Diagnosis of Kasabach-Merritt phenomenon in a newborn

Kasabach-Merritt Phenomenon (KMP) is a rare and potentially life-threatening coagulopathy that has high bleeding risk and is characterized by severe thrombocytopenia and hypofibrinogenemia. Timely diagnosis and intervention are essential, as untreated kaposiform hemangioendothelioma (KHE) or tufted angioma (TA) associated with Kasabach-Merritt phenomenon (KMP) can result in significant morbidity and mortality.. We report a case of a full-term female neonate who was noted to have a large vascular scalp mass at birth. The tumor was initially misidentified as an infantile and a congenital hemangioma. Upon specialty evaluation at a vascular anomaly center and additional diagnostics, the child was diagnosed with KHE and KMP. The patient was treated with oral sirolimus and tapering course of oral steroid, resulting in resolution of coagulopathy and substantial clinical improvement, and ultimately, underwent surgical tumor excision. This case highlights the importance of appropriate diagnosis and management of an infant with KHE with KMP.