Extradural arteriovenous fistulas involving the vertebral artery in neurofibromatosis Type 1: Case report

Sergio Paolini, Claudio Colonnese, Vittorio Galasso, Roberta Morace, Serena Tola, Vincenzo Esposito, Giampaolo Cantore

Research output: Contribution to journalArticle

Abstract

Spinal extradural arteriovenous fistulas (AVFs) are rare lesions that may be associated with neurofibromatosis Type 1 (NF1). In these patients, the shunt typically occurs between the V2 segment of the vertebral artery and the epidural venous plexus. Previously, reported cases have been treated either by using endovascular embolization or, sporadically, by open surgery. In surgical reports, proximal deafferentation or manipulation of the venous portion of the shunt - including suture, resection, or open embolization of the epidural ectasia - was attempted with variable results. The authors report on a case of a young patient with NF1 who underwent emergency surgical disconnection of a cervical extradural AVF after previously unsuccessful endovascular and surgical therapy. The lesion drained into a giant intrathecal varix, causing severe myelopathy. After surgery, the patient recovered almost completely. This experience clarified the surgical anatomy of these malformations and showed that, when surgery is necessary, the optimal treatment providing complete and permanent cure of this condition is direct closure of the epidural shunt pedicle.

Original languageEnglish
Pages (from-to)181-185
Number of pages5
JournalJournal of Neurosurgery: Spine
Volume8
Issue number2
DOIs
Publication statusPublished - Feb 2008

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Keywords

  • Extradural arteriovenous fistula
  • Neurofibromatosis
  • Vertebral artery

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery
  • Neurology

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