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.