Spinal arteriovenous shunts presenting before 2 years of age: analysis of 13 cases

Background: A minority of patients with spinal arteriovenous malformations (SAVM) are symptomatic as neonates or infants. We analyzed the clinical and anatomic factors associated with this early presentation, and reviewed our experience in treating patients with these lesions. Methods: A retrospective review of clinical records and imaging studies was performed for patients with SAVM who presented at less than 2 years of age and who were examined by the diagnostic and interventional neuroradiology service at our institution. Clinical, imaging and treatment data had been entered prospectively into a department database. Results: Thirteen patients were identified that had SAVMs that were either diagnosed or that became symptomatic before 2 years of age (nine boys, four girls). These represented 13% of the SAVM seen during the same period of time. The mean age at presentation was 6.9 +/- 6.4 months. Of the 13 patients, 11 had neurologic symptoms attributable to the spinal lesion. The presentation was nonhemorrhagic in ten patients and associated with hemorrhage in three. Ten lesions were fistulas [spinal cord arteriovenous fistulas (SCAVFs)] (77%); two were of the nidus type of malformation. There were syndromic associations in eight patients: hereditary hemorrhagic telangectasia (HHT) in six patients, all but one of these lesions were intradural high-flow perimedullary SCAVFs (46% of overall SAVM and 56% of SCAVF), and spinal arteriovenous metameric syndrome in two patients. One patient had Hirschprung's anomaly. Eight patients underwent endovascular treatment alone, one had surgery and embolization, and four were not treated. In all patients undergoing embolization, n-butyl cyanoacrylate liquid adhesive was used. Of those patients who underwent endovascular treatment, the lesion was completely obliterated in 7 and a 90% reduction in nidal size was achieved in the remaining 2. There was one treatment complication (infection) which resolved with medical therapy. No procedure-related permanent morbidity or mortality was seen. Follow-up data were available (mean 31.4 months) in ten patients (nine treated). All treated patients were either stable or improved, with none showing further deterioration following treatment. Conclusions: Factors associated with early presentation in neonates and infants with spinal arteriovenous shunts include the presence of high-flow, solitary fistulas and HHT. Specifically, the presence of SCAVF in a child less than 2 years of age is highly suggestive of HHT. Despite the aggressive nature of these lesions, many are amenable to endovascular treatment, and this is associated with a favorable posttreatment course in most cases.