Cholangiodysplastic pseudocirrhosis: light and electron microscopic examination.

The familial type of cholangiodysplastic pseudocirrhosis is presented. This chronic liver disease is caused by malformation of the intrahepatic bile ducts. The female infant was 5 months old when the diagnosis was established. The liver biopsy was studied by light and electron microscopy. Electron microscopic examination revealed active proliferation of ductual cells and progression of fibrogenesis, findings consistent with the rapid and fatal course of the disease. In the case presented an acute cholangitis occurred, but after healing the progression of the original process led to hepatic insufficiency. It is suggested that cholangiodysplastic pseudocirrhosis is a chronic, progressive liver disease the course of which might be hastened by the complication of cholangitis; the process itself causes liver cirrhosis without inflammation.