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Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations

From the Departments of Neurology (Drs. Fullerton and Ferriero, A.R. Aminoff), Pediatrics (Drs. Fullerton, Ferriero, and Gupta), Neurosurgery (Dr. Gupta), and Radiology (Dr. Dowd), University of California, San Francisco.

Address correspondence and reprint requests to Dr. H.J. Fullerton, Department of Neurology, University of California, San Francisco, Box 0114, San Francisco, CA 94143-0114; e-mail: hjfc{at}itsa.ucsf.edu

Objective: To assess neurodevelopmental outcome after endovascular treatment of vein of Galen malformations (VOGM).

Methods: Outcome of patients who underwent endovascular treatment for VOGM between 1983 and 2002 was assessed by chart review and parental questionnaires. Development was classified as normal, minor delay (slow initial acquisition of milestones but no permanent disability), or significant delay (slow or incomplete acquisition of milestones with some permanent disability) using an adaptation of the Denver Developmental Questionnaire.

Results: Twenty-seven patients were identified: five presented prenatally (by ultrasound), 16 as neonates, and 6 after the neonatal period. The most common presenting features were congestive heart failure (CHF; 16/27) and hydrocephalus (8/27). The 16 patients with CHF all presented either prenatally or neonatally; 4 died acutely, 6 had significant delay, and 6 had no or minor developmental delay. Of those presenting in the perinatal period without CHF, all survived, two of five were significantly delayed, and three of five had no delay. Of those presenting after the neonatal period, all survived and only one of six had delay. By angiographic classification, outcome was worse for those with choroidal VOGM (3/13 died; 5/13 had significant delay) than for those with mural VOGM (2/10 had significant delay; none died). For the entire series, 52% of all cases (61% of survivors) had no or minor delay.

Conclusions: Fourteen of 27 children who received treatment for VOGM had a favorable outcome. Features associated with worse outcome were perinatal presentation, presence of CHF, and choroidal angioarchitecture.

Received March 28, 2003. Accepted in final form July 29, 2003.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the November 25 issue to find the title link for this article.

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