Assuming the right approach in cerebrofacial arteriovenous metameric syndrome: a case report.

Abstract

Cerebrofacial arteriovenous metameric syndrome (CAMS) is a rare nonhereditary disease, amounting to only 0.5% of all cerebral vascular malformations. It is characterized by vascular malformations in a metameric distribution involving the cerebrofacial region. We present a case of a 14-year-old girl in whom the first vascular lesion appeared on the tip of her nose at the age of 18 months. This lesion reoccurred on several occasions during her childhood, with exacerbations coinciding with common rhinitis and several nose bleeds. She was unsuccessfully treated with laser therapy and topical selective beta-blockers prior to further investigations. Combined cerebrofacial arteriovenous malformation (CAMS I+II) was diagnosed by magnetic resonance imaging of the paranasal sinuses, orbits, and brain with cerebral angiography and confirmed by digital subtraction angiography. Clarifying the correct diagnosis enabled us to abort a potential surgical approach and to assume an expectant approach unless the patient develops neurological symptoms. Timely, complete, and adequate imaging investigations are necessary in cases of facial vascular malformations. This report for the first time describes the specific angioarchitecture involving two types of CAMS.