Successful Management of Neonatal Kaposiform Haemangioendothelioma and Kasabach-Merritt Phenomenon Using Sirolimus and Surgical Excision

Abstract

: Kaposiform haemangioendothelioma (KHE) is a rare vascular tumor, occurring predominantly in infancy, with an incidence of 0.091 per 100,000 children. Typically presenting as a solitary large cutaneous lesion with no distant metastases. Children with KHE are at high risk of developing the Kasabach-Merritt phenomenon (KMP), a consumptive coagulopathy. KMP is defined as profound thrombocytopenia, hypofibrinogenemia, and elevated D-dimer. KMP has a mortality of 10 - 30%. Due to its rarity, there is little robust evidence for KHE management. Rapamycin (mTOR) inhibitors are a safe and effective treatment. This report is of a newborn full-term infant with no antenatal complications, presenting with abdominal cutaneous vascular malformation and, subsequently, coagulopathy.