Intrahepatic Choledochal cyst (Type V) with Biliary Cirrhosis

Choledochal cyst are congenital bile duct anomalies.These cystic dilatations of the biliary tree can involve extrahepatic biliary radicles,intrahepatic biliary radicles,or both.

AGE: Most patients with choledochal cysts are diagnosed during infancy or childhood, although the condition may be discovered at any age

SEX:Choledochal cysts are more prevalent in females than males, with a female-to-male ratio in the range of 3:1 to 4:1.

CLASSIFICATION –  By Todani and coworkers.

Type I cysts are the most common and represent 80-90% of choledochal cysts. They consist of saccular or fusiform dilatations of the common bile duct, which involve either a segment of the duct or the entire duct.

Type IA is saccular in configuration and involves either the entire extrahepatic bile duct or the majority of it.

Type IB is saccular and involves a limited segment of the bile duct.

Type IC is more fusiform in configuration and involves most or all of the extrahepatic bile duct.

Type II choledochal cysts appear as an isolated diverticulum protruding from the wall of the common bile duct. The cyst may be joined to the common bile duct by a narrow stalk.

Type III choledochal cysts arise from the intraduodenal portion of the common bile duct and are described alternately by the term choledochocele.

Type IVA cysts consist of multiple dilatations of the intrahepatic and extrahepatic bile ducts. Type IVB choledochal cysts are multiple dilatations involving only the extrahepatic bile ducts.

Type V (Caroli disease) consists of multiple dilatations limited to the intrahepatic bile ducts.

COMPLICATIONS: Infants and children may develop pancreatitis, cholangitis, and histologic evidence of hepatocellular damage.

Adults in whom subclinical ductal inflammation and biliary stasis may have been present for years may present with one or more severe complications such as hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, and cholelithiasis. Cholangiocarcinoma is the most feared complication of choledochal cysts, with a reported incidence of 9-28%.