly done , assisted / , the ventilatory 's flow and pressure delivery must synchronize with patient effort during all three phases of breath delivery : breath initiation , flow delivery , and breath termination ( cycling ) . dys - synchronous interactions can overload ventilatory muscles ( ' imposed ' loads ) , compromise alveolar ventilation , overdistend alveolar units , disrupt sleep patterns , and cause patient discomfort , prompting additional sedation . carlucci and colleagues have recently studied patient - ventilator interactions in 69 acutely ill patients receiving non - invasive ventilation ( niv ) for a variety of reasons . they grouped these patients according to obstructive or restrictive pathophysiologies and carefully measured respiratory system mechanics ( including esophageal pressure - a surrogate for pleural pressure ) . the patients were then initiated on niv according to a standard protocol and were assessed for triggering dys - synchronies defined as missed triggers , delayed triggers , or double triggers . importantly , they found that these dys - synchronies were common ( 58% of patients experienced them with missed triggers being the most frequent ) . trigger dys - synchronies were associated with niv intolerance and their prevalence was similar among the different patho - physiologic patterns . this study is important as it underscores that patient - ventilator trigger dys - synchrony is common and can be a major barrier to niv effectiveness . this study also underscores the fact that the causes of trigger dys - synchrony are complex and affect all types of patients requiring assisted / supported mechanical ventilation [ 3 - 5 ] . like invasive mechanical ventilation , niv trigger dys - synchrony can stem from insensitive / poorly responsive breath triggering mechanisms . however , other factors also involved likely include ventilatory muscle loading patterns and their effects on the ventilatory controller , ventilatory muscle function , lung volumes at end - expiration ( that is , intrinsic positive end - expiratory pressure ( peep ) ) , gas exchange effectiveness , and cortical inputs to the patient 's ventilatory control center such as pain [ 3 - 5 ] . in addition , the triggering process with niv can be further hampered by the mask system with its inherent leaks and claustrophobia exaggerating dyspnea and neural drive . importantly , this study did not address issues of flow synchrony or cycle synchrony , two other forms of patient ventilator interactions likely to impact niv tolerance but not well studied in the current literature . flow synchrony is defined as the ability of the ventilator to provide flow in accordance with patient demand as manifest by the ventilatory muscle contraction pattern . if flow is synchronous with that contraction pattern , the inspiratory muscle pressure time profile conceptually should resemble a near normal pattern . instead it means providing flow to ' re - shape ' the inspiratory muscle 's pressure - time or pressure - volume profile to a more physiologic shape . in general , the variable flow features of pressure targeted breaths tend to flow synchronize easier with patient effort than clinician set flow - volume targeted breaths . an assisted / supported mechanical breath termination shorter than the neural inspiratory time ( machine ti < neural ti ) can lead to muscle activity beyond the machine 's flow delivery phase , which can lead to high muscle loading , excessive tidal volumes and/or triggering of a second breath . in contrast , when mechanical breath cycling terminates after the inspiratory effort has ended ( machine ti > neural ti ) , dyspnea and expiratory muscle recruitment may occur in an effort to terminate the breath . importantly , it is worth noting that dys - synchronous interactions often result in anxiety and dyspnea , which can stimulate overall ventilatory drive . thus , improving synchrony in one area ( for example , triggering ) can help facilitate achieving synchrony in other areas ( for example , flow demand ) . in summary , patient - ventilator interactions are complex and are important to recognize and manage during both invasive and non - invasive mechanical ventilation . carlucci and colleagues have demonstrated how common the trigger dys - synchronies are during niv and that understanding them requires assessment not only of respiratory system mechanics , but also the complex interactions of neural drive , gas exchange , muscle function , and muscle loading . much research is needed in this area as dys - synchronies of all types can lead to overloaded muscles and dyspneic patients requiring sedation - two factors that clearly can lead to prolonged mechanical ventilation days and poor outcomes .

patient - ventilator trigger dys - synchronies are common with the use of assisted forms of mechanical ventilatory support , including non - invasive mechanical ventilatory support ( niv ) . future system designs need to address this in order to improve the effectiveness of niv .

postanginal septicaemia in the early 20 century ; originally associated with a mortality reaching 90% it involves a classic triad of oropahryngeal sepsis , internal jugular vein ( ijv ) thrombophlebitis and metastatic abscess formation ( 1 ) . clinical findings can therefore vary considerably from neck pain and swelling associated with thrombophlebitis to pleuritic chest pain , haemoptysis , dyspnoea or arthralgia as a result of septic thromboemboli . we report a case of predominantly external jugular vein ( ejv ) involvement in an adolescent male with negative blood culture results . a twelve year old boy presented to the children s assessment unit with a 24 hour history of unilateral face and neck swelling on a background of seven days progressive sore throat and dysphagia . minor systemic upset was also noted including headache , vomiting and non specific abdominal pain . a provisional diagnosis of tonsillitis had been made five days earlier by the local gp and a course of erythromycin and penicillinv commenced . examination revealed tender swellings over the left temporal area , angle of the mandible and upper third of the sternocleidomastoid extending into the submandibular region . examination of the oral cavity was complicated by trismus , however no obvious tonsillar enlargement , erythema or exudate was recorded . initial blood results showed an elevated c - reactive protein of 155mg / l and white cell count of 16.6(10 * 9l ) . initial imaging of the neck comprised an ultrasound scan ; this demonstrated normal right sided vasculature but abnormal left ejv and retromandibular veins . these veins appeared dilated with increased wall vascularity and showed no recordable signal on doppler imaging , an appearance in keeping with an inflammatory thrombophlebitis . to note an mri brain with venography ( mrv ) sequences provided greater anatomical delineation ( figure 1 ) . despite a coincidental finding of a smaller calibre left venous sinus ; thrombus was not seen to extend intracranially . lastly an echocardiogram was sought confirming a normal heart with no septal defects , vegetations or thrombus . mrv brain images demonstrate bilateral sagittal , straight , transverse and sigmoid sinuses with no evidence of thrombus extension . both right and left ijv as well as right ejv are clearly patent . the left ejv could not be demonstrated . treatment was started promptly on clinical suspicion of ls and consisted of intravenous ( iv ) metronidazole , benzyl penicillin and dexamethasone ; anticoagulation was not given . the patient remained in hospital on iv antibiotics for a total of 14 days , the noted swellings slowly decreased in size , blood results normalized and the trismus resolved . he remained afebrile throughout , developed no subsequent complications and was discharged home to complete seven weeks of oral antibiotics . despite ijv thrombophlebitis forming a main diagnostic criterion for ls , we report a case in which the primary vascular involvement is that of the ejv , with a corresponding normal ijv . studies have previously described ls with thrombosis of the carotid artery , intracranial venous sinus and abdominopelvic vessels ( 1 ) ; only three have ever described involvement of the ejv . this unusual finding helps to highlight the variable nature of this condition and the diagnostic confusion it may herald . to note there are reported cases of ls in which there was no demonstrable vascular involvement . numerous theories have tried to explain the spread of organisms from oropharynx to the surrounding vein ; haematogenous via the tonsillar vein , secondary to lymphangitis or via direct spread through deep neck spaces . ( 3 ) the incidence of ls within developed countries is now thought to lie around one per million per year , significantly reduced from the pre antibiotic era , however a resurgence over the last decade has recently been proposed . mortality has been recorded at 6.9% however morbidity and complications remain high as the early diagnosis can often be missed and treatment delayed . ( 3 ) although classically described as following an oropharyngeal infection ls has been reported developing from other primary infective sites including the middle ear , sinuses , mastoid and teeth ( 1 ) . the development of sepsis in ls is often delayed , presenting three to seven days from signs of the primary infection , which may have all but resolved ( 4 ) , making accurate history taking and a high index of clinical suspicion ever more important . ls is classically but not exclusively associated with an obligate oropharyngeal gram negative bacillus ; fusobacterium necrophorum . other causative pathogens described in the literature include streptococcus , staphylococci and bacteroides species ( 5 ) . it has however been reported that in almost 13% of cases no pathogen is isolated on culture ( 6 ) . ls can also be associated with thrombophillia ( thought to be endotoxin induced ) ( 5 ) . the role of anticoagulation however remains controversial with no conclusive literature evidence ; the decision should therefore be made on clinical grounds ; assessing severity of symptoms , poor treatment response and degree of thrombus extension . a recent history of a sore throat in conjunction with sepsis and or a suggestive and often declining clinical picture should always prompt early antibiotic therapy whilst a diagnosis of ls is sought ; hopefully preventing the development of late embolic complications . differential diagnoses to consider include infectious mononucleosis , parapharyngeal abscess , lymphoma , pneumonia ( including atypical forms ) and other embolic sources ( e.g. infective endocarditis ) .

oropharyngeal infections are routinely encountered within general practice and accident and emergency departments . most settle with simple analgesia and antibiotics ; occasionally such patients may develop lemierre s syndrome ( ls ) a rare and potentially fatal sequela that can be easily overlooked . we aim to highlight the main symptoms , pathology , investigations and management .

supravalvar mitral ring is a circumferential ridge or membrane arising from the left atrial wall overlying the mitral valve and frequently attached to the mitral valve.1 variable in thickness and extent , the ring ranges from a thin membrane to a thick discrete fibrous ridge.2 the membranous variety may be difficult to detect because the membrane often adheres to the anterior mitral valve leaflet while remaining just proximal to the posterior mitral leaflet . adhesion to the valve may impair the opening of the leaflets , and this impairment may be the main mechanism of mitral valve inflow obstruction in some patients . in other patients , the ring may be large enough to protrude into the mitral valve inflow and cause obstruction . the supramitral ring may initially be incomplete and eccentric , allowing for an unobstructed flow through the mitral valve . however , turbulence can cause a progressive increase in the supravalvar membrane or ridge thickness , worsening mitral inflow obstruction.3 the same mechanism is responsible for the acquired variety of supravalvar mitral stenosis , which occurs after mitral annuloplasty for the repair of mitral regurgitation . we herein present a case of a supraventricular ring in a 17-year - old male , who presented with syncope . about 15 years ago , he had undergone a cardiac operation for ventricular septal defect ( vsd ) closure and mitral valve repair . in chest x - ray both lungs were clear , and the electrocardiogram result showed normal sinus rhythm , had undergone right bundle branch block , and right ventricular hypertrophy . transthoracic echocardiography , transesophageal echocardiography , and finally cardiac catheterization revealed ejection fraction of 5055% , pulmonary hypertension with pulmonary artery pressure ( pap ) of 80/40 mm hg , pulmonary capillary wedge pressure of 23 mm hg , gradient in the mitral valve plane of 40 mm hg , no residual vsd , no patent ductus arteriosus ( pda ) , bicuspid aortic valve , no aortic insufficiency , no aortic stenosis , no sub - aortic web , normal coronary arteries , mild mitral regurgitation , moderate to severe mitral stenosis , supra mitral valve ring , normal left ventricle size , moderate right ventricle enlargement , and moderate systolic dysfunction . there was a free floating high redundant obstructive membrane in the left atrium that was attached to the mid portion of the anterior mitral valve leaflet and also in the medial and base of the posterior mitral valve leaflet and large papillary muscles in the left ventricle ( figure 1 ) . it also extended just adjacent to the lower limb of the left atrium appendage mouth with a small orifice ( 6 mm ) in the lateral side and also multiple small fenestrations ( by 2 dimensional echocardiography ) , resulting in a severely restricted diastolic flow ( mean peak gradient = 9.3 mm hg ) . this web was not found in mri cine turbo - flash images acquired on a 1.5 t scanner ( magnetom vision - siemens ) in the four - chamber view ( 3a ) and the transverse plane , short - axis view ( figure 2 ) . the patient s ascending aorta , descending aorta , and aortic arch were tortuous with moderate narrowing of the descending aorta distal to the subclavian artery with a systolic gradient of about 30 mm hg , which was suggestive of insignificant coarctation of the aorta . after heparinization , extracorporeal circulation was established between the venae cava and the ascending aorta . following a median sternotomy , there was a long supravalvar muscular band , which was resected ( figure 3 ) . supravalvar mitral ring rarely occurs as an isolated defect ; other congenital heart defects coexist in 90% of patients . the mitral valve itself is often abnormal and stenotic at the valvar or subvalvar level ; fusion of leaflets , a small valve orifice , and abnormal papillary muscles are common abnormalities . shone s complex is a combination of four congenital heart defects : supravalvar mitral ring , parachute mitral valve , subvalvar aortic stenosis , and aortic coarctation . other common associated lesions in patients with supravalvar mitral ring are ventricular septal defect ( vsd ) , pda , atrioventricular ( av ) canal defect , and tetralogy of fallout.4 uncommonly associated defects include atrial septal defect , cor triatriatum , left superior vena cava , unroofed coronary sinus , partial anomalous pulmonary venous drainage , pulmonary venous obstruction , double - orifice mitral valve , and wolff - parkinson - white syndrome.5 lesions such as transposition of the great arteries , atrioventricular discordance , and double - outlet right ventricle are occasionally complicated by a supravalvar left atrioventricular valvular ring . obstruction to the mitral inflow results from the reduced area of the mitral valve orifice . when clinically significant , a diastolic pressure difference occurs between the left atrium and the left ventricle . left atrial and pulmonary venous pressures increase , leading to exudation of fluid into the pulmonary interstitial , which increases lung stiffness . breathlessness and tachypnea are secondary to the interstitial edema and diminished pulmonary compliance.6 in severe cases , frank pulmonary edema can occur.7 an associated atrial septal defect may decompress the left atrium , reducing or masking the severity of the mitral valve obstruction . associated lesions such as vsd or pda , which increase the left ventricle output , exacerbate the manifestations of mitral inflow obstruction.8 conversely , a supravalvar mitral ring may be difficult to detect in conditions with a diminished pulmonary blood flow such as tetralogy of fallot.9 persistently elevated pulmonary venous hypertension leads to pulmonary arterial hypertension , rise in pulmonary vascular resistance , and eventual failure of the right ventricle and tricuspid regurgitation . it also deserves of note that predilection for race , age , and sex has not been found yet . treatment is the surgical resection of the web , but balloon dilatation is reported to have been successfully performed under fluoroscopic and transesophageal echocardiographic guidance by some authors.10 preoperative identification of a supravalvular mitral ring is the target for obtaining good surgical results . for the identification of this congenital heart disease ,

supravalvar mitral ring is a rare congenital heart defect of surgical importance . the condition is characterized by an abnormal ridge of the connective tissue on the atrial side of the mitral valve . it often substantially obstructs the mitral valve inflow . we herein introduce a case of a supravalvar mitral ring in a 17-year - old male , who was admitted to our hospital with cardiac syncope . he had undergone a cardiac operation for ventricular septal defect ( vsd ) closure and mitral valve repair 15 years before . transthoracic echocardiography , transesophageal echocardiography , and finally cardiac catheterization revealed a neglected supravalvular mitral ring . the ring was resected in a second operation , and the patient was discharged from the hospital symptom free .

with an incidence rate of 0.92% , maternal puerperal lower extremity nerve injuries are rare . lateral femoral cutaneous neuropathy ( meralgia paraesthetica ) is the most common , followed by femoral neuropathy . nerve injuries are more likely to occur in nulliparae , in cases of prolonged stages of labour and assisted vaginal deliveries . the presented case involved a 32-year - old woman with bilateral heel numbness due to bilateral neuropathy of the medial calcaneal nerve due to external compression during vaginal delivery . a 32-year - old woman presented to our neurology outpatient clinic with tingling and numbness in both heels . she continuously complained about these sensations ever since she had given vaginal birth to her first child 3 months earlier in a hospital . spinal analgesia ( lumbar level , bupivacaine / sufentanyl bolus , followed by a continuous administration of 8 ml / h ) was used during labour . the first stage of labour was prolonged , and because of fetal compromise during the expulsion phase , a vacuum delivery system ( palmpump kiwi ) , together with episiotomy , was used for fetal extraction . after delivery , the epidural analgesia was continued during the closure of the episiotomy with sutures . the patient did not use any other medication and had no history of neurological disorders or risk factors for neuropathy ( diabetes , hypothyroidism or hereditary liability to nerve pressure palsy ) . on examination , she had hypoaesthesia in the area of both heels . the strength of both the leg and foot muscles was normal , and so were the deep tendon reflexes . , a near - nerve needle conduction study of the medial calcaneal nerve was not performed . a diagnosis of bilateral neuropathy of the medial calcaneal nerve , most likely due to longstanding pressure on both heels with epidural analgesia as a predisposing factor , was made . three months after presentation ( 6 months after delivery ) , her numbness had diminished , but had not completely disappeared . bilateral neuropathy of the medial calcaneal nerve due to external compression during delivery using epidural analgesia is very rare . only 1 patient with numb heels has been reported in a large study , describing 2,615 women who received epidural anaesthesia during delivery . however , no details about delivery or analgesia in that patient were mentioned . in epidural analgesia during delivery , a motor block is unwanted , and thus voluntary muscle movements are possible , which makes longstanding external compression of nerves uncommon . in our patient , prolonged labour with spinal analgesia and a continuation of analgesia during episiotomy probably masked signs of external compression on both heels . the medial calcaneal nerve arises from the tibial nerve at the medial side of the ankle , perforates the laciniate ligament , travels downwards , passing the bony projection below on the inner side of the ankle , and supplies the skin over the medial aspect of the heel . patients with risk factors , such as diabetes , are at a greater risk of developing neuropathy during epidural anaesthesia . however , also without any risk factors , the clinician should be aware of the absence of the normal reaction to longstanding pressure on a nerve during epidural analgesia . preventative measures like soft gel pads or intermittent posture changes could possibly prevent neuropathy of the medial calcaneal nerve in long - lasting epidural analgesia . bilateral medial calcaneal nerve neuropathy as a result of external compression is a rare complication of epidural obstetric analgesia .

we describe the case of a 32-year - old woman who developed bilateral heel numbness after obstetric epidural analgesia . we diagnosed her with bilateral neuropathy of the medial calcaneal nerve , most likely due to longstanding pressure on both heels . risk factors for the development of this neuropathy were prolonged labour with spinal analgesia and a continuation of analgesia during episiotomy . padded footrests decrease pressure and can possibly prevent this neuropathy .

symptomatic supravesical obstructive uropathy in a patient with dual functioning kidneys is classically characterized by bilateral hydroureters / hydronephrosis and an empty urinary bladder . this obstruction may be secondary to metastatic abdomino - pelvic and retroperitoneal malignancies , ureteric calculi and retroperitoneal fibrosis [ 25 ] . the evidence for obstruction may be partially or totally absent on ultrasound or computerized tomography , in one or both kidneys [ 68 ] . our recent experience with a uraemic 56-year - old caucasian showing only unilateral moderate right - sided hydronephrosis is presented . a 56-year - old caucasian male patient with a past medical history that included hypertension and coronary artery disease and a recent serum creatinine of 88.4 mol / l , was diagnosed 10 days earlier with urinary tract infection ( uti ) . two days before presentation to us , he developed worsening anorexia , nausea with vomiting and oliguria . his father had prostate cancer and died at age 75 years from colon cancer ; a sister had breast cancer and a maternal uncle had throat cancer . physical examination revealed a blood pressure of 165/89 mmhg , pulse rate of 56/min and respiratory rate of 16/min . he was not dehydrated nor orthostatic . except for trace ankle oedema and bilateral costo - vertebral angle punch tenderness , mol / l , co2 14 mmol / l and potassium 5.5 mmol / l . urinalysis showed 510 wbc per high - power field ( phf ) and 25 rbc phf . alanine aminotransferase ( alt ) , aspartate aminotransferase ( ast ) and total creatine kinase ( ck ) levels were normal . a post - void urinary bladder scan had demonstrated only 60 ml of residual urine . a renal ultrasound showed unilateral moderate right - sided hydronephrosis with suspected mass effect on the inferior urinary bladder . the left kidney appeared normal , measuring 16.2 cm 8.1 cm , with preserved cortical thickness . the next day , with increasing vomiting , serum creatinine 894.6 mol / l , phosphorus 2.6 mmol / l , potassium 6.2 mmol / l and oliguria ( table 1 ) , he started daily haemodialysis . a non - contrast computerized tomography examination , on day 2 , again showed unilateral right - sided hydronephrosis , bilateral nephric stranding and urinary bladder wall thickening suspicious for transitional cell cancer ( figure 1 ) . cystoscopy , on day 3 , revealed a sessile urinary bladder tumour , which was resected . the right ureter was successfully cannulated and a right ureteric stent was placed with prompt urine drainage . the next day , with a strong push from nephrology , the patient consented to a percutaneous left nephrostomy procedure despite the normal appearing left kidney . a percutaneous left nephrostogram revealed a previously unrecognized mild hydronephrosis / hydroureter with obstruction at the ureterovesical junction . the pathology report revealed high - grade urothelial carcinoma , grade 3 of 3 , with invasion of the muscularis propria . a patient with dual functioning kidneys presenting with uraemic symptoms and suspected to have obstructive uropathy must be presumed to necessarily have bilateral renal obstruction [ 610 ] . this is without prejudice to the findings on conventional renal imaging with ultrasound or computerized tomography [ 3,610 ] . there are false negative tests with these imaging modalities , the so - called syndrome of non - dilated obstructive uropathy or non - dilated obstructive nephropathy [ 610 ] . clinical conditions associated with the absence of hydronephrosis on ultrasound and computerized tomography despite obstructed kidney(s ) include acute early obstruction , the presence of retroperitoneal fibrosis or infiltrative metastatic abdomino - pelvic cancers , dehydration or septic shock and severe oliguria [ 210 ] . our patient was not dehydrated and was not hypotensive but was severely oliguric ( table 1 ) . we note that we did not rule out the presence of retroperitoneal fibrosis in our patient . the classic picture of bilateral hydronephrosis with hydroureters and an empty urinary bladder , in symptomatic uraemia following supravesical obstruction , in patients with dual functioning kidneys , is well acknowledged . however , the presentation of new - onset symptomatic uraemia concurrent with only unilateral hydronephrosis / hydroureter on conventional imaging ( ultrasound , computerized tomography ) should raise the plausibility of non - apparent obstruction of the contra lateral kidney . in such instances , the more sensitive albeit invasive percutaneous nephrostogram of the apparently normal appearing kidney is therapeutic and will lead to greater renal salvage [ 68 ] . we would like to remind practicing providers that symptomatic uraemia presenting in the setting of suspected obstructive uropathy must be assumed to imply bilateral renal obstruction , regardless of the results / interpretations of any form of conventional renal imaging . therefore , necessarily , every attempt to decompress both kidneys must be the rule . this approach would result in early and improved renal salvage . left undiagnosed and therefore untreated , this potentially reversible cause of renal failure can lead to irreversible renal failure if bilateral , , or to significant residual loss of renal function if missed on one side only . we note that we were not able to carry out any split renal functional testing after recovery as the patient 's primary attention at this point was to find out treatment options for his cancer .

bilateral hydronephrosis is classic for supravesical obstructive uropathy causing uraemia with dual functioning kidneys . recently , a patient presented with uraemia and metastatic urinary bladder carcinoma but only unilateral right - sided hydronephrosis . a right ureteral stent was placed retrograde and no further intervention was planned since the left kidney appeared normal , and since the left ureteric orifice was not visualized . we insisted on a left percutaneous nephrostomy which was successful with prompt urine return . a left nephrostogram revealed unrecognized hydroureter / hydronephrosis . following haemodialysis , kidney function normalized at 3 weeks . for symptomatic uraemia from obstruction , an antegrade and/or a retrograde decompression must be attempted bilaterally to improve renal salvage .

reversible focal lesions in the splenium of the corpus callosum ( scc ) have been found in patients with various conditions including seizures , antiepileptic drug toxicity or withdrawal , viral encephalitis , hypoglycemia , wernicke 's encephalopathy , marchiafava - bignami disease , sympathomimetic - induced kaleidoscopic visual illusion syndrome , hemolytic uremic syndrome , altitude brain injury , and acute axonal injury . analysis of the current literature revealed that the most frequent cause of reversible focal lesions of the scc is influenza virus - associated encephalitis / encephalopathy ( iaee ) . the abnormality has been reported in association with various viruses ( e.g. , epstein - barr virus , rotavirus , influenza virus ) ( 1 - 3 ) . the brain lesions described in patients with iaee include restricted diffusion involving the cerebral cortex and subcortical white matter in various locations , symmetric lesions in the brainstem , basal ganglia , thalamus , and cerebellar white matter with or without brain edema , and mild brain atrophy . transient restricted diffusion of the scc in patients with iaee has also been described ( 4 ) . here , we report an adult who presented with non - specific dizziness and lesions of the scc on magnetic resonance imaging ( mri ) who was diagnosed with hemorrhagic fever with renal failure syndrome ( hfrs ) with serologically proven hantaan virus infection . a previously healthy 53-yr - old man presented with a 6 - 7-day history of fever up to 40 and dizziness . the initial diffusion - weighted images revealed high signal intensity in the corpus callosum with a low signal on the apparent diffusion coefficient ( adc ) map ( fig . 1 ) . the next day , he developed thrombocytopenia ( 4010/l ) and was transferred to our emergency room for further evaluation . he stated that he had been tying rice straw into sheaves several days earlier , but not using any drugs , like metronidazole , antiepileptic drug or alcohol , and had not been exposed to toxins or experienced weight loss . on neurologic examination , he was slightly confused , and scored 24/30 on the mini mental status exam ( mmse ) . the cranial nerve , motor , and sensory examinations , deep tendon reflexes , and cerebellar function tests were normal . his blood tests showed marked thrombocytopenia ( 2110/l ) and azotemia ( bun / cr 23 mg / dl/1.5 mg / dl ) . he was started on cefotaxime and clindamycin for a suspected infection and transfused with fresh frozen plasma , but his fever ( > 38.0 ) was sustained . two days after admission , he was alert and his fever had subsided , but the oliguria , azotemia ( bun / cr 49 mg / dl/3.0 mg / dl ) , and thrombocytopenia ( 610/l ) had worsened ( figs . 2 , 3 ) . four days later , the thrombocytopenia had recovered , although the azotemia ( bun / cr 82 mg / dl/7.1 mg / dl ) had not improved . he was diagnosed with hfrs based on the detection of hantaan virus antibody in his serum . six days later , he was alert , and his electroencephalograph ( eeg ) was normal . his platelet count had stabilized ( 31110/l ) , and a lumbar puncture was performed . the cerebrospinal fluid ( csf ) analysis revealed no cells , a nor mal protein level of 26.0 mg / l , a normal glucose level of 80 mg / dl , and no organisms on gram staining . the mri findings had resolved completely in follow - up studies conducted on day 14 ( fig . hemorrhagic fever with renal failure syndrome occurs mainly in europe and asia and is characterized by a fever and renal failure associated with hemorrhagic manifestations . hfrs is caused by airborne contact with secretions from rodent hosts infected with viruses belonging to the genus hantavirus in the family bunyaviridae . the clinical features consist of a triad of fever , hemorrhage , and renal insufficiency ( 5 ) . other common symptoms during the initial phase of the illness include , myalgia , abdominal and back pain , and diarrhea . in most cases , mild neurological symptoms such as headache , vertigo , and nausea the pathogenesis is largely unknown , but findings from several studies have suggested that immune mechanisms play an important role . after the infection , marked cytokine production , kallikrein - kinin activation , complement pathway activation , and increased levels of circulating immune complexes occur . damage to the vascular endothelium , capillary dilatation , and leakage are clinically significant features of the disease . myelin sheaths in the scc have a relatively high water component , and the scc is more susceptible to cytotoxic edema than are other brain areas ( 2 ) . reversible brain lesions have been attributed to the transient development of intramyelinic edema due to the separation of myelin layers , which is a possible mechanism for the transiently decreased diffusion of the scc lesion ( 2 , 11 ) . the influx of inflammatory cells and molecules , possibly combined with related cytotoxic edema , might have decreased the adc ( 2 , 12 , 14 ) . the most common causes of reversible focal lesions of the scc are viral encephalitis , antiepileptic drug toxicity / withdrawal , and hypoglycemic encephalopathy . they are not specific to iaee and have been reported secondary to various infectious agents , including rotavirus , measles , herpesvirus , salmonella organisms , mumps , varicella - zoster virus , adenovirus , o157 escherichia coli - associated hemolytic - uremic syndrome , legionnaires ' disease , and unknown pathogens ( 4 , 9 , 14 ) . the similar clinical and imaging features suggest a common mechanism induced by different pathological events leading to the same results ( 1 ) . the most likely causes of these transient lesions of the scc have been explained as a rapidly resolving intramyelinic infiltrate or the influx of inflammatory cells and macromolecules , combined with related cytotoxic edema , which is very similar to the pathogenesis of hfrs ( 2 , 7 , 9 , 10 , 13 ) . this argues for a non - specific cause related to vasogenic edema , probably secondary to the inflammatory response ( 2 , 7 ) our patient was diagnosed with hfrs , and his mri showed scc lesions . we report a reversible scc lesion associated with hfrs . to our knowledge , this is the first case of virus - associated encephalitis / encephalopathy in which the pathogen was a hantaan virus . although it is not certain whether the neurologic symptoms in this patient might have been caused by fever , or scc lesions , or both , we suggest that hfrs patients with neurologic symptoms like dizziness and mental slowing should be considered to have structural brain lesions and to require brain imaging studies .

this is the first case of virus - associated encephalitis / encephalopathy in which the pathogen was hantaan virus . a 53-yr - old man presented fever , renal failure and a hemorrhagic tendency and he was diagnosed with hemorrhagic fever with renal failure syndrome ( hfrs ) . in the course of his illness , mild neurologic symptoms such as dizziness and confusion developed and magnetic resonance images revealed a reversible lesion in the splenium of the corpus callosum . this case suggests that hfrs patients with neurologic symptoms like dizziness and mental slowing should be considered to have structural brain lesions and to require brain imaging studies .

