WOFAPS 2025 8th World Congress of Pediatric Surgery

TengHui Zhan, Rong Zhang

Fujian Maternity and Child Health Hospital

Background: Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm predominantly affecting infants and young children, often associated with Kasabach-Merritt phenomenon (KMP) characterized by thrombocytopenia and coagulopathy. While medical management with sirolimus and corticosteroids is the first-line treatment, endovascular intervention for refractory cases in neonates remains largely unexplored.

Case presentation: A 1-month-old female presented with a hemangioma on the left face and neck, initially treated with oral propranolol. The mass increased progressively, leading to feeding difficulties and respiratory distress three days before admission. CT imaging revealed a 9.0×7.8×5.8cm mass causing nasopharyngeal and oropharyngeal compression. Laboratory findings showed progressive thrombocytopenia consistent with KMP, and airway obstruction worsened. Following diagnosis of Kaposiform hemangioendothelioma, treatment included tracheal intubation, platelet transfusion, methylprednisolone, and sirolimus therapy, followed by transcatheter arterial embolization (TAE) to reduce tumor vascularity. Postoperatively, airway obstruction symptoms gradually resolved, platelet counts stabilized, methylprednisolone was tapered, ventilation was discontinued, and sirolimus dosage was adjusted based on blood concentrations.

Conclusion: Combined endovascular therapy and pharmacological management may be feasible and effective in treating neonatal KHE with KMP and airway compromise. The short-term outcomes in this case were favorable, though long-term efficacy requires extended follow-up evaluation.