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Special Feature
July 2000

Radiological Case of the Month

Arch Pediatr Adolesc Med. 2000;154(7):744. doi:

Figure 1. Abdominal ultrasonogram reveals numerous, 1- to 4-cm ovoid hypoechoic hepatic lesions.

Figure 2. Abdominal computed tomographic scan shows numerous, spherical intrahepatic lesions (left) that enhance with intravenous contrast medium (right).

Hepatic hemangiomatosis (HH) is a rare condition of infancy with the potential for severe, life-threatening complications. Hepatic hemangiomatosis may occur as part of diffuse neonatal hemangiomatosis, characterized by hemangiomata of the skin and at least 2 visceral organs. Hepatic involvement in diffuse neonatal hemangiomatosis occurs in nearly two thirds of cases.1 Less commonly, HH may be present without any other organ involvement.2,3 Most patients have signs and symptoms of high-output congestive heart failure (CHF).3 The hemangiomata serve as multiple arteriovenous fistulae, decreasing peripheral vascular resistance and increasing cardiac output. Other complications of HH are jaundice, Kasabach-Merritt syndrome, hemorrhage, and hypofibrinogenemia.4,5 Hepatic hemangiomatosis is clinically and radiographically indistinguishable from hepatic hemangioendothelioma, and differentiation depends on histologic evaluation. Hemangioendotheliomas have a predominance of cellularity to vascular space.6 Hemangioendothelioma is the most common benign hepatic tumor of childhood and the most common hepatic tumor of the first year of life.7 The importance of distinction between HH and hepatic hemangioendothelioma rests on the potential for sarcomatous change in the latter condition.8