Infantile Hepatic Hemangioendothelioma (IHHE) – An Unusual Presentation

Hepatic tumours are relatively rare in children, accounting for 1% 4% of all paediatric solid tumors, commonest amongst them are hepatoblastoma, neuroblastoma, and vascular tumors (hemangioendothelioma/haemangiomas). Infantile Hepatic hemangioendothelioma (IHHE) is classically characterised by hepatomegaly, jaundice, congestive cardiac failure with high cardiac output, skin haemangiomas, haemolytic anemia, thrombocytopenia(kasabach – Merritt syndrome), normal Alfafeto protein (AFP) levels and hypothyroidism. We report a case of infantile hemangioendothelioma in a 5 day old female neonate with high output cardiac failure, unusually high alpha-fetoproteins, thrombocytosis and polycythaemia, which posed a diagnostic dilemma. Diffuse IHHE is a rapidly growing benign tumor which can lead to poor clinical outcome if not identified early. Serum AFP can’t be relied to exclude IHHE.