Introduction: Over the years, patients presented with cerebrofacial vascular malformations which occurred in clusters. The syndromic presentation suggested angio-architectural abnormality, which started to develop during embryogenesis. The segmentation of the neural tube could provide a rational explanation for such a neuropathological phenomenon. Based on this theory, cerebrofacial arteriovenous metameric syndrome (CAMS) was derived. This describes clinical association between arteriovenous malformations of the face, retina, and brain. In this case's presentation, the syndromic presentation of the patient provided further evidence to the theory.
Case report: The authors report a case of a male neonate presenting with heart failure at birth. He was found to have a left sigmoid dural arteriovenous fistula (dAVF), an upper lip arteriovenous malformation (AVM), and a capillary hemangioma over the nose. The neuropathological clustering of vascular lesions is suggestive of CAMS. The patient was successfully treated with endovascular treatment and showed normal development during our follow-up assessment.
Conclusion: With the evolving evidence for the embryo-developmental theory for the clustering of angio-pathological lesions, further study and development of pathophysiology should be continued in following this track of theory. The success of endovascular intervention has warranted a favorable treatment option.