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.
The Middlesbrough youngster suffers from a variety of conditions including portal hypertension, congenital hepatic fibrosis, polycystic kidney and Caroli 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.
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).
Of the fibropolycystic liver diseases, patients with congenital hepatic fibrosis and Caroli disease
often develop recurrent acute cholangitis and portal hypertension secondary to peribiliary fibrosis (Figure 1).
5-8) More than 300 cases of Caroli disease
and Caroli syndrome (Caroli disease
associated with congenital hepatic fibrosis) have been reported in the world literature, (9) and cholangiocarcinoma has been found in 7% to 14% of the cases.
Last year, Marley Gill was diagnosed with portal hypertension, congenital hepatic fibrosis, polycystic kidney disease and Caroli disease
- serious genetic conditions affecting his liver and kidneys.
5] Females are more prone to Caroli disease
represents the Type V Todani choledochal cyst; communicating cavernous ectasia of the IBDs.
An association with congenital abnormalities of the biliary tree, such as a choledochal cyst or Caroli disease
, and with polyposis cull and ulcerative colitis has previously been reported.