Die Durafistel – ein unscheinbares morphologisches Korrelat mit großen Konsequenzen

Zusammenfassung

Durale arteriovenöse Fisteln (DAVFs) sind unphysiologische Gefäßverbindungen innerhalb der Dura mater und repräsentieren 10 – 15 % aller arteriovenösen Shunts des zentralen Nervensystems. Die Ätiologie der DAVF ist bis heute unbekannt. Die Symptomatik ist sehr variabel, bedingt durch die Richtung des Blutflusses, das transportierte Blutvolumen sowie die spezifische Lokalisation der Fistel. Die Diagnose kann durch die Diversität in der klinischen Präsentation und die oft unauffällige Schnittbildgebung erschwert sein. Um permanente neurologische Defizite zu verhindern, ist es wichtig, bei unklarer neurologischer Symptomatik an eine DAVF zu denken und die entsprechende Diagnostik, die MRT/MRA sowie die konventionelle Angiografie als Goldstandard beinhaltet, einzuleiten. Die derzeit gültige Klassifikation nach Merland, Djindjian und Cognard zieht die Heterogenität der klinischen Symptome, die therapeutischen Implikationen sowie die vitalen Komplikationen in Bezug auf jeden Subtyp in Betracht. Während DAVFs vom Typ I anterograd in einen zerebralen Sinus drainieren und hauptsächlich funktionelle Defizite verursachen, steigt das Risiko für eine intrazerebrale Blutung bei retrograder Drainage (Typ II) oder bei Abfluss in eine kortikale (Typen III und IV) bzw. perimedulläre Vene (Typ V). Vor allem bei DAVFs des Typs IIb – V kann nur die Fistelokklusion anhand von transvenöser Embolisation, transarteriellem Verkleben bzw. Partikelembolisation oder Operation die Entstehung schwerwiegender Folgen verhindern. Im Folgenden werden die anatomischen Grundlagen und die unterschiedliche Symptomatik, die durch eine DAVF entstehen kann, illustriert und systematisch im Hinblick auf Diagnostik und Therapie diskutiert. Wir hoffen damit, das Bewusstsein für dieses seltene, aber klinisch wichtige neurologische Krankheitsbild zu schärfen.

Abstract

Dural arteriovenous fistulas (DAVFs) are abnormal arteriovenous shunts located within the dura mater representing approximately 10 – 15 % of all arteriovenous shunts in the central nervous system. The aetiology of spontaneous DAVFs remains to be elucidated. The symptoms associated with DAVFs can be highly variable and dependent upon the direction of the blood flow, the amount of arteriovenous shunting and the specific location of the fistula. Considering the diversity of clinical presentation in the setting of unremarkable imaging results, diagnosing a DAVF can be difficult. To avoid permanent neurological deficits due to DAVFs, it is important to consider the possibility of a DAVF whenever one encounters unclear neurological symptoms and to initiate appropriate diagnostic procedures including intraarterial DSA and MRI/MRA. The current DAVF classification accounts for the disparity of clinical symptoms, therapeutic/interventional implications as well as vital complications depending on each particular fistula subtype. While type I DAVFs drain anterogradely into a cerebral sinus and mainly cause functional deficits, the risk for severe intracerebral bleeding increases when DAVFs drain retrogradely (type II), or into cortical (types III and IV), perimedullar or radiculo-medullar veins (type V), respectively. In particular in the case of type IIb to V DAVFs, the appropriate treatment option is a complete fistula occlusion by transvenous embolization, transarterial glue or particulate embolization or surgery. In the following we systematically explain the differential anatomy underlying DAVFs and discuss possible symptoms and necessary diagnostic and therapeutic means. In that, we are seeking to increase attention for this rare, but clinically relevant neurological disease.

Schlüsselwörter

Angiografie - CT/MRT - Durafistel - endovaskuläre Therapie - heterogene Symptomatik

Key words

angiography - CT/MRI - dural arteriovenous fistula - endovascular therapy - heterogeneous symptoms

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