Authors: Sara AlShaker, Imran Jivraj, Haiying Chen, Prakash Muthusami, John Phillips, Dan DeAngelis

Author Disclosure Block: S. AlShaker: None. I. Jivraj: None. H. Chen: None. P. Muthusami: None. J. Phillips: None. D. DeAngelis: None.

Abstract Body:

Purpose: We describe a rare amblyogenic congenital hemangioma of the forehead and brow requiring early surgical management.
Study Design: Observational case report
Methods: The patient’s clinical and surgical records were reviewed including patient history, clinical examinations, and investigations. A thorough review of the literature was also performed.
Results: An otherwise healthy boy was born with a large disfiguring vascular mass involving the forehead and eyebrow which obstructed the left visual axis. MRI demonstrated a T1-hyperintense facial and periorbital mass that avidly enhanced on contrast administration without intracranial or orbital extension. MRA demonstrated rich arterial supply arising from the left external carotid and left ophthalmic arteries while MRV demonstrated bilateral superior ophthalmic vein drainage. There was no evidence of thrombocytopenia, coagulopathy, high output heart failure, or other vascular lesions. The lesion remained stable in size over the subsequent three weeks and customized head gear was designed to elevate the mass away from the visual axis. At 23 days of age, following intraoperative ultrasound-guided ligation of the arterial supply to the lesion, surgical resection to clear the visual axis was performed necessitating intraoperative transfusion. Pathology revealed a large vascular lesion involving dermis and subcutis consisting of lobules of small vascular channels with many large interlobular vessels. Pathologic analysis revealed pericytes and CD31-positive GLUT1-negative endothelial cells consistent with a congenital hemangioma 1,. At six months of age, the patient had a markedly improved appearance with mild residual hemangioma and normal visual development.
Conclusions: Congenital hemangiomas are very rare benign congenital vascular tumours occurring on the face and extremities which are clinically and pathologically distinct from the more common infantile hemangiomas 2,3. They must be distinguished from malignant vascular tumours such as angiosarcomas, Kaposiform hemangioendotheliomas and other benign vascular tumours. Congenital hemangiomas are fully developed at birth and may be identified on routine second trimester unltrasonography 4,5. They are classified as rapidly-involuting, partially-involuting, or non-involuting based on their clinical course over the first year of life. 6,7 In the majority of cases, these lesions are observed for signs of regression, and surgery can be offered for persistent lesions. Our case highlights the challenging management of a large amblyogenic congenital hemangioma of the face which was successfully managed with customized headgear and subsequent resection.