upper gastrointestinal bleeding ( ugib ) is a major cause of mortality and morbidity accounting for about 56.5/100,000 hospitalization with a mortality rate of 8.2% . aorto - esophageal fistula ( aef ) , an abnormal anatomical communication between the aorta and esophagus is a rare cause of ugib . as the thoracic aneurysm grows in size , the increasing tension on its wall will erode the aorta and rupture into the surrounding esophagus leading to uncontrolled bleeding . as ulcer related or variceal bleeding constitutes for most of the ugib bleeding , even in elderly who are at risk of aef and the inability of the esophagogastroduodenoscopy ( egd ) to detect most cases , aef is often not diagnosed timely . all these factors make the timely diagnosis of aef challenging , and at times missed altogether leading to catastrophic consequences . we present a case of aef which was initially treated as variceal bleeding and later promptly diagnosed with computed tomography ( ct ) . a 70-year - old male patient with hypertension with regular ethanol use , presented to a local hospital with complaints of hematemesis and melena from the past 1-day . he was hemodynamically stable on presentation with initial hemoglobin level of 7.2 g / dl . he underwent timely egd , which showed grade 1 mid and distal esophageal varices without any active bleeding . subsequently , his condition deteriorated with the development of hypotension and transferred to our medical center for further management . weiss tear without active bleeding along with the presence of large blood clots in the gastric fundus . as the source of bleeding was not clear despite two egd and clinical evidence of gi bleeding , he underwent emergent colonoscopy which once again failed to detect any source lower gi bleeding . however , later in the day , he developed massive hematemesis and hematochezia leading to persistent hemorrhagic shock . emergent intubation was done for airway protection , massive transfusion protocol was initiated , and a ct angiogram was performed . it revealed type b aortic dissection with rupture and the active extravasation of blood into the esophagus , confirming the diagnosis of aef [ figure 1 ] . he underwent emergency percutaneous thoracic endovascular aortic repair with aortic stent placement [ figure 2 ] . his shock resolved hemoglobin stabilized and did not have any more episodes of bleeding thereafter . repeat egd showed a 3-cm opening in the midesophagus with ragged and irregular borders , not seen earlier egds . his hospital course was complicated by the infection of the endograft which was replaced and fortified with an omental flap and being is presently being , followed by for reconstruction surgeries . ( a ) communication and extravasation between aortia and esophagus ( blue arrow head ) in the arterial phase . ( b ) accumulation of blood in lower esophagus in venous phase ( yellow arrow head ) . ( c ) significant accumulation of blood in the stomach in the kidney delay images ( red arrow head ) . ( d ) sagittal reconstruction images shows commination ( blue arrow ) and extravasation ( yellow arrow ) of blood between aorta and esophagus endovascular graft in the thoracic aorta aorto - esophageal fistula is an abnormal anatomical communication between the aorta and esophagus . in 1818 , dubrueil described this condition for the 1 time in a french soldier who ingested a beef rib , leading to massive hematemesis . almost a century later , chiari described the triad of midthoracic pain , herald hemorrhage , and fatal hematemesis seen in these patients . the exact incidence of this condition is not known , as the vast majority of these patients die before a definitive diagnosis can be made . in the past , infections such as tuberculosis and syphilis , leading to aortitis were the common cause of this condition . in this antibiotics era , thoracic aortic aneurysm constitutes for more than half of these cases . other causes described in the literature are cancer , acid reflux disease , trauma , caustic ingestion , and esophageal biopsies . aefs are classified as primary if they are caused by the spontaneous erosion of the aortic wall into the esophagus and as secondary if they occur as a complication following aortic or esophageal surgeries . owing to the increasing number of interventions of the aorta , secondary aef is 10 times more common than primary . the herald bleeding described by chiari is only seen in half of these patients . once the bleeding starts , rapid extravasation from aorta will cause a sudden drop in blood pressure . , the blood pressure will raise again dislodging the fresh and loose clot around the aef leading to terminal bleeding . commonly used resuscitation fluids including pseudo random binary sequence lack adequate clotting factors add to the compromise of clot integrity . unlike in other structures , the lax mediastinum around the aorta and esophagus prevent autotamponade , to prevent persistent extravasation of blood . these factors can contribute to torrential bleeding , the final hematemesis as described by chiari . the risk of aortic aneurysm increases with age , with an incidence of 12.5% in elderly americans . the sensitivity of egd to identify the source of bleeding in other causes of ugib is 90% , but for aef it is < 10% . it may show pulsatile mass , bluish discoloration , or simply blood clotting in the esophagus in one - third of cases . a ct arteriogram is diagnostic in most cases , which show active extravasation of blood from the aorta to esophagus . once diagnosed , aef should be treated with emergent aortic stenting to control the bleeding . owing to contamination from the gi tract , one - quarter of stents become infected . apart from broad - spectrum antibiotics and antifungals , once the infection is controlled , the complex aortic and esophageal reconstruction is done in multiples stages . aef , once considered to be uniformly fatal , is now a treatable condition with timely intervention . aorto - esophageal fistula is a rare , but life - threatening condition presenting as ugib . a high level of suspicion is needed , as routine tests performed for ugib typically only detect one - third of cases . avoiding over - resuscitation can be helpful in preventing subsequent hemorrhages .

aorto - esophageal fistula is a rare cause of upper gastrointestinal bleeding . thoracic aneurysm , the most common cause of this condition , will slowly increase over time and can erode the wall of the aorta creating a fistula and leading to torrential bleeding . high clinical suspicion is required for timely diagnosis as common investigations routinely done for gastrointestinal ( gi ) bleeding , including esophagogastroduodenoscopy , fails to detect most cases . the classical triad of midthoracic pain , herald bleeding and fatal hematemesis described in this condition is seen in only one - third of cases . physician should be wary of this condition , especially in elderly patients with uncontrolled gi bleeding and who are also at risk of thoracic aneurysm . computed tomography angiogram detects most cases and emergent endovascular repair with stents controls the initial bleeding . later , both the aorta and the esophagus are repaired and reconstructed in staged procedures .

in june 2014 , a 61-year - old female was admitted to keimyung university dongsan medical center because of a severe dry cough that had lasted for over 20 days . according to her medical history , she had undergone a hysterectomy in 2005 , and the pathologic diagnosis was leiomyoma of the uterus . in 2006 , chest computed tomography ( ct ) findings demonstrated multiple nodules in both lungs ( fig . microscopic examination revealed a proliferating spindle cell tumor showing relatively bland nuclei , no pleomorphism , and no mitoses . in addition , immunohistochemical staining analysis was positive for smooth muscle actin , desmin , vimentin , estrogen receptor ( er ) , and progesterone receptor ( pr ) , and negative for cd34 , pan - ck , and s100 proteins . the result of the pathologic study was benign leiomyoma , and the nodules were diagnosed as pulmonary benign metastasizing leiomyoma ( pbml ) . , the patient did not return to our outpatient clinic for follow - up . upon the patient s return , a posteroanterior chest x - ray showed a very large mass on the right cardiac border ( fig . chest ct demonstrated a 101-mm mass on the right lower lobe that compressed the posterobasal segmental bronchus ( fig . a ct - guided needle biopsy of the huge mass revealed it to be leiomyoma . after right thoracotomy , a very large mass , 100 mm in size , originating from the visceral pleura of the right lower lobe was observed ( fig . the mass was in contact with the parietal and diaphragmatic pleura , but no invasion was noted . near the mass , multiple fine , scattered , whitish nodules measuring less than 1 cm were found on the diaphragmatic , parietal , and visceral pleura . we performed an excision of the mass in the right lower lobe and excised fine nodules from the parietal and diaphragmatic pleura for biopsy . intrapulmonary nodules found in the middle lobe and upper lobe were not excised , because they had not increased in size . the necrotic lesion showed severe cellular atypia , frequent mitoses ( more than 15 per 10 high power fields [ hpfs ] ) , and continuity of the leiomyoma and leiomyosarcoma ( fig . 2c ) . immunohistochemical staining revealed that the tumor was positive for smooth muscle actin ( sma ) , er , and pr , but negative for cd34 , pan - ck , s100 , and hmb45 ( fig . the nodular lesions taken from the parietal and diaphragmatic pleura showed no malignant cells . the patient was discharged 8 days after the operation without complications . large heterogeneous hypermetabolic masses were found from the left para - aortic area to the left pelvic cavity ( fig . 3 ) , and we diagnosed them as metastatic leiomyosarcoma . additionally , increased fluorodeoxyglucose ( fdg ) uptake was observed in the peribronchial lymph nodes , the mediastinum , and the supraclavicular lymph nodes . two months later , en - bloc resection of the retroperitoneal masses with left nephrectomy was performed , and the pathologic diagnosis was metastatic leiomyosarcoma . we decided to continue observing fdg uptake in the mediastinal and peribronchial lymph nodes rather than prescribing chemotherapy , because metastasis of leiomyosarcoma to the lymph nodes is rare . a chest ct scan done 1 year after surgery demonstrated no changes in the pulmonary nodules and no recurrence of the mass . benign metastasizing leiomyoma ( bml ) results from metastasis of a uterine leiomyoma ; thus , bml patients have a history of uterine leiomyoma . the condition may occur at any age , but occurs most often in women from 30 to 74 years old . bml can occur in a number of different areas of the body , including but not limited to the lungs , para - aortic lymph nodes , abdominal lymph nodes , heart , breast , liver , and esophagus . the most common site of occurrence is in the lungs , in which case it is known as pbml . the pathogenesis of pbml is unknown , but the most common theory is hematogenous spreading of leiomyoma during uterine surgery . immunohistochemical staining of affected tissue is positive for desmin , sma , pr , and er . most cases of pbml do not require treatment , but pbml that metastasizes or increases in size does need to be treated , as it can cause respiratory failure , chest pain , or hemoptysis . surgical excision is the treatment of choice , but this is only possible in cases of resectable pbml . in some cases of pbml that a repositive for er or pr , reducing hormone release via hysterectomy , bilateral adnexectomy , and/or hormone therapy may decrease the tumor s size . the incidence of the sarcomatous transformation of benign uterine leiomyomas is only 0.1%0.8% , and is most often seen in women in their 50s . pbml can also undergo malignant transformation ; excluding our case , just 1 case of the malignant transformation of pbml has been reported . radiologically , it is difficult to differentiate between normal pbml and pbml that has become malignant . on fdg pet - ct , heterogeneous fdg uptake of the tumor may reflect necrosis within the leiomyosarcoma . in our case , pathologically , the current criteria used to differentiate a malignant smooth muscle tumor from a benign mass are the presence of necrosis , a high mitotic index ( > 5 mitoses seen in 50 hpfs ) , nuclear atypia , cellularity , and an ill - defined tumor border . however , in primary pulmonary leiomyosarcoma ( ppl ) , progress is slow , and metastasis of ppl occurs late in the disease process . if the condition is diagnosed early , wide excision with negative margins ( r0 ) is the standard treatment . the 5-year survival rate in ppl patients who undergo complete resection is over 50% , and recurrence is rare . lymph node resections are generally not necessary because ppl rarely shows lymph node involvement . for advanced lesions , depending on the size and grade of the tumor , radiation therapy , chemotherapy , or both may be indicated as an adjuvant therapy . however , the median survival of patients with an unresectable sarcoma or metastatic disease is about 12 months . overall , early diagnosis and complete mass resection are vital in cases of the malignant transformation of pbml . however , preventive resection or diagnostic excisional biopsy is not recommended for all pbml patients , since the occurrence of a malignant change is extremely rare if there is a very large , newly developed mass that is increasing in size rapidly , or if a malignant mass is found on pet - ct , diagnostic mass resection should be considered .

pulmonary benign metastasizing leiomyoma ( pbml ) is defined as metastasis of a leiomyoma to lung tissue . it was first reported in 1937 . p bml is known as a benign disease , but can undergo malignant transformation . only 1 case of the malignant transformation of pbml to leiomyosarcoma has been reported previously . in this report , we present a case of malignant transformation of pbml .

musculoskeletal stiffness and limited range of motion ( rom ) can restrict functional joint movement , particularly during ankle supination following neurological diseases1 . ankle dorsiflexion rom restrictions impair dynamic balance and gait , which may contribute to secondary injuries ; reduced ankle rom and tight calf muscles can cause poor gait , inefficient energy use , impaired balance , and an increased risk of falls2 . musculoskeletal transformations manifesting as abnormal joint stiffness and limited rom result in numerous restrictions in the function and joint movements of patients3 . limited passive ankle dorsiflexion rom during knee extension can alter foot positioning and result in compensatory foot movements , leading to an abnormal gait , which can result in ankle sprains and lower extremity overuse injuries4 . studies have reported that balance - training programs that alter the somatosensory input , such as the use of an unstable surface to induce equalized weight distribution , can be helpful in hemiplegic patients5 . kim et al . used an ankle board with a slope in their center to achieve efficient ankle stretching and increased dorsiflexion6 . they suggested that stretching exercises using their device resulted in a greater increase in foot pressure compared to that with stretching exercises using existing inclined boards during ankle dorsiflexion stretching6 . therefore , we investigated the effects of an unstable inclined board on the active and passive ankle rom in patients with ankle stiffness . the study included 10 female patients ( mean age , 22.8 5.3 years ; height , 158.9 3.0 cm ; weight , 61.2 4.7 kg ) with ankle stiffness caused by diseases or medical conditions . the patients were selected based on ankle stiffness scores obtained using a symptom checker tool . the patients were assigned randomly to two groups ( both n = 5 ) and their height and weight were measured . the patients were not cognitively impaired and were capable of independent walking for 20 m without difficulty . patients were excluded if they had a history of or current neurological conditions . before participating , the purpose and methods of the study were explained to the patients , and all provided informed consent , according to the principles of the declaration of helsinki . active and passive ankle dorsiflexion were measured using a standard 31.75 4.45 cm clear plastic goniometer , with one increment , a 360 scale , and a bubble level at each end . group 1 performed the ankle dorsiflexion stretching exercise using a wooden inclined board , and group 2 performed ankle dorsiflexion stretching exercises using an air - cushioned inclined board that provided an unstable surface . rubber sheets were attached to the inclined boards to prevent falls due to sliding . the patients performed ankle dorsiflexion stretching exercises for 5 min , with a 10-min rest between each set , five times a day for 1 week . the active and passive ankle dorsiflexion angles were measured bilaterally using the goniometer before and after the study . independent and paired t - tests were used to assess within- and between - group differences in ankle dorsiflexion , with spss for windows ( spss , chicago , il , usa ) . the amount of active and passive dorsiflexion did not differ between the two groups before the exercises , with active dorsiflexion angles of 18.2 6.4 and 19.3 4.2 and passive dorsiflexion angles of 23.4 5.5 and 22.6 5.7 in groups 1 and 2 , respectively ( both p > 0.05 ) . the stretching exercises significantly ( p < 0.05 ) increased the active and passive ankle dorsiflexion angles in both groups compared to those at baseline . the active dorsiflexion angle was significantly increased ( p < 0.05 ) in group 2 compared to that in group 1 after the exercises . the active dorsiflexion angles before and after the exercises were 18.2 6.4 and 22.1 5.0 in group 1 and 19.3 4.2 and 28.1 6.9 in group 2 , respectively . the passive dorsiflexion angles did not differ significantly between the groups after the exercises ( p>0.05 ) and were 23.4 5.5 and 32.2 5.3 in group 1 and 22.6 5.7 and 35.1 6.0 in group 2 before and after exercising , respectively . the air - cushioned inclined board provided an unstable surface for performing ankle dorsiflexion stretching exercises . the results showed that ankle stretching exercises using the unstable inclined board significantly increased the active dorsiflexion angle compared to that with exercises using a wooden inclined board . we believe that the use of the air - cushioned inclined board provides somatosensory input and subsequently stimulates active ankle dorsiflexion by promoting automatic postural control . sensory proprioception of the ankle and sensory compression of the plantar are important in the control of sway7 . an increase in the number of somatosensory inputs affects the muscles , and the use of an unstable support surface result in many different somatosensory inputs that limit compensatory action7 . performing cognitive tasks in a standing posture reduces internal focus and induces external focus , which promotes automatic postural control8 . stretching improve the flexibility of tendons , and changes in the mechanical condition of tendons depend on the type of stretching performed9 . , the passive dorsiflexion angles did not differ significantly between the two groups after ankle stretching exercises . however , stretching exercises significantly increased the active and passive ankle dorsiflexion angles in both groups compared to those at baseline . these results suggest that passive ankle exercises using a wooden inclined board only stretch muscle and tissue , while exercises using an unstable inclined board stretch muscle and tissue , and stimulate activation of the ankle dorsiflexor . active ankle dorsiflexion was more effectively improved with ankle stretching exercises using an unstable inclined board than with exercises using a wooden inclined board .

[ purpose ] the present study assessed the effects of using an unstable inclined board on the active and passive ankle range of motion in patients with ankle stiffness . [ subjects ] the study included 10 young female patients with ankle stiffness . [ methods ] the patients were divided into the following two groups : a group that performed ankle dorsiflexion stretching exercises using a wooden inclined board and a group that performed stretching exercises using an air - cushioned inclined board ( unstable inclined board ) . active and passive ankle dorsiflexion angles were measured bilaterally using a goniometer . [ results ] both inclined boards significantly increased active and passive ankle dorsiflexion . after performing ankle stretching exercises , active dorsiflexion significantly increased the unstable inclined board compared to that using the wooden inclined board . however , the passive dorsiflexion angles did not differ significantly between the two groups after ankle stretching exercises . [ conclusion ] the use of an unstable inclined board might stimulate activation of the ankle dorsiflexors in addition to stretching muscle or tissue . active ankle dorsiflexion was more effectively improved with stretching exercises using an unstable inclined board than with exercises using a wooden inclined board .

renal tubular involvement in sjgren 's syndrome ( ss ) usually manifests with fanconi syndrome , distal ( type 1 ) renal tubular acidosis ( rta ) , nephrogenic diabetes insipidus , and hypokalemia . , we describe a case a young female with ss who presented with features of gitelman syndrome and hypokalemic paralysis . a 29-year - old female presented with weakness of both upper and lower limb weakness since 15 days . she had a history of dry eyes and dry mouth for the past 6 months . on examination , laboratory evaluation showed hemoglobin - 9.5 g / dl , platelet 2.95 lakh / mm . blood urea nitrogen was 7 mg / dl , serum creatinine was 0.65 mg / dl , serum sodium was 134 meq / l , serum potassium was 1.81 meq / l and serum chloride was 84.3 urine chloride ( spot ) was 36.7 meq / l , urine creatinine ( spot ) 23.5 mg / dl , and urine calcium ( spot ) 1.23 mg / dl . this patient satisfied the american european consensus criteria for diagnosis of primary sjgren 's syndrome ( ss ) . ten days after admission , she was discharged on spironolactone and prednisolone . on follow - up this patient presented with muscle weakness and her laboratory reports showed hypokalemia and metabolic alkalosis with normal blood pressure . her urinary chloride levels were above 20 meq / l , which ruled out extra - renal causes of metabolic alkalosis . the presence of hypomagnesemia and absence of hypercalciuria ( urinary calcium creatinine ratio < 0.2 ) ruled out bartter syndrome . hypokalemic alkalosis , high urinary chloride , hypomagnesemia , absence of hypercalciuria in background of no history of diuretic use favored the diagnosis of gitelman syndrome . tubular involvement in ss is usually distal tubular dysfunction , type i ( distal ) renal tubular acidosis and nephrogenic diabetes insipidus . proximal tubular abnormalities are less frequent , and rarely fanconi 's syndrome has been reported in patients with ss . acquired gitelman syndrome is relatively rare . to the best of our knowledge , only five cases of acquired gitelman syndrome have been reported in english literature so far . among these five cases , four had ss , one was a case of chronic sialoadenitis . acquired gitelman syndrome secondary to ss presenting with hypokalemic weakness is very rare . only two cases have been reported so far . though gitelman syndrome is an inherited disorder , given the paucity of reports , we believe that ss presenting as acquired gitelman syndrome may be relatively rare . acquired gitelman syndrome should be considered in differential diagnosis of renal involvement in patients with ss .

we report a case of a young female patient who presented with weakness of upper and lower limbs . on evaluation , she had hypokalemia , hypomagnesemia , metabolic alkalosis and hypocalciuria . anti - ro ( ssa ) antibody was positive . she had an acquired gitelman syndrome due to primary sjgren 's syndrome ( ss ) . ss presenting with features of gitelman syndrome is very rare .

balance is very important in daily activities of the elderly and greatly affects their quality of life . due to age - related changes , balance ability deteriorates . osteoarthritis is a degenerative disease that causes severe pain in elderly patients with low pain thresholds . research has shown that it results in a high disability score , low quality of life , and limited range of motion , in addition to weak muscle strength , poor balance ability , and high risk of falls1,2,3,4,5 . as osteoarthritis causes severe pain and functional disorders , in general , the clinical treatment is focused on pain management and amelioration of related disorders . the treatments include muscle strength training , electrical stimulation , manual therapy , aquatic therapy , balance therapy , and pharmacotherapy6 . as balance ability and falls are closely related in the elderly , exercise therapies need to focus on the maintenance of such ability7 . horseback riding exercise is one such therapy . in research conducted by arajo et al . 8 where elderly individuals performed horseback riding exercise for eight weeks , the researchers reported that the exercise significantly improved the participants balance and muscle strength . unlike other interventions , horseback riding exercise can arouse the interest of patients and motivate them while improving their balance , bone density , muscle strength , and mental state9,10,11 . according to some studies , horseback riding exercise increased the static muscular contraction of the rider against the movement of the horse and improved the muscle strength in the quadriceps femoris and knee flexors of young riders12 , 13 . most people do not indulge in horse riding because of where they live ( i.e. , urban rather than rural areas ) or the costs and risks involved in horse riding . hence , simulator horseback riding exercise equipment using virtual reality has been developed for use at home and in small spaces . previous studies reported that horseback riding exercise significantly increased oxygen intake , minute ventilation , met , calorie consumption , and muscle14 , 15 . although various therapeutic approaches have made with horseback riding exercise in the previous studies there is a lack of research on the effects of horseback riding exercise equipment on the balance and gait abilities of elderly patients with osteoarthritis . experimental procedures were performed in accordance with the protocols established by the institution of animal care and use committee ( iacuc ) at the daegu university . include criteria was aged 65 years or over ( table 1table 1.general characteristics of the subjectsvariablessubjects ( n=15)gender ( m / f)6/9age ( years)76.5 7.7height ( cm)160.6 9.7weight ( kg)54.7 9.8mean sd ) , and with a history of none falls in previous year , lower pain for perform experiment , and visual or auditory damage , nervous system or of the vestibular organ problems , who ca nt understand the contents of the experiment were excluded . after providing information study all subjects received a participation agreement . in this study , the subjects , while providing conventional physical therapy , 8 weeks three times a week the movement of the horse riding equipment , was carried out for 30 minutes . before performing horse riding simulation exercise , subjects were educated about exercise program through a research assistant s demonstration . in this study , used the slimrider ( shinhwa , mx-0004se , korea ) , which is a horse riding simulator . while performing a horse riding equipment exercise , subjects were effort to maintain the correct posture . exercise intensity was increased gradually according to the state of the subjects while performing the exercise , the research assistant were always observed to prevent falling . in order to evaluate the balance of the elderly , it was using the frt ( functional reach test ) and sbbsf ( short form berg balance scale ) . the spss for windows ( version 18.0 ) was used for data analysis of the study . in order to compare the differences between before and after , it was analyzed using the paired t - test . in this study , sfbbs scores and range of frt increased significantly after horse riding simulator exercise ( table 2table 2.comparison of measurement values at pre - test and post - testvariablesprepostsfbbs ( score)18.6 5.822.3 4.4**frt ( cm)19.7 9.526.3 7.5*mean sd . osteoarthritis causes severe pain in elderly patients who have a low threshold for pain and high disability scores , thereby resulting in a low quality of life , increased risk of falls , and poor balance ability1 , 2 , 6 . in the present study , the static , dynamic , and functional balance abilities of osteoarthritis patients who undertook horseback riding exercises on exercise equipment were measured using a frt and sfbbs . the findings are in accordance with those of study that stroke patients performed horseback riding exercise for 35 min five times a week for eight weeks and arajo8 in which elderly patients performed horseback riding exercise for eight weeks . they are also in accordance with the results of han jun - young16 in a study of stroke patients who performed horseback riding exercise for 12 weeks that an eight - week - long horseback riding exercise significantly improved the bbs scores of osteoarthritis patients . in general elderly , the average bbs score is 20.60 4.0817 . in the present study , after eight weeks of the exercise , the average score was 22.3 4.4 , higher than the average . the results suggest that horseback riding exercise equipment can improve dynamic and functional balance abilities . furthermore , according to the frt results , the static balance ability of the participants improved . these results are in line with the research findings of yasuhiro et al.18 and kim sung - gil11 where the frt scores improved significantly after 12-week - long and 8-week - long horseback riding exercise , respectively . an increase in the frt score is considered an improvement in static balance ability . based on the findings of the present study , horseback riding exercise equipment seems to improve the static balance ability of the elderly .

[ purpose ] the aim of this study was to examine the effects of horse - riding simulator exercise on balance in elderly with knee osteoarthritis . [ subjects and methods ] fifty elderly patients with knee osteoarthritis were recruited , a horse - riding simulator group performed exercise three times a week for eight weeks . and each exercise was performed for 30 minutes . [ results ] the horse - riding simulator group showed significant differences after the intervention in short form berg balance scale , functional reaching test . [ conclusion ] the results of this study indicate that horse - riding simulator exercise is effective on knee osteoarthritis . therefore , horse - riding simulator exercise can be used balance training for knee osteoarthritis .

we report 2 patients infected with panton - valentine leukocidin ( pvl)positive mrsa t034 . each patient had a medical history typical of that reported for community - acquired mrsa of other lineages , which in most cases are pvl positive ( 8) . the first patient , a previously healthy 36-year - old male physiotherapist , sought medical care in march 2006 for a small abscess in his axilla . culture of the abscess grew mrsa . presence of meca gene was confirmed by pcr ( 9 ) . during the next 2 months , his youngest child , adopted from china , had been found to be mrsa positive ( throat , perineum , and a small wound ) a month earlier during routine screening for adopted children . during subsequent screening of the family , the older sister , adopted from south korea , both parents were negative for mrsa at that time , which suggests that the father was newly infected when his abscess developed and that he had not acquired the strain abroad . also , spa typing indicated that the children carried different strains from that of the father and from each other ( t286 , t1434 ) ( 10 ) . subsequent screening of family members for mrsa on several occasions found only the father to be repeatedly positive . the second patient , a 43-year - old male clerk , also previously healthy , sought medical attention during the summer of 2007 for a mrsa - infected elbow wound . follow - up examination determined that he carried mrsa also in the perineum and in a chronic external otitis eczema . the patients lived in geographically distinct areas in the western part of sweden and had no connection to each other . no animal contact ( e.g. , pets , farming ) was reported by the 2 patients , their family members , or other close contacts . both patient strains carried pvl , confirmed by identification of the luks - lukf genes ( 11 ) , and were resistant to digestion with restriction endonuclease smai when typing by pfge was attempted . they produced -hemolysin according to phenotypic detection methods that used rabbit blood agar with hot cold analysis , which further indicated their animal origin ( 12 ) . their drug - susceptibility profiles differed ; 1 was resistant to doxycycline and the other was resistant to ciprofloxacin , erythromycin , and clindamycin . these strains carry pvl , a toxin partly responsible for the increased virulence of several of the mrsa clones in the community ( 8) . despite several recent publications concerning st398 mrsa , few have reported pvl in this lineage , which is believed to be of animal origin ( 2,4,6,13 ) . most previous reports have described asymptomatic carriage in persons exposed to occupational hazards ( e.g. , veterinary personal and pig farmers ) ( 1,2,7 ) . however , severe clinical infections have been described ( 46 ) . in our patients these strains caused repeated infections that needed medical attention , even hospitalization . since neither patient had even a remote connection to animals and we found no common source of infection , these strains may already be more common in our region than we had thought . these case reports suggest that strains of this lineage may impose a threat in the community , even to patients with no obvious animal contact .

panton - valentine leukocidin ( pvl)positive methicillin - resistant staphylococcus aureus ( mrsa ) , sequence type 398 is believed to be of animal origin . we report 2 cases of infection due to pvl positive mrsa , spa type t034 , in patients in sweden who had had no animal contact .

the selective melatonin receptor agonism effect of ramelteon is useful for insomnia.1 ) here we wanted to present a refractory chronic migraine case , who had significant improvements in migraine after using ramelteon . the precipitating factors were the stress , increase in physical activities and sudden cold weather . the moderate or severe pain intensity was aggravated by or causing avoidance of increased physical activity . the residual symptom between the migraine episodes were the head tense feeling , but not achieveing headache severity . the computed tomography revealed no significant findings of other causes for headache , such as tumor or hemorrhage . she ever received many kinds of medications , such as aspirin , nonsteroidal anti - inflammatory drugs , triptans , ergots , anticonvulsants and glucocorticoids . however , the severity of migraine still remained moderate ( migraine disability assessment test [ midas ] scores , 19 ) and the associated symptoms were moderate headache , dizziness , nausea , and sensitivity to sound . the migraine also exacerbated her insomnia problems , with fragmented sleep and inadequate sleep duration as 12 hours ( insomnia severity index [ isi ] score , 23 ) . she did nt have any comorbid mental disorder except insomnia . due to the fear of abuse potential related to hypnotics after 2 weeks treatment of ramelteon 8 mg / day , her sleep duration prolonged to 45 hours with less fragmentation . in addition , her migraine severity started to decline ( midas scores , 11 ) . after 6 months of ramelteon 8 mg / day treatment , the migraine severity continued to relieve ( midas scores , 6 ) with stable sleeping quality ( isi score , 14 ) . in this case , we found significant improvements in migraine severity and insomnia after the use of ramelteon . since the patient did nt have insomnia before the first onset of migraine . therefore we could speculate that the ramelteon had significant effects in migraine in such mild prolongation of sleep duration . the melatonin might relieve the headache via the following possible mechanisms , such as anti - inflammatory effect , free radical scavenging , reduction of pro - inflammatory cytokine , membrane stabilization , nitric oxide synthase activity and dopamine release inhibition , gaba and opioid analgesia potentitation , glutamate neurotoxicity protection , neurovascular regulation,2 ) cytoprotection and antiallodynic action.3 ) the pineal gland , the primary source of serotonin and melatonin , might also play a significant role in the analgesic effect.4 ) however , the clinical trial of melatonin administration showed no significant improvements in migraine severity,5 ) which suggested the modulation of melatonin receptor might be a better option for the analgesic effects . the modulation of serotonin system by the ramelteon would also relieve the pain sensation.2 ) in addition , the chemical structure of ramelteon was similar with that of indomethacin , which is a kind of non - steroidal anti - inflammatory medicine.2 ) in the study of animal model , the antinociceptive and antiallodynic actions of melatonin have been observed in different kinds of pathways , such as intrathecally or intracerebroventricular routes . the use of ramelteon , a kind of mt1 and mt2 agonist , could also relieve the pain even lack of free radical scavenger effects.3 ) the state - of - art treatment of refractory chronic migraine also includes the ramelteon due to selective m1 receptor agonism.6 ) from the successful experience in this case , we can consider the use of ramelteon for refractory chronic migraine with insomnia .

the selective melatonin receptor agonism effect of ramelteon is useful for insomnia . here we wanted to present a refractory chronic migraine case , who had significant improvements in migraine after using ramelteon . the possible mechanism for the ramelteon in the migraine relief might be related to melatonin effects .

