Multimodal treatment strategies for complex pediatric cerebral arteriovenous fistulas: contemporary case series at Barrow Neurological Institute

J Neurosurg Pediatr. 2015 Jun;15(6):615-24. doi: 10.3171/2014.11.PEDS14468. Epub 2015 Mar 27.


OBJECT Pediatric cerebral arteriovenous fistulas (AVFs) are rare but potentially lethal vascular lesions. Management strategies for these lesions have undergone considerable evolution in the last decade with the advent of new endovascular, surgical, and radiosurgical technologies. This study sought to review current treatment strategies and long-term clinical outcomes at a high-volume cerebrovascular institute. METHODS A retrospective chart review was performed on patients with a diagnosis of cerebral AVF from 1999 to 2012. Patients with carotid-cavernous fistulas, vein of Galen malformations, and age > 18 years were excluded from final analysis. Medical history, surgical and nonsurgical treatment, and clinical outcomes were documented. Pre- and postoperative angiograms were analyzed to assess for obliteration of the fistula. RESULTS Seventeen patients with pial AVFs (29.4%), dural AVFs (64.7%), or mixed pial/dural AVFs (5.9%) were identified. The majority of lesions were paramedian (70.6%) and supratentorial (76.5%). The study population had a mean age of 6.4 years, with a slight male predominance (52.9%), and the most common presenting symptoms were seizures (23.5%), headaches (17.6%), congestive heart failure (11.7%), and enlarging head circumference (11.7%). Among patients who underwent intervention (n = 16), 56.3% were treated with endovascular therapy alone, 6.3% were treated with open surgery alone, and 37.5% required a multimodal approach. Overall, 93.8% of the treated patients received endovascular treatment, 43.8% received open surgery, and 12.5% received radiosurgery. Endovascular embolysates included Onyx (n = 5), N-butyl cyanoacrylate (NBCA; n = 4), or coil embolization (n = 7) with or without balloon assistance (n = 2). Complete angiographic obliteration was achieved in 87.5% at the last follow-up evaluation (mean follow-up 3.1 years). One infant with incomplete AVF obliteration died of congestive heart failure, and 1 patient with complete obliteration died of acute sinus thrombosis, with an overall complication rate of 18.8%. CONCLUSIONS Pediatric cerebral AVFs are challenging neurosurgical lesions. Although advancements in endovascular therapy in the last decade have greatly changed the natural course of this disease, a multidisciplinary approach remains necessary for a large subset of patients. Surgeon experience with a thorough analysis of preoperative imaging is paramount to achieving acceptable clinical outcomes.

Keywords: ACA = anterior cerebral artery; AP = anteroposterior; AVF = arteriovenous fistula; AVM = arteriovenous malformation; DSA = digital subtraction angiography; ECA = external carotid artery; GKS = Gamma Knife surgery; GOS = Glasgow Outcome Scale; ICA = internal carotid artery; MMA = middle meningeal artery; NBCA; NBCA = N-butyl cyanoacrylate; OA = occipital artery; Onyx; PCA = posterior cerebral artery; STA = superficial temporal artery; VA = vertebral artery; arteriovenous fistula; dural; endovascular; multimodal; pial; stereotactic radiosurgery; vascular disorders.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Algorithms
  • Arizona
  • Arteriovenous Fistula / complications
  • Arteriovenous Fistula / diagnosis
  • Arteriovenous Fistula / surgery
  • Arteriovenous Fistula / therapy*
  • Carotid Arteries / pathology
  • Carotid Arteries / surgery*
  • Cavernous Sinus / pathology
  • Cavernous Sinus / surgery*
  • Cerebral Angiography
  • Chemoembolization, Therapeutic* / methods
  • Child
  • Combined Modality Therapy / methods
  • Dura Mater / blood supply
  • Endovascular Procedures*
  • Female
  • Humans
  • Intracranial Arteriovenous Malformations / complications
  • Intracranial Arteriovenous Malformations / diagnosis
  • Intracranial Arteriovenous Malformations / surgery
  • Intracranial Arteriovenous Malformations / therapy*
  • Magnetic Resonance Imaging
  • Male
  • Medical Records
  • Pia Mater / blood supply
  • Radiosurgery*
  • Retrospective Studies
  • Seizures / etiology
  • Treatment Outcome
  • Vein of Galen Malformations / therapy