Partial Hepatectomy and Total Cyst Excision is Curative for Localized Type IV-A Biliary Duct Cysts – Report of Four Cases and Review of Management

Abstract

With the increasing use of newer modalities such as CT cholangiography and MRCP offering an accurate delineation of the ductal anatomy of choledochal cysts, the incidence of type IV-A biliary duct cysts has increased. Although the management of the more common type I cysts is well established, that of type IV-A cysts is still controversial. The localized/unilobar variety of type IV-A cysts is a unique entity amenable to curative surgical management. Between Jan 2000 and Jan 2005, 10 of 25 cases with choledochal cysts were diagnosed as having type IV- cysts. Four of these were localized or unilobar variants affecting only one lobe of the liver. Three cases had a left ductal involvement and one had a right ductal involvement of their intrahepatic component. We describe the presentation and curative surgical management of these patients with a unilobar variety of type IV-A cysts and discuss the management options for type IV-A cysts in the literature. One child with previous surgical treatment for presumed type 1 cyst, diagnosed as having a type IV-A right ductal cyst complicated with liver abscess, underwent right hepatectomy, two children had a left hepatectomy and one child had a left lateral lobectomy. All four children had uncomplicated intraoperative and postoperative courses. After 4-8 years' follow-up, all are symptom-free. There were no biliary tract or hepatic abnormalities on radiological surveillance with MRCP. In some series, the incidence of type IV-A choledochal cysts is equal to or higher than that of type 1 cysts. MRCP and intraoperative cholangiogram can help to confirm the anatomical varieties and associated ductal strictures. In the unilobar/uniductal varieties, complete cyst excision can be safely achieved in children through an additional hepatectomy/lobectomy. This can result in a curative status, leaving the child free from complications such as cholangitis, abscess, hepatolithiasis and the risk of biliary duct malignancy.