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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.

Golitz  LERudikoff  JO'Meara  OP Diffuse neonatal hemangiomatosis.  Pediatr Dermatol. 1986;3145- 152Google ScholarCrossref
Selke  ACCornell  SN Infantile hepatic hemangioendothelioma.  AJR Am J Roentgenol. 1969;106276Google ScholarCrossref
DeLorimier  AASimpson  EBBaum  RSCarlsson  E Hepatic-artery ligation for hepatic hemangiomatosis.  N Engl J Med. 1967;277333- 337Google ScholarCrossref
Blumenfeld  TAFleming  IDJohnson  WW Juvenile hemangioendothelioma of the liver: report of a case and review of the literature.  Cancer. 1969;24853- 857Google ScholarCrossref
Skopec  LLLakatua  DJ Non-immune fetal hydrops with hepatic hemangioendothelioma and Kasabach-Merritt syndrome: a case report.  Pediatr Pathol. 1989;987- 93Google ScholarCrossref
Othersen  HBWatanatittan  S Giant hemangiomatosis of the liver in infancy.  Am Surg. 1978;4420- 25Google Scholar
Stocker  JT Hepatic tumors in children. Such  FJed. Liver Disease in Children. St Louis, Mo Mosby–Yearbook Inc1994;901- 929Google Scholar
Kirchner  SGHeller  RIMKasselberg  AGGreene  HL Infantile hepatic hemangioendothelioma with subsequent malignant degeneration.  Pediatr Radiol. 1981;1142- 45Google ScholarCrossref
Mahboubi  SSunaryo  FPGlassman  MSPatel  K Computed tomography in the management and follow-up of infantile hemangioendothelioma of the liver in infants and children.  J Comput Tomogr. 1987;11370- 375Google ScholarCrossref
Goldberg  SJFonkalsrud  E Successful treatment of hepatic hemangioma with corticosteroids.  JAMA. 1969;2082473Google ScholarCrossref
Clemmensen  O A case of multiple neonatal hemangiomatosis successfully treated by systemic corticosteroids.  Dermatologica. 1979;159495- 499Google ScholarCrossref
Edgerton  MT The treatment of hemangiomas.  Ann Surg. 1976;183517- 532Google ScholarCrossref
Laird  WPFriedman  SKoop  CESchwartz  GJ Hepatic hemangiomatosis: successful management by hepatic artery ligation.  AJDC. 1976;130657- 659Google Scholar
Vorse  HBSmith  EILuckstead  EFFraser  JJ Hepatic hemangiomatosis of infancy.  AJDC. 1983;137672- 673Google Scholar
Shannon  KBuchanan  GRVotteler  TP Multiple hemangiomas: failure of corticosteroid therapy and successful hepatic artery ligation.  AJDC. 1982;136275- 276Google Scholar
Rotman  MJohn  MStowe  SInamdar  S Radiation treatment of pediatric hepatic hemangiomatosis and coexisting cardiac failure.  N Engl J Med. 1980;302852Google ScholarCrossref
Stanley  PGrinell  VSStanton  REWilliams  KOShore  NA Therapeutic embolization of infantile hepatic hemangioendothelioma with polyvinyl alcohol.  AJR Am J Roentgenol. 1983;1411047- 1051Google ScholarCrossref
Burrows  PERosenberg  HCChuang  HC Diffuse hepatic hemangiomas.  Radiology. 1985;15685- 88Google Scholar
Tarazov  PGPolysalov  VNRyzhkov  VK Hemangiomatosis of the liver and spleen.  AJR Am J Roentgenol. 1990;1551235- 1236Google ScholarCrossref
Touloukian  RJ Hepatic hemangioendothelioma during infancy: pathology, diagnosis and treatment with prednisone.  Pediatrics. 1970;4571- 76Google Scholar
McLean  RHMoller  JHWarwick  WJSatran  LLucus  RV Multinodular hemangiomatosis of the liver in infancy.  Pediatrics. 1972;49563- 573Google Scholar
Corbella  FArico  MPodesta  AFVilla  ABeluffi  GBianchi  E Infantile hepatic hemangioendothelioma treated by radiotherapy.  Pediatr Radiol. 1983;13297- 300Google ScholarCrossref