Bringing high-grade arteriovenous malformations under control: clinical outcomes following multimodality treatment in children

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  • 1 Departments of Neurological Surgery,
  • 3 Radiation Oncology,
  • 6 Radiology and Biomedical Imaging, and
  • 8 Pediatrics;
  • 4 Pediatric Stroke and Cerebrovascular Disease Center, Department of Neurology; and
  • 5 Center for Cerebrovascular Research, Department of Anesthesia and Perioperative Care, University of California, San Francisco, California;
  • 2 Department of Neurosurgery, Baylor Medical Center, Houston, Texas; and
  • 7 Department of Neurosurgery, Barrow Neurological Institute, Phoenix, Arizona
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OBJECTIVE

Brain arteriovenous malformations (AVMs) consist of dysplastic blood vessels with direct arteriovenous shunts that can hemorrhage spontaneously. In children, a higher lifetime hemorrhage risk must be balanced with treatment-related morbidity. The authors describe a collaborative, multimodal strategy resulting in effective and safe treatment of pediatric AVMs.

METHODS

A retrospective analysis of a prospectively maintained database was performed in children with treated and nontreated pediatric AVMs at the University of California, San Francisco, from 1998 to 2017. Inclusion criteria were age ≤ 18 years at time of diagnosis and an AVM confirmed by a catheter angiogram.

RESULTS

The authors evaluated 189 pediatric patients with AVMs over the study period, including 119 ruptured (63%) and 70 unruptured (37%) AVMs. The mean age at diagnosis was 11.6 ± 4.3 years. With respect to Spetzler-Martin (SM) grade, there were 38 (20.1%) grade I, 40 (21.2%) grade II, 62 (32.8%) grade III, 40 (21.2%) grade IV, and 9 (4.8%) grade V lesions. Six patients were managed conservatively, and 183 patients underwent treatment, including 120 resections, 82 stereotactic radiosurgery (SRS), and 37 endovascular embolizations. Forty-four of 49 (89.8%) high-grade AVMs (SM grade IV or V) were treated. Multiple treatment modalities were used in 29.5% of low-grade and 27.3% of high-grade AVMs. Complete angiographic obliteration was obtained in 73.4% of low-grade lesions (SM grade I–III) and in 45.2% of high-grade lesions. A periprocedural stroke occurred in a single patient (0.5%), and there was 1 treatment-related death. The mean clinical follow-up for the cohort was 4.1 ± 4.6 years, and 96.6% and 84.3% of patients neurologically improved or remained unchanged in the ruptured and unruptured AVM groups following treatment, respectively. There were 16 bleeding events following initiation of AVM treatment (annual rate: 0.02 events per person-year).

CONCLUSIONS

Coordinated multidisciplinary evaluation and individualized planning can result in safe and effective treatment of children with AVMs. In particular, it is possible to treat the majority of high-grade AVMs with an acceptable safety profile. Judicious use of multimodality therapy should be limited to appropriately selected patients after thorough team-based discussions to avoid additive morbidity. Future multicenter studies are required to better design predictive models to aid with patient selection for multimodal pediatric care, especially with high-grade AVMs.

ABBREVIATIONS ARUBA = A Randomized Trial of Unruptured Brain AVMs; AVM = arteriovenous malformation; DSA = digital subtraction angiography; mRS = modified Rankin Scale; NBCA = N-butyl cyanoacrylate; SAIVM = Scottish Audit of Intracranial Vascular Malformations; SM = Spetzler-Martin; SRS = stereotactic radiosurgery; Supp-SM = supplemented SM.

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Contributor Notes

Correspondence Nalin Gupta: UCSF Benioff Children’s Hospital, University of California, San Francisco, CA. nalin.gupta@ucsf.edu.

INCLUDE WHEN CITING Published online April 10, 2020; DOI: 10.3171/2020.1.PEDS19487.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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