Aims: To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years. Materials and Methods: Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management. Results: Initial examination of the external genitalia showed a "clover-leaf" appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a "high" position (n = 14) or at a relatively normal or "low" position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls. Conclusions: GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.

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Congenital duodenal obstruction is traditionally managed by laparotomy. Laparoscopic duodenoduodenostomy (LDD) in neonates is considered a technically demanding operation requiring advanced pediatric laparoscopic skills. To the best of our knowledge, there are no reports of LDD from the Indian subcontinent. Here, we report two cases of successful LDD in neonates and review the relevant literature.

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Introduction: Vascular complications are very common in pediatric living donor liver transplants. We present our experience in vascular complications in biliary atresia patients undergoing liver transplantation. Materials and Methods: All the patients who have undergone living donor liver transplant for biliary atresia from January 2003 to March 2013 were retrospectively analyzed. P value < 0.05 was considered to be statistically significant. Results: Total 110 patients have undergone living donor liver transplantation for biliary atresia between January 2003 and March 2013. There were 56 males and 54 females. Median age at transplant was 13.5 months. Eleven were primary transplants and 99 were post KASAI procedure. One hundred left lateral, four left lobe, and four right lobe grafts were used. Twenty-two patients developed vascular complications. Twelve patients developed hepatic artery thrombosis. Eleven patients of hepatic artery thrombosis were managed with redo hepatic artery anastomosis and one patient managed with radial artery interposition graft. Five patients developed portal vein stenosis and were managed by portal vein stenting. Five patients developed portal vein thrombosis and portal vein thrombectomy and re-anastomosis were done. One patient developed stenosis at the site of venous anastomosis and was managed by stenting. One patient developed both hepatic artery thrombosis and portal vein thrombosis and eventually succumbed to these complications. Out of five cases who died in this study, two had vascular complications. Graft/recipient weight ratio (GRWR) greater than 2.5 was significantly associated with vascular complications (P = 0.017). Conclusion: Vascular complications are frequently seen in liver transplantation for biliary atresia. Large for size grafts, weight less than 10 kg, age less than 1 year, and prolonged warm ischemia time is significantly associated with vascular complications.

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We present a low-birth weight newborn who presented with bilious vomit and had a "double bubble" sign on plain abdominal X-ray. Intraoperatively, we found annular pancreas with malrotation and type I choledochal cyst. To the best of our knowledge, this is the first reported case with a combination of these anomalies.

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Background and Aims: Symptomatic portal hypertension (PHT) as a complication of the choledochal cyst (CDC) is well-known, but the actual incidence of PHT in CDC has not been studied. This study was undertaken to evaluate the incidence of PHT in patients of CDC and correlate portal pressure (PP) with liver histology and blood nitric oxide (NO) levels. Materials and Methods: In this cross-sectional study, PP was measured after surgical access but before any mobilization of the cyst by directly cannulating a tributary of portal vein (preoperative PP) and at completion of surgery before closure (postoperative PP). Blood sample for NO and liver function tests (LFTs) was taken before surgery and during subsequent follow-up at 1-month, 3 months, and 6 months. Liver histology was assessed under parenchymal, bile duct, and portal parameters. Results: Measurement of PP and blood levels of NO was done in 20 patients. Mean preoperative PP was 16.45 ± 7.85 mmHg, and the median pressure was 14 mmHg (range 9-43). Mean of the postoperative PP was 14 ± 6.87 mmHg, and median pressure was 11.5 mmHg (range 7-37). The mean level of NO in the preoperative period was 11.85 ± 4.33 μmol/l, and median was 11.605 (range 5.24-22.77) μmol/l. NO levels at the first follow-up (1-month postoperative) were 5.96 ± 4.56 μmol/l and median value of 4.9 (range 1.74-23.56) μmol/l. Likewise, the mean and median values of NO at 3 months were 5.59 ± 7.15 μmol/l and median value of 3.71 (range 1.49-34.74) μmol/l. The mean and median levels of NO at 6 months postoperative were 5.08 ± 2.22 μmol/l and median of 4.59 (range 2.32-12.46) μmol/l. The fall in PP immediately after surgery was consistent and statistically significant (P = 0.001). There was statistically significant fall in the NO levels in the postoperative period as compared to the preoperative levels (P = 0.002). Bile duct proliferation was significantly correlated with PP (P = 0.05). Blood levels of NO closely followed the PP in the preoperative period and fell to baseline in subsequent follow-up. There was no statistically significant correlation between age at presentation, LFT and postoperative complications with either PP or NO levels. Conclusions: In this study, all patients with CDC had some degree of PHT. Measurement of PP and liver histology should be part of standard management protocol to take timely preventive measures so as to avoid life-threatening manifestations of PHT.

