Spontaneous perforation of the bile duct

We present a classic but rare case of spontaneous perforation of the bile duct in infancy and a previously undescribed treatment technique. The patient, a male age 5 weeks, was admitted with abdominal distention, ascites, and conjugated hyperbilirubinemia. Ultrasound revealed ascites but did not provide visualization of the gallbladder. Although hepatobiliary scintigraphy with technetium [dimethyl iminodiacetic acid (HIDA scan)] showed normal uptake peritoneal excretion suggested perforation of the common bile duct (CBD). Exploratory laparotomy revealed 200 cm3 dark amber ascitic fluid in the peritoneal cavity and cholestasis of the liver. Intraoperative cholangiogram performed via the gallbladder showed a large perforation at the cystic duct/CBD junction. The perforation was large and leakage of contrast prevented demonstration of the distal CBD despite our attempt to primarily repair the perforation. The CBD was explored; a T-tube was placed. T-tube cholangiogram demonstrated flow of contrast into the duodenum. A large leak remained at the cystic CBD junction. A cholecystectomy was performed and a vascularized flap of the gallbladder wall was used to repair the CBD over the T-tube. The T-tube was clamped intermittently beginning 3 weeks postoperatively. T-tube cholangiogram performed 6 weeks postoperatively revealed no extravasation and normal intra- and extrahepatic biliary tree. The T-tube was subsequently discontinued and liver function tests remained normal at 6 months follow-up.