the patient was a 41-week - gestational - age infant girl with a birth weight of 3300 g. after an uneventful pregnancy , the patient was delivered spontaneously and healthy until this episode . at 35 days of age , the patient was admitted to our hospital with a one - day history of anorexia , vomiting and recurrent diarrhea . on admission , conscious level was glasgow coma scale e2 v2 m2 , and blood pressure was 104/flat . physical examination revealed a distended and tender abdomen . laboratory values were significant for a c- reactive protein ( crp ) of 1.7 mg / dl ( normal range was less than 0.3 mg / dl ) . the patient was suspected intestinal obstruction due to enteritis , antibiotics were started ( abpc / mcipc and amk ) . 4 hours after admission , she was pale with a temperature of 39.2 c and 220 per minutes pulse . blood pressure was low , and the fevers , vomiting , and diarrhea persisted and worsened despite antibiotics . she failed to respond to medical managements and died 27 hours after admission due to multiple organ failures . the cyst was unilocular , contained watery fluid , did not communicate with the adjacent intestinal lumen ( fig . microscopic finding of the ileocecal duplication cyst had an undefined mucosal lining though it resembled pyrolic glands or duodenal glands . duplications of the alimentary tract can be located anywhere between the mouth and the anus.4 they are most commonly found in the ileum ( 30% ) , ileocecal valve ( 30% ) , duodenum ( 10% ) , stomach ( 8% ) , jejunum ( 8% ) , colon ( 7% ) and rectum ( 5% ) with the half of the all lesions may contain ectopic gastric mucosa.5,6 most alimentary tract duplications are cystic ( 80%90% ) , with the remainder being tubular.7 they have three common features , hollow structure that has a wall of smooth muscle often shared with the contiguous intestinal tract , lined by mucous membrane similar to some part of the alimentary canal , and usually attached to some part of the alimentary tube.8 the clinical symptoms are highly variable from minor digestive problems to intestinal obstruction , gastrointestinal bleeding , or perforation.9 most patients present before the age of 2 years with obstructive symptoms , pain , or an abdominal mass . the presence of gastric or pancreatic mucosa can lead to peptic ulceration , perforation , and hemorrhage . early diagnosis and treatment reduces the risk of dangerous complications , such as bowel perforation , bleeding , obstruction , and malignant alteration . resection of duplication alone is the treatment of choice , and in uncomplicated cases laparoscopic resection is advised . surgically treated intestinal duplications have good prognosis , however , such duplications can have a fatal consequence if not properly treated . possible complications like bowel perforation , bleeding , obstruction , and malignant alterations are the reason why all duplications should be surgically treated at the time of diagnosis.10 alimentary tract duplications can be easily mis - diagnosed as other disorders.5 pyloric and duodenal duplications can mimic hypertrophic pyloric stenosis or choledochal cyst.11,12 ovarian cyst should be considered in female patients , and adolescent patients can sometimes have a presumptive diagnosis of crohn s disease.5 yousefzadeh et al13 showed that duplication of the colon and rectum clinically presented as abdominal pain , vomiting and chronic constipation , a significant number of patients were thought to have hirschsprung s disease . in our fatal case , there are two reasons for this diagnostic difficulty : first , fevers , vomiting , and recurrent diarrhea persisted and worsened despite antibiotics . the patient was suspected intestinal obstruction due to enteritis , however , actually the ileocecal duplication cyst led to intestinal obstruction and necrotizing ileum ; second , the patient died in an instant due to multiple organ failures . if this patient was considered for exploratory laparotomy , she might be able to be alive . however , the patient was getting worse very quickly due to multiple organ failures and showed intractable psvt . exploratory laparotomy was impossible . it is demonstrated a high association of other anomalies in these patients including skeletal and urologic abnormalities,13,14 however , in this case , there were no other congenital anomalies . sudden unexpected death of infants associated with duplication cysts is extremely rare with only 4 cases reported in medical literature to date ( table 1 ) . puligandla ps et al5 and byard rw15 reported the patients presented with group b streptococcal sepsis and clostridium perfringens sepsis , respectively . kibayashi et al16 reported the patient died at home after being diagnosed as having gastroenteritis . in present case , though blood , stool and cyst cultures were negative , the patient had high fever and crp level elevated . infection is suspected to be a risk factor for sudden and unexpected death in infants with duplication cysts . in all cases , this potential diagnosis should be borne in mind for a patient who complains of abdominal symptoms with an unknown cause , and duplication cyst should be recognized as a fatal cause in infant .

duplications of the alimentary tract are very rare . a one - month - old female presented with symptoms of anorexia , vomiting and continuous watery diarrhea . the plain abdominal radiograph showed thickened intestinal wall and signs of small bowel obstruction . the fevers , vomiting , and continuous wartery diarrhea persisted despite antibiotics , and worsened . the patient failed to respond to medical managements , 27 hours after admission , the patient died due to multiple organ failures . the autopsy was performed , small bowel obstruction due to an ileocecal duplication cyst ( 3 3 cm ) was recognized . the ileocecal duplication cyst was attached to the ileum which was changed edematous and necrotic . this potential diagnosis should be borne in mind for a patient who complains of abdominal symptoms with an unknown cause , and duplication cyst should be recognized as a fatal cause in infant .

enteric duplications ( eds ) are uncommon anomalies that can occur at any point of the gastrointestinal tract . the small intestine is the most common location ; retroperitoneum is an extremely rare site . in general , diagnosed in the neonatal period or during infancy , they are increasingly diagnosed prenatally ; early prenatal detection is possible . , there have been seven reported cases of retroperitoneal ed cyst in the english literature . a female newborn , vaginally born at 39 weeks of gestation from a 32-year - old mother , gravid 3 , para 3 . prenatal ultrasound at 22 weeks of gestation objectified an abdominal cystic mass located in the left upper abdominal quadrant , associated with fetal pyelectasis . birth weight was 4000 g , length was 51 cm , and head circumference was 35 cm . postnatal ultrasound found a retroperitoneal para - aortic liquid - filled mass measuring 60 mm 33 mm 22 mm . magnetic resonance imaging ( mri ) confirmed the presence of a retroperitoneal cyst occupying the upper left retroperitoneal space ; with mass effect displacing the left kidney down [ figure 1 ] . peroperative finding was a retroperitoneal cyst above the left adrenal , displacing the left kidney down , measuring 70 mm 30 mm ; with no communication with any portion of the alimentary tract . histopathologic examination revealed an ed cyst lined by small intestinal epithelium , with no ectopic gastric mucosa . the postoperative period was uneventful ; the patient was discharged on the 5 postoperative day . magnetic resonance imaging showing retroperitoneal cyst occupying the upper left retroperitoneal space ; with mass effect displacing the left kidney intraabdominal duplications account for two - thirds of localization , among them , jejunoileal duplications account for 65% . eds occur early in intrauterine life ; their pathogenesis remains unclear ; several hypotheses have been proposed . reported 38 cases of ed cysts ; only one was retroperitoneal communicating with a nonfunctioning right kidney . eds are a part of fetal intraabdominal cysts which evoke several differential diagnoses including ovarian cysts , renal cysts , choledochal cysts , hepatic cysts , and mesenteric cysts . marchitelli et al . found high concordance between prenatal and postnatal findings in fetal intra - abdominal cystic lesions ( 90.4% ) . double - wall sign and peristaltism are suggestive of ed and help to differentiate it of differential diagnosis . postnatal ultrasound helps diagnosis showing an anechoic fluid - filled mass , rarely an echogenic mass is found due to complication ( hemorrhage , necrosis ) . more serious complications may occur : ulceration , perforation , severe hemorrhage , and malignant changes associated with the presence of ectopic gastric mucosa .

retroperitoneum is a very uncommon site of enteric duplication ( ed ) . we report a new case of retroperitoneal ed cyst suspected in utero . prenatal ultrasound showed an abdominal cystic mass . noncommunicating retroperitoneal ed cyst measuring 70 mm 30 mm was resected . histopathologic examination confirmed the diagnosis .

, neither buccolingual width nor angulation can be properly visualized on the most traditional radiographs . in the following case report , an innovative simplified method without radiation is used to fabricate a three - dimensional model to assess the available bone for implant placement in the mandible . even a minor variation in comparison to ideal placement causes difficulties in fabrication of final prostheses . the surgical guide is essential to establish a logical continuity among diagnosis , prosthetic planning , and surgical phase . a patient of age 27 years reported to the department of prosthodontics for the replacement of missing left first molar . following steps were performed to achieve a three - dimensional model : intraoral impression was recorded with addition silicone putty and light body [ figure 1]fabrication of acrylic assembly for aligning both intraoral and extraoral impressiona rectangular block of acrylic was made to which impression tray was pressed to make indentations . after applying separating medium , the counter block of acrylic was made to which extraoral impression was attached [ figure 2 ] . in prototype , [ figures 24 ] plastic rod was used for supporting the extraoral impressionreplacing the plastic rod with metal plate and key system the plastic rod was replaced by a metal plate in the first generation [ figure 5 ] for better stability and better customization . a key system was incorporated to adjust the length and angulation according to each patient 's mandible . on the top of the metal plate , addition silicone putty placed on the acrylic plate can be customized each time to record the extraoral impression . the extraoral impression should record the lower border of the mandible to the medial extent as much as possible [ figure 6]fabrication of three - dimensional model the intraoral impression was poured first to get a cast . after the initial setting was over , the entire assembly was poured , and a three dimensional model of the mandible was fabricated [ figure 7 ] . intraoral impression was recorded with addition silicone putty and light body [ figure 1 ] fabrication of acrylic assembly for aligning both intraoral and extraoral impression a rectangular block of acrylic was made to which impression tray was pressed to make indentations . after applying separating medium , the counter block of acrylic was made to which extraoral impression was attached [ figure 2 ] . in prototype , [ figures 24 ] plastic rod was used for supporting the extraoral impression replacing the plastic rod with metal plate and key system the plastic rod was replaced by a metal plate in the first generation [ figure 5 ] for better stability and better customization . a key system was incorporated to adjust the length and angulation according to each patient 's mandible . on the top of the metal plate , acrylic addition silicone putty placed on the acrylic plate can be customized each time to record the extraoral impression . the extraoral impression should record the lower border of the mandible to the medial extent as much as possible [ figure 6 ] fabrication of three - dimensional model the intraoral impression was poured first to get a cast . after the initial setting was over , the entire assembly was poured , and a three dimensional model of the mandible was fabricated [ figure 7 ] . intraoral impression with acrylic assembly prototype ( extraoral impression ) prototype - extraoral and intraoral impression together with acrylic assembly first generation metal plate assembled metal frame three - dimensional model the accuracy of this method was verified with computed tomography ( ct ) scan . the cross section where the mandibular premolar teeth were disappearing from the ct image was taken ( 14 mm depth ) as a reference and measured to get the available bone width buccolingually ( 8.7 mm ) . this measurement was same when compared with the available bone in the sectioned cast at the premolar area , at a depth of 14 mm ( premolar root length ) . on the three - dimensional model , the surgical guide was fabricated [ figure 8 ] . this surgical template can dictate the implant body placement that offers the best combination of support for the repetitive forces of occlusion and esthetics . three - dimensional model with surgical guide this article had won the best table clinic award in 35 ips conference conducted in new delhi 2007 by army dental corps .

the primary factor that determines the success of implant is the amount of available bone in the edentulous site . diagnostic imaging and techniques help to develop and implement a cohesive and comprehensive treatment plan according to the available bone . in the following case report an innovative simplified method ( poor man ct ) is used to fabricate a three dimensional model to assess the available bone for implant placement in mandible .

bezoars are masses formed by the condensation of debris or stomach contents in the gastrointestinal tract . there are four major types of bezoars : a trichobezoar consists of hair , a phytobezoar of vegetable and fruit residues , a lactobezoar is formed from dairy products , a pharmacobezoar is caused by medications . a high - fiber diet , incomplete mastication , reduced gastric secretion , and decreased gastrointestinal motility from autonomic neuropathy can predispose patients to phytobezoar formation . it occurs more frequently in the small intestine , particularly in the terminal ileum and jejunum . the complications caused by bezoars are mechanical irritation , gastrointestinal obstruction , and , in severe cases , peritonitis caused by bowel perforation . bezoars are responsible for 0.44% of cases of mechanical intestinal obstruction , which occurs mainly in the stomach and small intestine . contrast - enhanced computed tomography ( ct ) is the method of choice to identify the location , severity , and etiology of intestinal obstruction . we describe our experience regarding a large - bowel obstruction caused by phytobezoar in a patient with a transplanted kidney . a 39-year - old man was admitted to our hospital for constipation , distension , and severe acute abdominal pain in the lower left quadrant . his medical history included alport syndrome resulting in progressive chronic kidney disease ( ckd ) , hypertension , and iron deficiency anemia under medical treatment . twelve years ago , he received a successful deceased - donor kidney transplant [ figure 1 ] . at the time of admission , vitals were stable , and he was afebrile . chest x - ray was normal and abdominal x - ray did not show signs of bowel obstruction . coronal computed tomography scan shows transplanted kidney in left iliac fossa ( ) non - contrast ct ( ncct ) of the abdomen was done ( toshiba aquilion 64-tsx-101a / hc ) as the patient had ckd ( serum creatinine level = 7.71 mg / dl ) . ct scan revealed a focal circumferential thickening of the sigmoid colon with an ovoid and encapsulated intraluminal mass measuring 4.7 3.6 cm [ figure 2 ] . dilatation of the proximal segment with abundant fecal material was seen [ figure 2 ] . based on the ct findings , particularly mottled gas pattern and encapsulated wall of the mass , we made the diagnosis of phytobezoar . hence , because of acute intestinal obstruction and to avoid peritonitis , the patient underwent urgent exploratory laparotomy . ( a ) coronal computed tomography scan shows an ovoid intraluminal mass containing mottled gas pattern with encapsulating wall and associated wall thickening ( arrows ) ; note some dilated bowel loops ( stars ) and an abundant fecal material ( arrowhead ) . ( b ) the mean attenuation value of the intraluminal mass is about 10.21 hu the obstructive intraluminal mass in the sigmoid colon was identified ( approximately 4 4 cm in size ) and manually broken down with evacuation of the obstructive material along with large amounts of liquid fecal material . the postoperative course was uneventful and the patient was discharged on the seventh postoperative day . at 3-month follow - up , phytobezoars are concretions of poorly digested fruit and vegetable fibres that are found in the alimentary track and are mostly composed of indigestible cellulose , tannin , and lignin from ingested vegetables and fruits . normally , they are found in the stomach and may enter into the small bowel . the most important risk factors for phytobezoar formation are excessive consumption of fruits rich in fibers , poor dental health , insufficient mastication , diabetic gastroparesis , kidney failure , hypothyroidism , use of drugs which affect gastric motility , and previous gastrointestinal surgery . in our case , other symptoms include abdominal distension , vomiting , constipation , diarrhoea , anorexia , and weight loss . if treatment is delayed , colonic bezoars can be complicated by intestinal obstruction and perforation with consequent peritonitis . plain abdominal radiography and ct scan are used for radiological evaluation of the patients presenting with intestinal obstruction . simple abdominal x - ray may reveal air - fluid levels associated with mechanical obstruction , and occasionally it is possible to see the outline of bezoar , which is difficult to differentiate from abscess or feces within the colon . on abdominal ct , 89% of bezoars appear as spherically - shaped masses containing air - fluid levels . ct is also helpful in discriminating bezoars from other causes of bowel obstruction such as gastrointestinal malignancies , gallstone ileus , etc . in our case indeed , many authors have highlighted some ct features to allow easier differential diagnosis between these two entities . small bowel feces tends to be more amorphous and tubular in shape without evidence of an encapsulating wall , whereas phytobezoars have a well - defined ovoid shape and an encapsulating wall , such as in our case . chen et al . showed that a higher grade of obstruction is commonly associated with phytobezoar impaction without mesenteric fatty stranding and intraperitoneal fluid ; moreover , they found that a combination of the food debris length < 9.5 cm and the mean attenuation value < 11.75 hu of the obstructed bowel at the transition point was suggestive for phytobezoar , such as in our patient . although these features are typically found in the small bowel , they may also be considered very useful in the large bowel . conservative management is considered in uncomplicated cases of colonic bezoars and consist of digital evacuation , enemas and manual disimpaction . if conservative approach fails , a colonoscopy can be attempted for bezoar removal with contextual mechanical fragmentation and extraction using several instruments . although endoscopy has an acceptable success rate and fewer complications than surgery , surgical approach is mandatory in case of endoscopic treatment failure , sigmoid volvulus , hematochezia , intestinal obstructions , perforations , or peritonitis . when surgical approach is chosen , the first step is intestinal decompression and fluid - electrolyte replacement ; later , open or laparoscopic abdominal exploration may be performed . in our case , the patient underwent laparotomic surgery and phytobezoar was manually broken down with evacuation . bezoar is a rare disorder , however , it must be suspected in case of mechanical intestinal obstruction . our case underlined the importance of early detection and removal of a bezoar to prevent complications such as obstruction , perforation , or peritonitis .

bezoars are masses formed by the concretion of stomach contents or debris within the gastrointestinal tract . bezoars are rare and account for only 0.44% of all cases of gastrointestinal obstruction and mainly occur in the stomach or small intestine . intestinal obstruction caused by colonic bezoars is extremely rare . a 39-year - old man with a transplanted kidney came to the hospital because of abdominal pain , constipation , and distension . we performed an abdominal computed tomography scan and found an ovoid intraluminal mass with a mottled gas pattern in the distal sigmoid colon . subsequently , the patient underwent laparotomic surgery and removal of the bezoar . we report a rare case of large bowel obstruction due to colonic phytobezoar , which was confirmed intraoperatively .

it is widely accepted that mutation is the main causal factor for diverse tumorigenesis [ 1 , 2 ] . the recent development of next - generation sequencing ( ngs ) technologies has revolutionized the speed and throughput of dna sequencing , which facilitates the discovery of new driver mutations . for example , the arid1a gene is mutated in 83% of gastric cancers with microsatellite instability and the sf3b1 gene is somatically mutated in 9.7% of chronic lymphocytic leukemia patients [ 4 , 5 ] . in colorectal cancers , although the majority of recurrent mutations have been previously known , some novel mutations , such as the mutations in the sox9 and fam123b genes , were also identified by ngs technology , which may have implications for colorectal tumorigenesis . in melanoma , approximately 40% was found to have causal mutations affecting b - raf function . along with the well - known driver mutations , the novel mutations identified by ngs will be useful for predicting the prognosis and molecular characterization of cancers [ 8 , 9 ] . recently , a number of mutation calling tools , such as varscan , genome analysis toolkit , and mutect , have been developed to detect single - nucleotide variants ( snvs ) or indels from ngs data . however , snv calling from a single cancer case is not the final step for defining clinically meaningful mutations . to define the pathogenic snvs in human cancers , a commonly used approach is to identify the phenotype - associated recurrent mutations in the study group . due to the data size burden of ngs output , profiling the mutations associated with a certain phenotype or prognosis in a study group by a researcher who does not have a bioinformatics background or facilities is very difficult . currently , there is no user - friendly tool supporting methods for the detection of recurrent or phenotype - specific mutations . in this study , in order to support the definition of recurrent mutations or phenotype - specific mutations from ngs data of a group of cancers with diverse phenotypes , we aimed to develop a user - friendly tool , named mutation arranger for defining phenotype - related snv ( map ) . map software was designed as a standalone program , compatible in microsoft windows environments with a user - friendly graphic interface , and compiled codes of map can be easily installed . both variant call format and its annotation files , such as annovar output , can be used as input files for map . when a user loads the input file , called sample descriptor file ( gsf ) , map software displays the sample set information and filter options ( fig . 1 ) . once data are loaded , the user can filter the data using the ' analysis options ' tab in map software based on annotation information . by using the sample set and analysis options summary metrics include the mutation status for each sample , total mutation frequency , p - values for differences between phenotype groups based on clinical information , genomic position , reference / observed sequence , and additional information , such as read depth by default . map software also displays more detailed information in summary metrics based on the user - selected ' analysis options . ' in the ' analysis result ' tab , map software provides the frequency- or p - value - based filter function . this functionality supports detection of the recurrent mutations in study subjects and identification of the phenotype - specific mutation that occurs significantly in a certain phenotypic subclass . then , map software represents the user - selected mutations graphically in the ' visualization ' tab . to sort the variants by genomic position , p - value , or frequency , users can use the sort function in the ' analysis result ' tab . details of the program download , installation , and analysis procedures are available in the user manual . the major steps of map are as follows . - generating mutation summary metrics in study subjects- determination of recurrent mutations by frequency- determination of phenotype - specific mutations by association test- graphical illustration of user - selected mutations - generating mutation summary metrics in study subjects - determination of recurrent mutations by frequency - determination of phenotype - specific mutations by association test - graphical illustration of user - selected mutations mutation calling files from genome analysis toolkit can be used directly as input files for ' sample descriptor . ' alternatively , manually prepared standard variant call format is also available . annotation information files , such as annovar outputs , can be used as input files for sample descriptor . gsf is a comma - separated values text file that describes the input files , such as gatk output files and annovar output files , and phenotype information . when users load the input file , map software displays the sample set information and filter options in the ' source ' tab . if any file is not prepared properly , map software informs the user in the ' sample set files ' tab . after data are loaded , the user can filter the data using the ' analysis option ' tab based on annotation information . based on user - selected filters in the ' analysis options ' tab , summary metrics represent additional annotation information , such as gene name , function , known variant , and amino acid change . the additional annotations can be hidden using the snp filter option in the ' analysis option ' tab . these mutation metrics are exported into a comma - separated values text file for further investigation . in the ' analysis result ' tab , map software defines the recurrent mutations in study subjects by frequency . the frequency threshold can be determined arbitrarily by the user in the ' frequency filter ' function . map also defines the phenotype - specific mutations using the mutation metrics and phenotype information . phenotype - specific mutations that occur more frequently in one of two clinical conditions ( e.g. , drug - responder vs. non - responder ) are determined by chi - square test under the user - defined significance level . the graphic user interface is implemented in the software for users to easily handle large - scale data or access the analysis result ( fig . 2b and 2c ) . the recurrent or phenotype - specific mutations can also be demonstrated visually in heat - map style . for example , the left and right of the heat - map represent the sample name and mutational spectrum , respectively . the bottom of the heat - map represents the gene name by color for mutational spectrum . frequency or p - value plots are represented on top of the heat - map ( fig . 2b and 2c ) . map is a user - friendly program with multiple functions that supports the determination of recurrent or phenotype - specific mutations and provides graphic illustration images to the users . its operation environment , microsoft windows , enables more researchers who can not operate linux to define clinically meaningful mutations with ngs data from cancer cohorts .

next - generation sequencing ( ngs ) is widely used to identify the causative mutations underlying diverse human diseases , including cancers , which can be useful for discovering the diagnostic and therapeutic targets . currently , a number of single - nucleotide variant ( snv)-calling algorithms are available ; however , there is no tool for visualizing the recurrent and phenotype - specific mutations for general researchers . in this study , in order to support defining the recurrent mutations or phenotype - specific mutations from ngs data of a group of cancers with diverse phenotypes , we aimed to develop a user - friendly tool , named mutation arranger for defining phenotype - related snv ( map ) . map is a user - friendly program with multiple functions that supports the determination of recurrent or phenotype - specific mutations and provides graphic illustration images to the users . its operation environment , the microsoft windows environment , enables more researchers who can not operate linux to define clinically meaningful mutations with ngs data from cancer cohorts .

primitive neuroectodermal tumor ( pnet ) , a highly aggressive tumor of the kidney is a rare malignancy with only few cases being reported in literature . boys and men are more likely to suffer rpnet ( renal pnet ) , and the sex ratio ( male : female ) is about 3:1 . it usually presents at an advanced stage , with distant metastasis and is associated with a poor prognosis . we report in this paper , a case of primary renal pnet in a young male . a 39-year - old male presented with complaints of hematuria and heaviness in the right flank since 15 days . physical examination revealed a soft lump in the right hypochondrium . ultrasound examination of the abdomen showed an irregular cystic structure in the right kidney at lower pole with few internal echoes . ct scan of the abdomen demonstrated a large significantly enhancing right renal lower polar mass with necrosis with thrombosis in right renal vein . on the basis of these clinico - radiological findings , diagnosis of right side renal cell cancer gross examination revealed a friable , grayish - white , lobulated mass ( 7 5 cm ) with multiple foci of hemorrhage and necrosis . the mass extended from mid to lower pole of the right kidney and infiltrated into the renal pelvis and was limited to the renal capsule the renal vein and ureter were free of the tumor . there was a thrombus inside the renal vein , which was not adherent to intima of renal vein . microscopically , the tumor was composed of monotonous sheets of round cells with scant cytoplasm , hyperchromatic vesicular nuclei , coarse chromatin and few conspicuous nucleoli , transverse by thin fibrous bands infiltrating and entrapping the renal glomeruli and tubules [ figure 1 ] . on immunohistochemistry , the tumor cells were positive for cd99 [ figure 2a ] , fli-1 [ figure 2b ] , nse [ figure 2c ] , and synaptophysin [ figure 2d ] , but negative for desmin , ck , ema , and lca . a diagnosis of pnet was made based on the above findings , and patient was started on vac ( vincristine , adriamycin , and cyclophosphamide ) alternating with ie ( ifosfamide and etoposide)-based adjuvant chemotherapy regimen which he tolerated well . after 6 cycles of chemotherapy , a pet scan showed absence of any metabolically active focal uptake in whole body . chemotherapy with vac ( vincristine , actinomycin d , and cyclophosphamide ) alternating with ie ( ifosfamide and etoposide ) for a year was planned . section showing malignant round cells arranged in pattern sheets infiltrating and entrapping the renal glomeruli and tubules { figure 1a : h&e ; 100 , figure 1b : h&e ; 200 } ( a ) the tumor cells express membranous expression of cd99 { dab ; 200 } ( b ) the tumor cells express nuclear positivity for fli-1{dab ; 200 } ( c ) the tumor cells express cytoplasmic positivity for neuron - specific enolase ( nse ) { dab ; 200 } ( d ) the tumor cells expressing cytoplasmic positivity for synaptophysin { dab ; 200 } the primitive neuroectodermal tumor ( pnet ) , was first recognized by arthur purdy stout in 1918 , and is a member of the family of small round - cell tumors . pnet arising in the kidney was first reported by mor in 1994 . primitive neuroectodermal tumors occurs preferentially in the soft - tissues of the paravertebral region and chest wall , less frequently in extremities , with a slight male predominance . rpnet appears to be an unique clinical entity that behaves more aggressively than pnet arising at other sites . the presenting symptoms and images of rpnet are non - specific and similar to other renal tumors . rpnet show a male predominance with 85% cases being diagnosed during the second to fourth decade . it is an aggressive tumor that tends to recur locally and to metastasize to lymph nodes , lung , liver , bone , and bone marrow , which entail a worse prognosis . the differential diagnosis of rpnet includes other small round cell tumors like neuroblastoma , adult nephroblastoma , malignant lymphoma , rhabdoid tumor , small cell carcinoma and monophasic , poorly differentiated , round cell variant of synovial sarcoma , carcinoid , rhabdomyosarcoma , clear cell sarcoma of the kidney , the small cell variant of osteosarcoma , and desmoplastic small round cell tumor . grossly , rpnet are typically very large tumors with about 65% measuring > 10 cm in diameter . they tend to be grayish in color , encapsulated , and contain focal areas of hemorrhage or necrosis . microscopically , the tumor is composed of sheets of round cells ; individual cells have a round nucleus with a distinct nuclear membrane , fine powdery chromatin , and 1 or 2 small nucleoli . the cytoplasm is ill - defined , scanty , usually pas - positive , and mitosis are variable . perivascular pseudo rosettes and homer - wright rosettes are common . to better address the diagnosis , immunohistochemical markers to be used to confirm diagnosis are cytokeratin ( for nephroblastoma , small cell carcinoma , and synovial sarcoma ) , lca ( for lymphoma ) , nse / chromogranin a ( for neuroblastoma ) , and cd-99 for pnet . cd99 ( the product of mic2 gene ) greatly aids in recognizing the es / pnet family of tumors . many pnet also stain for neural markers , including nse , leu-7 , s-100 protein , synaptophysin , and pgp9.5 . the most frequent translocation in pnet is t(11;22)(q24;q12 ) , detected in approximately 90% of cases , resulting in ews - fli fusion gene . the overall survival in patients who had localized rpent was longer than that in the patients who had rpent with regional nodes or distant metastases . the role of radiotherapy is not clear , but it may be advocated in locally advanced disease and involvement of gerota 's fascia . post - operative chemotherapy for rpent is usually used and can improve the prognosis of rpnet . the most commonly used chemotherapeutics are adriamycin , etoposide , dactinomycin , vincristine , cyclophosphamide , and ifosfamide . a multimodality management is recommended .

primitive neuroectodermal tumor of the kidney is a rare entity . very few cases of primary renal pnet have been reported to date . most literature about rpnet is isolated case reports . we report a case of rpnet in a 39-year - old male with a pre - operative diagnosis of renal cell carcinoma with renal vein thrombosis . the patient underwent radical nephrectomy with thrombolectomy , and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells . tumor cells were positive for cd 99 and fli-1 , hence confirming the diagnosis of primitive neuroectodermal tumor . post - surgery , patient was given vac / ie - based adjuvant chemotherapy . in view of highly aggressive nature of this tumor , prompt diagnosis and imparting effective chemotherapy regimen to the patient is required , and it is important to differentiate pnet from other small round - cell tumors because of different therapeutic approach .

the clinical manifestation is typical and it is characterized by abduction of the affected arm , flexion of the elbow and pronation of the forearm . bilateral luxatio erecta represents an extremely rare condition , and just a few cases were reported in medical literature . because of a high incidence of related complications such as neurovascular damages early recognition and treatment of this type of dislocation is required by physicians . in this article we describe a case of a bilateral luxatio erecta that was successfully treated without long - term complications . a 70-year - old female was accepted in our emergency department because of a bilateral shoulder pain that onsets after a sudden fall happened in her house . the anamnestic research showed that the patient tumbled downstairs ; while clinging to both lateral banisters , she slipped down banging her face and chest to the ground . at physical examination both shoulders were abducted and elevated approximately 90100 from the horizontal plane , with flexed elbows and with hands below the head . the patient was unable to adduct and lower the arms , and there was immediate pain when attempting passive movements . no vascular deficit was found , and the patient complained of slight paresthesias on both arms , with superficial and deep sensation deficit on both arms . an x - ray examination was performed showing a bilateral inferior dislocation of humeral heads in relation with the glenoid fossa ; no fracture was associated ( fig . 1 ) . 1bilateral luxatio erecta bilateral luxatio erecta close reduction under sedation was achieved without difficulty by pushing the humeral head superiorly with an axial traction - countertraction maneuver on both shoulders . x - ray control revealed a correct anatomic reduction of dislocations and after the reduction no vascular damage were found while paresthesias were still present on the left arm ( fig . 2 ) . both arms were immobilized with bilateral slings in total adduction , intrarotation with the supinated arms . 2post - reduction x - ray the progressive mobilization of the shoulders started after 2 weeks with passive movements and pendulum codman s exercises . after 3 weeks the patient gave up complaining paresthesias on the left arm and active movements were allowed . after 8 weeks she recovered the complete function of both shoulders and after a one - year follow - up treatment the patient showed a complete range of motion of both arms . inferior dislocations are rare ( 0.5% of all shoulder dislocations ) and bilateral inferior shoulder dislocations are exceptional : only 10 cases have been described so far in the literature . according to the literature , luxatio erecta is due to an indirect mechanism such as a violent abduction force on abducted limb resulting in impingement of the proximal shaft of the humerus against acromion . direct axial loading from superior direction is another different mechanism , though less common . in our case the first mechanism has to be taken into consideration ; in fact , while the patient was sliding in a sitting position downstairs , her hands were holding onto both banisters and the arms were abduced . when she reached the ground floor the hands were blocked around the end of the banisters and her head and body were thrown forward by gravity . the clinical presentation of an inferior shoulder dislocation is typical : the extremities are held over the head or abduced in a fixed position with the elbow flexed . any passive movement is possible , and the humeral head is palpable on the chest wall . x - ray shows the humeral head located below the rim of the glenoid and the humeral shaft is parallel to the scapular spine . in order to reduce inferior glenohumeral dislocation , it is recommended a traction counter - traction maneuver under sedation followed by immobilization in a desault bandage for at least 2 weeks . there is a high incidence of associate tears of the rotator cuff ( 12% of patients ) and a slap lesion has been described by schai and hintermann after a post - reduction arthroscopic assessment view . fracture of the acromion , clavicle , inferior glenoid fossa and greater tuberosity represent a complication that should need a surgical treatment . neurovascular damages are also commonly associated with inferior shoulder dislocation [ 5 , 7 ] ; garcia et al . described a case of bilateral luxatio erecta complicated by an axillary vein thrombosis . in general , neurologic injuries ( 60% of cases ) of brachial plexus , axillar , radial and ulnar nerves are more common than vascular injuries but they tend to resolve after reduction with an excellent prognosis , suggesting the neuropraxia as the main mechanism of the injury [ 5 , 9 , 10 ] .

