Spontaneous perforation of right hepatic duct in a child with choledochal cyst

A 5-year-old girl with choledochal cyst (Alonso Lej, type I) and spontaneous perforation of the right hepatic duct during an attack of acute pancreatitis was treated by two-stage surgery. Biliary panperitonitis was diagnosed by abdominal paracentesis, bilirubin and amylase levels in the collected fluid being 41.3 mg/dl and 1812 Somogyi U, respectively. The initial surgery involved simple peritoneal drainage and decompression of the bile duct, followed by removal of a choledochal cyst and the common hepatic bile duct, including the biliary stricture of the left hepatic duct, and biliary reconstruction 5 months later. Cholangiogram after the initial surgery demonstrated pancreaticobiliary maljunction and stricture in the left hepatic duct and the common hepatic duct. Regurgitation of pancreatic juice into the bile duct was shown by the extremely high amylase level (90100 Somogyi U) in the bile drained through a T-tube. The patient is currently well, with normal liver and pancreatic function tests. The experience in this patient is significant in that it should be known that spontaneous perforation of the bile duct may occur at the right hepatic duct in choledochal cyst, causing biliary peritonitis.