Intact Choledochal Cyst With Spontaneous Common Hepatic Duct Perforation

Abstract

A 3-month-old girl admitted for lower respiratory infection developed constipation and abdominal distension 3 days later. On examination, she had mild abdominal distension with absent bowel sounds. Abdominal sonography showed a normal biliary tract and pancreas. Computed tomography of abdomen revealed minimal localized fluid collection in the right upper quadrant. Abdominal paracentesis done 24 hours later revealed bile-stained ascitic fluid. Exploratory laparotomy on the same day revealed intact cystic dilation of common bile duct (type 1). Operative cholangiography revealed leakage of contrast from the distal common hepatic duct with a 4-mm perforation that was sutured securely (Fig. 1). Complete excision of the cyst, cholecystectomy, Roux-en-Y hepatojejunostomy, and jejunojejunostomy were performed (Fig. 2).FIGURE 1: Sutured perforation site at the distal common hepatic duct.FIGURE 2: Excised intact choledochal cyst with gallbladder.Both spontaneous bile duct perforation and common hepatic duct perforation are rare in children (1). The unusual combination of common hepatic duct perforation coexisting with an intact choledochal cyst has not been reported previously. Clinical presentations of spontaneous perforation of bile duct and choledochal cyst are similar. Our case suggests a common underlying pathogenesis, and supports the theory that these 2 separate entities are related to weakness of the biliary canal wall during embryogenesis with pancreaticobiliary malunion (2,3). Pathobiology of spontaneous biliary perforation could be similar to that of choledochal cyst as is evident in the present case.