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 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.