Surgical treatment of type IV choledochal cysts.

The benefit of total resection of the dilated bile duct has remained unclear. We describe here our surgical management of 13 patients with type IV choledochal cysts. All six younger patients (25-35 years old) underwent resection of the extrahepatic bile duct (EHBD) and hepaticojejunostomy (HJ), whereas three of the seven older patients (50-68 years old) underwent resection of the EHBD resection and HJ, with the remaining four older patients undergoing total resection of the dilated bile duct and removal of a pancreatobiliary maljunction (PBMJ) in the form of a S4a+S5 hepatectomy (so-called Taj Mahal) and/or pancreas head resection with second portion pancreaticoduodenectomy. No malignancies were detected in the dilated bile duct after resection in the younger patients, but cancer of the gallbladder and/or the dilated bile duct was found in two (27.5%) of the older patients. No cancers were detected during the long-term follow up (1974-2008) in those patients who underwent EHBD resection plus partial hepatectomy, but cancer developed in the remnant duct in one of the older patients who underwent EHBD resection alone. Based on our findings, we recommend that type IV choledochal cysts should be treated by total excision of the dilated bile duct, including the PBMJ, due to its frequent association with malignancy, and to prevent the development of cancer in the remnant duct and improve the long-term survival rate.