luxatio erecta is an uncommon form of glenohumeral dislocation . cases of bilateral inferior shoulder dislocation ( luxatio erecta ) are very rare , and only ten cases have been described in literature . we describe a case of a woman with bilateral luxatio erecta of the shoulders treated with close reduction , immobilization and rehabilitation . the patient had fallen downstairs while clinging to both lateral banisters .

spider nevus ( also known as spider angioma or vascular spider ) is a common benign vascular anomaly that may appear as solitary or multiple lesions . spider nevi are represented by telangiectases that consist of a large arteriole from which radiate numerous small vessels that resemble spider 's legs . they are found in the distribution of the superior vena cava , that is , on the face , neck , arms , and upper trunk . spider nevi are usually < 2 cm in diameter and rarely grow to assume large size more than 2 cm . we hereby report a case of very large spider nevus in a patient with hepatitis c virus ( hcv ) related liver cirrhosis . a 48-year - old - female presented with a history of abdominal distension and jaundice of 3-month duration . physical examination revealed pallor , icterus , pedal edema , hepatospleenomegaly and tense ascites . she had multiple classical spider nevi over the chest . also , she had a large spider nevi situated on the forehead , measuring 8 cm in largest dimension with palpable dilated vessels and venous hum [ figure 1 ] . deep compression on the central area of the lesion produced complete disappearance with blanching and when released refilling of vessels occurred . laboratory tests revealed a hemoglobin level of 9.8 g / dl , a white - cell count of 4240/mm , and a platelet count of 77,000/mm . anti - hcv antibody for hcv was positive with hcv rna of 1.6 10 iu / ml . an ultrasound abdomen was suggestive of liver cirrhosis with portal hypertension ( portal vein diameter of 14 mm ) , ascites and splenomegaly . thus , a diagnosis of cirrhosis liver with portal hypertension was made ( ctp-9 , class b , and meld 11 ) . she was put on diuretics for the control of ascites but developed hepatic encephalopathy and renal dysfunction . diuretics were stopped and in view of refractory tense ascites , she was taken up for a transjugular intrahepatic portosystemic shunt . however , during the procedure she had massive intrahepatic bleed and in spite of intensive resuscitative measures she died 48 hours later . spider nevi can be seen in pregnancy , in patients with thyrotoxicosis , rheumatoid arthritis , oral contraceptive use and most commonly , liver cirrhosis . these frequently appear in alcoholic cirrhotic or when liver function deteriorates in patients of the chronic liver disease . found elevated levels of vascular endothelial growth factor and basic fibroblast growth factor in patients with liver cirrhosis , especially in those with spider nevi . in patients with nonalcoholic cirrhosis , the levels of substance p are elevated which may play an important role in the pathogenesis of spider nevi by causing vasodilatation . most remain small in size and rarely do they assume the size as seen in our patient . the present case is unique because of its very large size , rare presentation over the forehead and palpable mass like vascular lesion in a patient with nonalcoholic cirrhosis . electrodesiccation and laser treatments under local anesthesia are effective therapeutic procedures for facial spider nevi . spider nevi , however , usually do not require any treatment in cirrhotic patients due to a high risk of bleeding from these vascular lesions .

spider nevi are benign vascular lesions mostly seen in patients with decompensated liver cirrhosis . mostly , these are seen in the superior vena cava distribution and are small with pinhead size central vessel . giant spider nevus is rarely seen and hence this report .

a 22 years old female patient was admitted to our outpatient clinic with a complaint of dyspnea for the previous three years . a right bundle branch block was detected on her electrocardiogram . in the transthorasic echocardiography ( tte ) , a 22 mm secundum type atrial septal defect ( asd ) , right heart dilatation and pulmonary arterial hypertension ( pap 45 mmhg ) were detected . transesophageal echocardiography ( tee ) showed that the defect was 25 mm , the superior rim was 16 mm and the aortic rim was 8 mm in size . the defect was closed through a right femoral route with a 30 mm asd occluder after balloon sizing with tte . in the cineangiography and tte early after the procedure , the device was observed to be in the correct position ( fig . the tte revealed that the device was not in the defect and it had embolized to the pulmonary artery . the patient was referred to the cardiovascular surgery unit and underwent surgery promptly . under general anaesthesia the pulmonary artery was incised along its vertical axis and the occluder device was removed ( fig . the right atrium was opened and the defect was then closed with a pericardial patch . secundum asd is the most common type , which accounts for 50% to 70% of all asds and located in the fossa ovalis . sinus venosus type asd is located at the upper portion of the interatrial septum where the vena cava superior opens . a shunt between atria leads to chronic right ventricular volume overload , which in turn causes lower filling rates of left ventricle . surgical or percutaneous closure in asymptomatic patients is indicated in the case of right ventricular volume overload and if the pulmonary to systemic flow ratio increases beyond 1.5 . in adult patients , the defect should be repaired as soon as possible after it is detected because patients who undergo repair after 25 years of age have a shorter life span than a control population . the most important factor for success in percutaneous closure is the correct patient selection . in patients with secundum asd , percutaneous closure can be performed safely with careful evaluation of anatomical factors like defect size and sufficiency of the rims . in a previous report , it was shown that atrial defects up to 43 mm in diameter can be closed percutaneously . a sufficient anterior rim is not necessary to bring the device into a stable position . conversely , deploying the device in patients with deficient cranial rims can be difficult and may lead to an unstable position of the device and increased risk of device embolization . the most common type of complications in percutaneous closure is device embolization , with a rate of 0.5% to 1 % . in a study with 450 patients , seven cases of device embolization into pulmonary artery in the first 12 hours one hour after intervention we detected device embolization into the pulmonary artery after performing echocardiography since the patient had dyspnea . after that , emergency surgery was performed within one hour with a median sternotomy and under cardiopulmonary bypass . , snares can be used to take out the device , but most of the time , surgical extraction of the device and asd patch closure is needed . percutaneous asd closure is a widely used and has been performed successfully throughout the world in recent years . however , it should be kept in mind that improper patient and device selection may result in deadly complications like device embolization , cardiac perforation and tamponade .

an atrial septal defect is the most common type of congenital heart disease among adults . surgical repair or percutaneous closure of the defect is the treatment options . even though percutaneous closure seems to be less risky than surgical repair , it may result in fatal complications like device embolism , cardiac perforation and tamponade . herein we report a case of the embolism of a device into the pulmonary artery after one hour of percutaneous closure in which the embolized device was surgically removed and the defect was closed with a pericardial patch .

a number of skin disorders have been described in patients with acquired immunodeficiency syndrome ( aids ) . among them , secondary infections are common , but their incidence has decreased considerably following the introduction of combined therapies targeted to the human immunodeficiency virus ( hiv ) . some clinical presentations may be puzzling , particularly in secondary syphilis exhibiting lesions showing a marked tendency to polymorphism . a 29-year - old woman with a three - year history of aids presented with polymorphic papules on the face and abdomen . the skin lesions were asymptomatic but the patient complained of discrete malaise , stiff neck , myalgia headache , and mild fever . a biopsy specimen was taken from a papule on the abdomen . a series of 5-m thick sections were cut from the formalin - fixed paraffin - embedded biopsy . an immunohistochemical assessment was performed using a rabbit polyclonal antibody directed to treponema pallidum ( 1:200 biocare medical , walnut creek , ca , usa ) . the envision ( dakopatts , glostrup , denmark ) polymer - based revelation system and fast red ( dakopatts ) staining were used . negative immunohistochemical controls were performed by omitting or substituting the primary and the secondary antibodies in the laboratory procedure . the dermoepidermal junction contained a band - like infiltrate composed mostly of lymphocytes , histiocytes , and plasma cells . a deeper cell infiltrate of similar composition extended along the microvasculature , hair follicles , and sweat glands . the typical spiral , corkscrew , and threadlike spirochetes were highlighted by the red chromogen , and the contrast with the clear background was striking ( figure 1a , b , c ) . their presence inside the lichenoid infiltrate was associated with a dense superficial and deep perivascular cuff of spirochetes . the latter slender spirochetes were clustered in the dermal stroma ( figure 1a ) and in rims confined to the perivascular areas ( figure 1b , c ) . in addition , some t. pallidum were evident in the cytoplasm of cells , particularly endothelial cells ( figure 1c ) . dermal homing of t. pallidum on immunohistochemistry : ( a ) multiple interstitial clumps of spirochetes ( 200 ) ; ( b ) vascular trapping of spirochetes ( 400 ) ; ( c ) prominent accumulation of spirochetes in the microvasculature wall ( 400 ) . early syphilis . dermal homing of t. pallidum on immunohistochemistry : ( a ) multiple interstitial clumps of spirochetes ( 200 ) ; ( b ) vascular trapping of spirochetes ( 400 ) ; ( c ) prominent accumulation of spirochetes in the microvasculature wall ( 400 ) . in the present case , skin immunohistochemistry shed some light on the diagnosis of syphilis in an aids patient and showed the dermal homing and the microvascular tropism of t. pallidum . it is acknowledged that during a five - year period after inoculation , t. pallidum spreads to every organ . a long time later , it commonly re - emerges as a chronic and deadly illness . at any stage in its evolution , syphilis may mimic a number of other unrelated diseases . during the past decade , a sizable proportion of the population with syphilis corresponded to gay men coinfected with hiv . when the clinical diagnosis of syphilis is not established , a skin biopsy sometimes is submitted to the dermatopathologist without any relevant information . at the conventional histological examination , the diagnostic clues for syphilis are not always obvious because the disease presentation depends on both the host immunological response to the infection and the diverse angioinvasive propensity of the t. pallidum strains indeed , the histochemical silver stain may be difficult to interpret owing to heavy background staining . immunohistochemistry using an antibody directed to t. pallidum was reported to improve the histological diagnostic accuracy of syphilis . the present finding was assumed to illustrate the migration of t. pallidum toward the microvasculature during early syphilis . in summary , t. pallidum were abundant and heavily clustered in some specific portions of the skin . the peculiar homing of t. pallidum in the skin appears quite specific as it has not been reported for any other infectious microorganism .

during the past decade or so , the incidence of syphilis has increased in most parts of the world . in some urban regions , a coinfection with human immunodeficiency virus is disclosed in nearly 50% of the cases . owing to the polymorphism of the lesions , the clinical diagnosis may be puzzling . the homing patterns and migration paths of treponema pallidum in the skin during early syphilis represent the preliminary steps preceding dissemination to other organs . immunohistochemistry directed to t. pallidum is a convenient means for reaching the diagnosis and for exploring the dissemination process . the present case illustrates the dermal clustering and the vascular spread of t. pallidum in a woman with acquired immunodeficiency syndrome .

the epstein barr virus ( ebv ) infects b cells . in most cases , ebv - positive patients develop a severe form of infectious mononucleosis which typically manifests with fever , tonsillopharyngitis , lymphadenopathy and hepatosplenomegaly . deficiencies may be characterized by abnormal activity of lymphocyte function leading to hemophagocytosis and multi - organ failure . although ebv - associated hlh carries a high mortality rate , quick diagnosis and appropriate treatment can subside the disease . we describe here a patient who developed hepatosplenomegaly and pancytopenia at 2 years of age but achieved automatic recovery . , there was a clinical suspicion of ebv - induced hlh and the patient was given the option of treatment with rituximab . she received antibiotic treatment due to fever and chills . during the hospitalization period , the patient recovered and developed hemophagocytosis with pancytopenia as well as sores on the lip . the patient underwent bma and nothing abnormal was found . in spite of treatment , the disease did not subside and she developed fever , abdominal distention and edema in her lower extremities . physical examination showed that the patient had decreased deep - tendon reflexes ( dtr ) and was unable to walk . clindamycin , ceftazidime , dexamethasone , ivig ( intravenous immunoglobulin ) and g - csf were administered . since the patient did not achieve full recovery , she was transferred to our center . based on clinical symptoms and lab tests ( table 1 ) , bma was performed once again and the result raised suspicion of ebv - induced secondary hlh . rituximab was injected once and the patient developed a blood ebv load of zero within one week . in order to control abnormal stimulation of the immune system , the patient was investigated for mutations in il-2-inducible t cell kinase ( itk ) and negative test results were received . ( the patient was negative for mutations in il-2-inducible t cell kinase ( itk ) ) . she is now considered to be an acceptable candidate for transplantation from an hla matched sibling donor . hlh can be divided into two subgroups : primary or familial hlh and secondary hlh . the primary form is an inflammatory disease which is similar to secondary one on the basis of symptoms . primary hlh usually arises in young children less than one year old ( 70% cases ) ; however , in rare cases it can also occur in adults ( perforin gene mutations have been identified in patients with primary hlh ) . for example , type 2 is caused by mutations in genes 21 - 22 and type 5 is due to mutations in the munc18 - 20 that may be accompanied by hypogammaglobulinemia . hypogammaglobulinemia was not confirmed in the patient because it was not possible to check mutation ( the related tests were sent to germany to examine the type 5 disease ) . ebv may also be accompanied by hypogammaglobulinemia for a certain period secondary hlh is associated with immunologic stimulation caused by malignancies and bacterial or congenital infections . the most common causes of secondary hlh are viral infections by ebv , cmv , prob19 and hiv . however , it may also be seen in patients with immune system defects . , 4 secondary hlh is a known complication of ebv infection , particularly in patients with x - linked lymphoproliferative disease . the incidence of x - linked lymphoproliferative syndrome is 1 in 1,000,000 male infants . diagnostic criteria for hlh disease diagnosis of hlh requires the presence of all 5 major criteria . if the patient meets only 4criteria but the clinical suspicion for hlh is high , one should initiate treatment , because delays may be fatal . alternative criteria 1 or a combination of 2 and 3 may substitute for 1 major criterion . adapted from henter ji , elinder g , ost a.semin oncol . 1991 ; 18:29 - 33 . it means that ebv - infected b cells stimulate cytotoxic t lymphocytes leading to hypercytokinemia and stimulation of histolytic cells . more recently , it has been found that chronic stimulation by ebv may cause chronic hlh in the patient . on the other hand , ebv causes stimulation , generation and uncontrolled secretion of t - and nk- cells , as well as generation of il2 , infa and il6 . there is another mechanism by which ebv stimulates membrane protein ( lmp-1 ) in cells . similar to xlp , lmp-1 may cause acquired immune deficiency , leading to hlh accordingly . treatment follows two goals : first , to suppress immune response using ivig , steroid and hlh protocol . second , to inhibit undesirable influence of immune response and cytokine - stimulated cell activation using immunochemotherapy . in cases with neurological symptoms the level of viral dna which is measured by quantitative pcr may be useful to demonstrate treatment response predicting mortality . the dna level reached zero in our patient . using ivig prior to start of protocol 2004 it has also been recommended in the treatment of ebv - induced secondary hlh monthly . decrease of b cells , which have been contaminated by ebv , actually causes decrease of viruses , as it may be useful for our patient as well . finally , treatment - refractory patients , transplant candidates , patients with familial type of hlh , or recurrent disease undergo chemotherapy for 8 weeks . patients who respond favorably to treatment do not undergo transplantation but those who experience relapse and exhibit neurological symptoms are considered for transplantation . severe infection of ebv is not associated with life - threatening condition , but our case study needs urgent treatment . in patients with long - term ebv infection , pancytopenia , coagulopathy and hepatosplenomegaly , bma will be performed and the serum ferritin level will be measured as well . for patients with secondary hlh , rituximab can be used when hlh is associated with ebv to inhibit the immune response . thus , the main aim of treatment is to reduce the rate of mortality in patients with hlh .

hemophagocytic lymphohistiocytosis ( hlh ) is one of the complications of epstein - barr virus ( ebv ) infection . although the patients who have developed hlh following ebv have normal immune system , there are a few patients with ebv - induced immune deficiency who develop hlh as well . here , we describe the case of a 10-year - old girl with neurological complications caused by ebv - induced hlh . the patient received rituximab , leading to weakening inflammation associated with ebv infection and suppression of disease through quick treatment .

macroscopically , renal cell carcinomas ( rccs ) generally present as either solid or cystic mass . among cystic rccs , multilocular cystic rcc represents a rare entity that was recognized in 1982 , with a reported incidence of 1 - 4% of all rccs . renal leiomyomas are benign tumors of the kidney originating from smooth muscle cells of the renal capsule , pelvis , calices , or blood vessels . although small renal leiomyomas can be found in about 5% of autopsy specimens , clinical incidence of these lesions is much lower . in this report , we discuss the first case of simultaneous occurrence of a multilocular cystic rcc and a leiomyoma in the same kidney . a 38-year - old man was referred for treatment of a right renal cystic mass found incidentally . physical examination on admission revealed no palpable mass , and laboratory data were negative . computed tomography of the abdomen showed a about 5.4 4.6 cm poorly enhancing cystic mass with enhancing septa in the polar area of the right kidney ( fig . since multilocular cystic rcc could not be excluded , the patient underwent right laparoscopic radical nephrectomy . macroscopically , in the mid pole , a well - circumscribed mass showing a multicystic feature with thin fibrous septa and without expansile tumor nodules was noted . rccs show cystic changes on imaging studies in 4 - 15% of cases [ 4 , 5 ] . since it is often difficult to differentiate cystic rccs from benign cystic lesions , a definite diagnosis can in most cases only be established by histopathologic examination . these include : ( 1 ) intrinsic multiloculated growth ; ( 2 ) intrinsic unilocular growth ; ( 3 ) cystic necrosis , and ( 4 ) origin from the epithelial lining in a pre - existing cyst . because multilocular cystic rcc are more likely to be discovered incidentally , this feature may contribute to their excellent prognosis compared to that of conventional rccs . since the majority of rccs originate from the proximal tubules , the characteristics and/or environment of the proximal tubules are considered to play an important role in the pathogenesis of rcc . recently , imura et al . carried out a detailed immunohistochemical analysis of multilocular cystic rcc cases . they found that in a high proportion of cases , multilocular cystic rcc reacted strongly with the distal nephron markers , but none reacted preferentially with proximal nephron markers . these results illustrate that multilocular cystic rcc originates from the distal nephron , although the precise pathogenesis of cystic formation in multilocular cystic rcc has not been elucidated clearly . renal leiomyomas are benign tumors arising from the mesenchymal ( or connective ) tissue of the kidney . tumors may be subcapsular ( 53% ) , capsular ( 37% ) , or located in the renal pelvis ( 10% ) . a variety of structural patterns have been described at imaging : solid , cystic , and both cystic and solid [ 9 , 10 ] . in 1990 , steiner et al . the first group comprises small cortical or subcortical neoplasms that are usually asymptomatic , less than 2 cm in size , and are often detected incidentally during autopsy or surgery . the second group consists of larger neoplasms that arise from the renal capsule or blood vessels and may be symptomatic . unfortunately , it is almost impossible to clinically distinguish renal leiomyomas from their malignant counterparts . because of their small size ( usually less than 2 cm ) , these smooth muscle - containing tumors are commonly diagnosed during autopsy , and clinical manifestations become apparent only when they grow larger , hemorrhage , or undergo cystic or sarcomatous degeneration . since the finding of primary synchronous renal neoplasms is very uncommon , especially when they have a different histogenesis , up to now , only a few primary , synchronous rccs with different histotypes have been reported . the coexistence of multilocular cystic rcc and leiomyoma has not been documented . this present case is the first report of synchronous renal neoplasms with different histogenesis ( multilocular cystic rcc and leiomyoma ) in a patient without any symptoms . the significance of the relationship between multilocular cystic rccs and leiomyomas is not well understood and needs further exploration .

we present an unusual case of concurrent occurrence of a multilocular cystic renal cell carcinoma and a leiomyoma in the same kidney of a patient with no evident clinical symptoms . a 38-year - old man was found incidentally to have a cystic right renal mass on computed tomography . laparoscopic radical nephrectomy was performed under a preoperative diagnosis of cystic renal cell carcinoma . histology revealed a multilocular cystic renal cell carcinoma and a leiomyoma . this is the first report of this kind of presentation .

pregnancy course was regular , except for maternal consumption of mebendazol at 5 weeks ' gestation to treat a pinworm infection . maternal history was negative for consumption of any known teratogenic class drug during the whole pregnancy . clinical examination at birth was normal , except for complete congenital anonychia of left middle finger ( fig.1 ) . x - ray was performed , showing an absence of the left middle finger distal phalanx ( fig.2).the rest of the nails on the fingers and toes were normal . parents were not consanguineous and have normal fingernails and toenails . the baby was discharged on the third day of life , and was found healthy and thriving at three - month follow - up visit . final diagnosis was that of a sporadic co. x - ray showing absence of left middle finger distal phalanx . congenital onychodystrophy ( co ) , also called as iso kikuchi syndrome , was first described by iso in 1969 1 and later by kikuchi in 1974 2 as a clinical syndrome involving dysplasia / absence of fingernails with underlying bone abnormalities . since these first observations , clinical criteria have been expanded to include a number of additional associated conditions derived from small series , case reports , and retrospective reviews over the next 30 years . co clinical criteria are the following : unilateral or bilateral hypoplasia of the index fingernails and/or other fingers including toenails 3 ( up to total anonychia of hands and feet ) ; radiographic abnormalities of the distal bony phalanx of the affected fingers ; congenital occurrence , which can be both sporadic or hereditary 4,5 . unilateral or bilateral hypoplasia of the index fingernails and/or other fingers including toenails 3 ( up to total anonychia of hands and feet ) ; radiographic abnormalities of the distal bony phalanx of the affected fingers ; congenital occurrence , which can be both sporadic or hereditary 4,5 . genetic loci responsible for the condition are still under investigation : linkage to the known keratin gene clusters on 12q12 and 17q21 has been excluded by krebsova et al . in 2000 ; 7 a putative isolated congenital nail dysplasia locus , designated ndic , has been identified on 17p13 , although the identified region harbors no genes known to be involved in skin or nail abnormalities 7 . several acquired isolated nail disorders which may represent differential diagnoses of co are presented in table1 8 . differently from all these conditions , co presents as a congenital hypoplasia , dysplasia , or absence of one or more fingernails , and is typically accompanied by underlying phalanx bone disease . acquired isolated nail disorders 8 several heterogeneous multisystem pathologies may also come with ungueal abnormalities ; these are summarized in table2 813 . 813 triangular nail lunula fingernails and toenails hypoplasia 8 absent or hypoplastic patellae elbow dysplasia , often involving posterior subluxation of the radial head iliac horns dysplasia arrested or reduced nail growth thickened and over - curved nails with absence of cuticle differently from the above - mentioned conditions , classic co does not come with involvement of systems other than nails and relative phalanges . kikuchi originally suggested fetal grip as a causative mechanism of nail and phalanx ischemia , leading to dysplasia or even complete resorption of such structures 14 . this theory , however , is not consistent with the recent acquisitions on developmental biology stating that limbs and bones develop early in fetal life , prior to fetus ' ability to exert a significant grip . more recently , several pathogenetic mechanisms have been proposed for this condition : selective abnormal fetal vascular supply from palmar digital artery causing in utero ischemic injury : the ischemic damage would mainly be seen on the radial side of the affected finger due to the smaller caliber of the artery on that side 15 , which should be more protected than the ulnar one;16 in utero dysplastic change in the crescent - shaped cap of the distal phalanx , a theory that would account for the frequent y shape of the dysplastic distal phalanx , when present;17 genetic mutations causing impairment of the wnt signaling pathway , an evolutionarily conserved signal transduction pathway that plays a pivotal role in embryonic development , growth regulation of multiple tissues , and cancer development;18 fetal exposure to teratogens , particularly antiepilectic drugs : phenytoin 19 , valproate , and carbamazepine20 selective abnormal fetal vascular supply from palmar digital artery causing in utero ischemic injury : the ischemic damage would mainly be seen on the radial side of the affected finger due to the smaller caliber of the artery on that side 15 , which should be more protected than the ulnar one;16 in utero dysplastic change in the crescent - shaped cap of the distal phalanx , a theory that would account for the frequent y shape of the dysplastic distal phalanx , when present;17 genetic mutations causing impairment of the wnt signaling pathway , an evolutionarily conserved signal transduction pathway that plays a pivotal role in embryonic development , growth regulation of multiple tissues , and cancer development;18 fetal exposure to teratogens , particularly antiepilectic drugs : phenytoin 19 , valproate , and carbamazepine20 a literature research showed no proven association between the use of mebendazole during pregnancy and co. conversely , helminth infection during pregnancy is associated with poor cognitive and gross motor outcomes in infants , so that measures to prevent helminth infection during pregnancy should be reinforced;21 anthelminthic therapy is actually recommended in infected pregnant women , and it has been associated to a decreased rate of maternal anemia and low birth weight 22 . nevertheless , clinical follow - up is needed in order to exclude other conditions associated with multisystem pathology ; a complete family history may also be warranted to determine sporadic or hereditary transmission of such a condition .

key clinical messageclassic co ( also called iso kikuchi syndrome ) represents a benign , isolated condition associated with normal patient outcome . nevertheless , clinical follow - up and/or further clinically - based tests are needed to exclude other nail diseases associated with multisystem pathology ; complete family history is also important to determine sporadic or hereditary transmission of such condition .

studies have shown the incidence of injury from squash to range from 35.5 to 80.9 per 100,000 players . trauma to the head ( including the eyes ) is the most common cause of squas - related hospital presentation , comprising 48.7% of all emergency department presentations . craniofacial and spinal fractures typically result from high - energy blunt force to the skeleton , as seen in motor vehicle accidents . this is the first case reported of a patient sustaining such extensive head , neck and spinal injuries from a squash - related injury . a 55-year - old man was brought in by ambulance after colliding with the corner of the wall and floor of a squash court , without the use of protective eye - glasses or mouth - guard . he was chasing a ball toward the corner of the court when he fell and hit the junction of the wall and floor with his head . on examination in the emergency department , the patient had a glasgow coma scale ( gcs ) of 14 , with confusion to time and place . initially , the patient reported diplopia at primary position but examination by the surgical team elicited normal eye movements with nil complaint of diplopia . there was significant boggyness to palpation of the forehead with a palpable defect of the frontal bone . the cervical spine was slightly tender over / c1 - 2 and c5 - 6 . upper limb examination was limited by pain maximal over the distal radii but bilateral arm weakness was noted with nil sensory dysfunction . primary and secondary surveys did not elicit any further findings . a non - contrast computed tomography ( ct ) imaging of the facial bones [ figure 1 ] showed a le fort type ii fracture , with comminuted fractures of the roofs of the orbit bilaterally and a depressed fracture of the left orbital floor . there was also fracture of the right maxilla at the junction of the maxilla and zygomatic arch , extending towards but not involve the right orbital floor . on the left , there was fracture of the anterior part of the left zygomatic arch which extended into the anterolateral wall of the left maxillary sinus . a large , depressed fracture of the frontal bone was also evident , extending superiorly from the superior lateral wall of the right orbit and extending across the frontal bone and inferiorly toward the mid - portion of the roof of the left orbit . the fracture involved the inner and outer tables of the frontal bone and was depressed by at least 3.5 mm . there was also a comminuted fracture of the nasal bone , which was not significantly displaced . anterior and anterolateral views of three - dimensional ct reconstructions demonstrating lefort type ii , frontal , orbital and zygomatic fractures ct brain showed tiny subarachnoid or intraparenchymal haemorrhages but there was no evidence of extensive intra- or extra - axial haemorrhage . imaging of the cervical spine [ figure 2 ] showed an undisplaced fracture of the posterior arch of c1 on the right side and a type 3 dens fracture with posterior displacement of 4 mm but no contact with the spinal cord . anterolateral view 3 dimensional ct reconstruction demonstrating type 3 dens fracture with posterior displacement and fracture of c7 spinous process subsequent ct of the thoracic spine showed a comminuted t6 vertebral body fracture with loss of vertebral height and extension into the left costovertebral joint . there was a small bony fragment projecting posteriorly into the spinal canal and making contact with the cord , but there was nil evidence of compression . associated with this finding were two to three hyperdense foci within the spinal cord likely to represent small contusions . the above findings were confirmed on magnetic resonance imaging ( mri ) of the spine , which also demonstrated a small cortical buckle at the t6 level without significant cord impingement . the patient underwent craniotomy , obliteration of the frontal sinus and open reduction internal fixation ( orif ) of the frontal bone . orif of the maxilla bilaterally , orif of the infra - orbital rim and supra - orbital rim bilaterally and inter - maxillary screw fixation was also performed . the patient was admitted to the neurosurgical intensive care unit post - operatively and underwent cervical fusion with odontoid screw fixation of the c2 fracture one week after surgical correction of the head injuries . post - operative ct scans demonstrated complete bony union in the c2 fracture site [ figure 3 ] and uncomplicated fixation of the facial bone fractures [ figure 4 ] . post - operative lateral x - ray of the cervical spine in extension demonstrating union of dens fracture with overall alignment in - tact post - operative coronal ct demonstrating surgical correction of multiple craniofacial fracture sites our patient suffered injuries to multiple sites in the cranium , facial bones and the vertebrae that have previously not reported in the literature . the thinnest and weakest segment of the orbit is the floor , which is the most common site of fracture . our patient suffered a le fort type ii fracture , which is pyramid shaped and passes through the posterior alveolar ridge , lateral walls of maxillary sinuses , inferior orbital rim and nasal bones . he also suffered a depressed frontal bone fracture involving the inner and outer tables , an extremely rare complication of sport seen in only 1.3% of sports related craniofacial fractures . spinal injury is associated with sports in approximately 8.7% of cases , usually in relation to contact sports such as wrestling . our patient had a fracture of the posterior arch of c1 on the right side and a type 3 dens fracture , which required cervical fusion . there was also fracture of the c7 cervical process , from forceful flexion of the cervical spine . we report the first case of extensive head and spinal injury resulting from collision with the wall of a squash court . the injuries ranged from the frontal bone to the thoracic vertebra and are usually seen in high velocity trauma .

squash is a popular racquet sport not usually associated with severe head or spinal injury . the incidence of squash - related injury ranges from 35.5 to 80.9 per 100,000 players , with the most common sites being the lower - limbs and eyes . we present a case of extensive traumatic craniomaxillofacial and vertebral injury resulting from collision on a squash court , without use of protective gear . the patient sustained fractures of the frontal bone , orbits , maxillae , zygomas , the first and second cervical vertebrae and the spinous process of the seventh cervical vertrebra . this is the first case of squash - related injury with such extensive craniofacial and vertebral involvement . this unique case required multiple surgical procedures as well as an extensive admission to the intensive care unit and highlights the risk of significant craniomaxillofacial trauma in sports not usually associated with such injuries .

