Hepatic hemangioendothelioma. Angiographic appearance and apparent prednisone responsiveness

A 6-month-old girl was seen because of multiple cutaneous hemangiomas, hepatomegaly, and failure to thrive. A liver scan with technetium Tc 99m sulfur colloid showed two filling defects in the left lobe. Diffuse nodular hepatic lesions characteristic of hemangioendotheliomas were disclosed by celiac angiography and confirmed by biopsy. Congestive heart failure, present in many infants with this syndrome, was not manifest. Prednisone was administered, 2 mg/kg, on alternate days. Liver size and cutaneous hamangiomas promptly regressed in size and "catch up" linear growth occurred during six months of treatment. Congestive heart failure did not develop. Sixteen months after treatment, liver size and a second hepatic scan were normal.