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DOI: 10.3233/PNR-14191
Vein of Galen Malformations: Long-term functional outcome in 6 cases
Subject Editor:
Publication History
06 May 2014
30 June 2014
Publication Date:
29 July 2015 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00029169/201404/lookinside/thumbnails/10.3233-pnr-14191-1.jpg)
Abstract
PURPOSE: Vein of Galen Malformations (VOGMs) are rare congenital arteriovenous fistulas that were previously associated with high mortality and morbidity prior to the advent of endovascular treatment approaches. The long-term functional outcome and quality of life of infants and children treated with these approaches, however, are not well characterized.
METHODS: The long-term outcomes of a cohort of pediatric patients treated at University of California at San Francisco from 1986–2013 (N = 39) for their VOGMs were assessed using a self-report survey-based approach. The Pediatric Quality of life Inventory (PedsQL), the Functional Disability Inventory (FDI) and the World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0) were employed. The results of the surveys were analyzed.
RESULTS: We received six surveys from three male and three female patients with a median age of 17.5 years (range 7–22 years). The median length of follow-up since first treatment was 17 years (range 5–19 years). The PedsQL summary scores of the patients in our sample were only slightly lower than norms from healthy controls (78.4 vs. 83.0). The cases in our sample were on an average mildly affected in the domains of physical health, emotional health, social functioning and school functioning. The FDI and WHODAS results revealed that survey respondents had minimal or no disability.
CONCLUSION: The long-term neurodevelopmental outcomes of children with VOGM may be good following endovascular treatment. Better long-term follow up is needed, as evidenced by the low rate of survey response.