Hypoplasia of the right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis.

Hypoplasia of the right hepatic lobe is a rare and usually asymptomatic anomaly, which sometimes may be associated with portal hypertension but rarely with retroperitoneal fibrosis. We report one such rare case of hypoplastic right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis. A 45-year-old nonalcoholic male presented with a chief complaint of hematemesis. Serum investigations were normal and inconclusive, so computed tomography was performed which showed right hepatic lobe hypoplasia, esophageal varices, and retroperitoneal fibrosis. Since all other causes of acquired liver atrophy had been ruled out, we considered this case to be of congenital origin. Although it is a rare anomaly, it should be kept in mind to avoid fatal surgical complication. [ABSTRACT FROM AUTHOR]