Tailgut cyst in an infant with imperforate anus: a case report

Tailgut cyst [TGC] is a rare congenital lesion that originates from remnants of the embryonic post-anal gut. It presents as a multilocular presacral mass mainly in young women. Microscopically, the cyst lining is composed of different types of epithelium such as stratified squamous, transitional, or glandular. We present a term female newborn referred to our hospital for evaluation and management of imperforate anus. During dissection of the presacral space to release the rectum, a multicystic mass adherent to the distal part of rectum was detected and completely excised. Histopathology confirmed the TGC diagnosis. TGC is a very rare lesion, but it should be considered in differential diagnosis of any presacral mass, even in infancy. Complete excision is the preferred treatment and can be done more easily neonatally or in infancy

[ investigation of diagnostic accuracy of intraoperative frozen sections in Hirschsprung's disease]

Widespread use of one-stage pull-through technique for Hirschsprung's disease and its excellent results are dependent on the accuracy of intraoperative frozen sections [IOFS]. The purpose of the present study is to determine the incidence and sources of probable errors during this process. This cross-sectional study included all patients who were admitted from 2000 to 2010 in Dr Sheikh Children Hospital and had undergone IOFS for diagnosis of Hirschsprung's disease. This cross-sectional study included all patients who were admitted from 2000 to 2010 in Dr Sheikh Children Hospital and had undergone IOFS for diagnosis of Hirschsprung's disease. Frozen section can be a reliable method for evaluation of suspected Hirschsprung's patients with consideration that the favorable outcome is totally dependent on quality and number of prepared frozen sections for careful evaluation by pathologist

[Diagnostic accuracy of polyclonal stool antigen for detection of helicobacter pylori infection in children]

Helicobacter pylori infection has various clinical features. One of the most common presentations of this infection is upper abdominal pain. Complications such as gastritis, gastric ulcer, gastric carcinoma and MALT lymphoma mandate early diagnosis of H.P infection by a low cost and non invasive manner. The aim of this study was to evaluate the diagnostic role of H.P stool antigen detection as a simple and non invasive method for diagnosis of this infection. Upper endoscopy with gastric biopsy was done on all patients between 6 months to 18 years old with upper abdominal pain. Stool test was done by polyclonal anti-h.p antibody. Results of stool Test were compared with results of RUT and histologic examination Overall 110 patients [57 boys, 53 girls] were studied, 32 patients were Helicobacter pylori positive and 78 patients were Helicobacter pylori negative based on RUT and histologic examination. Stool antigen testing was positive for 39 patients. Sensitivity and specificity of stool Ag were 100% and 91% respectively Stool Ag test has high sensitivity and negatity predictive value for diagnosis of Helicobacter pylori infection

[Results of one stage transanal resection of large bowel in hirschsprung's disease]

Hirschspnmg's disease is a developmental disorder of the enteric nervous system, characterized by functional obstruction. Absence of ganglion cells in the distal colon resulting in a functional obstruction. The aim of this study was to evaluate the results and complications of one-stage transanal endorectal pull-through [OTEPT] in the management of patients with Hirschsprung's disease. A total of 40 children [31 boys and 9 girls] aged 3 days to 60 months underwent OTEPT procedure over a period of two-years at Dr. Sheikh pediatric Hospital. This study was approved by the local committee of Medical Ethics. Median follow up period was 9.10 months [range, 3 to 33 months]. Mean operation time recorded 101.60 mins [range, 25 to 270 minutes]. Hirschsprung's disease developed conventional in 55% [short segment in 30%], Ultra-short in 32.5% and long segment in 12.5%. Mean length of resected bowel measured 17.31 cm, 2 patients underwent laparatomy because of extension of aganglionic segment beyond the sigmoid colon. Mean NPO time and need to rectal tube after the operation were 4.35 and 4.41 days, respectively. 8 patients required urinary catheters after the operation with the mean remaining time of 3.30 days, 20% needed ICU care after surgery and mean hospital stay was 5.76 days. Post-operative complications included entrocolitis [n=2] and abdominal distension [n=1]. One patient expired due to non surgical reason [heart failure]. OTEPT procedure is a safe method with low incidence of postoperative complications. In Hirschsprung's disease confined to the rectosigmoid region, the hazard and morbidities associated with laparatomy and colostomy are avoided with this technique

