Unusual case of acute cholangitis in adults: congenital cystic dilatation of the bile ducts

We report 20 year old woman who was admitted with an acute cholangitis with a history of abdominal pain since 12 month. Clinical examination found the Charcot's cholangitis triad: jaundice, fever and right upper quadrant abdominal pain. Laboratory studies revealed an elevated leukocyte (14 000/mm3), cholestasis with elevated total bilirubin value 139 mg/l and direct bilirubin 85 mg/l. An abdominal ultrasound demonstrated a cystic dilatation of the extra and intra hepatic biliary tree. An MRI showed a malformed cystic dilatation of the intrahepatic bile ducts and the common bile duct (Todani type IVa). We performed a laparotomy by subcostal incision, a cholecystectomy with side-to-side anastomosis between the choledochal cyst and the second portion of the duodenum were made. The patient suffered of an early biliary fistula treated spontaneously with any medical or surgical intervention. She was discharged 7 days after the surgery. The outcome until 10 months after still uneventful.