Multimodale Bildgebung der vertebrobasilären Dolichoektasie

Eine vertebrobasiläre Dolichoektasie kann sowohl einen raumfordernden Effekt ausüben als auch vaskuläre Ereignisse wie z. B. Schlaganfälle verursachen. Die exakte Diagnose der Erkrankung ist Voraussetzung für die korrekte Therapieentscheidung, da vaskuläre Ereignisse bei vertebrobasilärer Dolichoektasie einer speziellen Behandlung bedürfen. Dieser Artikel vermittelt dem Leser die nötigen Kenntnisse zu Anatomie, Pathophysiologie sowie Bildgebung.

Abstract

Vertebrobasilar dolichoectasia (VBD) is characterized by ectasia, elongation, and tortuosity of the vertebrobasilar arteries, with a high degree of variability in clinical presentation. The disease origin is believed to involve degeneration of the internal elastic lamina, thinning of the media secondary to reticular fiber deficiency, and smooth muscle atrophy. The prevalence of VBD is variable, ranging from 0.05 % to 18 %. Most patients with VBD are asymptomatic and their VBD is detected incidentally; however, it is important to recognize that the presence of symptoms, which can lead to clinically significant morbidity and sometimes mortality, may influence clinical management. The most important clinical presentations of VBD are vascular events, such as ischemic stroke and catastrophic intracranial hemorrhage, or progressive compressive symptoms related to compression of adjacent structures, including the cranial nerves, brainstem, or third ventricle, causing hydrocephalus. The imaging diagnostic criteria for computed tomography and magnetic resonance (MR) imaging include three quantitative measures of basilar artery morphology: laterality score, height of bifurcation, and basilar artery diameter. The authors review the relevant anatomy and disease origin of VBD; pertinent imaging findings, including intraluminal thrombus and relation to the cranial nerves; and imaging pitfalls, such as the hyperintense vessel sign on MR images and artifacts related to slow flow in the dolichoectatic vessel. In addition, clinical manifestations, the role of radiology in diagnosis and management of this condition, and available management options are reviewed.

Kernaussagen

• Der primäre pathophysiologische Mechanismus der Dolichoektasie wird heute in gestörten Umbauvorgängen in der Gefäßwand sowie in einer Anomalie des Bindegewebes innerhalb der Arterienwand gesehen. Diese wiederum resultiert aus einem Ungleichgewicht der Aktivität von Matrixmetalloproteinasen und Antiproteasen im Bindegewebe.

• Die Kriterien von Smoker u. Mitarb. enthalten 3 quantitative Parameter der Morphologie der A. basilaris: den Lateralitäts-Score und die Höhe der Bifurkation als Surrogatmarker für die Tortuosität bzw. Elongation sowie den Durchmesser der A. basilaris.

• Die schwierige Abgrenzung eines langsamen Blutflusses von einem intraluminalen oder wandständigen Thrombus auf FLAIR-Bildern kann als limitierender Faktor der FLAIR-Technik bei VBD gewertet werden.

• Die meisten VBD-Patienten zeigen keine Symptome und ihre VBD wird als Zufallsbefund diagnostiziert. Patienten mit VBD sind mit einer höheren Wahrscheinlichkeit asymptomatisch als Patienten mit einer Dolichoektasie im anterioren Hirnkreislauf. Die klinischen Manifestationen lassen sich in Kompressionssymptome und vaskuläre Ereignisse unterteilen. Zu Letzteren zählen die transitorische ischämische Attacke, der ischämische Schlaganfall und die intraparenchymale oder subarachnoidale Blutung.

• Die am häufigsten beschriebenen ischämischen Läsionen sind mit einer Größenordnung von 41 % im Hirnstamm lokalisiert und betreffen zumeist den Pons, gefolgt vom Territorium der A. cerebri posterior, dem Thalamus, dem Zerebellum und anderen Regionen. Ischämische Schlaganfälle infolge von VBD-assoziierten Veränderungen in den tiefen kleinen Arterien und einer Okklusion von perforierenden Arterien – als „Erkrankung der kleinen Gefäße“ bzw. „multiple Lakunen“ bezeichnet – machen ⅔ aller ischämischen Schlaganfälle aus.

Schlüsselwörter

vertebrobasiläre Dolichoektasie - Pathophysiologie - Bildgebungsmerkmale - Schlaganfall - Kompressionssymptome

Keywords

vertebrobasilar dolichoectasia - pathophysiology - imaging findings - stroke - compressive symptoms

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