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Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Spinal arteriovenous shunts presenting before 2 years of age: analysis of 13 cases
Sean Cullen1, Hortensia Alvarez, Georges Rodesch
1Department of Diagnostic and Therapeutic Neuroradiology, Bicetre Hospital, Kremlin-Bicetre, France.
Insights
Spinal arteriovenous malformations (SAVM) presenting in infants are often high-flow fistulas, frequently associated with hereditary hemorrhagic telangiectasia (HHT). Endovascular treatment offers a favorable outcome for these pediatric spinal vascular lesions.
Area of Science:
- Pediatric Neuroradiology
- Vascular Neurology
- Interventional Radiology
Background:
- Spinal arteriovenous malformations (SAVM) are rare, with a small subset presenting symptomatically in neonates and infants.
- Understanding factors associated with early presentation is crucial for timely diagnosis and management.
- This study reviews clinical and anatomical factors influencing early SAVM presentation and treatment outcomes.
Purpose of the Study:
- To analyze clinical and anatomical factors associated with early presentation of spinal arteriovenous malformations (SAVM) in patients under two years of age.
- To review the institution's experience in treating these pediatric SAVM cases.
- To identify specific characteristics that predict early-onset SAVM.
Main Methods:
- Retrospective review of clinical records and imaging studies for patients with SAVM presenting before two years of age.
- Prospective data collection on clinical, imaging, and treatment details.
- Analysis of patient demographics, lesion types, associated syndromes, and treatment modalities.
Main Results:
- Thirteen pediatric patients (13% of total SAVM) presented before age two, with a mean age of 6.9 months.
- Neurologic symptoms were present in 11 patients; 10 lesions were spinal cord arteriovenous fistulas (SCAVF), and 8 had syndromic associations (6 with HHT).
- Endovascular treatment with n-butyl cyanoacrylate achieved complete obliteration in 7/9 treated patients, with a favorable outcome (stable or improved) in all treated cases.
Conclusions:
- High-flow solitary fistulas and hereditary hemorrhagic telangiectasia (HHT) are associated with early SAVM presentation in infants.
- The presence of spinal cord arteriovenous fistulas (SCAVF) in young children strongly suggests HHT.
- Endovascular treatment is effective and associated with favorable outcomes for pediatric SAVM, despite their aggressive nature.
Background And Purpose:
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.
Conclusion:
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.
