Investigations generally reveal cholestatic jaundice serum b

Investigations generally reveal cholestatic jaundice (serum bilirubin < 8 mg/dL) with near-normal transaminase levels. Abdominal USG can be a useful adjunct to diagnosis (generalized ascitis or localized collection in gall bladder fossa); however, confirming the diagnosis preoperatively is difficult. Absence of pneumoperitoneum on erect abdominal radiography adds to the difficulty in diagnosis. Abdominal CT scan reveals the same findings as ultrasound does, and perforation is extremely difficult to detect. CT, magnetic resonance cholangiopancreatography, cholangiography, 99mTc diisopropyl iminodiacetic AG 013736 cholescintigraphy, or hepatobiliary scintigraphy may confirm the diagnosis. Scintigraphy reveals radioactivity in the free peritoneal cavity. These methods are not feasible in most rural and peripheral areas of India, making preoperative diagnosis difficult. Herein, we describe the importance of meticulous clinical examination and erect abdominal radiography. Although routine abdominal paracentesis is not recommended in infants and children, we suggest that in a jaundiced child, the diagnosis of spontaneous biliary perforation should be considered if there is a triad of: (1) signs of peritonitis; (2) absence of pneumoperitoneum on erect abdominal radiography; and (3) bilious abdominal paracentesis under ultrasonographic guidance. Prompt recognition and surgical management is mandatory before infection because the condition may be fatal if untreated. A laparoscopic technique should be considered first if possible. Sterile biliary ascitis and bile staining are encountered with flakes over the site of perforation. The most common site of perforation is at the junction of the cystic duct and CBD, seen as a hole in the anterior aspect of the CBD. Perforation of the left intrahepatic bile duct has been reported. Perforation is typically small and allows a slow efflux of bile, and may present with pseudocysts. The optimal strategy is to drain the area and place a T-tube through the perforation initially, because the presumed obstruction causing the perforation is inspissated bile or biliary sludge, which resolves by itself after recovery from the acute state. The T-tube also facilitates the postoperative assessment of the anatomy of the biliary tree and APBDJ, and also eliminates choledochal cyst. Few studies have performed initial cholecystostomy drainage. A surgical cholangiogram or an intraoperative cholangiogram (if feasible) is performed to rule out APBDJ, although it was not feasible in our case. This method delineates intra- and extrahepatic biliary system, filling defects, free flow into the duodenum, and extravasation of dye if present. The healing time (perforation seals with drainage) is ∼4 weeks (supported by our case) after which the T-tube is removed. Aggressive surgical intervention to suture the site of perforation is avoided because the bile duct is friable and congenitally weakened, although primary repair without any external drainage has been reported. Successful management by excision of the gall bladder and CBD through Roux-en-Y hepaticojejunostomy in patients with APBDJ has been reported; however, this carries a high morbidity and mortality risk because the patients are dehydrated and malnourished. Finally, surgical treatment always depends on the clinical findings during examination and treatment should be individualized according to case. In addition, APBDJ is associated with an increased risk of choledochal cyst and malignant degeneration in later life. Hence, frequent follow-up in later life is crucial.
Introduction Colonic atresia (CA) is a rare entity with an incidence of one in 20,000 live births and accounts for approximately 1.8–15% of intestinal atresia. Neonates with CA typically present with predictably marked and progressive abdominal distension within 24–48 hours after birth. The neonate typically passes little or no meconium. Hirschsprung disease (HD), a congenital intestinal neuropathy characterized by the absence of ganglion cells extending from the rectum proximally for a variable distance, presents with the failure to pass meconium within 48 hours after birth and often progresses to abdominal distension and vomiting. We report a case of type I sigmoid atresia misdiagnosed as HD in the neonatal period. Diagnosis was missed even on a contrast enema in the postoperative period. The atresia (web) was later diagnosed when colonoscopy was performed to relieve the suspected fecal impaction after colostomy closure. Such complications emphasize the importance of a high index of suspicion for this condition.