port - wine stains ( pwss ) are congenital vascular malformations comprised of ectatic dermal capillaries and affecting 0.3 - 1% of newborn infants . acquired pwss are an uncommon entity developing later in life , but morphologically and histologically similar to the congenital lesions . we hereby report a case of 41-year - old male patient who acquired pws about a year ago . a 41-year - old male presented to the outpatient department of dermatology of our tertiary care hospital with complaint of asymptomatic reddish patches involving the lower part of right leg for 10 months . the patch started from near the ankle and gradually spread to involve whole of the medial aspect of right leg over a period of 10 months . we could not elicit any history of antecedent mechanical or thermal trauma , drug intake , topical application or excessive ultraviolet exposure . dermatological examination revealed a diffuse partially blanchable erythema extending from the right ankle and involving chiefly the medial aspect of right leg up to the knee [ figure 1 ] . there were also a few discrete irregularly shaped partially blanchable reddish - purple macules present on the anterior aspect of right leg [ figure 2 ] . a diffuse partially blanchable erythema extending from the right ankle and involving the medial aspect of right leg up to the knee discrete irregularly shaped partially blanchable reddish - purple macules present on the anterior aspect of right leg on the basis of the clinical examination , a provisional diagnosis of acquired pws was made and the patient investigated . the histopathological examination revealed multiple dilated , variably sized capillaries lined by a single layer of endothelial cells throughout the dermis showing lobular arrangement at places [ figure 3 ] . color doppler of the limb did not reveal any abnormality of the underlying arteries and veins . ( a ) multiple dilated variably sized capillaries in the dermis ( h and e , 40 , arrows ) . ( b ) high power showing groups of capillaries lined by a single layer of endothelial cells ( h and e , 250 , arrow ) laser therapy was discussed with the patient , but he declined treatment for the lesion . pwss are congenital vascular malformations usually presenting at birth in the form of pink - red to purple macules , which become darker , raised , and nodular as the person ages . the most accepted hypothesis is a defective embryological maturation of the sympathetic fibers , resulting in a loss of normal sympathetic control of the cutaneous vessels which leads to ectasia . various factors have been proposed for the occurrence of acquired pwss . of these , trauma has been found to be the most important causative factor in majority of the cases . trauma - induced pws was first described by fegeler in 1949 and hence called fegeler syndrome . in a review of 59 patients with acquired pws , trauma was found to be a causative factor in 17 ( 29% ) cases . it has been proposed that injury may result in loss of a previously effective sympathetic regulation of the cutaneous blood flow , leading to development of the lesions . reports of acquired port - wine stain few cases of acquired pws have also been proposed secondary to oral medications like isotretinoin , oral contraceptive pills , simvastatin , and metformin . isotretinoin causes skin fragility and frictional trauma , while simvastain and metformin have been shown to promote angiogenesis by upregulation of vascular endothelial growth factor . isolated cases of acquired pws following frostbite injury , obstruction of the peritoneovenous shunt , herpes zoster infection , cerebral arteriovenous malformation , spinal root compression , and solid brain tumor have also been reported . no definite cause was found for the development of lesion in our patient . in such cases , a likely possibility of having a latent vascular anomaly which manifests clinically after unnoticed trauma an unusual finding in our patient was the presence of a lobular arrangement of dilated vessels in the dermis which has not been observed in previous cases of congenital or acquired pws . interestingly , there are multiple reports of development of pyogenic granulomas ( pg ) within pws which also shows a lobular pattern of dilated vessels . it has been proposed that the pg probably results from an underlying arteriovenous anastamosis frequently associated with the pws . though , there was no clinically appreciable pyogenic granuloma like lesion , the distinct histological features seen in our patient might be attributed to a similar pathogenetic mechanism . as regards therapy , it includes treatment with krypton , argon , argon - pumped , tunable dye , neodymium : yttrium - aluminum garnet , copper vapor , and pulsed dye lasers . acquired pws tends to have a quicker and better response to pulsed dye therapy than congenital pws . the case report demonstrates a rare occurrence of a vascular malformation in an adult patient . although no definite cause could be appreciated for the development of lesion in our patient , further reports and future insights into the pathogenesis of such cases would broaden our knowledge regarding this infrequently reported phenomenon . this is the first report of an acquired port - wine stain from india and previous reports of such cases have also been reviewed .

port - wine stains ( pwss ) are congenital vascular lesions caused by progressive ectasia of blood vessels located in the vascular plexus of the dermis . acquired pwss develop later in life but are identical in morphology and histology to the congenital pwss . less than 75 cases of acquired pwss have been reported in the published literature , of which there has not been a single report from india so far . various factors have been proposed for its pathogenesis like trauma , actinic exposure , drugs , tumors , and herpes zoster infection . we report an acquired port - wine stain in a 41-year - old male . the causative factors , treatment , and previous reports of this uncommon entity have also been reviewed .

since the first fontan procedure was introduced by fontan in 1971 , the surgical technique has evolved and the survival rate has shown dramatic improvement . recently , fontan surgery has been performed as an operation of the final stage for congenital heart disease with single ventricle physiology1 ) . in fontan circulation therefore , the pulmonary vascular resistance ( pvr ) must be lower than the antegrade transpulmonary flow resistance2 ) . thus , even a mild increase in the pvr may interfere with pulmonary circulation which in turn may result in decreased preload of ventricle , diminished cardiac output , and declined exercise tolerance3,4 ) . loss of pulsatility of pulmonary flow , reduced growth of the pulmonary vasculature , micro and macro thromboembolism in pulmonary circulation and reduced ventricular function are underlying mechanisms that induce the high pvr in fontan circulation5,6,7 ) . several studies8,9,10,11,12 ) reported the pulmonary vasodilating therapy had an effective influence on fontan patients so we want to determine whether inhaled iloprost as a pulmonary vasodilator might have a role in the clinical symptoms , hemodynamics and exercise capacity of fontan patients . ethical approval for this study and permission for acquiring , analyzing and reporting of patient 's data were obtained in accordance with the guidelines of the institutional review board of gachon university gil medical center ( no . an 18-year - old female with pulmonary atresia with intact ventricular septum status post fontan surgery at age of three years complained of aggravated dyspnea on exertion ( doe ) . a physical examination showed regular heart beat without murmur and mild pitting edema on the pretibial area , liver and spleen were not palpable . recently , she felt more difficult to climb the stairs due to shortness of breath . the inhaled iloprost was administered at 60 microgram / day divided by six times for 12 weeks . pre and post medication , laboratory test - including pro brain natriuretic peptide , cardiac catheterization , bicycle ergometer test , nyha class , and 6-minute walking test - were performed . after 12 weeks , the patient 's systemic blood pressure and o2 saturation were increased . six - minute walking distance was increased from 380 to 395 m and nyha class changed from iii to ii . , the cardiac index and stroke volume were increased without significant change of pulmonary pressure and pvr . the results are shown in table 1 . a 22-year - old male with double outlet right ventricle after 12 weeks , the patient 's systemic blood pressure , o2 saturation , and nyha class were not changed significantly . , the cardiac index and stroke volume were also increased without significant change of pulmonary pressure and pvr . an 18-year - old female with pulmonary atresia with intact ventricular septum status post fontan surgery at age of three years complained of aggravated dyspnea on exertion ( doe ) . a physical examination showed regular heart beat without murmur and mild pitting edema on the pretibial area , liver and spleen were not palpable . recently , she felt more difficult to climb the stairs due to shortness of breath . the inhaled iloprost was administered at 60 microgram / day divided by six times for 12 weeks . pre and post medication , laboratory test - including pro brain natriuretic peptide , cardiac catheterization , bicycle ergometer test , nyha class , and 6-minute walking test - were performed . after 12 weeks , the patient 's systemic blood pressure and o2 saturation were increased . six - minute walking distance was increased from 380 to 395 m and nyha class changed from iii to ii . , the cardiac index and stroke volume were increased without significant change of pulmonary pressure and pvr . a 22-year - old male with double outlet right ventricle status post fontan surgery at age of five years complained of mild doe only . after 12 weeks , the patient 's systemic blood pressure , o2 saturation , and nyha class were not changed significantly . , the cardiac index and stroke volume were also increased without significant change of pulmonary pressure and pvr . despite the increased survival rate of patients who has congenital heart disease with single ventricular physiology due to advanced surgical technique , the fontan surgery is still a palliative procedure . development of the fontan failure state might occur gradually over time ; ventricular dysfunction , aggravated cyanosis , hepatic dysfunction , coagulopathy , protein loosing enteropathy , plastic bronchitis , arrhythmia etc.1 ) . progressive decreasing cardiac output , and increasing central venous pressure can be also observed in fontan physiology with age1 ) . most patients who underwent cardiac transplant long after fontan surgery had elevated pvr before and immediately after the operation13 ) . indeed , pvr is an important factor in the progressive decline in efficiency of the fontan physiology . the loss of pulsatile blood flow of pulmonary circulation of fontan patients is positively correlated with high pvr . the lowering of pvr may increase pulmonary blood flow and preload reserve in fontan physiology , and for this reason the selective pulmonary vasodilating therapy is interesting as a means to improve the fontan circulation8 ) . in addition , nonpulsatile pulmonary blood flow may reduce capillary recruitment and endothelial function . as a result of endothelial dysfunction , impairment in production of nitric oxide , prostacyclin and prolonged overexpression of endothelin-1 level can be observed5,14 ) . most studies were based on high level of endothelin-1 in fontan patients9,10 ) , the endothelin receptor antagonist induced improvement of clinical , hemodynamic parameters and exercise capacity . experiences with sildenafil therapy on fontan patients have also been reported11,12 ) . in a recent study , rhodes et al.15 ) reported on the effect of inhaled iloprost on exercise function of fontan patients . in this article the authors administered a single treatment with iloprost nebulizer just before exercise and concluded that iloprost improves the peak oxygen pulse and peak vo2 of patients with fontan physiology . in our two case studies , we found that 12 weeks of iloprost therapy showed an association with improvement of clinical , hemodynamic parameters and exercise capacity in patients with fontan physiology . first case was considered fontan failure because of aggravated doe , functional class and pitting edema . unlike previous study using iloprost once , we used iloprost for three month period and positive results were achieved without major side effects . in addition , we found that iloprost was effective for fontan patients with or without symptoms . although more advanced curable treatments of fontan physiology have not been developed , pulmonary vasodilator therapy on fontan circulation is one of good suggestion for symptomatic treatment of fontan failure and improvement of the cardiac output .

decreased exercise capacity after fontan surgery is relatively common and the failure of the fontan state gradually increases with age . however , there is no further treatment for patients with fontan circulation . pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the fontan problem . inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator . we experienced two cases of fontan patients treated with inhaled iloprost for 12 weeks . the first patient was an 18-year - old female with pulmonary atresia with an intact ventricular septum , and the second patient was a 22-year - old male with a double outlet right ventricle . fifteen years have passed since both patients received fontan surgery . while the pulmonary pressure was not decreased significantly , improved exercise capacity and cardiac output were observed without any major side effects in both patients . the iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of fontan patients .

angiomyolipoma is a benign tumor that is histologically composed of groups of mature adipose tissue intermixed with convoluted thick - walled blood vessels , interlacing bundles , and irregularly arranged sheets of smooth muscle , with the kidney as the most frequent site of involvement . extrarenal angiomyolipoma can occur in organs such as the liver , lung , uterus , and skin . only two instances of this tumor arising in the tongue have previously been reported [ 1 , 2 ] . a 61-year - old woman was referred to our hospital with a mass in the left proglossis that was painless , but had enlarged slowly for 5 years . the mass measured 20 mm 20 mm and was soft and dome shaped with a dark violet - colored surface ( figure 1 ) . the mass was well - demarcated and easily dissected with an ultrasonic surgical aspirator ( figure 2 ) . histopathological examination showed an encapsulated lesion composed of a proliferation of an intricate mixture of mature adipose tissue , blood vessels , and smooth muscle ( figure 3(a ) ) . the patient 's postoperative course was uneventful , and there were no signs of recurrence at followup at 18 months ( figure 4 ) . koizumi et al . reported a case of angiomyolipoma in the centre of the tongue . described a case of a angiomyolipomatous hamartoma arising in the left lateral border of the tongue . in the present case , angiomyolipoma arose in the proglossis and it had a diagnosis of hemangioma because of its hardness and color . because the three principal components in angiomyolipoma , regardless of its location , vary greatly in proportion and distribution , its heterogeneity may cause diagnostic confusion . the differential diagnosis includes lipomatous or myolipomatous tumours , angiomyoma , angiolipoma , hemangioma , fibroma , and fibrolipomatous hyperplasia . angiomyolipoma could be considered a hamartoma , but there is no consensus that these lesions are a single entity . ide et al . reported that the present lesion may not be a classic oral angiomyolipoma because it was poorly circumscribed and not encapsulated . they suggested the term angiomyolipomatous hamartoma to designate this lesion . in agreement with ide et al . , we diagnosed the present case as an angiomyolipoma because the mass had enlarged gradually with active proliferation and was well - encapsulated . in particular , tongue cancer and hemangioma are tumors with many blood vessels in the proposed area of surgical excision . for preservation of the nervous system and avoidance of bleeding , an ultrasonic surgical aspirator is effective in the resection of these tongue tumors [ 35 ] . in our case ,

a 61-year - old woman was referred to our hospital with a mass in the left proglossis . the lesion was excised with a provisional diagnosis of hemangioma . the mass was well - demarcated and easily dissected with an ultrasonic surgical aspirator . the histopathologic diagnosis was angiomyolipoma arising in the tongue . there were no signs of recurrence at followup at 18 months .

it is an unusual " atypical " antipsychotic , as the " atypical " profile of the new antipsychotics clozapine , olanzapine , quetiapine , and risperidone has been linked to combined antagonism of serotonin 2 ( 5-ht2 ) and dopamine 2 ( d2 ) receptors , whereas amisulpride has negligible affinity for 5 ht2 receptors and is specific for dopamine d2 and d3 receptors in the limbic rather than striatal structures . there are reports of amisulpride being used for the management of bipolar disorders . however , there are very few reports of mania induced by amisulpride . here we describe a young male who developed mania while on amisulpride , a newer antipsychotic drug introduced in india . a 18-year - old unmarried hindu male presented with a history suggestive of schizophrenia for the last 2 years . the illness had an acute onset with complaints of suspiciousness , hearing voices not heard by others , violent abusive behavior , disturbed biological functions , and decreased self care . he was started on risperidone 4 mg along with trihexyphenidyl 2 mg and lorazepam 6 mg per day . gradually , over a period of 1 month , he showed improvement in the symptoms . subsequently , trihexyphenidyl and lorazepam were tapered and he was maintained on risperidone 4 mg per day for the next 1 and a half year . however , he would still be lethargic , would prefer to remain alone and would be inattentive at class . he would not interact with others and not take active part in various household functions . he was prescribed amisulpride for the negative symptoms along with risperidone 4 mg that was continued . it was initiated at 50 mg per day and was increased to 100 mg per day after 4 days of initiation it . after 10 days of 100 mg dose , the patient developed a manic episode , characterized by decreased need for sleep , over talkativeness , hyperactivity , persistent elevated mood , disinhibited behavior , over - grooming , and distractibility . amisulpride was stopped and he was prescribed lorazepam 4 mg on as and when required basis . the patient was followed up after 1 week and during this time there was substantial improvement in his manic symptoms with ymrs score of 17 . to the best of our knowledge of mania due to first amisulpride was reported by murphy in 2003 . however , in the case described by murphy , the patient was also on citalopram , an antidepressant , though its discontinuation did not led to improvement in manic symptoms . also , the patient was initiated on olanzapine immediately which would also probably have antimanic effects . in our case , use of the naranjo adverse drug reaction probability scale and edward 's criteria both indicate a probable relationship between the manic episode and short - term exposure to amisulpride therapy . the manic symptoms with amisulpride can be postulated to be due to the fact that amisulpride in low doses ( < 10 mg / kg ) , preferentially blocks presynaptic d2/d3 receptors , resulting in enhanced dopamine transmission . increased dopamine metabolites and increased dopamine transmission this case report highlights the fact that close monitoring of patients on amisulpiride should be done for manic symptoms . this is also important because of the fact that atypical antipsychotic drugs ( including amisulpride ) are used for the treatment for bipolar disorder . open label studies have shown that amisulpride may be useful for patients with bipolar disorder . also , as low doses enhance dopamine transmission , and high doses reduce dopamine transmission , it might be prudent to start amisulpride at the recommended target dose at the initiation of therapy .

amisulpride is an atypical antipsychotic used for the management of schizophrenia and other conditions like dysthymia . it has also been used for the management of bipolar disorders as an add on therapy . here , we report a patient of schizophrenia who developed a manic episode while on amisulpride .

acoh ( 3.00 mmol ) was added to a solution of galactoside 20 or 13 ( 1.57 mmol ) in acetone ( 3.5 ml ) and meoh ( 4.7 ml ) at 0 c . after 8 h , the reaction was quenched by the addition of nahco3 ( 5.95 mmol ) and then filtered . after concentration of the filtrate under reduced pressure , the resulting oil was purified by flash column chromatography ( etoac in hexane ) to afford alcohol 21 ( from 20 ) or 26 ( from 13 ) as a colorless oil . pph3 ( 1.86 mmol ) , diad ( 1.86 mmol ) , and dppa ( 1.86 mmol ) were added sequentially to a cooled solution of alcohol 21 or 26 ( 0.90 mmol ) in thf ( 20 ml ) . concentration under reduced pressure followed by purification of the residue by flash column chromatography ( etoac in hexane ) afforded azide 22 ( from 21 ) or 25 ( from 26 ) as a colorless oil . tfa ( 0.50 ml , 6.6 mmol ) was added dropwise over 5 min to a solution of azide 22 ( 200 mg , 0.19 mmol ) in ch2cl2 ( 5 ml ) at rt . after 30 min , the reaction mixture was concentrated under reduced pressure to afford pentaol 9 as a colorless oil ( 115 mg , quant . ) : [ ]d + 12.4 ( c 0.5 , cdcl3:cd3od , 2:1 ) ; max(film ) ( cm ) 3282s br ( oh ) , 2114s ( n3 ) , 1696 m ( c = o ) ; h nmr ( 300 mhz , cdcl3:cd3od , 2:1 ) 0.85 ( t , j = 6.0 hz , 3h ) , 1.181.39 ( stack , 22h ) , 1.401.71 ( stack , 4h ) , 1.44 ( s , 9h ) , 3.26 ( a of abx , jab = 12.6 hz , jax = 4.9 hz , 1h ) , 3.513.64 ( stack , 3h ) , 3.653.98 ( stack , 7h ) , 4.89 ( d , j = 3.3 hz , 1h ) ; c nmr ( 100 mhz , cdcl3:cd3od , 2:1 ) 14.3 ( ch3 ) , 23.2 ( ch2 ) , 26.4 ( ch2 ) , 28.6 ( ch3 ) , [ 29.9 , 30.2 , 32.4 , 32.8 ( ch2 , resonance overlap ) ] , 51.7 ( ch ) , 51.8 ( ch2 ) , 68.3 ( ch2 ) , 69.3 ( ch ) , 70.4 ( ch ) , 70.6 ( 2 ch , resonance overlap ) , 72.5 ( ch ) , 75.3 ( ch ) , 80.1 ( c ) , 156.8 ( c ) ; ms ( tof es+ ) m / z 627.3 ( [ m + na ] , 100% ) ; hrms ( tof es+ ) calcd for c29h56n4o9na [ m + na ] 627.3945 , found 627.3956 . tfa ( 1.0 ml , 13.2 mmol ) was added dropwise over 5 min to azide 22 ( 400 mg , 0.38 mmol ) at rt . the resulting colorless oil was used in the next step without further purification ( 192 mg , quant . ) . tfa ( 0.50 ml , 6.6 mmol ) was added dropwise over 5 min to azide 9 ( 114 mg , 0.19 mmol ) at rt . the resulting colorless oil was used in the next step without further purification ( 96 mg , quant . ) .

a concise route to the 6-azido-6-deoxy--galactosyl - phytosphingosine derivative 9 is reported . orthogonal protection of the two amino groups allows elaboration of 9 into a range of 6-n - derivatized -galactosyl ceramides by late - stage introduction of the acyl chain of the ceramide and the 6-n - group in the sugar headgroup . biologically active glycolipids 6 and 8 have been synthesized to illustrate the applicability of the approach .

ventriculoperitoneal and cystoperitoneal ( cp ) shunts are commonly used for the treatment of hydrocephalus and symptomatic intracranial cysts . abdominal complications , including intestinal volvulus , pseudocyst formation and migration into the gastrointestinal tract have been reported , accounting for 25% of shunt - related complications . bowel perforation by the shunt catheter is uncommon and accounts for 0.010.07% of all abdominal complications [ 13 ] . breast - related complications represent a class of thoracic shunt complication and are characterized by breast cerebrospinal fluid ( csf ) pseudocyst formation , csf galactorrhoea and shunt obstruction . we report a rare case of acute mastitis caused by enteric organisms passing through a cp shunt catheter , which had penetrated the transverse colon , and was related to a previous failed attempt to remove the shunt resulting in a divided catheter . the patient is a 56-year - old woman who underwent a surgical treatment of a lateral ventricle meningioma at the age of 29 . the postoperative course was uneventful and she was well without shunt - related problems for 26 years . she developed a brain abscess related to the shunt catheter at the age of 55 . a surgical removal of the catheter was attempted , but was not technically possible , and only the segment from the clavicle to the breast was removed with ligation of the two remaining ends . a year later , she was taken to a local hospital with continuous right - breast pain . the diagnosis of acute mastitis was made and she was referred to our institution for further examination . on presentation , her temperature was 36.7c , and laboratory tests revealed a decreased platelet count of 103 000/l , elevated ast of 97 u / l , alt of 72 u / l and c - reactive protein level of 1.4 mg / dl . cultures taken from the abscess showed enteric organisms including enterococcus avium , klebsiella oxytoca and bacillus . abdominal ct showed that the shunt catheter was in the splenic flexure of the transverse colon with no other abnormalities ( fig . 2 ) . colonoscopy was performed to survey the colon and the shunt catheter was found inside the lumen at the splenic flexure ( fig . acute mastitis caused by enteric organisms which had migrated through the shunt catheter after penetration into the transverse colon was the presumptive diagnosis , and the catheter removed under general anesthesia . the breast to the abdomen segment of the catheter was exteriorized through the right - anterior chest wall without laparotomy . the shunt catheter ( white arrow ) extended through the right - rectus sheath ( a ) , crossed the midline to the left side of the abdomen ( a , b ) and was inside the splenic flexure of the colon ( b , c ) . figure 3:colonoscopy showed the distal catheter within the transverse colon , 40 cm proximal to the anal verge ( a ) . an inflammatory polyp was present at the penetration site in the colon wall ( b ) . the shunt catheter ( white arrow ) extended through the right - rectus sheath ( a ) , crossed the midline to the left side of the abdomen ( a , b ) and was inside the splenic flexure of the colon ( b , c ) . colonoscopy showed the distal catheter within the transverse colon , 40 cm proximal to the anal verge ( a ) . an inflammatory polyp was present at the penetration site in the colon wall ( b ) . the penetration of shunt catheters into the intestine at the distal end is rare with a reported incidence between 0.01 and 0.07% . the mortality rate may be high , up to 15% , due to intracranial or intra - abdominal infections . several factors have been found to be responsible for this rare complication , including chronic irritation of the gastrointestinal tract , prior abdominal surgery or silicone allergy [ 2 , 3 ] . csf leakage and retrograde flow from the peritoneal cavity to the breast may cause breast - related complications . this is the first report of case with acute mastitis caused by enteric organisms migrating through a cp shunt catheter that had penetrated the transverse colon . the diagnosis of bowel perforation by a shunt may not be easy to make , unless the shunt protrudes from the anus [ 5 , 6 ] . prolonged diarrhea of unknown etiology , as well as abdominal symptoms , serves as warning signs of possible bowel perforation . ct can play a crucial role in the diagnosis of bowel perforation . in the present case , these findings suggested that the infection was due to the retrograde flow from the colon . the development of mastitis was facilitated by previous division of the catheter , leaving a ligated end . presumably , the ligature became dislodged , leaving a direct connection from the breast tissue to the distal end of the catheter . previous study reports that the shunt catheter can be directly removed percutaneously in 69% ( 31/45 ) , whereas 17% ( 8/45 ) of patients require a laparotomy and repair of the bowel . if there is evidence of significant abdominal infection , such as an abscess or life - threatening peritonitis , the fistulous opening may not close spontaneously , warranting laparotomy . in the present case , previous insufficient surgical treatment such as a partial removal of the catheter contributed to this shunt - related complication . however , it is common surgical practice to permit the distal end of a shunt catheter to remain in situ in order to simplify the surgical procedure . although this practice is most commonly uncomplicated , it is not unreasonable to suspect that the tube may irritate the serosal surface of the colon leading to chronic inflammation . it is speculated that local inflammation owing to repeated irritation of the bowel wall by the catheter tip might have contributed to the subsequent perforation . in conclusion , a patient who presents with acute mastitis and has previously undergone shunt placement should undergo assessment of the abdominal segment of the catheter .

we report a rare case of acute mastitis caused by enteric organisms passing through a cystoperitoneal shunt catheter , which had penetrated into the colon . the patient is a 56-year - old woman who underwent shunt placement for cyst formation after surgery for meningioma at the age of 29 . after 26 years , she suffered from a brain abscess and an attempt was made to surgically remove the indwelling catheter . only part of the catheter could be removed , leaving a divided and ligated catheter in situ . a year later , she described right - breast pain . ct showed that the catheter had migrated into the colon , followed by colonoscopy confirming that the catheter had indeed penetrated the colon . the breast to the abdomen segment of the catheter was exteriorized through the right - anterior chest wall without laparotomy . a patient who presents with acute mastitis and has previously undergone shunt surgery should have a careful assessment of the entire catheter .

does it have any bearing on outcome in the icu or during the post - icu period ? although not evidence based in any prospective , controlled , randomized clinical trials , it is unlikely that anyone would dispute that under - feeding eventually leads to death . this parallels the lack of controlled studies on the use of parachutes when jumping out of aeroplanes at high altitude . nevertheless , a short period of starvation appears to be a part of clinical treatment in many icus . this is highlighted by a report from colleagues in the netherlands presented in this issue of critical care . intensive care medicine is a comparatively young speciality , and within our arsenal of treatments the amount of evidence - based knowledge is often embarrassingly low . an illustration of this is seen in the guidelines for the surviving sepsis campaign , in which the evidence for different treatment modalities was evaluated in a formalized way . when we come to icu nutrition , there is little evidence at hand in terms of prospective , randomized , controlled clinical trials ; this was highlighted in recently published canadian guidelines . . some authors even call it ' poisonous nutrition ' and ban it from use in the icu . most investigators and authorities in the field would advocate enteral nutrition before parenteral , merely on the grounds that it is the natural way to feed and is cheaper . the evidence in favour of enteral over parenteral nutrition , or the other way around , is weak ; furthermore , it is obsolete because it was generated more than 10 years ago , with obscure indications for parenteral nutrition and with no blood sugar control . in studies comparing enteral and parenteral nutrition in patients whose attending physician is unsure regarding which modality will be optimal , the results demonstrate a very low feeding success rate with enteral nutrition and that complications are related to the duration of nutritional treatment , regardless of the mode of administration . in general terms it has repeatedly been shown that when patients are given only enteral nutrition , the success of feeding is below 70% . successful feeding may be defined in terms of the percentage of prescribed calories , number of days with at least 80% of prescribed calories delivered , or any other measure . the study from the netherlands presented in this issue adds further evidence to a number of publications that demonstrate that clinical practice is equivalent to delivering as little as 50% of the prescribed kilocalories . does this make any difference ? probably , a very large study population would be required to demonstrate that patients do worse when they are administered only 50% of the prescribed dose of antibiotics . the safety margin for bacterial kill when administrating antibiotics is such that reducing doses to half would require 500010,000 patients to show a difference in a prospective study . still no - one would advocate such a regimen . however , the side effects of antibiotics create renal insufficiency , liver insufficiency and drug fever , and predispose to development of multi - resistant bacteria . nevertheless , no - one would deliberately give only 50% of the prescribed dose , stating that it does not matter whether 50% or 100% of the dose is administered . when it comes to nutrition , however , many of us do this . in the study presented in this issue , the post hoc analysis shows that any action that reflects an interest in nutrition , such as placing the tube in any other position than the stomach , using some type of enhanced formula , or having a percutaneous feeding device , increases success rates with nutrition . these specially treated patient groups were small , and findings in these groups should perhaps not be used as a basis for clinical recommendations , but it is clear that an enhanced interest in nutrition and delivery of nutrition will increase success rates . so , the main reason for the systematic under - feeding that is practiced in icus is probably a lack of interest by the attending physician . binnekade and coworkers do not give any information on success rates of feeding in relation to outcome . this is a difficult area , and studies that try to link nutritional practice to outcome must be designed carefully . there are examples in the literature of investigators jumping to conclusions based on insufficient information . nevertheless , in any patient in whom a huge energy deficit is built up , resulting in malnutrition , increased risk for complications can be predicted . in addition , evaluation of nutritional protocols in terms of success rate , nutrition related complications and outcome must be encouraged . systematic under - feeding of icu patients may be used as a marker of suboptimal care . wherever it is considered a human right not be hungry , the burden of producing proper evidence should be imposed on anyone who suggests that half the feed is good enough .

systematic undernutrition of intensive care unit patients is common and neglected . is this inevitable or can better routines and protocols make a difference ? the necessity of feeding may be regarded as self - evident , but more evidence is obviously needed to strengthen this issue . in rich countries it should be a human right not to be hungry .

