Background and importance: Cauda equina radicular arteriovenous fistulas are rare "low flow" shunting lesions characterized by direct communication between the radicular artery and vein of a cauda equina nerve root. None have been associated with hereditary hemorrhagic telangiectasia and a high-flow cauda equina radicular arteriovenous fistula has never been reported. We present a unique case of a high-flow cauda equina radicular arteriovenous fistula in a patient with hereditary hemorrhagic telangiectasia. Marked flow-induced vascular remodeling posed significant diagnostic and therapeutic challenges which will be highlighted in this report.
Clinical presentation: A 39-year-old female with genetically confirmed hereditary hemorrhagic telangiectasia presented with progressive thoracic myelopathy secondary to a high-flow single-hole arteriovenous fistula below the conus. The feeding artery, arising from the anterior spinal artery, and draining vein had a paramedian course, favoring the diagnosis of a cauda equina radicular arteriovenous fistula (supplied by a proximal radicular artery) over a filum terminale arteriovenous fistula. Transarterial embolization was attempted but significant elongation and tortuosity of the anterior spinal artery precluded microcatheter access to the fistulous point. Surgical disconnection was successfully performed. The intraoperative findings supported the diagnosis of cauda equina radicular arteriovenous fistula. Delayed neurologic deterioration secondary to overshooting venous thrombosis was observed. She recovered after the initiation of therapeutic anticoagulation.
Conclusion: To the best of our knowledge, we hereby report the first high-flow cauda equina radicular arteriovenous fistula. The accurate differentiation of cauda equina radicular arteriovenous fistula from filum terminale arteriovenous fistulas, while challenging, is important to avoid treatment-related complications. Careful preoperative planning, the use of specialized endovascular and surgical techniques, and meticulous postoperative care can ensure the safe and complete disconnection of high-flow cauda equina radicular arteriovenous fistulas.
Keywords: Arteriovenous fistula; cauda equina; endovascular techniques; filum terminale; hereditary hemorrhagic telangiectasia.