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

Abstract

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.