Two Cases of Infantile Hemangioendothelioma of the Liver in Neonates

ABSTRACT

Infantile hemangioendothelioma(IHE) of the liver is a rare benign vascular tumor that presents most commonly in infants before the age of 6 months. IHE presents as abdominal mass, cutaneous hemangiomas, unexplained jaundice, bleeding disorders, or congestive heart failure. Death often results from congestive heart failure despite appropriate treatment with digoxin and diuretics. IHE also is associated with Kasabach-Merritt syndrome, anemia, intraperitoneal hemorrhage secondary to rupture, consumptive coagulopathy and vascular malformation involving brain, skin, gut, and other organs. Although children with asymptomatic lesions may experience spontaneous regression within a year, symptomatic lesions shoud be treated aggressively because this disease can progress rapidly and may be fatal. Treatment options are divided into medical treatment, interventional therapy including embolization, and surgical resection. Corticosteroid may hasten involution by inhibiting proliferation of endothelial and smooth muscle cells, and this trial is warranted in most cases before invasive procedures are used. If steroid therapy is unsuccessful, early definitive treatment using embolization or ligation of the hepatic artery, resectional surgery, and orthotopic liver transplantation shoud be considered. We experienced symptomatic IHE in two neonates. In the first case, she showed respiratory failure and consumptive coagulopathy, and symptoms were aggravated despite steroid therapy, so a left lobectomy was performed. In the second case, he presented high output cardiac failure, and was successfully treated by the coil embolization of left hepatic artery. This coil embolization of hepatic artery for treating IHE was the first case in Korea we know of.