CUTANEOUS HEMANGIOMAS AND PERSISTENCE OF EMBRYONIC VASCULARIZATION

Objectives: The association of cutaneous hemangiomas and vascular malformations was first described in 1978. This disorder, one of the most frequent neurocutaneous syndromes is known as “cutaneous hemangiomas: vascular anomaly complex, ”Pascual-Castroviejo type II syndrome”, and ”PHACE”. It is most often associated with internal vascular anomalies anywhere in the body, particularly in head, neck, heart, aortic arch, abdomen and spine, and with cerebellar malformations. Persistent trigeminal artery, absence of hypoplasia of carotid and/ or vertebral arteries are very common congenital vascular malformations. Persistence of the primitive vascularization in the neck has not been previously reported in this disease.

Methods: A 2-year-old girl presented with a facial hemangioma that appeared first at the age of one week. She was studied with MR and MR arteriography (MRA).

Results: MRA revealed: a) absence of the left internal carotid, b) hypoplasia of the right external carotid artery, c) vascularization of the left cerebral hemisphere via the basilar artery through an enlarged posterior communicating artery, d) hemangioma on the left side of the neck, and e) embryonic appearance of both vertebral arteries that appeared without finishing their maturation and without obliteration of the longitudinal neural arteries.

Conclusion: Imaging features of this patient show the various vascular abnormalities associated with this syndrome.