Surgical Management of Kaposiform Hemangioendothelioma with Shoulder Contracture after Remission of Kasabach-Merritt Phenomenon

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy frequently associated with Kasabach-Merritt phenomenon (KMP), a severe consumptive coagulopathy. Although KMP often resolves with corticosteroids and vincristine, residual lesions may lead to fibrosis, functional impairment, and recurrence of KMP. We report a five-year-old boy with KHE of the right chest wall who developed shoulder contracture after hematologic remission of KMP. Surgical resection of the residual lesion was performed during a hematologically stable phase. The tumor was resected safely without significant intraoperative bleeding or hematologic deterioration. One year postoperatively, shoulder abduction improved from 120 degrees to 170 degrees, pain had resolved, and no recurrence was observed. Surgical resection during hematologic stability may be an effective option for functional recovery in post-KMP KHE.