Hepatic hemangioendotheliomas in infancy

Abstract

Multicentric hepatic hemangioendotheliomas are vascular lesions of the liver that usually present in the infant with hepatomegaly, high output congestive heart failure and cutaneous hemangiomas. The diagnosis, pathologic and physiologic conditions and treatment were discussed. Two of the patients we studied and 117 from the literature were reviewed. A total of 38 patients survived and 57 died with or without medical treatment. Thirteen patients survived and five died after ligation of the hepatic artery. Five patients survived and three died after embolization of the hepatic artery. (formula; see text) Of 119 patients with MHH, 81 (68 per cent) had congestive heart failure; 40 survived and 41 died. Of 56 patients with localized hepatic angiomas, 19 had congestive heart failure. Only two of the patients died of heart failure. Fifty localized hepatic angiomas in infancy that were treated with local resection or lobectomy were reviewed. Forty-six patients survived and four died of hemorrhage. Four patients survived and two died with or without medical treatment. Plans for management of multiple hepatic hemangioendotheliomas and localized hepatic angiomas in infancy were proposed.