the treatment of chronic hepatitis c ( chc ) has evolved in the last 15 years from monotherapy with interferon alpha ( ifnalpha ) to the combination treatment with pegylated ifn ( peg - ifn ) plus ribavirin for 24 - 48 weeks . up to now , several viral , host and drug - related reactions in response to ifnalpha - based therapy have been identified . recent studies suggest that liver inflammation in chc is controlled by several mechanisms , including host regulatory immune responses and viral polypeptides interacting with cells involved in innate and adaptive immunity . it is well known that cutaneous side effects of treatment with ifnalpha alone or ifnalpha plus ribavirin in patients with chc have been widely reported , beyond the fact that the virus itself can cause skin lesions . however , the cutaneous side effects during therapy of chc are of inflammatory type with local erythema , edema and , much less frequently , necrosis at the injection skin sites . by contrast , skin side effects of such drugs have few data available in the literature , although the number of reports has been increasing in the last years , including cosmetic filler site injections . roughly one half of these patients had cutaneous findings , either alone or with systemic involvement . more recently there have been reported dermatological diseases in patients receiving the combination of ifn / ribavirin or ifn / ribavirin / amantadine for the treatment of chc . a 44-year - old man with chc genotype 1a and a viral load of hcv > 800,000 iu / ml , a2-f1 on liver biopsy according to metavir criteria , was treated for the hepatic disease . peg - ifnalpha-2b 1.5 g / kg weekly and 1,000 mg of ribavirin daily was introduced . in the 40th week the patient reported sudden intense pain , pruritus , erythema and skin hypertrophy in the left deltoid area over the seahorse tattoo and in a scar on his face ( fig . 1 ) . a skin biopsy on the scar and tattoo showed granulomatous dermatitis ( fig . chest x - ray was normal and the level of angiotensin enzyme converter was 50 iu / ml ( range 18 - 55 iu / ml ) . we intend to call attention especially to dermatologists to the possibility of a granulomatous tattoo reaction as a side effect during chc treatment with peg - ifnalpha-2b plus ribavirin . until now , eight cases of sarcoidosis as a complication of ifn therapy in chc have been reported in the english language literature . the most relevant topic in this case is the occurrence of disease on a tattoo area . six months later retreatment using peg - ifnalpha-2a 180 g once a week plus ribavirin 1,000 mg daily was tried . in the 6th week , cutaneous symptoms relapsed in an unbearable way . the patient did not accept to continue therapy , since hydrocortisone cream was prescribed again but no satisfactory pruritus and pain improvement was noticed . a prompt regression of skin reaction occurred after stopping therapy . sarcoidosis is a granulomatous disorder of unknown etiology and whose epidemiology suggest a genetic tendency face to infectious agents being supposed to result of immune system deregulation leading to non - caseating granulomas as an immune reaction to an unknown persistent antigenic stimulus . ifn has been linked to pulmonary macrophage activation , a characteristic feature of sarcoidosis which has been assumed an exaggerated t helper 1 ( th-1 ) immune response to a variety of exogenous antigens . it seems very likely that a potent immunoregulatory protein for th-1 response such as ifn may induce the disease . in january 2003 the eighth case of ifn - related sarcoidosis was reported with a review of the literature . chest x - ray revealed hilar lymphadenopathy in three patients , with reticulonodular shadows in another three , while the patient with only cutaneous involvement had a normal chest x - ray . the case we report presented with cutaneous sarcoidosis during chc therapy and spontaneous regression of the lesions was noted with treatment discontinuation . we intend to emphasize , especially to dermatologists , the risks of a granulomatous tattoo and other cutaneous sites during chc treatment with peg - ifnalpha-2b plus ribavirin . thalidomide has been also shown to have specific activity to the inflammatory mediators of sarcoidosis and to be an alternative beneficial therapy .

the treatment of chronic hepatitis c ( chc ) has evolved in the past 15 years and combination of pegylated interferon plus ribavirin is its current standard therapy . however , several side effects are commonly observed and frequently lead to transient or definitive interruption of treatment . although sarcoidosis in its systemic or cutaneous form is a very rare side effect in such circumstances , some cases have been reported even with conventional interferon . this brief review of the literature and description of a case of sarcoidosis occurring in a tattoo and a scar patient 's face , during treatment with pegylated interferon alpha-2b plus ribavirin , is an educative report directed in special to dermatologists . the lesion improved after drug interruption and recurred after retreatment with pegylated interferon alpha-2a . we conclude that this side effect must call the attention of doctors to seek for the diagnosis and therapy as soon as possible in such circumstances . no differences were noticed neither with alpha-2a nor alpha-2b pegylated interferon employment .

management of suspected scaphoid fractures with normal x - rays at emergency department ( ed ) presentation is a common clinical challenge . there has long been concern that failure to identify and immobilise a scaphoid fracture might result in avascular necrosis or non - union with significant functional impact for the patient and potential medico - legal risk for the practitioner . traditional management would see the patient immobilised in a plaster cast for 714 days followed by clinical examination and re - x - ray to identify fractures not visible on the initial films . that approach can been challenged on two fronts : whether cast immobilisation while awaiting clinical review is justified and the role of alternative imaging modalities at or near the time of initial presentation . the aim of this project was to characterise current management of adult patients with possible occult scaphoid fracture in australasia . this was an internet - based survey of directors of emergency medicine training ( demt ) throughout australasia as identified from the australasian college for emergency medicine web - site ( www.acem.org.au ) . demts are the designated supervisors of specialist training at hospitals accredited for training , both metropolitan and rural ( one / hospital ) . as such , they are easily identifiable , experienced specialists with a good understanding of clinical practice within the ed in which they work . participants were sent an invitation to participate in the confidential on - line survey by e - mail ( where one could be found ) or by post . data were collected using internet - based survey software ( www.surveymonkey.com ) and included the most common management strategy used for patients with possible occult scaphoid fracture ( from a range of options , see table 1 ) and whether there was a written ed guideline regarding management of such cases . the outcome of interest was the most common management strategy for possible occult scaphoid fractures . table 1management options and reported frequencymanagement strategyfrequency ( n , % ) backslab ( half - cast ) with assessment and re - x - ray at 714 days23 , 38%plaster cast ( full ) with assessment and re - x - ray at 714 days19 , 32%backslab with early ct ( within 7 days)6 , 10%backslab with early bone scan ( within 7 days)5 , 8%backslab with early mri ( within 7 days)2 , 3%support bandage with early mri ( within 7 days)1 , 1.7%support bandage with early ct ( within 7 days)1 , 1.7%plaster cast ( full ) with early ct ( within 7 days)1 , 1.7%plaster cast ( full ) with early bone scan ( within 7 days)1 , 1.7%support bandage with re - assessment at 1 week , with further tests only if symptoms or signs persist1 , 1.7%support bandage with assessment and re - x - ray at 714 days0plaster cast ( full ) with early mri ( within 7 days)0support bandage with early bone scan ( within 7 days)0othersame / next day ct1 , 1.7% management options and reported frequency this project was approved by western health under the nhrmc quality assurance guidelines . the most common management reported was immobilisation in a backslab ( 23 , 38% ) or full cast ( 19 , 32% ) with clinical assessment and re - x - ray in 710 days . ct scan within 7 days was used by 9 ( 15% ) , bone scan within 7 days by 6 ( 10% ) and mri within 7 days by 3 ( 5% ) . eighty - three percent of sites reported not having a written guideline / protocol for this condition . the rate of occult scaphoid fractures in cohorts of patients with clinical evidence suggestive of scaphoid fracture but normal initial x - rays is reported to be 016% [ 24 ] , weighted average 3.7% ( 95% ci 2.55.6% ) . in the subgroup who prove to have a fracture , the rate of delayed / non - union is low ( 1020% ) . traditional treatment for suspected occult scaphoid fracture is cast immobilisation with clinical re - assessment and re - x - ray at 710 days . that approach , however , has been suggested to be over - treatment , with a very low rate of significant fractures at the cost of significant time lost from work or school [ 57 ] . it has been suggested that symptomatic treatment with follow - up within 2 weeks for these cases is sufficient [ 6 , 8 , 9 ] . we found that most australasian eds continue to use plaster immobilisation for this patient group ( backslab or full cast , 70% ) and that uptake of alternative imaging modalities is only moderate ( 30% ) . the high use of immobilisation is at odds with reported uk practice , where only 46% of eds reported routinely using plaster immobilisation for x - ray - negative suspected scaphoid fracture . ct , mri and bone scan have all been shown to be effective in the diagnosis of occult scaphoid fractures , but to date no studies have shown additional benefit from early advanced imaging for hard clinical outcomes . at present , the logical role for advanced imaging is for patients with persistent symptoms and normal follow - up x - rays . there is a clear need for further research comparing combinations of the various immobilisation and imaging strategies and reporting clinical outcomes ( including time off work and time immobilised ) and cost - effectiveness outcomes . there is no evidence that lack of access to advanced imaging impacts adversely on clinical outcome . the weight of evidence would suggest that this is over - treatment and that a simple bandage is sufficient . as this was a survey , the management strategy reported may not be that actually used . the traditional approach to management of possible occult scaphoid fracture of immobilisation with re - x - ray at 710 days remains the most commonly used in australasia , despite evidence that this is probably over - treatment with significant consequences for patients .

aimto characterise current management of adult patients with possible occult scaphoid fracture in australasian emergency departments.methodsinternet-based survey of directors of emergency medicine training throughout australasia . data collected included the most common management used in ed for patients with possible occult scaphoid fracture and whether there was a guideline regarding management of such cases . data are reported as descriptive statistics.results61 responses were received ( response rate 73% ) . the most common management reported was immobilisation in a backslab ( 23 , 38% ) or full cast ( 19 , 32% ) with clinical assessment and re - x - ray in 710 days . ct scan within 7 days was used by 9 ( 15% ) , bone scan within 7 days by 6 ( 10% ) and mri within 7 days by 3 ( 5% ) . very few sites were using same day / next day ct or mri . eighty - three percent of sites reported not having a guideline / protocol for this condition.conclusionthe traditional approach to management of possible occult scaphoid fracture of immobilisation with re - x - ray at 710 days remains the most commonly used in australasia , despite evidence that this is probably over - treatment with significant consequences for patients . the place of advanced imaging for investigation of potential scaphoid fractures requires further research .

acute appendicitis , one of the commonest surgical emergencies , affects nearly 7% of the world 's population and accounts for about 1% of all surgical operations . faecoliths formed by mineral deposits layered with faecal debris and lodged in the appendix are called appendicoliths . the prevalence of faecoliths in the general population is 3% , and appendicoliths are seen in 10% cases of acute appendicitis . a 25-year - old male presented to us with pain in the right flank for 1 day . urinalysis was normal , and abdominal ultrasound raised a suspicion of small calculi in the right kidney . non - contrast computed tomography ( ncct ) abdomen revealed a few subcentimetric calculi in both kidneys without hydronephrosis or hydroureter . [ figure 1c and d ] ( mineral core ) , composed mainly of calcium and struvite on spectral analysis [ figure 1a and b ] . he underwent laparoscopic appendectomy using a 10-mm supra - umbilical camera port and two 5 mm ports at the supra - pubic region and the left iliac fossa , respectively . a pre - ileal appendix was found hidden in flimsy adhesions posterior to the terminal ileum . an incidental meckel 's diverticulum was also found [ figure 1f ] , which was not pathological and was left alone . the mesoappendix was cut using harmonic scalpel ( johnson and johnson make ) and the appendix was cut at the base between ligatures [ figure 1e ] . it was removed using a glove bag after enlarging the supra - umbilical incision because of the large stone size 2.5 cm 3.0 cm [ figure 1 g and h ] . ncct abdomen ( a and b ) the ct spectral analysis of the appendicolith shows the highest peak of the histogram corresponding to struvite - calcium ( c ) multiplanar reconstruction ( mpr ) coronal image showing appendicolith 2.50 cm size ( longitudinal ) ( d ) axial image showing appendicolith 2.10 cm size ( transverse ) ( e - h ) laparoscopic appendectomy , procedure , specimen and appendicolith ( e ) multiple ligations of base of appendix ( f ) meckel 's diverticulum ( g ) size of appendicolith 3 cm on longest axis ( h ) size of appendicolith 2.5 cm on the perpendicular axis acute appendicitis was first reported by fitz in 1886 , and wangensteen and bowers proposed the theory of an obstructive component as a causative factor in 1937 . other proposed aetiologies include lymphoid hyperplasia , constipation , trauma , diet , genetic predilection , hypersensitivity and mucosal ulceration . appendicolith on plain abdominal x - ray is a reliable sign of appendicitis ( 70% ) . however , computed tomography ( ct ) is more sensitive , detecting even non - calcified faecoliths . on ct , appendicoliths appear as laminated bodies with gas in centre or homogenous opacity . when symptomatic , they carry 90% probability of acute appendicitis and 50% higher risk of perforation and abscess formation . some authors have found good correlation ( 65 - 100% ) between faecoliths on ct and appendicitis , while others have not . in a retrospective review by lowe et al . , an appendicolith detected on ct had a sensitivity of 65% , specificity of 86% , and positive predictive value of 74% for the diagnosis of appendicitis . despite appendicoliths being common , a giant appendicolith ( > 2 cm ) is extremely rare and only sporadically reported . others include a 2.1 cm appendicolith reported by garg and a 2.2 cm stone by kaluarachchi . our case is probably the largest documented stone ( 3 2.5 cm ) and the first whose chemical composition has been determined preoperatively by spectral analysis . the authors have not received any resources from a third party , directly or indirectly , to complete this work . the authors have not received any resources from a third party , directly or indirectly , to complete this work .

acute appendicitis is one of the commonest surgical emergencies worldwide . there is considerable variation in prevalence of appendicoliths with appendicitis . most of the patients with appendicoliths are asymptomatic and they are not pathognomic for acute appendicitis . however , appendicoliths show increased association with perforation and abscess formation . appendicolith are quite common , being present in 3% of general population and in nearly 10% cases of appendicitis . however , giant appendicoliths measuring over 2 centimeters ( cms ) are extremely rare . computed tomography ( ct ) has increased their pre - operative diagnosis considerably . use of spectral analysis can give us the details of composition of the stone pre - operatively . we present a young male diagnosed pre - operatively on non - contrast computed tomography ( ncct ) to have a giant calcium struvite appendicolith . on laparoscopy he had a 3 cm stone and an incidental meckel 's diverticulum and underwent appendectomy . the case is presented for the unique size of the appendicolith alongwith review of literature .

it is commonly treated with conservative surgery ; prognosis is rapidly improved by performing concomitant chemotherapy after the initial surgery.1,2 gliomatosis peritonei is the metastatic implantation of mature glial tissue within the peritoneal cavity of patients with ovarian teratomas.3 as second - look or secondary debulking surgery is not generally performed for immature teratoma , as gliomatosis peritonei is not usually discovered in a patient who has completed chemotherapy and shows no evidence of disease.4 this is a rare case of a patient with ovarian teratoma recurrence in the contralateral ovary . the first and later occurrences were treated with conservative surgery , using local resections or cystectomies . gravia 0 , para 0was referred to us because of a lower abdominal mass ; magnetic resonance ( mr ) imaging revealed a tumor 20 cm in size . tumor marker tests revealed cancer antigen 125 = 279 u / ml , ca19 - 9 = 130.6 u / ml , squamous cell carcinoma = 3.9 ng / ml , and alpha - fet protein = 50.6 ng / ml . after providing informed consent , she selected conservative surgery . we performed a right salpingo - oophorectomy , partial omentectomy and peritoneal biopsy , and found lint - like scattered mass lesions 2 mm in size at the vesi - couterine pouch and the douglas pouch . the tumor is a mass with cystic regions of many sizes , with solid parts and adipocyte - laden parts ( fig . the diagnosis was stage ii c(a ) immature teratoma grade2 of the right ovary with gliomatosis peritonei . pathologically , the mass contained squamous epithelium , adipose tissue , bone and cartilage tissue , and immature neuroectodermal tissue , and was therefore classified as a grade 2 immature teratoma ( fig . peritoneal lesions , which were composed of mature glial cells , were diagnosed as gliomatosis peritonei using hematoxylin and eosin ( h&e ) stain ( fig . immunohistochemically , the glial cells were positive for glial fibrillary acidic protein ( gfap ) , which is expressed by numerous cell types of the central nervous system , including astrocytes , ependymal cells and glial cells ( fig . sixteen months after the first operation , the patient underwent cystectomy of a left ovarian tumor and a douglas pouch biopsy . pathological diagnosis was mature cystic teratoma of left ovary and gliomatosis peritonei ( fig . sixty months after the first operation , mr imaging revealed a left cystic ovarian tumor with solid parts 7 cm in size . pathologically , the mass contained immature neuroectodermal tissue , and so was classified as a grade 1 immature teratoma and gliomatosis peritonei ( fig . 2f and g ) . 71 months after the first operation , cystectomy of a left ovarian tumor5 cm in size and biopsies of the douglas pouch and left external iliac lymph node8 mm in size were carried out . the pathological diagnosis was endometrial cyst of left ovary , gliomatosis peritonei and glial implant in lymph node ( fig . although this patient has never conceived a child , she has maintained a regular menstrual cycle , despite multiple surgeries for ovarian teratoma and chemotherapy . immature teratoma of grades 1 and 2 are considered ovarian germ cell borderline tumors , according to the world health organization s classification . as they occur predominantly in women in their teens and 20 s , conservative surgery is performed as a standard ; concomitant chemotherapy after the initial surgery has led to rapid improvements in prognosis.1,2 jefferys et al produced a retrospective study of 47 patients with malignant ovarian germ - cell tumors who were treated by conservative surgery and adjuvant chemotherapy . during chemotherapy , 61.7% ( 29/47 ) of patients developed amenorrhea but 91.5% of these women resumed normal menstrual function upon completion of chemotherapy . twenty patients ( 42.6% ) attempted conception after chemotherapy , 19 of whom ( 95% ) were successful ; 14 healthy live births were recorded , with no documented birth defects.5 in patients with ovarian germ - cell tumors , a recurrence in the contralateral ovary still could be treated by a local resection or cystectomy followed by chemotherapy if fertility was desired , thereby preserving some normal ovarian tissue , if present.5 however , there is little information about recurrence in the contralateral ovary in patients with immature teratomas . in the present case , we re - performed conservative surgery for contralateral recurrence of an ovarian immature teratoma . as such cases are too uncommon to offer evidence for or against repeated fertility - conserving surgery , such surgery should be performed only after obtaining informed consent . histological grading of peritoneal implants , from grade 0 to grade 3 , is essential for therapeutic and prognostic considerations . grade 0 is defined by tumor consisting of only mature tissue with no mitotic activity . gliomatosis peritonei is the metastatic implantation of the mature glial tissue in the peritoneal cavity of patients with ovarian teratomas.3 the prognosis for gliomatosis peritonei should be very good and no further therapy should be necessary . peritoneal implants consisting of embryonic tissue and metastatic immature teratomas imply a poor prognosis.6 if no other teratomatous elements or malignant glial tissue can be found in the implants , the mature glial implants can be ignored and the methods of therapy should be judged only by the stage and grade of the primary ovarian teratoma.7 progression of the peritoneal implants may be as follows : ( 1 ) transforming fibroblastic and eventual disappearance ; ( 2 ) transformation to malignant tissue ( glial or teratomatous ) ; and ( 3 ) persistence without morphological changes.8 in the present case , gliomatosis peritonei was initially classified as grade 0 . because the peritoneal implants were not malignant and shrank over the 8 years , we thought them to be of the first stage ( 1 ) described above . although the gliomatosis peritonei shrank , a lymph node glioma was detected in the fourth surgery . presence of glial tissue in lymph nodes is rare , with very few reported cases . patients with intraperitoneal and lymph node metastases of mature glial tissue do not need therapy for such metastases . the prognosis for these patients is excellent , but they require long - term follow - up .

we report a patient who has maintained a regular menstrual cycle despite undergoing cystectomy and chemotherapy for contralateral recurrence of ovarian immature teratoma with gliomatosis peritonei . we initially performed a fertility - sparing right salpingo - oophorectomy , omentectomy and peritoneal biopsy for immature teratoma with gliomatosis peritonei , with adjuvant chemotherapy ; we performed a left ovarian cystectomy and peritoneal biopsy for mature cystic teratoma with gliomatosis peritonei 16 months after the first surgery , a fertility - sparing left ovarian cystectomy and peritoneal biopsy for contralateral recurrence of ovarian immature teratoma with gliomatosis peritonei 60 months after the first surgery , and a left ovarian cystectomy and peritoneal and external iliac lymph node biopsy for endometrial cyst with gliomatosis peritonei 71 months after first surgery . the peritoneal gliomatosis lesions gradually decreased through the 4 surgeries over 8 years . the patient has maintained a regular menstrual cycle and currently shows no evidence of disease .

nearly , 90% of metastatic tumors occur in jaw bones , especially premolar - molar region of the mandible , whereas metastases to soft tissues are extraordinarily rare and account for 0.1% of all oral malignancies . most metastatic tumors to the orofacial region are seen in patients aged between 40 and 70 years . metastasis to the soft tissues mostly involves gingiva and alveolar mucosal sites ( 54% ) followed by tongue ( 30% ) . in some cases , oral metastatic tumors are found to be the first sign of metastatic spread and in some instances ; they are an indication for unidentified primary tumor of the distant site . the primary site differs according to oral site colonization . in men , primary tumor of the lung followed by the prostate gland and kidney the breast is the most common primary tumor site affecting the jawbones and soft tissues ( 41% and 24.3% , respectively ) , followed by the adrenal and female genital organs . clinically , these metastatic lesions appear nonaggressive , mimicking reactive or benign lesions or even simple odontogenic infections . the clinical features of these lesions include a bony swelling with tenderness , pain , ulcer , hemorrhage , paresthesia and pathological fracture . it is important to diagnose the origin of the tumor as the management will depend significantly on the histological type . although infrequent , oral metastases are rarely encountered as the first sign of occult cancer . hence , this article emphasizes on detailed dentoalveolar examination and early diagnosis for finding the primary focus of metastatic tumor . a 60-year - old male patient presented with a swelling in the right lower back teeth region of the jaw since 3 months [ figure 1 ] . the patient complaints of pain for 10 days , radiating to the right temporal region . the patient was a known hypertensive since 4 years . a well - defined solitary swelling present on the right side of mandible on extraoral examination , a well - defined , solitary swelling measuring 4 cm 5 cm was noted on the right side of the mandible , which was hard in consistency and fixed to the underlying skin . no other pathologic findings were noticed during physical examination . on intraoral examination , firm , nontender swelling with ill - defined borders measuring 3 cm 3 cm was noted in the right mandibular premolar - molar region [ figure 2 ] . there was no ulceration , paresthesia or pathological fracture . on intraoral examination , firm , nontender swelling with ill - defined borders measuring 3 cm 3 cm was noted patient was advised for oral pantamograph and computed tomography ( ct ) scan . ct scan of the face with three - dimension reconstruction revealed a well - defined osteolytic lesion involving the ramus and body of the right half of the mandible , measuring 54 mm 45 mm 50 mm ( anterioposterior transverse craniocaudal ) . it showed speculated periosteal lesion with multiple irregular calcifications and extended into the lingual and mandibular soft tissues [ figure 3 ] . based on the clinical presentation , a differential diagnosis of inflammatory swelling , odontogenic cyst , odontogenic tumor , soft tissue tumor or metastatic tumor to the jawbones was given . computed tomography scan of the face with three - dimensional reconstruction revealed a well - defined osteolytic lesion with multiple irregular calcifications that extended into the lingual and mandibular soft tissues incisional biopsy was done for the histopathological diagnosis . histopathological examination of the h and e stained tissue showed diffuse fibrous connective tissue stroma invaded by the tumor cells which were cuboidal to short columnar with eosinophilic cytoplasm arranged in alveolar / follicular pattern of varying sizes [ figure 4 ] . bony trabeculae were evident in some areas [ figure 5 ] . based on these features , the lesion was diagnosed as a metastatic malignant tumor , probably metastasizing from the lung . ( h&e stain , 100 ) tumor islands along with few bony trabeculae were evident in some areas . ( h&e stain , 40 ) x - ray chest posterior - anterior view revealed consolidation of the right lower zone [ figure 6 ] . the ct scan of the chest with contrast revealed dense soft tissue mass lesion with speculated margins and focal pleural thickening noted at the right posterior basal segment . x - ray chest posterior - anterior view revealed consolidation in right lower zone of the lungs treatment for the patient was palliative external beam radiotherapy on linac given to a total dose of 3000 cgy in 10 fractions to jaw by rl / ll for 12 days which was well - tolerated by the patient . chemotherapy included injection cisplatin ( 75 mg)-intravenous ( iv ) ; injection etoposide ( 150 mg)-iv ; tablet vomiset ( 8 mg ) ; tablet ultracet . the common primary sources of tumors metastatic to the oral region are the breast , lung and kidney . the actual incidence of these cancers is unknown since oral metastatic disease is usually a manifestation of advanced disease . the mean age of occurrence is 54 years with slight male predilection . in our case , also the patient was aged about the same age ( 60 years ) as supported in the literature . most of the patients are aware of primary tumors before the metastatic spread to the oral cavity . in our case , the most common primary sites for oral metastases were the lung , kidney , liver and prostate for men ; breast , female genital organs , kidney , and colorectum for women . in our case too , lung was found to be the primary site . the clinical presentation of the metastatic lesions differed between the various oral sites . in the jawbones , most of the patients complain of rapidly progressing swelling , pain , paresthesia , difficulty in chewing , dysphagia , disfigurement and bleeding . our patient presented with features such as firm , nontender swelling with ill - defined borders . there was no paresthesia , difficulty in chewing , dysphagia , bleeding and pathological fracture . pathogenesis of oral metastasis is unclear but thought to be a multistage process in which cells detach themselves from the primary tumor and get transported by lymphatic or blood vessels . recent studies state that cancer cells metastasizing to bone have shown to alter the physiologic balance between both bone resorption and bone formation . treatment of oral metastasis depends upon its presentation and the stage during which it has been identified . management includes chemotherapy , radiotherapy , surgical excision or a combination of the techniques under local anesthesia . an average survival time for lung cancer metastasis is 4 months to 1 year with a maximum survival rate of 5 years .

oral cavity is an uncommon site for metastasis and is usually an evidence of widespread disease . the clinical presentation of the metastatic lesions differs between the various sites in the oral region . metastatic tumors account for 13% of all oral malignancies . the jawbones , particularly the mandibular molar area is more frequently affected than the oral soft tissues ( 2:1 ) . here , we report a case of a 60-year - old male patient with metastasis from lung to the mandible , where the metastasis was detected before primary tumor .

they may be seen in midline or para - axial location , from the brain to the sacral area . they display a particular tendency to locate in the sacrococcygeal and pre - sacral regions . reported incidence of tumors at these sites the oropharyngeal cavity is exceedingly rare for teratomas , 2% of all teratomas , present as large masses protruding from the oropharyngeal area . though oropharyngeal teratomas have a benign histopathology , they are potentially lethal as they may cause airway obstruction and respiratory compromise . so , principles of management in such patients comprise of the immediate postpartum establishment of a secure airway , if needed via tracheostomy and complete surgical resection of the mass . a 1980 g female infant born to 22-year - old mother after 38 weeks gestation presented with a growth in oral cavity associated with respiratory distress . the mother had undergone ultrasonography examination during pregnancy , but the report of the same was unavailable . on examination , cleft palate was present and a palatal tumor of size 4 cm 3 cm was found in the cleft area [ figure 1 ] . it was hampering feeding . computed tomography scan showed mass lesion in the palatal region ( attached to the palate , more on the right side ) . no intracranial extension [ figure 2 ] . computed tomography scan image after due preparation , the patient was operated , and the mass was excised in toto [ figure 3 ] . histopathologic examination revealed a teratoma composed of mature glial tissue , choroid plexus , glands lined by respiratory type , columnar mucin secreting , and melanin pigment bearing epithelium . pools of myxoid matrix bearing physaliferous cells are present ( notochord like / chondroid areas ) [ figures 4 and 5 ] . microscopic picture 1 microscopic picture 2 following excision , patient recovered faster and gained weight . now , the patient is under routine follow - up , and palatoplasty has been done . teratomas are the tumors which contain tissues derived from all three germ layers . these contain tissues foreign to the anatomical site of origin . most of the teratomas in the pediatric age group are benign , but reports of malignant teratoma do exist . basic histological classifications which are widely used ( arnold 's system ) are : dermoid tumors : these are composed of ectoderm plus mesoderm . this is the most common form of teratomateratoids : these are poorly differentiated tumors and contain all three germ layersteratomas : these also contain tissue from all three germ layers . they are histologically more identifiableepignathus : these are tumors which contain fully developed organs and appendages . the large cervical and nasopharyngeal teratomas which obstruct fetal swallowing of amniotic fluid may contribute to polyhydramnios . this is the most common form of teratoma teratoids : these are poorly differentiated tumors and contain all three germ layers teratomas : these also contain tissue from all three germ layers . they are histologically more identifiable epignathus : these are tumors which contain fully developed organs and appendages . the large cervical and nasopharyngeal teratomas which obstruct fetal swallowing of amniotic fluid may contribute to polyhydramnios . other imaging technique such as magnetic resonance imaging , is useful in finding out the anatomical relationship of these tumors . clinical differential diagnoses of oropharyngeal teratoma are cystic hygroma , lymphangioma , duplications and neuroblastoma . moreover , the histopathological differential diagnosis includes hamartoma , dermoid cyst , and a heterotopic gastrointestinal cyst . with growth , oral teratomas that is why , the respiratory distress may be less . the neonates with oral teratomas the basic principle of management of oropharyngeal teratomas is the establishment and maintenance of a secure airway . if an oropharyngeal teratoma is diagnosed antenatally , elective delivery or lower segment cesarean section is carried out . if orotracheal / nasotracheal intubation is failed / or difficult , tracheostomy must be done . surgical goal is to remove the complete mass in toto , as a residual tumor may cause recurrence . associated cleft palate can be repaired when the child grows up , that is , at the age of 1 years . during follow - up , afp should be monitored . in our case , all the afp levels were within normal range . early diagnosis , the establishment of the good airway , complete excision of tumor and timely follow - up should increase the survival of newborns with oral teratomas .

for congenital teratomas , oropharyngeal cavity is one of the rarest sites ( 2% of all teratomas ) . they are rarely picked up by prenatal ultrasonography . postnatally , newborns present with respiratory distress and at this point role of pediatricians is very crucial in establishing secure airway after which they need to be carefully evaluated and surgically managed . we present a female neonate with palatal teratoma which was treated successfully with surgery .

