Burger I M, Siclari F, Gregg L, Gailloud P
Division of Interventional Neuroradiology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA.
AJNR Am J Neuroradiol. 2007 Nov-Dec;28(10):2017-22. doi: 10.3174/ajnr.A0719. Epub 2007 Sep 26.
Bilateral vertebrobasilar junction agenesis is an exceptional anatomic variation. This article explores the angiographic characteristics of this variant and its embryologic mechanisms.
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.
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.
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.
双侧椎基底动脉连接部发育不全是一种罕见的解剖变异。本文探讨了这种变异的血管造影特征及其胚胎学机制。
报告了2例双侧椎基底动脉连接部发育不全的观察病例。展示了1例椎基底动脉连接部动脉粥样硬化疾病病例,以突出该病变与所报告变异的区别特征。
在报告的2例病例中,双侧椎动脉(VA)的远端节段和基底动脉(BA)的近端部分均缺失。此外,基底动脉与大脑后动脉(PCA)的远端连接也不存在。因此,基底动脉的其余部分与其通常的血液供应来源隔绝,其血液供应由持续存在的颈动脉 - 基底动脉吻合提供。
双侧椎基底动脉连接部发育不全的潜在发育机制可能涉及C1的前根动脉。寰前动脉的这一分支通常会发育为成人的远端椎动脉和近端基底动脉。基底动脉与大脑后动脉缺乏颅部连接可能继发于近端椎基底动脉发育不全以及由此导致的基底动脉内顺行血流不足。或者,基底动脉近端和远端连接均缺失可能是由于一种类似但未知的发育机制所致。从临床角度来看,这种血管异常在我们的2例患者中是偶然发现的,这一发现与该变异假定的先天性本质相符。