Case Of the Week (COW) 13 September 2015
Caroli ‘s disease , Splenomegaly with Portal hypertension , Ascites Features of Caroli ‘s syndrom (caroli ‘s disease and congenital hepatic fibrosis)
Findings
Dilated intra hepatic biliary ducts with multiple intraductal calculi. Splenomegaly is present. Splenic vein appear enlarged. Multiple collaterals are noted in splenic hilum, spleno renal collaterals , coronary vein collaterals are present. Portal vein enlarged at its origin. Portal vein appear small in portal hilum. Ascites is present
Discussion:
Caroli disease is characterized by multifocal segmental dilatation of the large intrahepatic bile ducts, which retain their communication with the biliary tree. The inheritance is autosomal recessive. Two types of Caroli’ disease Caroli disease proper Caroli’s syndrome (Caroli disease with congenital hepatic fibrosis)
EMBRYOLOGY: A ductal plate is defined as a cylindric layer of cells that surrounds a branch of the portal vein and that develops during the 1st week of gestation. Biliary ducts are normally formed from remodeling and partial involution of these cylindric ductal plates Caroli disease is the result of ductal plate malformation of the large intrahepatic bile ducts . Caroli syndrome is due to the arrest of remodeling both in bile duct embryogenesis (early )and in development of the peripheral biliary ramification
RADIOLOGY: Saccular or fusiform cystic dilatations of the intrahepatic bile ducts up to 5 cm in diameter, often containing calculi or sludge. Contrast-enhanced CT or gadolinium-enhanced MR imaging shows the central dot sign corresponds to a portal vein branch protruding into the lumen of a dilated bile duct or a linear hyperattenuating or high-signal-intensity focus containing a portal vein and bridging the cyst wall in carolis disease. In Caroli’s syndrome imaging shows cystic biliary dilatation along with features of congenital hepatic fibrosis such as right lobe atrophy with normal left medial segment and hypertrophic left lateral segment (be useful in distinguishing congenital hepatic fibrosis from cirrhosis) and portal hypertension Complications: Caroli’s disease : Cholangitis or jaundice, Caroli’s syndrome: Hematemesis due to ruptured esophageal varices.
Contributed By:
Prof. S Babu Peter, Dr. Geetha, Dr. Dheebha V
Barnard Institute of Radiology
Caroli ‘s disease , Splenomegaly with Portal hypertension , Ascites Features of Caroli ‘s syndrom (caroli ‘s disease and congenital hepatic fibrosis)
Findings
Dilated intra hepatic biliary ducts with multiple intraductal calculi. Splenomegaly is present. Splenic vein appear enlarged. Multiple collaterals are noted in splenic hilum, spleno renal collaterals , coronary vein collaterals are present. Portal vein enlarged at its origin. Portal vein appear small in portal hilum. Ascites is present
Discussion:
Caroli disease is characterized by multifocal segmental dilatation of the large intrahepatic bile ducts, which retain their communication with the biliary tree. The inheritance is autosomal recessive. Two types of Caroli’ disease Caroli disease proper Caroli’s syndrome (Caroli disease with congenital hepatic fibrosis)
EMBRYOLOGY: A ductal plate is defined as a cylindric layer of cells that surrounds a branch of the portal vein and that develops during the 1st week of gestation. Biliary ducts are normally formed from remodeling and partial involution of these cylindric ductal plates Caroli disease is the result of ductal plate malformation of the large intrahepatic bile ducts . Caroli syndrome is due to the arrest of remodeling both in bile duct embryogenesis (early )and in development of the peripheral biliary ramification
RADIOLOGY: Saccular or fusiform cystic dilatations of the intrahepatic bile ducts up to 5 cm in diameter, often containing calculi or sludge. Contrast-enhanced CT or gadolinium-enhanced MR imaging shows the central dot sign corresponds to a portal vein branch protruding into the lumen of a dilated bile duct or a linear hyperattenuating or high-signal-intensity focus containing a portal vein and bridging the cyst wall in carolis disease. In Caroli’s syndrome imaging shows cystic biliary dilatation along with features of congenital hepatic fibrosis such as right lobe atrophy with normal left medial segment and hypertrophic left lateral segment (be useful in distinguishing congenital hepatic fibrosis from cirrhosis) and portal hypertension Complications: Caroli’s disease : Cholangitis or jaundice, Caroli’s syndrome: Hematemesis due to ruptured esophageal varices.
Contributed By:
Prof. S Babu Peter, Dr. Geetha, Dr. Dheebha V
Barnard Institute of Radiology