the original technique of staining the anterior capsule with trypan blue ( tb ) in eyes with no red reflex involves the injection of the dye with a cannula after filling the anterior chamber with an air bubble.1 the stability of the anterior chamber can be compromised by air escaping when the cannula is introduced and the dye is injected . moreover , the superficial tension of air bubble and capillary action between tb and the metal cannula can force the dye to build up within the aqueous meniscus surrounding the air bubble , making selective staining of the capsule difficult . several techniques using viscoelastic substances as an alternative to air have been presented to increase the stability of the anterior chamber.25 however , staining under viscoelastic material can be time - consuming and expensive , as it requires the mechanical spreading of tb onto the anterior capsule and the replacement of the viscous solution when an excessive diffusion of dye affects its transparency . we present an under - air staining technique of the anterior capsule using one drop of tb injected with a 30 g needle through the peripheral cornea . our procedure prevents the exit of air during the injection of the dye , resulting in increased stability of the anterior chamber . moreover , it allows selective staining of the capsule using the needle and avoids an excessive buildup of tb in the anterior chamber . connect a 30 g needle to a 2.25 ml luer lock syringe containing trypan blue 0.06% ( visionblue ; dutch ophthalmic research center international , zuidland , netherlands ) . make a standard side - port incision with a 15 blade . use a 25 g cannula connected to a 5 ml syringe to inject a big air bubble in the anterior chamber . introduce the 30 g needle bevel - up in the anterior chamber through the peripheral cornea between the side - port incision and the site chosen for the corneal tunnel . push the needle forward in the air bubble right between the anterior capsule and posterior corneal surface until the tip reaches the center of the pupil . unlike what occurs using a cannula , the air can not escape from the anterior chamber . inject gently , allowing the tb volume to grow within the needle bevel ( figure 1 ) . rotate the syringe 90 , letting the drop fall on the anterior capsule ( figure 2 ) . the needle tip allows precise control on the injected dye , similarly to a dropper . if the staining is not sufficient , it is possible to inject one more drop of tb on the unstained area . once the needle is removed , the perforation will close spontaneously in a few seconds , and if necessary an absorbent stick can be used to stop tiny leakages of air and/ or dye . inject viscoelastic substance in the anterior chamber through the side - port incision , allowing the air and the leftover dye to exit the eye . tb is the most used dye to perform capsulorhexis in eyes with no red reflex.6 the main staining techniques involve tb injection under air,1 under viscoelastic material,25 or directly under aqueous humor . an air bubble within the anterior chamber reduces the contact between the dye and the corneal endothelium and allows the delivery of undiluted tb onto the anterior capsule . however , the air bubble makes the anterior chamber unstable and prone to collapse when the cannula is introduced to inject the dye . in such an event , the anterior chamber has to be reinflated , thus increasing the duration of surgery . moreover , sudden reductions in volume of the anterior chamber can cause narrowing of the pupil and damage to the corneal endothelium . to prevent the air bubble from escaping the anterior chamber , it has been proposed that a small amount of high - density viscoelastic material be placed near the side - port incision.7 in our technique , we inject the dye in the anterior chamber , introducing a 30 g needle through the peripheral cornea . in this way , the air can not escape from the side - port incision , as often occurs when a cannula is used . once the needle is pulled out , the corneal perforation spontaneously closes in a few seconds . when the dye is injected with a blunt cannula through the side - port incision , the superficial tension of the air bubble spreads the dye into the thin liquid layer between the air and the anterior capsule.8 in this phase , a meniscus of tb forms between the metal cannula and the anterior capsule due to the capillary action and adhesive forces . to obtain the right staining , the anterior capsule must be swept thoroughly with the cannula , allowing the meniscus of tb to contact the largest possible area . if too much dye is injected , it can migrate back between the cannula and the capsule , escaping from the paracentesis , or it could build up in the aqueous meniscus surrounding the air bubble . to avoid this , toprak et al8 modified a cannula by bending it to increase the surface contact between the dye and the capsule . in our procedure , we use a very sharp - tipped needle , which reduces the adhesive forces with tb and allows the drop of dye to fall easily over the anterior capsule . many authors have described alternative staining techniques based on viscoelastic substances instead of air.25 these techniques have the advantage of making the anterior chamber more stable and of better dosing the amount of injected dye . however , when the anterior chamber is filled with viscoelastic substance , it is difficult to deliver the dye onto the capsule . the surgeon has to mechanically spread the dye on the largest possible surface of the capsule , and at times the dyed viscoelastic material or pockets of dye can affect the visibility of the capsule beneath . to avoid these problems , special cannulae have been proposed.2,4 rarely , an exchange of the viscoelastic material is needed to restore the transparency before the capsulorhexis . we injected tb with a 30 g needle in seven consecutive patients with white cataract and normal anterior chamber depth . in this small group of patients , we have not observed intra- or postoperative complications . after surgery , all patients showed marked improvement in their visual acuity . in six patients , we performed a regular capsulorhexis after injecting only one drop of dye . in a single patient , one more drop of tb had to be injected to build up the staining of the capsule . an additional corneal access and a sharp needle in the anterior chamber are the major disadvantages of our technique . in eyes with shallow anterior chamber or with excessive posterior pressure , the use of the 30 g needle technique could be dangerous and should be avoided . in conclusion , in a small series of seven patients , the staining of the anterior capsule using tb under air with a 30 g needle allowed for enhanced stability of the anterior chamber and the use of the smallest quantity of dye .

the original technique of staining the anterior capsule of the lens with trypan blue involves the injection of an air bubble in the anterior chamber . a drawback of this technique is the possible instability of the anterior chamber caused by the sudden exit of air when the dye is injected with the cannula through the side - port incision . other staining techniques that use viscoelastic substances to increase the stability of the anterior chamber and to dose the injected dye have been described . the authors present an under - air staining technique of the anterior capsule using one drop of trypan blue injected with a 30 g needle through the peripheral cornea . this procedure prevents the air bubble from escaping the anterior chamber and allows fast and selective staining of the capsule .

the associated edema which may develop within an hour is the leading cause of potential life - threatening airway compromise . the disease may show variation in terms of its presentation and clinical course , which needs to be addressed carefully before making any airway management plan . literature is convincing both for the active as well as conservative approach of airway management depending upon the patient 's clinical condition . the purpose of reporting this case is to highlight these issues in the management of adult patient with acute supraglottitis . a 52-year - old man presented in the emergency department with the complaints of sore throat for 1 day . the past medical history was unremarkable except the history of ischemic heart disease for which he was taking regular clopidogrel , losartan , and sublingual trinitrates as required . he was febrile with the temperature of 37.6c , respiratory rate was 22 per minute , while the oxygen saturation on room air was 95% . there were no signs of airway obstruction including stridor , change of voice , and dyspnea related to change in position or lying down . the initial management was started with humidified oxygen , intravenous dexamethasone , and adrenaline nebulization . the fiberoptic nasolaryngoscopy showed the significant edema and inflammation of supraglottic region more on the right side as compared to the left . he was quiet comfortable with the breathing and maintaining the oxygen saturation on hudson mask . the rest of the physical and systemic examination was normal . on the basis of initial assessment however , consideration was given to nasolaryngoscopy findings and the fact that we did not have overnight otorhinolaryngology cover in our district hospital . it was also decided that conservative airway management of patient , either in our hospital or during shifting to the specialized unit , would be very high risk . the three anesthetists , out of whom two had consultant grades , were present for the case . patient 's was kept on spontaneous ventilation with the help of nasopharyngeal airway connected to the main circuit via 15 mm outer diameter connector . a surgical tracheostomy was done after infiltration of local anesthesia while the patient was kept anesthetized on inhalational anesthetic on spontaneous breathing pattern . the patient remained stable throughout the procedure and shifted to the intensive care unit on completion of the procedure . the unanticipated rapidly progressive course of the disease was confirmed by the computerized tomography scan of the neck done after 24 h , which showed the marked swelling of oropharynx reducing the size of airway to not more than a pin hole [ figure 1 ] . patient was shifted to specialized center on the first postoperative day where he was treated medically . rest of the course in the hospital was uneventful and then he was discharged home . the reported incidence in this population group is 12.9/100,000 annually and is higher than that in the pediatric population . signs and symptoms at the time of presentation show variation among the patient group involved . sore throat , dysphagia , and change in voice are the common findings in the adults while the children usually present with the cardinal signs of airway obstruction as stridor , drooling , and dyspnea . the current literature supports the conservative approach for the patients in which no airway compromise is suspected . patients having a rapid progressive course , diabetes , or symptoms of impending airway obstruction like stridor or muffled voice are likely to be managed with the active airway intervention . we recommend that before taking any decision on the approach to a patient , it is very important to consider the type of health care setup and facilities available . the disease process may show marked variation in terms of symptoms and progression as it was happened in our patient . our patient presented with sore throat and he did not have any sign or symptoms of respiratory distress but still had rapid onset of massive airway edema . a retrospective review of nine healthy adults found that the signs of upper airway obstruction are characteristically absent in early phase of potentially fatal supraglottitis . . it also aids in tracking the disease progress and the response to medical treatment . severe swelling of the epiglottis and its extension to the arytenoids are the two factors strongly associated with the airway intervention . the management of our patient was based on the nasolaryngoscopy finding at the time of presentation which showed the clinically significant edema of oropharynx . air way manipulation in such high risk patients should be done in the operation theatre with a surgeon ready for emergency tracheostomy . the anesthetic options , inhalational induction using sevoflurane or awake trachesotomy , should be individualized according to the patient condition and clinical expertise available . in adult patients having acute supraglottitis , clinical parameters are not the reliable indicators of the airway condition , which may deteriorate rapidly . a multidisciplinary approach involving the otorhinolaryngologist and anesthesiologist is of prime importance for the management of airway . either the prophylactic or conservative approach may be used depending upon the patient condition and the health care facilities available . it is also very important to anticipate the difficulty and all the preparations for an emergency tracheostomy should be there for securing of airway .

airway management of adult patients with acute supraglottitis is challenging . the sign and symptoms of the disease may show marked variation in terms of severity and progression . thorough evaluation is required before selecting any particular approach . we report the case of an adult patient with acute supraglottitis , in whom active airway intervention was planned . the clinical predictors were not suggestive of any airway compromise in our patient . however , the disease was found to have an unanticipated rapidly progressive course leading to the significant edema of the oropharynx and the surrounding structures as evidenced by the laryngoscope and computerized tomography scan findings .

a 15-year - old boy had complained of motility limitation in the right eye since a young age . his visual acuity was 20/20 in each eye , although an ocular motility examination revealed markedly limited abduction and mildly limited adduction in his right eye ( fig . 1 ) . on attempted adduction of the right eye , globe retraction ( fig . 2 ) with narrowing of the palpebral fissure was observed , and there mild upshoot and downshoot were also observed ( fig . an alternate prism cover test revealed a 16 prism diopters ( pd ) right esotropia ( et ) in the primary position , 25 pd et in right gaze , 10 pd et in left gaze at distance fixation , and 4 pd et at near fixation . he assumed a compensatory face turn to the right and did not complain of diplopia . duane 's retraction syndrome was inferred as the diagnosis until we noted that the patient 's right eye had slight enophthalmos . hertel 's exophthalmometry revealed a 1.5 mm difference between both eyes and the margin reflex distance 1 was 0.5 mm in the right eye . computed tomography of the orbits was performed and demonstrated an old medial orbital wall fracture with entrapment of the medial rectus muscle and surrounding tissues ( fig . however , there was no history of trauma or forceps delivery according to the patient or his parents . the patient 's ocular motor impairment was concluded to have arose from a long - term medial orbital wall fracture and was diagnosed as ' pseudo - duane 's retraction syndrome ' . because open reduction surgery for an old orbital wall fracture would be very difficult , and fruitless for this patient , we decided only to perform surgery on the eye muscle . the 5 mm recession of the right medial rectus muscle was successfully performed in order to correct the patient 's head turn and esotropia . postoperatively , the patient had orthotropia in the primary position , and the anomalous head posture disappeared , though there were still some limitations in both abduction and adduction in the right eye one year after the surgery . duane 's retraction syndrome is a well - known congenital ocular motility disorder caused by anomalous innervation and mechanics of the lateral rectus muscle , with secondary muscle changes sometimes superimposed . in 1976 , thomas duane reported five cases of pseudo - duane 's retraction syndrome showing similar , yet different , symptoms and sign as duane 's retraction syndrome.1 other authors preferred the names of ' inverse duane , reverse duane , or mirror - image duane retraction syndrome ' owing to the presentation of opposing clinical features compared with those of classic duane 's retraction syndrome . the etiology is , also , completely different from that of classic duane 's retraction syndrome . the primary lesion in pseudo - duane 's retraction syndrome is suspected to be the medial rectus muscle . most cases have been reported to result from the entrapment of the medial rectus muscle within the medial orbital wall due to trauma.1,3 congenital cases,4 recurrent pterygium,5 and myocysticercosis involving the medial rectus muscle6 have also been reported to be related with inverse duane 's retraction syndrome . in korea , jo et al.7 reported on inverse duane 's retraction syndrome with exotropia , and lew et al.8 reported a case of congenital inverse duane 's retraction syndrome with a tight medial rectus muscle . recently , a case of innervational etiology , such as bilateral inverse duane 's retraction syndrome , due to simultaneous co - contraction of both horizontal rectus muscles has been reported by khan.9 to the best of our knowledge , there is no report presenting very similar clinical features as those of classic duane 's retraction syndrome arising from the entrapment of the medial rectus muscle in a medial orbital wall fracture . this case would most appropriately be categorized as pseudo - duane 's retraction syndrome instead of inverse or reverse duane 's retraction syndrome . there might be some possibility that the patient acquired duane 's retraction syndrome prenatally , and that the medial orbital wall then fractured after birth . however , several pictures of this patient around one year of age revealed that there was no conspicuous head turn , and his family recalled that the limited eye movement was not noted until 2 - 3 years of age . the limitation of his eye movements , therefore , could be concluded as acquired due to unnoticed orbital trauma after one year of age . we report a case of pseudo - duane 's retraction syndrome caused by entrapment of the medial rectus muscle in an old medial orbital wall fracture presenting very similar clinical features as those of classic duane 's retraction syndrome . the importance of several tests such as the forced duction test and an imaging study should be emphasized in making the diagnosis for limited eye movement .

we report a case of pseudo - duane 's retraction syndrome with entrapment of the medial rectus muscle in an old medial orbital wall fracture presenting identical clinical symptoms as duane 's retraction syndrome . a 15-year - old boy presented with persistent limited right eye movement since a young age . examination showed marked limited abduction , mildly limited adduction , and globe retraction accompanied by narrowing of the palpebral fissure during attempted adduction in the right eye . he showed a right esotropia of 16 prism diopters and his head turned slightly to the right . a slight enophthalmos was noted in his right eye . a computed tomography scan demonstrated entrapment of the medial rectus muscle and surrounding tissues in an old medial orbital wall fracture . a forced duction test revealed a marked restriction of abduction in the right eye . a 5 mm recession of the right medial rectus muscle was performed . postoperatively , the patient 's head turn and esotropia in the primary position were successfully corrected , but there was still some limitations to his ocular movement . the importance of several tests such as the forced duction test and an imaging study should be emphasized in making a diagnosis for limitation of eye movement .

scurvy is a disease that played an important role in history and used to be the number one killer of sailors . it is still diagnosed in third - world countries because of malnutrition and in epidemic form in refugee camps . a 35-year - old patient was seen at the emergency department of a level 1 trauma centre with spontaneous bruisings on the lower extremity . initially , the patient was seen by an orthopaedic surgeon at an outpatient clinic with a painful knee without any preceding trauma . the procedure was postponed twice . at first , the arthroscopy was cancelled because the patient had the flu . the second time the orthopaedic surgeon postponed the operation because of extensive bruising on the right leg ( fig . 1 ) moreover , doctors of the internal medicine and dermatology department were consulted to explore the origin of the haematomas . 2 ) and a biopsy was performed . at the time of the current presentation at the emergency department , because of the progression of the haematomas to the patients left leg , the surgeon was consulted to rule out an active bleeding focus . figure 1:small bruises on the upper legs . the patient was an electrician , currently unemployed because of knee problems . since 10 weeks the haematomas on his right leg occurred after an episode of flu a month ago ( fig . ever since , he had been exhausted , and he had spent the majority of his time in bed . his dentist proposed to remove the decaying teeth . according to the patient , the bad teeth apparently , the patients eating regimen only consisted of bread , sometimes with spread , but mostly without any additions . for this eating habit on physical examination , we encountered a pale patient with haematomas on his upper legs , generally green / blue or yellow coloured . laboratory tests revealed anaemia with a haemoglobin drop of 2.5 points in 2 weeks ( from 8.6 to 5.7 mmol / l ) . no surgical cause for the haematomas was found . instead , the diagnosis of a severe vitamin c deficiency ( scurvy ) by malnutrition due to his eating disorder was considered . after laboratory blood tests were drawn to confirm the diagnosis , vitamin c supplements were started . it is regrettable that the united east india company ( voc ) lost more sailors on scurvy than on the battlefield during the seventeenth century . scurvy was one of the limiting factors for travelling long distances on ships because of the absence of fresh food , fruit and vitamins . scurvy was first described by hippocrates . later in 1753 , a surgeon of the royal navy described how citrus fruits enhanced the symptoms of scurvy . in 1930 , it was discovered that depletion of vitamin c level was the cause . vitamin c is needed for the synthesis of collagen , which is essential for firmness of the blood vessels . in patients with scurvy , the presentation of scurvy is often malaise , fatigue , stiff joints and bruising , mostly on the upper thighs and legs . the next stage consists of open wounds , jaundice , fever , loss of teeth and finally death . scurvy does not occur in most animals as they can synthesize their own vitamin c in contrast with the human , for example guinea pigs and certain monkey species . treatment is relatively simple with a versatile diet with citrus fruits , sweet peppers and tomatoes . the recommended daily quantity of vitamin c is 75 mg for women and 90 mg for man ( http://www.who.int/en/ ) , but up to 40% more can be necessary in smokers and during infection . nowadays , scurvy occurs predominantly in developed countries because of malnutrition , and frequently epidemics are reported from the lesser civilized areas . this present case report emphasizes that scurvy may still occur in developed countries and that patients with scurvy could be encountered by physicians of multiple medical disciplines . scurvy should be considered in risk groups , e.g. singles , patients with eating disorder , students , elderly , alcohol abusers and patients after gastric surgery [ 5 , 6 ] . recently , in wales ( uk ) in 2011 , a young patient died of scurvy ( http://www.bbc.com/news/uk-wales-31039895 ) . however , even in these modern timeframe , groups with a high risk of vitamin deficiencies can be discerned . in our patient , the biopsy could have been avoided , and supplements could have been initiated more quickly in case of higher awareness of the signs and symptoms of vitamin c deficiency .

scurvy is a disease that played an important role in ancient history and used to be a notorious cause of death in sailors . nowadays , scurvy is not a common diagnosis in the civilized world , but this case report indicates that this old - fashioned disease is not extinct at all and still exists but in a different patient category .

only a few cases of pulmonary vascular tumors diagnosed using endobronchial ultrasound - guided transbronchial needle aspiration ( ebus - tbna ) have been reported in the literature . they frequently present as filling defects in the pulmonary vasculature on computed tomography ( ct ) making it challenging to distinguish between vascular tumor and thrombotic emboli . although fluorodeoxyglucose - positive emission tomography ( pet ) scan may reportedly help differentiate malignant growth from benign emboli , pet scans are not routinely performed for this purpose . increased uptake on the pet scan increases the malignant suspicion of vascular tumors but in most cases ebus - tbna of mediastinal and hilar lymph nodes has proven value in diagnosis and staging for lung cancer . in addition , ebus - tbna may provide an opportunity to identify and sample endovascular abnormalities in the mediastinal and hilar area . a 43-year - old white male was diagnosed with leiomyosarcoma of proximal left thigh involving femoral vessels 2 years back . he was found to have pulmonary embolism involving right pulmonary artery for which he was started on anticoagulation and an inferior vena cava filter was placed prior to surgery . despite being on anticoagulation postoperatively , serial surveillance imaging continued to show persistent thrombus . eventually , an interval increase in size of the filling defect in right main pulmonary artery extending to the right lower lobe pulmonary artery was noted on contrast - enhanced chest ct [ figure 1 ] . at this point , intravascular metastatic tumor was suspected and the patient was referred to our interventional pulmonary service for diagnostic evaluation . the patient did not have any other imaging studies such as pet scan or magnetic resonance imaging of the chest . ebus ( bf - uc160f - ol8 ; olympus , tokyo , japan ) procedure was performed under general anesthesia . with the ultrasound and color flow doppler , an endovascular hyper - echoic lesion was identified in right pulmonary artery [ figure 2 ] . only one endovascular lesion was identified , which extended to the right lower lobe branch of the pulmonary artery . because of the nature of the lesion 's extension in the pulmonary artery , it was not possible to accurately size it but it was estimated to be around 20 mm in the largest diameter . ebus - tbna of this lesion using the 22 gauge ebus needle ( olympus , na-201sx-4022 ) was performed under general anesthesia with laryngeal mask airway using propofol intravenous drip and fentanyl intravenous intermittent injections . a core biopsy was obtained using the 22 gauge ebus needle and a total of five passes were performed during this procedure . rapid - on - site evaluation of fixed slides was bloody , and no malignant cells were identified . the procedure was performed without any complications and cytopathological and immunohistochemical evaluation confirmed the diagnosis of metastatic leiomyosarcoma [ figure 3 ] . the patient was started on gemcitabine plus docetaxel combination chemotherapy . ( a ) chest computed tomography ( ct ) image showing right pulmonary artery filling defect ( arrow ) diagnosed as pulmonary thrombotic emboli 2 years ago . ( b ) chest ct 2 years later showing enlarged right pulmonary artery filling extending to the right lower arterial branch consistent with vascular tumor ( arrow ) ( a ) endobronchial ultrasound ( ebus ) image of the right hilar area showing an vascular tumor of the right lower lobe pulmonary artery ( arrow ) . ( d ) ebus - guided transbronchial needle aspiration ( arrow ) of the right pulmonary artery tumor . the color doppler was used to ensure sampling the tumor area with the least blood flow ( a ) this is the tissue core biopsy of the right pulmonary mass lesion showing cells with abundant eosinophilic cytoplasm and spindled nuclei ( h and e , 200 original magnification ) . ( b ) these cells cytologically shows fine granular chromatin without prominent nucleoli ( papanicolaou stain , 600 original magnification ) . ( c ) immunohistochemical stains for smooth muscle actin shows strong immunoreactivity in these cells ( sma , 400 original magnification ) . ( d ) immunohistochemical stains for desmin also shows strong positivity in these malignant cells ( desmin , 200 original magnification ) historically , most cases of pulmonary artery tumors were diagnosed with surgery or during postmortem examination . successful diagnosis of primary pulmonary artery leiomyosarcoma with catheterization and biopsy has also been reported . in the last few years , several cases of primary and metastatic pulmonary artery tumors diagnosed by ebus - tbna have been reported without any procedure - related complications . although pulmonary artery hematoma has been described from ebus - tbna , the pulmonary artery was inadvertently punctured in that case during a routine biopsy of mediastinal lymph node . in addition , there have been reports of using ebus - guided trans - pulmonary vascular lymph - node sampling without any complications . here , we present the first case of metastatic leiomyosarcoma of pulmonary artery diagnosed with ebus - tbna . the case reported is a clear example of the challenges in diagnosis of pulmonary artery tumors due to their endovascular location and the potential risk of bleeding . the use of color flow doppler during ebus - tbna of pulmonary vascular tumor is essential to avoid area of higher blood flow and to perform the biopsy in the tumoral area of least blood flow . given the potential higher risk of bleeding , anticoagulation or clopidogrel therapy should probably be discontinued prior to this procedure . clinically , the importance of high index of suspicion is also demonstrated as the patient was initially diagnosed with pulmonary embolism . misdiagnosing patients with pulmonary artery tumor emboli as thrombotic emboli can result in delaying effective tumor therapy with chemotherapy or surgery . the diagnosis of pulmonary embolism in patients with malignant diseases can be challenging especially in ill patients where the wells score is already high . our patient was asymptomatic , and the chest imaging was performed as part of surveillance and staging of his sarcoma . intravascular metastasis was suspected as an alternate diagnosis after the presumed failure of anticoagulation therapy and increased of the pulmonary artery lesion size . ebus - tbna is a safe procedure and serious complications are extremely rare . until now , ebus - tbna of the pulmonary vascular lesions did not report any serious complications . ebus - tbna seems to be a safe , feasible , and minimally invasive tool for the diagnosis of vascular tumors such as metastatic pulmonary artery leiomyosarcoma .

leiomyosarcoma of the pulmonary vasculature is an extremely rare condition that has not been previously diagnosed by endobronchial ultrasound ( ebus ) bronchoscopy . we present the case of a 43-year - old white male with a history of leiomyosarcoma who was diagnosed with pulmonary embolism 2 years ago . as the filling defects on follow - up chest computed tomography continued to worsen despite anticoagulation , ebus - guided transbronchial needle aspiration ( ebus - tbna ) of the right pulmonary artery lesion was safely and successfully performed . cytopathological examination revealed the thrombus to be metastatic leiomyosarcoma . in experienced hands , and carefully selected cases , ebus - tbna seems to be a safe and effective in diagnosing thoracic endovascular lesions .

the patients were subjected to specialists opinion ( e.g. , ophthalmologist , ent or medical specialist ) and relevant investigations ( e.g. , fundus , x - ray skull , c.t . the cases suffering from organic mental disorders , chronic psychiatric disorders , epilepsy or drug dependence were excluded from the study . out of 3245 patients seen during the study period , 100 ( 3.2% ) were suffering from migraine . maximum patients belonged to age - group of 31 - 40 years ( 40% ) followed by 21 - 30 years ( 22% ) . a majority of migraineurs were married ( 76% ) , illiterate or just literate ( 55% ) , hindus ( 86% ) and housewives ( 56% ) [ table 1 ] . socio - demographic characteristics of patients with migraine family history of migraine was present in 12% cases whereas family history of depression was present in 6% . maximum patients had a total duration of migraine as one to three years ( 40% ) followed by less one year duration ( 32% ) . the commonest frequency of migraine was 1 - 2/week ( 54% ) or 2 - 3/month ( 26% ) . the provoking factors were reported in 18 patients ( 18% ) [ table 2 ] . migraine without aura ( previously called common migraine ) was the commonest variety ( 81% ) followed by migraine with aura ( previously called classis migraine ) ( 17% ) and complications of migraine ( 2% ) ( only two cases reported complications due to migraine both with fainting spells ) . the personality profile of the patients showed anankastic traits ( 25% ) , dysthymic traits ( 20% ) , histrionic traits ( 17% ) , anxious traits ( 14% ) , schizotypal traits ( 10% ) , cyclothymic traits ( 6% ) and others ( 8% ) . the physical comorbidity included hypertension ( 12% ) , cervical spondylosis ( 8% ) , refractive errors ( 7% ) , chronic sinusitis ( 3% ) and diabetes mellitus ( 2% ) . characteristic of migraine generalized anxiety disorder ( f41.1 ) was the commonest psychiatric diagnosis ( 34% ) followed by mixed anxiety and depressive disorder ( f41.2 ) ( 18% ) , 22 patients ( 22% ) did not have any psychiatric disorder [ table 3 ] . migraine is reported to be more common in persons between the age of 20 and 45 years . in the present study also , migraine was found to be more common among age group of 31 - 40 years ( 40% ) followed by 21 - 30 years of age ( 22% ) . this is also consistent with other studies[91416 ] and a majority of patients were married ( 76% ) . unilateral type of headache was much more common than the bilateral type and the headache was throbbing in nature in a majority of migraneurs . there is a wide range of age of onset of migraine reported in the literature ( 5 to 28 years ) with an average age at onset in mid - teens . they were fatigue , lack of adequate sleep , intake of alcohol , menstruation and oral contraceptives . the other provoking factors for migraine as reported in the literature e.g. , aged cheeses , caffeine , chocolate , concentrated sugar , dairy products , fermented , pickled food , fruits ( bananas , figs , raisins , pineapple etc . , ) vegetables ( onions , pea , pods , nuts , peanuts ) , yeast products , meats with nitrites , monosodium glutamate , saccharin etc . , could not be identified or reported by the patients in the present study . migraine without aura ( previously called common migraine ) was the commonest subtype seen ( in 81% ) . this is consistent with the reported findings that lifetime prevalence of migraine without aura is about 13% as compared to migraine with aura seen in less than 4% population . generalized anxiety disorder ( f41.1 ) was the commonest disorder followed by mixed anxiety and depressive disorder ( f41.2 ) and depressive episode ( f32 ) . mixed dissociative ( coversion ) disorders ( f44.7 ) ( fits and fainting ) were also seen among female migraineurs . previous clinical and community studies[1724 ] have also reported a strong association between migraine and depression as well as anxiety disorders and also vice - versa . the literature also indicates that patients with migraine are at reduced risk of suffering from anxiety , mood disorders and substance - related disorders compared with medication overuse headache sufferers . retrospective studies on the onset and course of these syndromes showed that the onset of anxiety generally preceded that of migraine whereas the onset of depression tended to occur after that of migraine . the studies on association of personality traits and migraine demonstrate increased prevalence of neuroticism - anxiety and depression as compared to healthy controls . but the present study , a cross sectional one , did not determine whether the onset of anxiety disorder and depression preceded or followed the migraine . health - related outcomes were worst in those with both migraines and a psychiatric disorder and intermediate in those with either condition alone . future long term follow up studies on larger sample are warranted to study this association . the present study confirms that majority ( 78% ) of patients with migraine had psychiatric disorders .

background : migraine is a common disorder which has psychiatric sequelae.objective:the objective of this study was to determine the clinical pattern and psychiatric comorbidity of migraine.materials and methods:100 cases of migraine seen over a period of one year were analysed to know the sociodemographic characteristics , clinical pattern and psychiatric morbidity.results:maximum patients were between 31 - 40 years of age group ( 40% ) , females ( 78.0% ) , married ( 76% ) and housewives ( 56.0% ) . family history of migraine was present in 12% cases . average age of onset was 22 years . unilateral and throbbing type of headache was most common . the commonest frequency was one to two per week . migraine without aura was commonest sub - type ( 80% ) . generalized anxiety disorder ( f41.1 ) was the most common psychiatric disorder ( 34% ) , followed by mixed anxiety and depressive disorder ( f41.2 ) ( 18% ) and depressive episode ( f32 ) ( 14% ) . in 22% cases , no psychiatric disorder could be elicited.conclusion:the present study confirms that majority patients with migraine had psychiatric disorders . this needs timely detection and appropriate intervention to treat and control the migraine effectively .

in this and the next issue of critical care , john haddad presents a comprehensive review on the contributions of transcription factors to lung injury . the topic is large and ever changing , and the complete coverage of nuclear factor-b ( nf-b ) and hypoxia - inducible factor ( hif)-1 is spread over two issues . in critical care the majority of these patients progress to the point at which they require intubation and mechanical ventilation , with an associated high mortality . recent studies have demonstrated that low tidal volume ( 6 ml / kg ) ventilation strategies dramatically reduce this mortality . however , many other clinical trials of various treatments have failed at either reducing the lung injury or accelerating the healing to the end - point of reduced mortality . in this age of genomics and proteomics we continue to explore the association of gene and environment . with respect to lung injury , we need to identify and understand the mechanisms that predispose patients to the excessive inflammation resulting from an overactive innate immune response that characterizes sepsis and lung injury . these include stimuli , signal transduction ( receptors , enzyme cascades , transcription factors ) , gene(s ) response and the measured clinical phenotype . john haddad , in his two - part review , identifies many ' clinical stimuli ' in cell culture , animal model and patient studies representing an oxidative stress that can generate a response via nf-b or hif-1 dependent signalling . nf-b was originally described in b lymphocytes , but it is now recognized as a member of the rel family of transcription factors and is a critical response element in many cytokine - dependent events or inflammatory conditions . as a result of this link , nf-b has become a major target for new therapeutic approaches in such clinical disease states as asthma , cancer , arthritis , and cardiovascular and neurodegenerative conditions . haddad discusses the roles of critical care conditions such as hyperoxia , haemorrhage and resuscitation ; the ' stress response ' to illness ( interleukin-6 , interleukin-8 , tumour necrosis factor , rantes [ regulated upon activation , normal t cell expressed and secreted ] ) ; and mechanical ventilation and ischaemia / reperfusion . in all of these conditions these dynamic variations in cellular redox or oxidative stress , if in disequilibrium , may regulate gene expression and lead to apoptosis ( cell death without inflammation ) , inflammation and lung injury . the master regulatory element of hypoxic conditions and adapting oxidative stresses to gene expression is hif-1 . hif-1 , a dna - binding protein , has increased stability and binding in hypoxic conditions and is degraded rapidly in normoxia . the accumulation of the -subunits allows for heterodimer formation and translocation into the nucleus during hypoxia . this process leads to selective upregulation of genes whose products are involved in hypoxia and inflammatory lung injury . these include erythropoietin , vascular endothelial growth factor ( vegf ) and glucose transporter . work by haddad has demonstrated that proinflammatory cytokines also activate hif-1 stability and dna binding . this effect was most profound in hypoxic conditions and was , in fact , greater than that in hypoxaemia alone . it is felt that hif-1 , via its action on vegf expression , is directly related to lung injury by endothelial barrier dysfunction mediated by vegf and recognized clinically as increased pulmonary vascular permeability . haddad discusses in detail how hypoxia and inflammatory stimuli initiate many signalling cascades via hif-1 to generate a response phenotype to these oxidative stresses . understanding the molecular signalling that couples oxidative stresses via nf-b or hif-1 to acute lung injury should generate new therapeutic options . haddad discusses the rationale for the use of tyloxapol to reduce proinflammatory cytokines , n - acetyl cysteine ( a glutathione precursor ) to reduce neutrophil - associated alveolitis in chronic conditions such as cystic fibrosis , and the use of pyrrolidine dithiocarbamate in transplantation to reduce neutrophil - associated oxidant lung injury . two new compounds , isohelenin and lisofylline , a phosphodiesterase inhibitor , are described as being able to reduce proinflammatory cytokines and ameliorate oxidant lung injury in animal models . as exciting as this emerging field is , with its predictable contribution to future ' bench to bedside ' discussions , a more complete mechanistic understanding and future clinical trials will assist in the realization of improved treatment and reduced mortality from oxidant - mediated lung injury . hif = hypoxia - inducible factor ; nf-b = nuclear factor-b ; vegf = vascular endothelial growth factor .

