Cerebrofacial venous metameric syndrome (CVMS) type I: constellation of imaging and clinical findings

A woman in her early 30s presented with swelling over the right supraorbital region and forehead that she had since birth (figure 1A). On examination, the swelling was soft, non-tender and increased in size on bending forwards. Doppler ultrasound scan revealed a few prominent venous vascular channels. Contrast-enhanced MRI showed a large deep venous anomaly (DVA) in the right frontal lobe. The DVA was seen draining into an ectatic venous pouch in the right basifrontal region. It further showed transosseous drainage into a venous sac in the subcutaneous plane (right supraorbital region) (figure 1B).