*2.5.4 Surgical management*

Given the risk of cholangiocarcinoma, highest in types I and IV, surgical intervention is indicated at the time of diagnosis of any type of choledochal cyst. The approach depends on type of lesion. For type 1 cysts, primary cyst excision with cholecystectomy and roux-en-Y hepaticojejunostomy reconstruction is procedure of choice. Type 2 disease is managed by simple diverticulectomy. Type 3 is managed by transduodenal cyst excision or marsupialization and sphincteroplasty. Types 4 and 5 may be treated by anatomic hepatic resection based on the extent and location of disease, however, liver transplantation is ultimately required in most cases.
