Abstract
Spinal vascular malformations are rare diseases with a wide variety of neurologic presentations. Their classification depends on the differentiation of shunting versus nonshunting lesions, the latter being the spinal cord cavernomas. In the shunting lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural vascular shunts from the pial vascular malformations: while those shunts that are fed by radiculomeningeal arteries (i.e., the counterparts of meningeal arteries in the brain) constitute the dural arteriovenous fistulas, the shunts that are fed by arteries that would normally supply the spinal cord (i.e., the radiculomedullary and radiculopial arteries) are the pial cord arteriovenous malformations (AVMs; whose cranial counterparts are the brain AVMs). Depending on the type of transition between artery and vein, the latter pial AVMs can be further subdivided into glomerular (plexiform or nidus-type) AVMs with a network of intervening vessels in between the artery and vein and the fistulous pial AVMs. The last step in the classification then describes whether the type of fistula has a high or a low shunting volume which will differentiate the “macro-” from the “micro”fistulas. The proposed classification is therefore based on a stepwise analysis of the shunt including its arterial anatomy, its nidus architecture and its flow-volume evaluation. The major advantage of this approach is that it leads to a subclassification with direct implications on the choice of treatment, thereby constituting a simple and practical approach to evaluate these rare diseases.
Zusammenfassung
Spinale Gefäßmalformationen sind seltene und vor der Einführung der Magnetresonanztomographie noch deutlich seltener diagnostizierte Erkrankungen, die unbehandelt zu einer meist progredienten Schädigung des Rückenmarks durch Veränderung der spinalen Zirkulation führen. In Abhängigkeit vom Typ der vaskulären Malformation kann diese Schädigung durch Blutungen (subarachnoidal oder intramedullär), venöse Stauung mit Kongestionsmyelopathie, Durchblutungsstörungen oder Raumforderungseffekte hervorgerufen werden. Auch ein „Steal“-Phänomen wird diskutiert. Während symptomatische vaskuläre Malformationen immer behandlungsbedürftig sind, ist die Behandlung inzidenteller spinaler vaskulärer Malformationen aufgrund ihres unklaren natürlichen Verlaufs weniger gesichert. Die Therapie spinaler vaskulärer Malformationen hängt im Wesentlichen von der Angioarchitektur ab, so dass diese der im vorliegenden Übersichtsartikel benutzten Klassifikation zugrunde gelegt wird. Prinzipiell unterscheidet sich die Einteilung der spinalen Gefäßmalformationen nicht von der Einteilung vaskulärer Malformationen des Gehirns. Während durale arteriovenöse Shunts von Gefäßen gespeist werden, die normalerweise die Dura versorgen (radikulomeningeale Gefäße), werden die pialen (oder intraduralen) arteriovenösen Shunts von Gefäßen gespeist, die normalerweise das Rückenmark versorgen. Die vaskulären Malformationen des Rückenmarks, die nicht mit einem arteriovenösen Kurzschluss einhergehen, sind die spinalen Kavernome.
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*Dedicated to Professor Armin Thron, MD, on the occasion of his 65th birthday.
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Krings, T. Vascular Malformations of the Spine and Spinal Cord*. Clin Neuroradiol 20, 5–24 (2010). https://doi.org/10.1007/s00062-010-9036-6
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DOI: https://doi.org/10.1007/s00062-010-9036-6
Key Words
- Spinal dural arteriovenous fistulas
- Spinal vascular malformations
- Spinal vascular anatomy
- Spinal cavernomas