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Cerebrofacial Arteriovenous Metameric Syndrome Type 2

Vasanthapuram, Varshitha Hemanth M.D.*; Varma, Ravi D.M.; Bothra, Nandini M.D.*

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Ophthalmic Plastic and Reconstructive Surgery: July/August 2020 - Volume 36 - Issue 4 - p e109
doi: 10.1097/IOP.0000000000001503
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A 3-year-old boy was brought by his parents with complaints of red eyes (Fig. A) since the age of 3 months. With time, progressive increase in the redness of the OS along with increase in the swelling on the left side of scalp was noted. There was no history of seizures. On examination, the vision was 6/6 in OU and extraocular motility was normal. There were no papillae, trantas spots, or limbal thickening; however, dilated conjunctival vessels were present in the temporal quadrant of the OS (Fig. B). The fundus examination was unremarkable, with no evidence of vascular malformation. Systemic examination did not show any vascular lesion. An MRI with contrast of brain showed well-defined heterogeneously enhancing (T2 weighted) hyperintense lesion in left superior ophthalmic fissure and posterior orbit (arrow in Fig. C, D). Similar lesion was present in the left temporal fossa, in the subcutaneous plane in the temporal area of scalp (* in Fig. C). The diploic space of the lesser wing of sphenoid bone and adjacent frontal bone showed asymmetric enhancement (double arrows in Fig. C, D). Prominence of left sphenoparietal sinus and pterygoid venous plexus, multiple venous anomalies in bilateral thalami, and bilateral cerebellar hemispheres were also noted (arrows in Fig. E). Based on the above findings, a diagnosis of cerebrofacial arteriovenous metameric syndrome Type 2 was made. Cerebrofacial arteriovenous metameric syndrome is a rare, nonhereditary condition with vascular malformations in a metameric distribution involving the craniofacial region (brain and face) with subtypes1–3 based on the distribution of lesions. Type 2 or “lateral prosencephalic group” involves the occipital lobe, thalamus, optic tract, and retina intracranially and the maxilla as its facial component. An embryonic developmental defect has been proposed to cause the condition. The most common presenting symptom is decreased vision; however, the patient in this study presented with just congestion of the eye and minimal scalp swelling. Other symptoms like mental retardation due to underdevelopment of the brain, seizures due to bleeding, and so on may be seen. The child is under periodic observation to note for progress in the lesion or the symptomatology, if any.

Clinical and Radiological features of Cerebrofacial arteriovenous metameric syndrome type 2.


1. Bhattacharya JJ, Luo CB, Suh DC, et al. Wyburn-Mason or Bonnet-Dechaume-Blanc as Cerebrofacial Arteriovenous Metameric Syndromes (CAMS). A New Concept and a New Classification. Interv Neuroradiol 2001;7:5–17.
2. O'Loughlin L, Groves ML, Miller NR, et al. Cerebrofacial arteriovenous metameric syndrome (CAMS): a spectrum disorder of craniofacial vascular malformations. Childs Nerv Syst 2017;33:513–6.
3. Jiarakongmun P, Alvarez A, Rodesch G, et al. Clinical course and angioarchitecture of cerebrofacial arteriovenous metameric syndromes. Three demonstrative cases and literature review. Interv Neuroradiol 2002;8:251–64.
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