MULTINODULAR HEMANGIOMATOSIS OF THE LIVER IN INFANCY

Abstract

Multinodular hemangiomatosis of the liver (MHL) is characterized pathologically by multiple hemangiomas, primarily in the liver but also in other organs. The hepatic hemangiomas act as small arteriovenous fistulae and their combined effect may result in a massive peripheral arteriovenous shunt and high output congestive cardiac failure. Patients with MHL may be recognized clinically by the classical triad of congestive cardiac failure, hepatomegaly, and cutaneous hemangiomas. All but 2 of 31 cases of MHL herein reviewed had the onset of symptoms prior to 6 months of age. High output cardiac failure were severe and resulted in a 70% mortality. Hepatomegaly was massive and out of proportion to the degree of cardiac failure. Therapy includes intensive treatment of congestive cardiac failure and judicious transfusion in anemic patients. Irradiation of the liver and corticosteroids have been utilized in attempts to speed evolution of the hepatic arteriovenous fistulae. The data are not sufficient to establish the efficacy of either of these treatment modalities.