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.