Omental-mesenteric myxoid hamartoma mimicking malignancy in a 14-month-old child [a case report]

The omental-mesenteric myxoid hamartoma [OMMH] is a very rare lesion, mainly seen in children and characterized by multiple omental and mesenteric nodules, which may be confused with malignant neoplasm. Microscopically, these lesions consist of a richly vascularized myxoid stroma with plump mesenchymal cells. This lesion has a benign clinical course without recurrence during follow up. We present a 14-month-old boy that was referred with history of abdominal distension, fever and vomiting for 3 months. Enhanced computed tomography [CT] revealed a huge well-demarcated hypodense and spherical mass which displaced bowel loops without obvious penetration to the intestinal walls. Histological and immunohistochemical examinations confirmed the diagnosis of OMMH. No evidence of recurrence was noted during 3 years follow up. OMMH is a very rare lesion and because of its aggressive appearance, differential diagnosis with malignancy is warranted. The clinical picture of our case also led to high suspicion of malignancy. However by consideration of histological and immunohistochemical findings we could achieve the correct diagnosis

ANCA-negative pulmonary-renal syndrome with pathologic findings suggesting thrombotic thrombocytopenic purpura

Background: Thrombotic thrombocytopenic purpura [TTP] is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. The disease classically spares the lung tissue, but recently some cases were reported that presented with respiratory involvement [adult respiratory distress syndrome or pulmonary-renal syndrome]. Presentation with pulmonary-renal syndrome has rarely been reported. Case Presentation: We report a 14-yaers-old boy admitted to the hospital because of a biochemical evidence of renal failure. Two days after admission he developed a high fever, hemoptysis and respiratory distress. Open kidney biopsy was performed and confirmed the diagnosis and finally the patient expired due to neurologic involvement. This case is presented to discuss the need to update the criteria for TTP diagnosis and considering TTP in the differential diagnosis of pulmonary renal syndromes

Cryptosporidium infection in pediatric patients with lymphohematopoietic malignancies

Cryptosporidium parvum is a common protozoan pathogen with worldwide distribution. It localizes on the intestinal cells and prolonged diarrhea in immunocompromised patients. The aim of this study was to estimate the prevalence and the clinical features of enteric cryptosporidiosis in pediatric patients with lymphohematopoietic malignancies. in this cross-sectional study stool samples were collected from 100 children [67 boys, 33girls] with lymphohematopoietic malignancies who underwent chemotherapy between the ages of 6 months and 17 years [mean age 7.5 years]. All of the specimens were examined for the oocysts of C. parvum by modified Ziehl Neelsen [MZN] staining technique and coproantigens of C. parvum by EL IS A. Cryptosporidium infection was detected in 22 patients. 16 [72.7%] of the infected patients were male and 6 [27.3] female. 7 [31.8%] patients were <5 years, 8 [36.4%] 5-10 years and 7 [31.8] >10 years old. Parasites were detected in 19/85 [86.4%] patients with ALL, 2 of 5 [9.1%] with AML, and 1 of 10 [4.5%] with NHL. Clinical symptoms were found in 11 [50%] of the patients. We found longer duration of chemotherapy in patients who were positive for Cryptosporidium infection [Mean=2067 days] in comparison to negative group [Mean=258.5 days] [ANOVA, f=2.82, P=0.04]. The incidence of Cryptosporidium infection was 22% among pediatric patients with lymphohematopoietic malignancies. We recommend evaluation of these patients with at least two different diagnostic methods in order to prevent possible life threatening outcomes

Pleuropulmonary blastoma: case report

Pleuropulmonary blastoma [PPB] is a rare and aggressive tumor that is emerging as a distinct entity of childhood disease .It is characterized by mesenchymal elements [including undifferentiated blastoma and often cartilaginous, rhabdomyoblastic, or fibroblastic differentiation] and epithelium-lined spaces. PPB may be exclusively cystic [type I], solid [type III] or both solid and cystic [type II]. A 5-month-old boy presented with a history of fever and respiratory distress. Chest radiograph and subsequent CT scan showed a large soft-tissue density occupying the left chest cavity. Radical resection of the mass was achieved by lobectomy. Histologic examination revealed PPB [type II]