title: Spinal arteriovenous fistulae in patients with hereditary hemorrhagic telangiectasia: A case report and systematic review of the literature.
abstract: BACKGROUND
  Although rare, spinal arteriovenous malformations (AVMs) are thought to be more prevalent in the hereditary Hereditary Hemorrhagic Telangiectasia (HHT) population.
  
  
  METHODS
  We report a case of a spinal AVM in a 37-year-old female with HHT treated with endovascular embolization. In addition, we report findings from a systematic review of the literature on the clinical characteristics, angioarchitecture, and clinical outcomes of HHT patients with spinal AVMs.
  
  
  RESULTS
  The patient is a 37 year-old female with definite HHT who presented with a one-year history of progressive gait difficulty. The spinal fistula was incidentally detected on chest computed tomography (CT). Spinal angiography demonstrated a large perimedullary arteriovenous fistula was supplied by a posterolateral spinal artery. The fistula was treated with detachable coils. The patient made a complete neurological recovery. Our systematic review yielded 25 additional cases of spinal AVMs in HHT patients. All fistulae were perimedullary (100.0%). Treatments were described in 24 of the 26 lesions. Endovascular-only treatment was performed in 16 cases (66.6%) and surgical-only treatment was performed in five cases (20.8%). Complete or near-complete occlusion rates were 86.7% (13/15) for endovascular treated cases, 100.0% (4/4) for surgery and 66.6% (2/3) for combined treatments. Overall, 80.0% of patients (16/20) reported improvement in function following treatment, 100.0% (5/5) in the surgery group and 84.6% (11/13) reported improvement in the endovascular group.
  
  
  CONCLUSIONS
  Spinal fistulae in HHT patients are usually type IV perimedullary fistulae. Both endovascular and surgical treatments appeared to be effective in treating these lesions. However, it is clear that endovascular therapy has become the preferred treatment modality.
authors.affiliation: Department of Radiology, Mayo Clinic, USA brinjikji.waleed@mayo.edu.
authors.forename: Waleed
authors.initials: W
authors.lastname: Brinjikji
authors.affiliation: Department of Neurology, Mayo Clinic, USA.
authors.forename: Deena M
authors.initials: DM
authors.lastname: Nasr
authors.affiliation: Department of Radiology, Mayo Clinic, USA.
authors.forename: Harry J
authors.initials: HJ
authors.lastname: Cloft
authors.affiliation: Department of Pulmonology and Critical Care Medicine, Mayo Clinic, USA.
authors.forename: Vivek N
authors.initials: VN
authors.lastname: Iyer
authors.affiliation: Department of Neurosurgery, Mayo Clinic, USA.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lanzino
mesh_terms: D000328:Adult; D001164:Arteriovenous Fistula / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy*; D004621:Embolization, Therapeutic / Q000379:methods*; D005260:Female; D006801:Humans; D013116:Spinal Cord / Q000098:blood supply*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*
keywords: Hereditary hemorrhagic telangiectasia; spinal fistula
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