Hepatic Hemangiomatosis of Infancy

The Management of Disseminated Eruptive Hemangiomata in Infants

“…Diagnostic evaluation of any infant with multiple cutaneous and/or visceral hemangiomas may include any or all of the following screening studies: (1) hematocrit and hemoglobin to detect anemia secondary to bleeding; (2) platelet count to check for thrombocytopenia (Kasabach-Merritt syndrome is rare but has been reported in these infants); (3) urinalysis to detect urinary tract bleeding; (4) stool examination for fresh or occult blood; (5) chest film and electrocardiogram if cardiac failure is suspected; (6) ophthalmologic examination if hemangiomas involving the globe are noted; (7) abdominal films for delineation and localization of a mass and for detection of hepatic calcifications often present in hemangiomas of the liver; (8) ultrasonography of the abdomen to localize the mass; (9) liver-spleen scan to define organomegaly further; (10) computerized tomography to detect intracranial lesions if involvement of the central nervous system seems likely; and (11) liver function tests in any jaundiced infant, since obstructive jaundice may occur rarely from encroachment of hemangioma on the porta hepatis. More complicated studies such as abdominal aortograms and celiac angiography may be required for more explicit delineation of an intraabdominal mass.…”

“…If congestive heart failure is suspected, cardiac output should be measured. Digitalis and diuretic therapy are recommended (1,4,6).…”

“…Frequent observation of the patient's health and developmental status is recommended. For the patient who does not respond to steroid therapy and decongestive measures, ligation of the hepatic artery may be effective, however, it is not without its hazards (4,6).…”

Radiological Case of the Month

“…3 Treatment with corticosteroids and digitalis improves the outcome of CHF in patients with HH [10][11][12] ; however, this treatment is not universally effective. 1,[13][14][15][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][14][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.…”

“…1,[13][14][15][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][14][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).…”