Cerebro-facial metameric syndrome (CAMS) is a rare entity comprising of vascular malformations of craniofacial structures in a metameric distribution. It is classified into 3 subtypes depending upon metamere involved into medial prosencephalic, lateral prosencephalic and rhombencephalic group. Retinal involvement is most commonly seen in cases reported with facial vascular malformations. Facial lesions are generally silent or may present as small, angiomatous lesion at the time of presentation. Here, we report a case of CAMS 1 & 2 presenting with facial asymmetry, and intraoral lesions which was diagnosed as CAMS on contrast enhanced computed tomography (CECT), and CT Angiography. CECT Angiography demonstrated involvement of muscles of mastication with presence of phleboliths, feeder vessels and draining veins. Magnetic resonance imaging (MRI) Brain with Angiography was done for intracranial lesions.
Gupta et al. (Wed,) studied this question.