with portal hypertension, congenital hepatic fibrosis, Caroli disease
and polycystic kidney disease.
Congenital hepatic fibrosis is one of the fibropolycystic diseases, which also includes Caroli disease
. The pathophysiology of Caroli Syndrome involves the presence of a progressive liver fibrosis and intrahepatic ductal dilatation that predisposes patients to portal hypertension, ascites, and biliary lithiasis .
Ductal plate is the embryological precursor of the biliary system and its malformations causes abnormal branching of the intrahepatic portal veins and progressive fibrosis of the portal tracts.1 In 1856, the liver-related clinical presentations were first described2 and in 1961, Kerr described different clinical manifestations of CHF.3 It belongs to the disorders grouped as fibropolycystic diseases (having abnormally high degree of fibrosis and presence of cysts in liver and kidneys) which include Caroli disease
, autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD).
The last group included intrahepatic paucity of bile ducts, Caroli disease
, veno-occlusive disease, alpha-1 antitrypsin deficiency and steatosis.
The differential diagnosis for simple cysts includes benign developmental hepatic cysts, biliary hamartomas, foregut cysts, Caroli disease
, and adult polycystic liver disease.
Additionally, exposure to chemical compounds, such as naphthenic acids, primary sclerosing cholangitis, anabolic steroids, ulcerative colitis, intrahepatic lithiasis, Caroli disease
and multiple liver cysts are other recognized predisposing factors for the development of cholangiocarcinomas in humans (AL-BAHRANI et al., 2013).
Liver involvement is a result of congenital hepatic fibrosis or Caroli disease
, which may progress to portal hypertension, hypersplenism with resultant thrombocytopenia and recurrent ascending cholangitis (Caroli syndrome).
Finally, the fibropolycystic liver diseases include various disorders, including congenital hepatic fibrosis, Caroli disease
, autosomal recessive polycystic kidney disease (ARPKD), and biliary hamartoma.
(5,6) Cholangiocarcinoma has been reported to occur as a complication of Caroli disease
, an extremely rare congenital disease of the intrahepatic biliary tree characterized by segmental cystic dilatation of the larger intrahepatic bile ducts, recurrent bacterial cholangitis, and biliary lithiasis.
The Middlesbrough youngster suffers from a variety of conditions including portal hypertension, congenital hepatic fibrosis, polycystic kidney and Caroli disease
. Chronic illness stops Marley from doing everyday things as a little boy, and he can no longer play football with his friends.
 Females are more prone to Caroli disease
This sign is highly suggestive of Caroli disease