Spontaneously ruptured choledochal cyst: Rare diagnosis on hepatic scintigraphy
A 47-day-old female infant with jaundice from Leh, India, was referred for a Tc-99m HIDA scan to rule out extrahepatic biliary atresia (EHBA). There was no history of direct or indirect trauma. On day 2 of life, the child developed fever and had a seizure. Over the next few days, she started having jaundice, distension of the abdomen, acholic stools and was found to be anemic. The child was treated as a case of ABO incompatibility with phototherapy in view of mother's blood group “O” and baby's “B.” Subsequent laboratory examination revealed conjugated hyperbilirubinemia (total 6.8 mg%, conjugated 5.4 mg%). Other hepatic and renal biochemical functions were mildly deranged. Serology for HBsAg and HIV was negative. First ultrasound (USG) on 12.08.10 revealed? portal venous thrombosis with cirrhosis, ascites and mass in relation to segment VI of the liver. Repeat USG on 03.09.10 showed cystic lesion in the region of porta hepatis with diagnosis of choledochal cyst and differential diagnosis of enteric duplication cyst. The child was treated with antibiotics and therapeutic ascitic fluid tap due to respiratory distress. Tc-99m HIDA scan was ordered to rule out EHBA, which showed adequate tracer uptake by the liver in 5 min. A photopenic area appeared at porta hepatis which subsequently Accumulated and held the activity []. Delayed imaging at 2 h showed slowly rising activity in the ascitic fluid []. This was indicative of choledochal cyst with leak into the peritoneal cavity. Immediate laparotomy was done and the leak was identified in a fusiform dilatation of the extrahepatic biliary duct with gall bladder opening into it (Type I). T-tube was placed into the perforation. The child recovered from the sepsis with biliary diversion and antibiotics, and was planned for definitive surgery. Choledochal cyst rupture is managed with major excision–reconstruction procedure unlike spontaneous perforation of the extrahepatic bile duct which is managed by temporary tube cholecystectomy or subhepatic drainage till subsidence of the biliary leak.[]