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Congenital pouch colon (CPC) in the female patient presents with highly variable and anomalous anatomy. We herein report the first case of CPC with uterus didelphys having normal anal opening, H-type vestibular fistula, two other fistulous communications between pouch colon and two vagina managed in a single stage with excellent postoperative outcome.

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Background: Though the outcomes in operated children with anorectal malformation (ARM) have greatly improved, postoperative soiling and constipation remain major issues. Among the various factors described for poor outcomes; misplaced bowel, hypoplastic sphincters and obtuse anorectal angle bear special mention. The aim of this study was to compare the stooling outcomes, type of anomalies and surgical procedure with postoperative pelvic magnetic resonance imaging (MRI). Materials and Methods: This was a cross-sectional study involving operated children of ARM who had at least 2 years of follow-up, and who were at least 3 years of age. The subtypes of ARM, surgical procedures, and functional outcomes were documented using the Krickenbeck classification. All children were subjected to a pelvic MRI. Results: Thirty-three eligible children were part of this study. Twenty-two patients underwent posterior sagittal anorectoplasty, seven patients underwent abdominoperineal pull-through (APPT) and four patients underwent perineal operations. Local abnormalities were present in 66% patients, and 34% had abnormalities of the spine detected on MRI. Poorer stooling outcomes were twice as common in children with local pelvic MRI abnormalities as compared to asymptomatic children. The highest incidence of local abnormalities were seen in patients treated with APPT (P = 0.0001). No significant difference in the pelvic MRI was seen among children who were constipated and those who had soiling. Conclusion: MRI is a useful imaging modality in operated children of ARM with poor stooling outcomes. Local abnormalities were the most common in children undergoing abdominoperineal pull-through procedure.

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Aim: The differentiation between neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA) is not always possible despite all the currently available diagnostic modalities. In this study, an attempt has been made to evaluate the role of nitric oxide (NO) levels in the peripheral blood to differentiate between the two conditions, one requiring early surgical intervention (EHBA) and the other amenable to conservative medical management (NH). Patients and Methods: Twenty patients who presented to the pediatric surgical service, over a 2 years period, with features of neonatal cholestasis were enrolled in the study. The diagnostic workup included documentation of history and clinical examination, biochemical liver function tests, ultrasonography, hepatobiliary scintigraphy (HS), and magnetic resonance cholangio-pancreaticography (MRCP). These patients did not show excretion on HS and intrahepatic ducts on MRCP. Hence, they were subjected to mini-laparotomy and operative cholangiography (OC). The EHBA patients were treated with the Kasai's portoenterostomy procedure, and the extrahepatic ducts were flushed with normal saline in NH patients. All patients were evaluated preoperatively for levels of NO in the peripheral blood by the Greiss reaction spectrophotometrically at 540 nm. Normal values were determined from a cohort of controls. The median (range) levels of NO in patients with EHBA and NH were compared, and the statistical significance of the difference was calculated by applying the Wilcox Rank Sum test. A P = 0.05 was considered as significant. Results: Of the 20 patients enrolled in the study, 17 patients were treated for EHBA (Group I) and the remaining 3 patients had patent ducts on OC and were thus diagnosed as NH (Group II). The mean age of the patients in Groups I and II was comparable: 2.79 ± 0.75 and 2.67 ± 0.58 months, respectively (P = 0.866). The median NO levels were significantly elevated in each of the two groups as compared to the controls (5.6 μmol/l, range 1.26-11.34 μmol/l); when compared among themselves, the NO levels were significantly higher in Group I, 64.05 μmol/l (range 24.11-89.43 μmol/l), when compared with Group II, 41.72 μmol/l (range 23.53-45.63 μmol/l) (P = 0.022). Conclusion: The serum levels of NO were found to be significantly higher in patients with EHBA as compared to those with NH. Hence, this may be a useful biochemical marker for the preoperative differentiation of EHBA from NH. However, a larger study is required for establishing the validity of the statistical significance.

