Management of congenital choledochal cyst with intrahepatic involvement.

The clinical features and surgical treatment of 75 patients with choledochal cysts with intrahepatic involvement (Type IV-A) were evaluated. Seventeen were in this series and 58 were reviewed from the literature. It was of interest that the incidence of Type IV-A choledochal cysts was nearly 30%, considerably higher than had been previously assumed. This type is commonly found in older children and young adults. Abdominal pain and fever indicative of biliary tract infection are more frequent seen than in patients with Type I, and a mass is not commonly palpable. Surgical treatment of Type IV-A must achieve bile drainage from the intrahepatic cysts as well as from the choledochal cyst. For this reason, as well as the prevention of later development of cancer in the wall of the cyst, hepaticoenterostomy at the porta hepatis with partial resection of the wall of the intrahepatic cyst (or partial hepatectomy if possible) combined with excision of the intrahepatic cyst is recommended as the procedure of choice for type IV-A cysts.