Choledochal Cyst

Abstract

Choledochal cysts are congenital, cystic dilatations of the biliary tree that can occur with an incidence of approximately 1:100,000–1:150,000 in the Western hemisphere and a reported incidence of 1:1,000 in the Asian population. The majority of patients with choledochal cysts remain asymptomatic and diagnosis is made due to an incidental finding. Only a minority presents with clinical signs of jaundice/obstruction, cholangitis/pancreatitis, or an acute abdomen due to secondary complications. Diagnostics include abdominal ultrasound and blood tests, an MRCP can further specify the anatomy if necessary and in cases of acute obstruction an ERCP with stenting is required. Choledochal cysts are premalignant conditions; therefore, the therapy consists of a complete choledochal cyst excision, early after diagnosis. This can be performed using “open” or “laparoscopic” surgery. The reconstruction afterwards includes a biliodigestive anastomosis, which can be either a hepatico-jejunostomy with a Roux-en-Y loop, or a hepatico-duodenostomy. Currently, there is no evidence for the superiority of any of these techniques. After choledochal cyst excision the lifetime risk for malignancies is reduced, but recent data suggest a lifelong elevated risk of up to 11% of cancer development even following operation. Therefore, lifelong follow-ups are necessary.