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July 2000

Radiological Case of the Month

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

Denouement and Discussion: Hepatic Hemangiomatosis

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

The natural history of HH parallels that of cutaneous hemangiomas, with spontaneous involution during early childhood. Asymptomatic patients require no specific treatment. However, follow-up imaging studies are indicated to evaluate resolution of the lesion and to exclude hepatic endothelioma and the potential for malignant change.9

In patients with CHF resulting from HH, therapeutic intervention is indicated. Initial treatment of CHF in patients with HH with diuretics and digitalis was disappointing, with mortality rates of nearly 90%.3 Treatment with corticosteroids and digitalis improves the outcome of CHF in patients with HH10-12; however, this treatment is not universally effective.1,13-16 Surgical removal is prohibited by the extensive hepatic involvement. Interruption of blood flow to the hemangiomata may be successfully accomplished by ligation of the hepatic artery,3,13-15 though ligation of the main hepatic artery at its origin from the celiac axis does not interrupt all arterial flow to the liver as there is collateral flow from the left gastric artery and the superior mesenteric artery.3 More recently, embolization has been accomplished with a variety of agents such as polyvinyl alcohol, silicone balloons, metal coils, and Spongostan (Ferrosan Co, Copenhagen, Denmark).17-19 Radiotherapy has been used for children who have not responded to medical therapy or have been too ill for surgery16,20-22; however, the possibility of long-term sequelae of hepatic irradiation makes radiotherapy an undesirable form of treatment in children.

Accepted for publication October 28, 1998.

Reprints: Beverly P. Wood, MD, KAM 211, USC-HSC, 1975 Zonal Ave, Los Angeles, CA 90089-9024.

References
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