Results
The patient was a male newborn from the first pregnancy of a clinically healthy woman. He was born at 38+6 weeks of gestational age by normal spontaneous delivery, weighing 2890 gm. His mother received regular prenatal examinations revealing no specific abnormality. A large (approximately 6 x 5 x 2 cm), firm, immovable mass with purpuric and bruised appearance was found over his left postauricular site (Figure 1A ). He was initially diagnosed with infantile hemangioma and treated with propranolol. However, although gauze soaked with epinephrine was used locally and directly pressed to stop the bleeding, hemorrhage from the hemangioma continued from the sixth day of birth. Laboratory data showed thrombocytopenia (platelet of 17000/uL), coagulopathy (international normalized ratio of prothrombin time was 2.04, activated partial thromboplastin time was greater than 150 sec) and hypofibrinogenemia (fibrinogen of 152.1mg/dL). These findings are consistent with Kasabach-Merritt phenomenon (KMP). Magnetic resonance imaging revealed a vascular-rich lesion with central scars, consistent with Kaposiform hemangioendothelioma (KHE) (Figure 2A ). Despite sirolimus 0.8 mg/m2 twice daily and transfusion with fresh frozen plasma, cryoprecipitate, platelets, the bleeding could not be stopped. Because of KMP with active bleeding and surgical operation not being feasible, transcatheter arterial embolization was performed at 9 days of age and 3.05 kg. Selective left common carotid arteriography demonstrated a single feeding artery to the mass lesion without collateral artery to adjacent organs (Figure 2B ). A 4F delivery catheter (Amplatzer Judkins Right catheter, Abbott Medical, MN, U.S.A.) was advanced over a 0.018” Terumo guide wire into the feeding artery of the mass lesion. A total of three Cook Embolization Coils, two 3 mm x 4 cm, one 3 mm x 5 cm, and one 3/2 mm x 2 cm Cook Tornado Embolization Microcoil (Cook Medical, Bloomington, IN, USA), were selected for embolization of the feeding artery. Repeat arteriography showed no obvious residual shunt after embolization (Figure 2C ). The bleeding was stopped, and he no longer needed a blood transfusion on the second day after embolization. Follow-up laboratory data revealed neither thrombocytopenia nor coagulopathy 1 week later. He was discharged from hospital on day 18 (Figure 1B ). At 3 months, the KHE had almost completely regressed (Figure 1C ), and then sirolimus was discontinued.