Cerebral Arteriovenous Malformations in Children Homepage

Authors

Edward Smith, MD
Zsombor Gal, BA
Ryan Kellogg, MD
Heather Spader, MD

Section Editors

Edward Smith, MD
Gianpiero Tamburrini, MD
Alfred Pokmeng See, MD

Editor in Chief

Rick Abbott, MD

Introduction

An AVM is a direct arterial-to-venous connection without intervening capillaries. AVMs are relatively common and can occur in the cerebral hemispheres, brainstem, and spinal cord (1). They can present with hemorrhage, seizure, headache, or progressive ischemia (“steal”), or they may be found incidentally. AVMs are among the most important vascular anomalies in the nervous system of children and usually require treatment that involves obliteration of the lesion. This can be achieved by surgery, radiation, embolization, or a combination of therapies (2). Surgical resection has been associated with favorable neurological outcomes in up to 90% of patients and very high rates (approaching 100%) of radiographic obliteration (110,111). Radiosurgery is associated with obliteration and favorable neurological outcomes in at least 80% of patients with smaller AVMs (103). Perioperative bleeding and new neurological deficits are known complications of treatment (110,112). Long-term disability and mortality may be observed in up to 7.2% and 5%, respectively, of children who undergo surgery (110,112).

Key Points

• AVMs common: AVMs are the most common symptomatic intracranial vascular abnormality in children (3). The prevalence of cerebral AVMs has been estimated to be between 5.47 and 18 per 100,000 people (4,5,6).
• High mortality rate in children: Hemorrhagic events from an AVM in childhood have been associated with a 12% to 25% mortality rate (7,8).
• CT and MR angiography used for screening: CT and MR angiography are employed as the initial preferred study for children presenting to the emergency department with nontraumatic ICH, followed by catheter-based DSA if an AVM is found (9).
• Treatment decisions vary by patient: Acuteness of presentation, AVM characteristics, and patient/family preferences are different for each patient with an AVM. Therefore, a detailed risk-benefit analysis should inform treatment decisions.
• Best treated by a multidisciplinary team: Treatment for pediatric AVMs should be performed at experienced centers with multidisciplinary teams able to offer all modalities of therapy (surgery, embolization, and stereotactic radiosurgery) whenever possible (10,11).