Intrahepatic bile duct cysts (Caroli's disease) with liver fibrosis in foals. A synopsis of signs and diagnosis of seven cases

摘要

Several forms of congenital cystic hepatic fibrosis with autosomal inheritance have been described in humans and domestic animals. We report seven cases of hepatic fibrosis with cystic bile duct dilatation in two- to four-months-old Freiberger foals.These cases closely resemble the Caroli's type II syndrome in humans. All, except one foal, showed pronounced hepatic encephalopathy with neurological disorders and laboratory findings of hepatic insufficiency. Pathologically gross and histological findings were similar in all foals. Necropsy revealed severely enlarged and firm livers with multiple thin walled cysts. Their weight was up to four fold higher than normal. Histologically all livers showed a diffuse portoportal bridging fibrosis with cysticbile ducts, forming a branching network. Prognosis is poor. Proper diagnosis of Caroli's type II syndrome justifies euthanasia. Breeding of carrier horses must be avoided to eradicate this hereditary disease.