Fibropolycystic diseases

These diseases are usually inherited and lead to the presence of cysts or fibrosis in the liver, kidney and occasionally the pancreas, and other organs.

Polycystic disease of the liver


This usuaUy presents in middle age with abdominal swelling or right hypochondrial discomfort. It can also be detected by ultrasound scanning or may only be discovered at autopsy. There mayor may not be hepatomegaly and bilateral irregular palpable kidneys. It is inherited as an autosomal dominant. The cysts are of variable size and consist of thin-walled cavities containing clear fluid or altered blood. Liver function is normal and complications such as oesophageal varices are very rare. The prognosis is excellent and is often dependent on whether the kidneys are involved.


Childhood polycystic disease is inherited in a different way from the adult type. It is an autosomal recessive condition presenting in the first few months of life. Renal involvement is common, with cystic changes in the renal tubules.

Congenital hepatic fibrosis

In this rare condition the liver architecture is normal but there are broad collagenous fibrous bands extending from the portal tracts. It is often inherited as an autosomal recessive condition but can also occur sporadicaUy. It usuaUy presents in childhood with hepatosplenomegaly, and portal hypertension is common. It may present later in life and can be misdiagnosed as cirrhosis. A wedge biopsy of the liver may be required to confirm the diagnosis. The outlook is good and the condition should be distinguished from cirrhosis. Patients who bleed do well after variceal sclerotherapy or a portocaval anastomosis because of their good liver function.

Congenital intrahepatic biliary dilatation (Caroli’s disease)

In this rare, non-familial disease there are saccular dilatations of the intrahepatic or extrahepatic ducts. It can present at any age (although usuaUy in childhood) with fever, abdominal pain and recurrent attacks of cholangitis with Gram-negative septicaemia. Jaundice and portal hypertension are absent. Diagnosis is by ultrasound, PTC or ERCP.

Solitary non-parasitic cysts

These are rare and probably a variant of polycystic disease.

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