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

Abstract

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 (134Â10 9 /L) on the 1st day of life progressed to the lowest level (107Â10 9 /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...

Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: a case series

“…Hsiao et al. 22 reported a patient with a large congenital haemangioma on the scalp highly suggestive, by the clinical pictures, of RICH. There is no description of the histological findings or Glut‐1 immunostaining.…”

“…There are three previous reports of coagulation abnormalities complicating a large congenital haemangioma 1,21,22 . In all reports, the authors considered the coagulopathy an example of KMP.…”

“…[18][19][20] Thrombocytopenia and coagulopathy complicating what appeared to be RICH-like lesions have been reported only three times. 1,21,22 We describe seven further cases and define the clinical characteristics that may help to distinguish this entity from the haematological findings associated with true KMP. The medical records, skin biopsy specimens, when available, and clinical photographs of patients who presented with RICH and thrombocytopenia early in the neonatal period were reviewed for this retrospective study.…”

“…Thrombocytopenia and coagulopathy complicating what appeared to be RICH‐like lesions have been reported only three times 1,21,22 . We describe seven further cases and define the clinical characteristics that may help to distinguish this entity from the haematological findings associated with true KMP.…”

Surgical management of large scalp infantile hemangiomas

“…[4] However, large scalp lesions can be associated with serious sequelae such as ulceration, bleeding, and cardiac failure, as well as unaesthetic outcomes such as abnormal growth of the cranial vault. [11] Other complications of scalp IHs include the disruption of the visual axis in large frontal lesions, disruption of the anterior hairline as the lesion involutes leaving behind a large region of alopecia, and the disruption of the aural anatomy that can become permanent if the lesion is not removed early. [20]…”