Neonates with severe infantile hepatic hemangioendothelioma: limitations of liver transplantation.

IHHE as the most common vascular tumor of the liver in infancy can present with acute postnatal liver and congestive heart failure. LTx may be a lifesaving option, but can be complicated by extrahepatic involvement and bleeding complications, especially in neonates. Here we discuss the benefit of LTx in cases of acute postnatal deterioration and massive extent of the hepatic tumor. Three infants with IHHE were transplanted at our institution between 2005 and 2007. Two were neonates with acute postnatal decompensation and progressive liver and heart failure within days. Treatment with steroids and chemotherapy was ineffective; resection surgery and interventional treatment were not considered appropriate. LTx was performed at the age of 7 and 24 days, respectively. An additional infant with a bilobar tumor that evolved more slowly was transplanted on day-of-life 56. Patients 1 and 2 had to be resuscitated during the LTx procedure because of massive bleeding and both died during the procedure. Patient 3 had a complicated post-operative course but is doing well one-yr post-LTx. Neonates with extended hepatic and extrahepatic involvement of IHHE should be evaluated carefully prior to LTx. Whenever possible, alternative interventional treatment options should be considered.