successful management of neonatal kaposiform haemangioendothelioma and kasabach-merritt phenomenon using sirolimus and surgical excision

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 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 have been shown to be a safe and effective treatment. this report is regarding a newborn term female who presented at birth with an abdominal cutaneous vascular malformation, subsequently developing life-threatening thrombocytopenia and coagulopathy. she was diagnosed with khe complicated by kmp, with an excellent response to sirolimus treatment, with subsequent lesion resection.