Bilateral segmental agenesis of the vertebrobasilar junction: Developmental and angiographic anatomy

BACKGROUND AND PURPOSE: Bilateral vertebrobasilar junction agenesis is an exceptional anatomic variation. This article explores the angiographic characteristics of this variant and its embryologic mechanisms. MATERIALS AND METHODS: Two observations of bilateral agenesis of the vertebrobasilar junction are reported. A case of atheromatous disease of the vertebrobasilar junction is shown to highlight characteristics distinguishing such a lesion from the reported variant. RESULTS: In the 2 reported cases, the distal segment of both vertebral arteries (VAs) and the proximal portion of the basilar artery (BA) were absent. In addition, distal connections of the BA with the posterior cerebral arteries (PCA) were also lacking. As a consequence, the remaining portion of the BA was isolated from its usual sources of blood supply, which was provided by a persistent carotid-basilar anastomosis. CONCLUSION: The developmental mechanism underlying bilateral agenesis of the vertebrobasilar junction likely involves the anterior radicular artery of C1. This branch of the proatlantal artery normally becomes the adult distal VA and the proximal BA. The lack of cranial connection of the BA with the PCA may be secondary to the proximal vertebrobasilar agenesis and the resulting paucity of antegrade flow within the BA. Alternatively, the absence of both the proximal and distal connections of the BA could be the result of a similar, yet unknown, developmental mechanism. From a clinical standpoint, this vascular anomaly was discovered incidentally in our 2 patients, a finding consistent with the assumed congenital nature of the variant.