Choledochal cysts

Abstract

Choledochal cyst is a rare congenital anomaly that left untreated can progress to cholangitis and biliary cirrhosis. Most commonly grouped by the Todani classification, five types of choledochal cysts have been described with types I and IV being the most common. Signs at presentation may include jaundice, fever, abdominal pain, and a right upper quadrant mass. Because of the widespread use of perinatal ultrasound, these biliary anomalies may also be diagnosed antenatally. Surgical management of a choledochal cyst should proceed in a timely fashion to avoid cholangitis, pancreatitis, and the small risk of cirrhosis. The goal of surgical intervention is complete cyst excision and biliary bypass. Cyst excision with roux-en-Y hepaticojejunostomy is generally effective for types I, II, and IV choledochal cysts. Type III cysts (choledochocele) may be treated using endoscopic sphincterotomy. Patients with type V cysts (Caroli’s disease) require partial hepatectomy or transplantation depending on the extent of biliary abnormalities.