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Colonic atresia is the rarest entity among the all intestinal atresias and may be associated with anterior abdominal wall defect, small intestinal atresia, Hirschsprung's disease, and other anomalies. Here, we are reporting a case of colonic atresia associated with annular pancreas, which has not been reported previously to the best of our knowledge.

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The case of a 3-year-old boy diagnosed to have Hirschsprung's disease with infantile nephropathic cystinosis is being reported. Both these conditions are etiologically and genetically different as per current understanding and available information. The association is incidental and has not reported before in the English literature.

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Aims: The aim was to compare biliary amylase, common channel, and gall bladder/liver histopathology between spherical and fusiform choledochal cysts. Materials and Methods: Children undergoing cyst excision with Roux-en-Y hepatico-jejunostomy over a 17 months period were prospectively studied. The common channel was assessed by three-dimensional (3D) magnetic resonance cholangiopancreatography (MRCP). Results: Among 22 patients (spherical = 10, fusiform = 12), there was a higher incidence of spherical cysts in infants (5/7-71.4%) and fusiform cysts in older children (10/15-66.7%) (P = 0.09). Common channel identified in 14 (64%) cases was long (>10 mm) in 5 (38.2%) (one spherical, four fusiform [P = 0.5]) with associated high biliary amylase levels (>500 IU/L) in four (one spherical, three fusiform) (P = 0.05). Exact point of junction of common bile duct with pancreatic duct was visible with coronal half-Fourier-acquisition single-shot turbo spin-echo and 3D sampling perfection with application with optimize contrast using different flip angle evolution sequence using a reformatted plane of axis, particularly coronal, and coronal oblique orientation. Biliary amylase was raised (>100 IU/L) in 4 (40%) in the spherical group, compared to 8 (67%) in the fusiform group. Mean biliary amylase was similar in gallbladder and cyst in spherical but higher in gallbladder in fusiform cysts. Mean biliary amylase was <500 IU/L in 85.7% infants. Five out of 7 infants had liver fibrosis or cirrhosis on histopathology (P = 0.05). There were no dysplastic changes in the gallbladder epithelium. Conclusion: Three-dimensional MRCP delineated the common channel in two-third cases especially in coronal and coronal oblique orientation. The long common channel may have an etiological role in fusiform cysts. Spherical cysts, especially in infants, have a higher incidence of obstructive cholangiopathy.

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We report a case of 14-year-old male, who presented to us with complaints of severe pain in abdomen, vomiting, and inability to pass feces and flatus. He was diagnosed as a case of peritonitis after careful history, examination, and investigations. The exact cause of peritonitis was not known. Exploratory laparotomy was done, and it was found that there was perforation of the right hepatic duct about 1 cm proximal to its confluence with the left hepatic duct. Perforation was closed around the T-tube. Postoperative T-tube cholangiogram was done after 3 weeks which confirmed the free passage of dye into the duodenum, and there was no leakage of dye. T-Tube was removed 4 weeks after the operation, and the patient was discharged in satisfactory condition. Postoperative follow-up was done for 3 months, and it was uneventful.

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Colonic duplications are rare congenital anomalies. Treatment of choice is complete resection that in case of a long tubular duplication requires total or subtotal colectomy. A simple surgical technique for treatment of complete colonic duplication is described, which avoids the complications of extensive colonic resection.

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