The pathogenesis of arteriovenous malformations: insights provided by a case of multiple arteriovenous malformations developing in relation to a developmental venous anomaly.

Abstract

Developmental venous anomalies (DVAs) are common anomalies of intracranial venous drainage that may occur in conjunction with other cerebral vascular malformations. The present case raises important questions regarding the association between anomalous venous drainage patterns and the development of arteriovenous malformations (AVMs). We present the case of a 24-year-old man with small AVMs fed by the superior cerebellar artery that drained directly into a large DVA of the cerebellum. The patient was managed conservatively and returned 10 years later with recurrent symptoms. A repeat angiogram demonstrated spontaneous thrombosis of the previously documented AVMs; however, new AVMs at a different site that was also fed by the superior cerebellar artery and drained into the same DVA had appeared. The AVMs were completely embolized, and the DVA was left intact. Recently, increasing attention has focused on the possible importance of venous outflow disturbance and venous hypertension in the pathogenesis and pathophysiology of AVMs. The potential mechanisms for this association and the implications of the present case are discussed, and the pertinent literature is reviewed.