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Abstract
In this review, we describe the pathophysiology, diagnosis, and treatment of spinal dorsal intradural arteriovenous fistulas (DI-AVFs), focusing on novel research areas. DI-AVFs compose the most common subgroup of spinal arteriovenous lesions and most commonly involve the thoracic spine, followed by lumbar and sacral segments. The pathogenesis underlying DI-AVFs is an area of emerging understanding, thought to be attributable to venous congestion and hypertension that precipitate ascending myelopathy. Patients with DI-AVFs typically present with motor, sensory, or urinary dysfunction, although a wide swath of other less common symptoms has been reported. DI-AVFs can be subdivided by spinal region, which in turn is associated with 4 distinct clinical phenotypes: craniocervical junction (CCJ), subaxial cervical, thoracic, and lumbosacral. Patients with CCJ and lumbosacral DI-AVFs have particularly interesting presentations and treatment considerations. High-value diagnostic findings on MRI include flow voids, missing-piece sign, and T2-weighted intramedullary hyperintensity. However, digital subtraction angiography is the gold standard for diagnosis and localization of DI-AVFs and for definitive treatment planning. Surgical disconnection of DI-AVFs is almost universally curative and frontline treatment, especially for CCJ and lumbosacral DI-AVFs. Endovascular techniques evolve in promising ways, such as improved visualization, distal access, and liquid embolic techniques. The pathophysiology of DI-AVFs is better understood using newly identified radiologic diagnostic markers. Despite new techniques and devices introduced in the endovascular field, surgery remains the gold-standard treatment for DI-AVFs.
Glossary
- 3D=
- 3 dimensional;
- AI=
- artificial intelligence;
- AVF=
- arteriovenous fistula;
- AVM=
- arteriovenous malformation;
- CCJ=
- craniocervical junction;
- DI-AVF=
- dorsal intradural arteriovenous fistula;
- DSA=
- digital subtraction angiography;
- ICG-VA=
- indocyanine green videoangiography;
- MRA=
- magnetic resonance angiography;
- n-BCA=
- n-butyl cyanoacrylate
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Associate Editor Rebecca Burch, MD.
- Received August 26, 2022.
- Accepted in final form March 6, 2023.
- © 2023 American Academy of Neurology
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