Spontaneous spinal epidural arteriovenous fistulae in neurofibromatosis type-1.

摘要: Abstract Background NF-1 is one of the most common autosomal-dominantly inherited genetic disorders with an incidence approximately 1:3500. We report a case and review literature to characterize spontaneous spinal AVF that occur in neurofibromatosis (NF-1). Case Report A 51-year-old woman presented progressive radiculomyelopathy. Angiography revealed terminating giant intraspinal epidural varix extending paraspinally through C3/4 neural foramen. Trapping attempted 18 years earlier prevented endovascular access for embolization, vigorous bleeding made direct surgical resection impossible. Therefore, as palliation, arterial feeding collaterals were occluded, surgically exposed tortuous veins packed coils. Laminectomies partial resulted subtotal occlusion clinical improvement. Conclusion The associated appears show dominant venous drainage extradural paraspinal plexus without evidence intradural drainage. vertebral artery typically origin fistula. numerous collateral feeders may give AVM-like appearance. Clinically, fistulae produce syndromic triad including symptoms NF-1, radiculomyelopathy, bruit. Treatment attack on fistula by either surgery or embolization. If, however, approach cannot be chosen, vessels combined laminectomies can result long-term symptomatic