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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 . |
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