the role of oxidative stress in regulating transcription factors and specific gene responses in critical illness is a new and emerging area . a better understanding of the proinflammatory oxidant stimuli of reactive oxygen species generation and how this generates the clinical phenotype of acute lung injury by regulating gene expression may allow the development of new therapeutic strategies . in his review john haddad describes the present data and role for transcription factors nuclear factor-b and hypoxia - inducible factor-1 in acute lung injury .

techniques and equipment to accomplish endotracheal ( et ) intubation were the precursor to modern day invasive mechanical ventilation . in recent years , however , the popularity of the et tube has waned . clinically , the et tube is seen as an impediment to spontaneous breathing , a transit route for bacteria to the lower airway , and with the advent of noninvasive ventilation a device to be avoided when possible . of particular interest has been the effect of the et tube on work of breathing and methods to eliminate this work . commonly , pressure support ventilation ( psv ) has been suggested as the technique of choice for eliminating imposed work due to the et tube . more recently , the technique of automatic tube compensation ( atc ) has become available to specifically address this issue . in this issue of critical care , maeda and colleagues compare the technique of atc , as provided by the drager evita 4 ( dragerwerks , lubeck , germany ) and the puritan bennett 840 ( carlsbad , ca , usa ) , versus psv in reducing imposed work of breathing in a lung model . before i comment on the merits of the study , clearly , before the advent of pressure support ventilation in the early 1980s , patients were successfully weaned using t - piece trials and intermittent mandatory ventilation , with no apparent untoward effects . in 1986 , shapiro and coworkers presented data from three normal volunteers breathing through et tubes at a constant tidal volume of 500 ml . that report is widely quoted but is limited by the use of unintubated normal individuals and the requirement for a constant tidal volume during increasing respiratory rates . additionally , close review of the data demonstrates that with a size 8.0 et tube the work of breathing in joules per minute does not become excessive until minute ventilation exceeds 15 l / min . brochard and colleagues compared work of breathing during assisted ventilation , four levels of pressure support , continuous positive airway pressure , and after extubation in an effort to determine the role of pressure support in overcoming the imposed work presented by the et tube . that trial evaluated both patients with normal lungs and those with chronic obstructive pulmonary disease ( copd ) . the authors concluded that the pressure support level that eliminated imposed work was between 3 and 14 cmh2o . interestingly , this was determined retrospectively by matching the work of breathing after extubation to the level of pressure support that resulted in equivalent work of breathing during mechanical ventilation . recent work suggests that the work of breathing postextubation may actually increase compared with work of breathing through the et tube , raising questions about the conclusion of the study by brochard and colleagues . weissman evaluated flow - volume loops in 18 postoperative patients intubated with size 7.0 and 8.0 et tubes and found that only ' minimal limitation to airflow ' occurred at volumes and frequencies associated with tidal breathing . that study is one of the very few that contemplate the idea that et tube resistance may not be important when the appropriate size is chosen and the patients pulmonary function has improved to the point that weaning may be considered . of course , there are numerous other studies that suggest that et tube resistance may represent a significant impediment to spontaneous breathing . however , it is important to note that although there remains concern about et tube resistance , and the literature is replete with lung model studies of increased work of breathing , there is not a single clinical trial that suggests that spontaneous breathing through the et tube results in untoward outcomes . the report by maeda and coworkers in this issue of critical care evaluates the two most popular methods of overcoming et tube resistance , namely psv and atc . the authors concluded that tube compensation could not overcome the pressure time product associated with triggering and that pressure support is as effective as atc at 100% . there are , however , tremendous disadvantages to a lung model study in comparing these techniques . calculating the pressure time product and work includes the work required to trigger the ventilator , which can only be overcome by direct measurement of tracheal pressure and triggering from the tracheal signal . this method has been proposed by many , and advanced by the group from gainesville . additionally , although the lung model allows consistency , it can not react to differences in gas delivery . in several human studies comparing psv with atc , the slow flow and longer inspiratory time associated with psv has been associated with dysynchrony . when used in a patient with copd , the patient 's high airway resistance and increased compliance allow even small amounts of psv to cause hyperinflation and auto - peep ( positive end - expiratory pressure ) . alterations in pulmonary mechanics , along with the preference of the copd patient for short inspiratory times and long expiratory times , result in neuromechanical dysynchrony during psv . the main proposed advantages of atc over psv are reduced work of breathing as patient demand varies , preservation of a normal , variable breathing pattern , and improved synchrony . in a recent trial of spontaneous breathing before extubation , haberthur and coworkers failed to show any advantage of atc over psv or t - tube trials . one issue not addressed in the study by maeda and coworkers is the role of expiratory compensation . one distinct difference in the operation of the drager evita 4 and puritan bennett 840 is that evita 4 provides both inspiratory and expiratory compensation for et tube resistance . in these cases , the airway pressure may be allowed to drop below peep to facilitate expiratory flow . new modes and new techniques are developed in the hope of resolving clinical problems and often concentrate on a short - term physiologic end - point . conventional wisdom suggests that the et tube is an impediment to efficient spontaneous breathing , yet clinical evidence during spontaneous breathing trials appears to argue to the contrary . the real question may be whether overcoming et tube resistance is necessary , not whether atc is as good as or better than psv . the future of atc , like many new techniques , may not be in overcoming et tube resistance , but as a method of support during spontaneous breathing that improves patient - ventilator synchrony as compared with psv . additional clinical studies are required to complement the excellent laboratory work by the group from osaka reported in this issue . atc = automatic tube compensation ; copd = chronic obstructive pulmonary disease ; et = endotracheal ; peep = positive end - expiratory pressure ; psv = pressure support ventilation .

concerns about the work of breathing imposed by the endotracheal tube have led clinicians to routinely use pressure support to overcome this resistive component . more recently , ventilator manufacturers have introduced systems to automatically overcome endotracheal tube resistance , regardless of tube diameter or patient demand for flow . despite the theoretical advantages , neither method appears to provide superior performance . stepping back , the real question may be , is overcoming endotracheal tube resistance really important ?

gastrointestinal lipomas are rare , mostly small and detected incidentally during endoscopic examination ( 1 ) . these tumours are benign proliferations of mature fatty tissue arising in the submucosa and predominately arise in the large intestine ( 51 - 70% ) preferentially on the right side . the majority of symptomatic lesions occur in adults in the sixth decade of life ( 2 ) . lipohypertrophy of the ileo - caecal valve is classified as a variant of normal ( 1 ) . the vast majority are asymptomatic but they can manifest due to intussusceptions , ulceration leading to haemorrhage or iron deficiency anaemia , intestinal obstruction or present as an abdominal mass . most are compressible and submucosal although they can rarely develop in the serosa and appear as a fold or polyp . they contain mature adipose tissue and usually have a thick capsule surrounding the tumour ( 2 ) . these changes if extensive can mimic a liposarcoma ; however , frank liposarcoma of the colon is extremely rare and requires the presence of lipoblasts ( 2 ) . mucosal biopsies can be unhelpful and yield either intact mucosa , granulation / inflammatory changes or fat necrosis . . it can be difficult to separate the two and even endoscopic ultrasound can be non - diagnostic and adequate histology sampling may require a resection ( 3 ) . where a biopsy diagnosis has been made endoscopic resection or limited resection can be undertaken removing the need for major resectional surgery ( 3 ) . where this is not technically feasible a minimally invasive approach using either mini - laparotomy or laparoscopic resection has successfully been reported ( 4,5 ) . a 74 year old man with a background of chronic obstructive airways disease ( coad ) was referred for urgent assessment by his general practioner with a 1 month history of lower abdominal pain and altered bowel habit . abdominal and rectal examination was unremarkable and routine blood testing including carcinoembryonic antigen ( cea ) was normal . a large ulcerating polypoidal lesion was found at his hepatic flexure ( figure 1 ) . colonoscopy demonstrating large pedunculated ulcerating lesion found at hepatic flexure histology from biopsies taken showed no evidence of neoplasia or dysplasia but was felt to most likely be unrepresentative of the endoscopic lesion . a staging ct scan of chest , abdomen and pelvis was arranged as part of pre - operative staging protocol and this demonstrated a large 3.3 x 4.3 x 3.4 cm heterogeneous enhancing colonic polyp , which predominately enhanced with fatty attenuation ( figure 2 ) . proximal to the lesion was circumferential colonic wall thickening and surrounding fat stranding suspicious of colonic malignancy . there was concern about colonic malignancy and following discussion at a mdt meeting he was offered a surgical resection . ct scan of the abdomen showing the lesion ( marked a ) a laparoscopic extended right hemicolectomy was performed under general anaesthesia . there were no complications and the patient made an excellent recovery and was discharged home . gross and microscopic histological examination showed a submucosal lipoma with areas of fat necrosis and surface ulceration with extensive granulation tissue . polypoidal tumour measuring 4 cm with an area of central ulceration gross slices of the tumour show adipose tissue expanding the submucosa . there are areas of fat necrosis and mucosal ulceration microscopy shows lobules of mature adipocytes . imaging may reveal tissue with the signal and uptake of adipose tissue but a confident diagnosis may not be possible . endoscopists , radiologists and pathologists need to be aware of this entity . careful discussion and review of the investigations at an mdt meeting may permit a pre - operative diagnosis . endoscopic resection can be used where technically feasible but where doubt remains or this is not feasible then laparoscopic or open resection may be required .

lipomas of the alimentary tract are rare tumours that can mimic malignant lesions . they are often small and asymptomatic although larger tumours can present with intusussception or as abdominal masses . we present a case of a transverse colon submucosal lipoma masquerading as a colonic adenocarcinoma leading to resection.a 74 year - old - man was referred urgently for assessment with altered bowel habits , and lower abdominal discomfort along with a positive faecal - occult - blood sample . colonoscopy demonstrated a large polypoidal lesion at the hepatic flexure with ulceration . biopsies were inconclusive . a staging ct scan confirmed a 3.3 x 4.3 x 3.4 cm polyp with colonic wall thickening suspicious of malignancy . an extended right hemi - colectomy was performed . histology showed a large submucosal lipoma with 12 reactive lymph nodes.colonic lipoma often present as incidental findings detected on either imaging or endoscopically whilst investigating other symptoms . their appearances can mimic colonic malignancy and surgical resection may be required .

acute intussusception is one of the most common causes of acute abdomen in infants . management includes both surgical and non - surgical methods , with gradually increasing importance recently being accorded to the latter . conservative measures , including hydrostatic reduction , are the methods of choice for initial management . though widely accepted , the expertise is still not widely available , especially in developing countries . a six - month - old child presented with complaints of repeated episodes of vomiting that contained only ingested milk . this was associated with three episodes of loose stools , initially watery and later with fresh red blood per rectum with jelly - like consistency . there was no history of fever , decreased urine output , lethargy , abnormal body movements , or difficulty in breathing . her immunizations were fully up to date with no history of rota virus immunization . on admission abdominal examination was remarkable for an ill - defined mass that could be felt in the right hypochondrium . she was initially kept nil per oral and started on conservative measures , including antibiotics . an urgent abdominal ultrasonography was done , which showed a round mass - like lesion giving a target appearance in the right hypochondrium ( suggestive of ileocolic intussusception ) . she was taken for immediate intervention , and as per surgical opinion , a hydrostatic reduction under radiological guidance was started . the child was first stabilized using intravenous fluids to treat dehydration , and a pre - procedure x - ray abdomen was performed to rule out intestinal perforation . the procedure was done in the ultrasonography room in the presence of a treating physician and a pediatric surgeon . without using any sedation , a 16 f foley catheter was inserted into the rectum in the supine position and the balloon was gently inflated while maintaining a tight anal seal . the patient s vitals , which included abdominal girth and distension , were monitored throughout the procedure . lactate solution , warmed to body temperature , was then slowly introduced into the catheter through a bag hanging at a height of approximately 100 cm . during reduction , the intussusceptum was observed under continuous ultrasound guidance as it proceeded to the cecum and then reduced across the ileocecal valve ( figures 1 - 4 ) . ileal loop ( star ) within ascending colon ( arrow ) , longitudinal view . this was associated with the patient s improved condition , which included less crying and irritability . the parents were told about the possibility of recurrence and asked to pay close attention for the onset of symptoms . acute intussusception is defined as the telescoping of the proximal bowel ( intussusceptum ) into the distal bowel ( intussuscipiens ) . the usual locations include the ileocolic region and ileocecal junction , which comprise the majority of cases . intussusception mostly presents between four months and two years , while the peak incidence is found around middle infancy . diagnosis usually needs a high suspicion on the part of the treating physician , as presentation can be quite varied , including irritability , vomiting , and poor feeding . characteristic manifestations include crying episodes , fresh red blood in stools ( hematochezia ) , and a mass in the abdomen . differential diagnoses include acute gastroenteritis , dysentery , sepsis syndrome , and volvulus , amongst others . the diagnosis is usually confirmed sonographically , which is highly accurate and has a reported sensitivity that exceeds 90% . various signs have been described in the literature , depicting intussusception as an abdominal mass with a target sign on the transverse section , a pseudokidney ( or sandwich sign ) on the longitudinal section , and a crescent - in - doughnut sign on axial images . the management of intussusception has seen a paradigm shift in recent years . in the past , the preferred non - surgical method involved use of a barium enema for reduction , followed by the air insufflation method . hydrostatic reduction using ultrasound - guided saline reduction has recently gained acceptance as the procedure of choice for initial nonsurgical management of intussusceptions in children . this popularity can be attributed to the avoidance of radiation exposure to the child and the treating team , as well as to the high success rates that have been achieved . there is still some controversy regarding the exact method of the procedure , which can be attributed to the poor training of physicians and to the general belief that intussusception is entirely a surgical problem , especially in developing countries . this procedure should be undertaken with a close liaison between pediatrics , pediatric surgery , and radiology teams to obtain the best results . the surgeon and physician should always be present at the time of reduction for clinical monitoring . after adequate optimization , including temperature controlled settings , the procedure should ideally be undertaken without any sedation . this is helpful for assessing a successful reduction using clinical criteria , as described later . we used ringer s lactate due to its near physiological constitution , avoiding the risk of chemical peritonitis due to a barium enema or tension pneumoperitoneum due to an air enema , as seen in cases of intestinal perforation . the risk of intestinal perforation following such a procedure is rarely encountered due to less hydrostatic pressure exerted and an even amount of pressure exerted on the bowel walls as compared to air insufflations . even if intestinal perforation occurs we chose 100 cm h2o pressure to lessen the risk of perforation , though different studies have used a wide range of pressures ( 75 to 125 cm h2o).6 , 9 the main ultrasonographical criteria of successful reduction is the flow of the fluid from the cecum into the terminal ileum . general improvement in the clinical profile of the patient including relief from crying , stable vitals , increasing abdominal distension during the procedure ( which indicates filling of the small bowel ) , and the disappearance of the abdominal mass , should also be given due consideration when deciding to terminate the procedure . a repeat ultrasound should always be done after the procedure as the chances of recurrence is high , especially in the immediate post - procedure period . , this method should be undertaken as teamwork , and not considered the work of a single specialty , which is the best way of treating the patients according to the standards of today .

we present a case of infantile intussusception treated successfully using hydrostatic reduction , highlighting the importance of non - surgical techniques as the first line of management for this entity . physicians should strive for mastery over such techniques by extensive training to prevent unwanted surgical procedures in such cases .

a rising trend in the incidence and prevalence of inflammatory bowel disease ( ibd ) in asia has been recognized for the past two decades . the thiopurine drugs azathioprine ( aza ) and mercaptopurine are widely used for the treatment of ibd and have proven to be effective in both inducing and maintaining remission of crohn 's disease and ulcerative colitis ; however adverse effects can occur in 928% of patients , and these adverse effects often necessitate a dose reduction or discontinuation . nevertheless , some reports suggested that neutropenia during aza therapy reduced the relapse rates in ibd patients [ 1 , 2 ] , and that the eradication of sensitized leukocytes , which was done by leukapheresis or bone marrow transplantation , improved ibd in some cases [ 3 , 4 ] . there have also been some cases of ulcerative colitis with prolonged remission following aza - induced pancytopenia [ 5 , 6 ] . a 22-year - old man with a known history of crohn 's colitis who had undergone fistulectomy for complex perianal fistula 2 years previously was admitted with a complaint of severe weight loss ( 25 kg during 2 years ) , diarrhea , abdominal pain and recurrent perianal tenderness . physical exam revealed fistular openings and subcutaneous abscess pockets with severe tenderness on the anus and buttock . he was not able to maintain a sitting or supine position , so his social activity was limited . contrast - enhanced computed tomography scan of the abdomen showed multifocal inflammatory wall thickening and thick - walled abscess pockets with the beaded appearance of subcutaneous small abscesses in the precoccygeal and perianal area ( fig . abscess drainage and a seton operation were performed , then aza was administered at a starting dose of 25 mg ; this was increased up to 50 mg after 2 weeks . he then revisited the hospital and presented with high fever ( 39.2c ) and myalgia . on general peripheral blood test , leukocytes were 180/l ( segmented cells 6% ) , hemoglobin was 6.6 g / dl and platelets were 48,000/l . after 2 weeks of administering human recombinant granulocyte colony - stimulating factor and broad - spectrum antibiotic therapy , his fever subsided and the cell count fully recovered . escherichia coli was found on blood culture , but it was sensitive to the antibiotics . after recovering from cytopenia , the frequency of bowel movements decreased and the perianal pain and oozing improved gradually . follow - up colonoscopy was performed 3 months later and demonstrated diffuse fibrotic scar instead of multiple ulcerations ( fig . he gained 10 kg within 6 months and is now on regular follow - up without clinical recurrence ( cdai score = 114 ) . therefore , a conventional immunosuppressive dose of less than 2.5 mg / kg / day causes predictable mild leukopenia . rarely however , sudden , severe and unexpected myelosuppression , which is possibly idiosyncratic , has also been reported when low to moderate doses ( < 2 mg / kg / day ) of the drug were used . in these cases , the cytopenic patients are vulnerable to the development of sepsis that necessitates hospital admission for intensive care . however , the preferential suppression of neutrophils develops when leukopenia occurs , which suggests that aza 's antiinflammatory effects are mediated through its effects on neutrophils as well . in fact , clinical relapse of ibd is characterized by increased neutrophil migration into the intestine . . moreover , some reports have described cases of ulcerative colitis with prolonged remission following pancytopenia . burke et al . reported prolonged remission in excess of 4 years following induced pancytopenia , where 3 of the 4 patients who developed severe neutropenia remained in remission for up to 21 months following that episode . first , given that altered host immunity plays an important role in ibd activity , it is possible that this patient 's host immunity was reset following the pancytopenia caused by aza . according to one report that analyzed the course of patients with crohn 's disease and ulcerative colitis and who underwent allogeneic stem cell transplantation for myeloid leukemia and myelodysplastic syndrome , 10 of 11 patients showed no ibd activity after stem cell transplantation , and the colonoscopy after complete discontinuation of prophylactic posttransplant immunosuppression revealed no pathologic findings . second , the possible benefit of granulocyte macrophage colony - stimulating factor ( gm - csf ) can be hypothesized . dieckgraefe and korzenik investigated the safety and possible benefit of gm - csf for the treatment of 15 patients with moderate to severe crohn 's disease . third , the original bowel flora is temporarily altered after total gut decontamination and long - term antibacterial treatment . therefore , the most likely theory seems to be the modification of a genetically determined immune abnormality that is responsible for the chronic intestinal inflammation by the reset of the immune system or modification of an altered microbial environment . however , it is unclear whether this mechanism can affect the long - term clinical course . some studies have reported that tpmt genotype or activity does not predict the development of aza - induced myelotoxicity . on the other hand , some reports have suggested a substantial correlation between low enzyme activity and the development of myelotoxicity [ 13 , 14 ] . reported that assessment of the tpmt genotype or activity can reduce the risk of myelotoxicity in approximately one - third of patients . in our case , the tpmt was the wild type and this result raised doubts whether tpmt can be a reasonable screening test to identify patients with a high risk of severe myelotoxicity due to an inherited deficiency of tpmt activity . in conclusion , we report here a patient who had crohn 's colitis and complicated perianal fistulas that required immunosuppression , and who achieved endoscopically determined remission that showed accelerated mucosal healing as well as clinical remission after aza - induced pancytopenia .

the immunosuppressant azathioprine ( aza ) is widely used in the treatment of inflammatory bowel disease ( ibd ) for both inducing and maintaining remission . however , the adverse effects of aza can often necessitate a dose reduction or discontinuation . bone marrow suppression is one of the most serious complications with aza treatment . on the other hand , some reports have suggested that neutropenia during aza therapy reduced the relapse rates of ibd patients , and there have been some cases where eradication of the sensitized leukocytes by leukapheresis or bone marrow transplantation improved the ibd , which may explain the relevant role of neutropenia in controlling disease activity . this report describes the case of a 22-year - old male patient who had crohn 's colitis and complicated perianal fistulas that required immunosuppression ; he achieved endoscopically determined remission and showed accelerated mucosal healing as well as clinical remission following the aza - induced pancytopenia .

the relationship between the clotting cascade and the promotion or inhibition of the anti - inflammatory response continues to be defined through basic research . the potential key role of proteins that were originally believed to be exclusively involved in coagulation in the inflammatory response provides an exciting potential mechanism(s ) for modification through the application of new therapies . the accompanying review by riewald and ruf outlines the available information on the possible steps involving some of the various proteins that are common to both coagulation and inflammation . the complexity of the multiple actions of the proteins in both inflammation and coagulation should excite the clinician with regard to potential new therapies that may be beneficial via activation of one or both of the concurrent pathways . however , before clinicians can administer new therapies that utilize new knowledge on the actions of coagulation cascade components , and hence improve the outcomes of their patients , several gaps in our current understanding must be addressed . this information is imperative if the clinician is to take basic knowledge ' to the bedside ' , where individual patients can benefit . administration of intrinsic or synthetic analogues of coagulation cascade components ( e.g. protein c , thrombin , and factor viia or xa ) , coagulation inhibitors ( e.g. heparin or analogues ) , or agonists or antagonists of the protease - activated receptors , in an attempt to alter the individual patient 's coagulation / inflammation balance , could theoretically produce responses ranging from beneficial to detrimental . knowledge of the ' most beneficial ' balance between augmenting or inhibiting the contribution of the clotting cascade components to inflammation would be required for determining the selection and dosing of potential new therapies . a safe assumption is that any new therapies that alter the intrinsic response to coagulation and inflammatory stimulants would also potentially produce detrimental effects on these , and potentially other , physiological responses . a simple example of such a potential for detrimental effects is the administration of protein c resulting in undesirable haemorrhage . this example illustrates that a broader knowledge of the impact of altering the coagulation/ inflammatory response will be required before clinicians can comfortably utilize new therapies in patient care . it is unlikely that any new therapies resulting from the new knowledge of the contribution of the clotting cascade to inflammation can be applied to all patients . it can be anticipated that certain patients would have contraindications to the new therapies or that dosages would need to be adjusted on the basis of pharmacokinetic or pharmacodynamic variables . the clinician will need to know what these contraindications and variables are before the new therapies can be used confidently . some , such as coagulation disorders , may be known and obvious , but any new strategies that could potentially alter the balance of a complex physiological response may be anticipated to be influenced by many , as yet unknown , factors in the individual patient . an example is the concurrent administration of heparin , which appears to antagonize the anti - inflammatory effect of antithrombin iii in septic patients . a more complete understanding of the impact of concurrent conditions , therapies and the patient 's genetic make - up on outcome will be required if we are to obtain the maximum benefit from new therapies . for any new therapy resulting from our expanded knowledge of the clotting cascade in the inflammatory response to be tailored to the needs of the individual patient , a method(s ) for rapidly and easily assessing the patient 's dynamic inflammatory response will be required . unless any new therapies have very benign toxicities , the need to assess the need , dosage and response to treatment will mandate sensitive and specific monitoring techniques . bedside or clinical laboratory techniques that could be applicable to most clinical environments would allow the benefit of any new therapies to be applied to the greatest number of patients . conversely , any new treatments that require complex , laborious monitoring techniques will have limited applicability . as our knowledge of the contribution of the intrinsic coagulation components increases , the development of many potential new therapies will hopefully emerge for clinical use . these may be totally new therapeutic entities or new strategies for using existing drugs or clotting factors . such developments will create the new dilemma for the clinician , namely that of trying to select between different treatment options for the individual patient . clinical trials outlining the potential anticipated benefit(s ) and the appropriate target patient population for each new therapeutic strategy will need to be completed . this will allow the clinician to ensure that the most appropriate strategy can be selected for the individual patient . the above - mentioned concerns aside , the clinician should be excited about the expanding knowledge of the role of coagulation cascade components in modifying the inflammatory response . such knowledge will hopefully result in new strategies and therapies to manage the septic patient .

the relationship between blood coagulation factors and the promotion or inhibition of the anti - inflammatory response continues to be defined through basic research . the potential key role of blood coagulation factors in the response during sepsis provides an exciting potential mechanism(s ) for modification through the application of new therapies . the complexity of the potential multiple actions of the proteins , such as protein c , should allow for development of new therapies to minimize the detrimental inflammatory response . however , several gaps in our current understanding must be bridged before the clinician can take the basic knowledge ' to the bedside ' , where individual patients will benefit .

a 45-year - old iranian woman was referred to the private clinic in the city of hamadan for implant consultation . her past medical history was not notable , and there was no evidence of systemic disease . she had no history of trauma to the mandible . in the extra - oral examination , intra - oral examination revealed normal oral mucosa , the absence of soft tissue expansion , and teeth of a normal color . all of the teeth were asymptomatic , with no pain or tenderness on percussion or palpation . the involved teeth were vital in an electric stimulation test . for assessment before implant insertion , cbct had been ordered . during evaluation of the implant insertion areas on promax3d cbct ( planmeca oy , helsinki , finland ) , a radiolucent - radiopaque mixed lesion located on the apices of the lower incisors was observed . on the axial , sagittal , and coronal cbct images , the extension of the lesion was observed from the mesial side of the right mandibular lateral incisor to the distal side of the left mandibular lateral incisor . it was a multifocal lesion in which solitary lesions were reached together and made a larger lesion . the total dimension of the lesion was about 16.6 mm in the mesiodistal direction and 6.9 mm in the longest superior - inferior direction . on the panoramic reconstructed cbct image , the lesion associated with the left mandibular lateral incisor was radiolucent , whereas the lesion on the apex of the left central incisor was mixed radiolucent - radiopaque and the lesion associated with the right central incisor was radiopaque with a radiolucent rim around the lesion of this tooth ( fig . the state of the lesion relative to the buccal and lingual cortical plates could be assessed , which might not be possible on the conventional radiographs . on the axial image , one of them was located at the mesial side of the right canine and the other between the left central and lateral incisors ( fig . cross - sectional images , the discontinuity of the lingual cortex was found at the area between the two central incisors on several consecutive sectional images ( fig . this cortical discontinuity was more obvious on the three - dimensional ( 3d ) cbct images ( fig . however , it should be considered that the cortical bone could be seen to have destruction on 3d images , even though it would have been thin without discontinuity on the cross - sectional images . on the digital periapical radiograph that was taken for periodontal ligament space widening was found , especially around the root of the left lateral incisor . based on the patient clinical and radiographic findings , a diagnosis of multifocal periapical cemento - osseous dysplasia was made . pcod is a specific lesion within this group of conditions that usually occurs in middle - aged black women.4 a systematic review of the literature on cod indicated an ethnic distribution of 59% , 37% , and 3% for blacks , asians ( japanese , chinese , and korean ) , and caucasians including indian cases , respectively , in case reports.8 zegarelli et al reported the incidence of pcod in the general population to be 2 - 3/1000.9 periapical cemento - osseous dysplasia generally does not cause cortical bone expansion or perforation.9 - 12 alsufyani and lam reviewed the clinical and radiographic characteristics of 118 patients with cod.13 they showed that 71.6% of 118 patients had no cortical expansion , 76% had intact lamina dura , and 93% had a normal periodontal ligament space . modern advances in imaging help the dentist to use the cbct with the appropriate field of view and spatial resolution ; therefore , the internal mineralized structure of the pathologic lesions can be investigated with a low radiation dose.14 axial cbct images clearly demonstrate the location and extension of the lesion . the expansion and perforation of the cortical plates can be evaluated on cbct even if they are slight . this report showed the discontinuity of the lingual cortex on some axial and cross - sectional cbct images . this characteristic of the lesion was an unusual feature among the cases of pcod reported up to now that may be due to its large size and needed to be checked in further regular follow - ups . this feature also could be found in an exaggerated fashion on the 3d cbct images . although 3d imaging could help the radiologist in diagnosing the lesion for the first look , it should be noted that it is not a reliable viewing modality for assigning the real status of the lesion in relation to its surrounding bone structures . in many cod cases that have been misdiagnosed and/or mismanaged , the lesions were identified in their early stages as a periapical rarefying osteitis such as periapical abscess , granuloma , or cyst , and unnecessary endodontic treatment was performed . therefore , vitality tests are especially important for differential diagnosis . in the radiolucent - radiopaque mixed stage and the radiopaque stage , the differential diagnosis might include chronic sclerosing osteomyelitis , cementoossifying fibroma , odontoma , cementoblastoma , and osteoblastoma.13 periapical cemento - osseous dysplasia is usually detected on routine radiological examinations . however , the differential diagnosis of our case could be calcifying cystic odontogenic tumor and cemento - ossifying fibroma . by using cbct discrimination of pcod from these lesions that exhibit similar internal calcification on conventional radiography high density mass in pcod which is centered in low density area is different from findings of calcifying cystic odontogenic tumor in which calcification is observed at or near the cyst wall.15 also , cemento - ossifying fibroma has more obvious concentric buccolingual expansion on multiplanar cbct image . unless pcod is symptomatic , treatment is usually not needed because development and maturation of the lesion is self - limiting . intervention may cause secondary infection of the cementum - like radiopacities , which may in turn induce osteomyelitis in these lesions.13 however , if pcod demonstrates unusual changes or becomes symptomatic , surgical intervention would then be needed.7 this case was considered as no treatment with only periodic follow - up check .

periapical cemento - osseous dysplasia ( pcod ) is a subtype of cemento - osseous dysplasia that usually occurs in middle - aged black women . this report described a case of a 45-year - old iranian woman who was diagnosed with pcod on the basis of cone beam computed tomographic ( cbct ) findings . cbct enabled detailed visualization of the bone changes . this report described the special radiographic characteristics of pcod , including discontinuity of the lingual cortex on the cbct sectional and three - dimensional images .