Congenital hepatic fibrosis of Riedel’s lobe causing portal hypertension

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CONGENITAL hepatic fibrosis is a rare but important cause of portal hypertension in children;1–6 it is even more rare as a cause of portal hypertension in adults. Parker1 reported two patients 33 years of age whom he studied, and the case of a patient 50 years old seen by Melnick;7 and McCarthy, Baggenstoss, and Logan3 reported five patients in whom portal hypertension, secondary to congenital hepatic fibrosis, became apparent in adult life. Because of the development of portal hypertension and the dangers of hemorrhage from gastroesophageal varices,8 the selection of patients for portal-systemic shunt procedures9 is of considerable importance. The excellent response to portal-systemic shunt for relief of the portal hypertension, and the fact that liver function usually remains good, signify that congenital hepatic fibrosis often has a favorable prognosis.

We recently treated an elderly man in whom bleeding developed from gastroesophageal varices secondary to portal hypertension and congenital hepatic fibrosis. An interesting and unique feature of the case was that the hepatic fibrosis involved only Riedel’s lobe. While Riedel’s lobe is the most common lobar deformity of the liver,10 its association with congenital hepatic fibrosis and portal hypertension has not, to our knowledge, been previously reported.

Report of a case

A 64-year-old man was admitted to the Cleveland Clinic Hospital on December 26, 1968, because of melena. He had been well until June 1968, when he began to note occasional tarry bowel movements. There had been no diarrhea, abdominal pain, or other symptoms; there was no history of jaundice.



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