Acronym of Craniofacial Arteriovenous Metameric Syndrome

See also Wyburn-Mason syndrome

Very rare. Form of non-hereditary congenital cerebro-retino-facial angiomatosis. Endothelial cells of the blood vessels of the facial, orbital, maxillary, mandibular and cerebral territoriess come from the neural crests of three large embryonic areas. A defect present before the migration of the neural crest cells to their final territory is likely to disseminate vascular lesions along the pathway of migration, giving segmental or metameric cerebro-facial syndromes (CAMS), variable depending on the region, the cells are originating from:

-        CAMS 1: corpus callosum, hypothalamus, olfactory tract, forehead, nose

-        Wyburn-Mason syndrome or CAMS 2: cortex and diencephalon, chiasma, optic nerve, retina, sphenoid bone, maxillary, cheek.

-        CAMS 3: cerebellum, temporal cortex, mandibula.

Anesthetic implications:

depending on the localisation of the arteriovenous malformation; in case of cerebral localisation, avoid high blood pressure and maintain  normoventilation; gentle laryngoscopy in case of maxillary or mandibular localisation.

References :

-        Lee AW, Chen CS, Gailloud P, Nyquist P.
Wyburn-Mason syndrome associated with thyroid arteriovenous malformation : a first case report.
Am J Neuroradiol   2007 ; 28 : 1153-4

Updated: July 2019