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Updated: Jul 13, 2026

Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Diagnosis and endovascular treatment of pediatric spinal arteriovenous shunts
1Department of Radiology and Neurosurgery, Brigham and Women's Hospital, Francis Street, Boston, MA 02115, USA.
Insights
Spinal arteriovenous shunts (SAVSs) in children are rare but serious. Key features include genetic links, male predominance, and frequent hemorrhage, yet they often respond well to endovascular treatment.
Area of Science:
- Neurology
- Pediatric Medicine
- Vascular Malformations
Background:
- Spinal arteriovenous shunts (SAVSs) are uncommon in pediatric patients, yet carry significant morbidity risks.
- Distinct clinical features characterize pediatric SAVSs compared to adult populations.
- Understanding these unique pediatric features is crucial for accurate diagnosis and management.
Purpose of the Study:
- To delineate the specific characteristics and clinical behavior of spinal arteriovenous shunts in infants and children.
- To highlight the association of pediatric SAVSs with genetic abnormalities.
- To evaluate the efficacy of endovascular treatment in this patient group.
Main Methods:
- Review of pediatric cases diagnosed with spinal arteriovenous shunts.
- Analysis of clinical presentation, associated conditions, and treatment outcomes.
- Comparison of pediatric SAVS features with those reported in adult populations.
Main Results:
- Pediatric SAVSs show a pronounced male predominance and higher frequency of hemorrhagic presentation than adults.
- Associations with genetic syndromes like hereditary hemorrhagic telangiectasia and Cobb syndrome are common.
- Despite severe symptoms, endovascular treatment frequently leads to anatomic cure or symptom stabilization.
Conclusions:
- Pediatric spinal arteriovenous shunts possess unique clinical and genetic associations.
- Early diagnosis and intervention, particularly endovascular therapy, can yield favorable outcomes in children.
- Further research is needed to fully elucidate the natural history and optimize management strategies for pediatric SAVSs.
Abstract:
Spinal arteriovenous shunts (SAVSs) are rarely diagnosed in infants and children, but they are important clinically because morbidity can be significant. Although these lesions do not form a distinct pathologic group separate from the SAVSs seen in older patients, experience with these malformations in the pediatric population has led to the identification of several important features that are characteristic of this group of SAVSs. Association with genetic abnormalities, heritable (hereditary hemorrhagic telangiectasia) and nonheritable somatic (spinal arteriovenous metameric syndrome or Cobb syndrome), is relatively common and likely underrecognized. Male predominance is more pronounced than in the adult population. Hemorrhagic presentation is more frequent than in adults, except in extremely young children. The natural history seems to be better than previously thought, with early rehemorrhage uncommon. Despite early presentation and severe symptoms, these lesions are frequently amenable to endovascular treatment, often with anatomic cure achieved and with improvement or stabilization of symptoms after partial targeted treatment.
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