Primary sclerosing cholangitis associated with liver atrophy

Of 27 patients with primary sclerosing cholangitis, 4 were found on investigation and subsequently at operation to have gross lobar atrophy. The disease was particularly severe in the hilar region. Three of the four patients were asymptomatic prior to the onset of jaundice. The presence of atrophy precipitated laparotomy to exclude cancer in two cases. Marked disparity in size between liver lobes precluded a hilar approach to relieve extrahepatic obstruction in two patients. Prolonged follow-up excluded coincident malignant disease. Selective liver atrophy should be considered part of the disease spectrum in primary sclerosing cholangitis.