Introduction: Kasabach-Merritt syndrome (KMS) is a life-threatening disease. We describe a combined medical and multistep, endovascular embolization that was successfully performed with surgery.
Case report: A 40-day-old female baby was referred because of an infiltrating pelvic mass. Blood tests showed severe anemia and thrombocytopenia with consumptive coagulopathy. The clinical aspect was pathognomonic for KMS. Administration of steroids and chemotherapy were started and coagulation parameters were normalized with tumor volume regression. Three months later, elective surgical treatment became possible, and dissection and ligature of the left internal iliac artery and sacral artery were performed. The main arterial supply of the mass was embolized with Spongostan®. Two months later, a second hybrid approach was adopted for embolization of the main vascular supply of the tumor occupying the left thigh. Via a third contralateral hybrid femoral approach and under fluoroscopy, selective catheterization of the left profunda femoris artery was performed. The arterial feeder of the tumor, localized at the left gluteus, was seen on a CT scan 2 months later and was embolized with Onyx-18.
Conclusion: Chemotherapy and a tailored hybrid approach comprising repeated endovascular embolizations seem to be a safe treatment in cases of unresectable and life-threatening tumors in small babies.
Keywords: Endovascular embolization; Kaposiform hemangioendothelioma; Kasabach-Merritt syndrome; Onyx-18.