To the Editor:
We would like to thank the authors for their letter1 and thoughtful commentary regarding our recent study.2 We are grateful for the opportunity to discuss issues pertinent to the interpretation of our observations, especially about the different prevalence of the association between developmental venous anomaly (DVA) and diffuse intrinsic pontine gliomas (DIPG).
We have demonstrated a frequency higher than 20% of DVA in adult patients harbouring a diffuse glioma2 but also in paediatric patients harbouring a DIPG3 as compared to a control group. Complimentary deoxyribonucleic acid sequencing of frozen cerebral DVA-associated glioma samples are underway to uncover somatic genetic changes, with a specific focus on postzygotic mutations in PIK3CA.4
The difference in prevalence the authors observe between their dataset (4.95% of extralesional DVAs in 101 DIPG patients) and our dataset (24.1% of extralesional plus intralesional DVAs in 162 DIPG patients) can be explained by several factors. Beyond those already raised by the authors in their letter, one may question the definition of a DVA and methodological aspects. We defined a DVA as a cluster of multiple tubular and radially oriented veins that converge into a single dilated venous collector, resulting in a fan-shaped appearance (ie, caput medusae),5 whatever the size of the DVA. In addition, image acquisition and processing may account for the observed differences. We encourage the authors to compare their result to a control group obtained under same image parameters since we observed a higher prevalence of DVAs in our DIPG group, than in our control group, and than in the general population.
However, the interesting and though provoking results presented by the authors highlight the need of a multicenter analysis of the DVA prevalence both in the adult and paediatric glioma with a multiple blinded imaging assessment followed by a face-to-face consensus evaluation in case of discrepancies.
The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.
1. Bray DP, Buster BE, Boucher Ab, Wrubel DM. Letter: high prevalence of developmental venous anomaly in diffuse intrinsic pontine gliomas: a pediatric control study. Neurosurgery. 2020;87(4):E525-E526.
2. Roux A, Edjlali M, Porelli S, et al. Developmental venous anomaly in adult patients with diffuse glioma? Neurology. 2019;92(1):e55-e62.
3. Roux A, Boddaert N, Grill J, et al. High prevalence of developmental venous anomaly in diffuse intrinsic pontine gliomas: a pediatric control study. Neurosurgery. 2019;86(4):517-523.
4. Roux A, Vikkula M, Pallud J. Letter: is developmental venous anomaly an imaging biomarker of PIK3CA mutated gliomas? Neurosurgery. 2019;86(1):E93.
5. Linscott LL, Leach JL, Jones BV, Abruzzo TA. Developmental venous anomalies of the brain in children – imaging spectrum and update. Pediatr Radiol. 2016;46(3):394-406; quiz 391-393.