Huge, alarming congenital hemangioma of the scalp presenting as heart failure and Kasabach-Merritt syndrome: a case report

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We report on a neonate with a huge congenital hemangioma of the scalp presenting as high-output heart failure and Kasabach-Merritt syndrome. The condition was successfully treated by means of surgical excision. A prematurely born (gestational age=36 weeks; birth weight=2,316 g), three-day-old female infant was referred to our institution for a huge scalp tumor (Fig. 1), already detected by fetal ultrasonography at six months of gestation. Head computed tomography scan revealed a hypervascular tumor of the scalp with strong heterogeneous enhancement. Magnetic resonance imaging demonstrated multiple feeding arteries, mainly consisting of an anastomosis of bilateral superficial temporal arteries and branches of the internal carotid artery. There was no evidence of intracranial hemorrhage. The patient had early heart failure signs including progressive respiratory distress and tachycardia. A small patent ductus arteriosus was present (1.2 mm in size). Thrombocytopenia (134 10/L) on the 1st day of life progressed to the lowest level (107 10/L) on day 3. On day 4, normocytic anemia (hemoglobin=11.5 g/dL) was present, prothrombin time was prolonged (16.3 seconds) as was partial thromboplastin time (79 seconds). Direct Coombs_ test was negative and both fibrin split products (80–160 mg/ml; normal<10 mg/ml) and D-Dimer (1.94 mg/ml; normal<0.5 mg/ml) were increased. The early heart failure signs improved soon after oxygen hood. Dopamine (5 mg/kg/min) and parenteral furosemide were administered beginning on the 3rd day of life. On the 5th day of life surgical excision following ligation of the feeding arteries was successful apart from the need for a whole-blood transfusion for surgery-related blood loss of approximately 300 mL. Indirect hyperbilirubinemia on the day subsequent to surgery complicated patient recovery, followed by the need for a double-volume blood-exchange transfusion in order to deal with a total bilirubin level escalating to 23.8 mg/dL. The patient_s thrombocytopenia resolved from the 14th day of life. Dopamine and furosemide were discontinued three days following surgery and the baby was discharged in good condition two weeks later. The histological findings from the excised lesion revealed a number of vascular channels located beneath the scalp and surrounded by fibrotic and myxoid stroma. Low cellularity and scant mitotic figures were noted without any malformed vessels. Hemangioma is present in more than 10% of normal newborns with a strong female predominance [4]. Approximately 60% of the hemangiomas of infancy appear on the head and neck [3], ninety-five percent will regress spontaneously without sequelae [1]. By contrast, 3–5% of hemangiomas involve vital organs or produce life-threatening complications requiring the need for aggressive therapy [5]. The management of hemangioma is generally divided into conservative treatment and intervention therapy. A response rate to treatment of 45%, but a cure rate of only 18% has been reported following prednisone therapy (3–10 mg/kg/day) for six to eight weeks [5]. Serious side effects of interferon-a limit its use to life-threatening cases [2]. We chose direct surgical intervention for our patient, mainly Eur J Pediatr (2007) 166:619–620 DOI 10.1007/s00431-006-0278-x