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Epidemiology, Diagnostics, and Management of Vein of Galen Malformation
Abstract
Background Vein of Galen malformation (VGM) is a rare congenital intracerebral vascular malformation. The aim of this study was to gain reliable data about epidemiology; clinical manifestations; and pre-, peri- and postinterventional treatment strategies in Germany. Methods This prospective epidemiologic study was conducted by Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland, a hospital-based German nationwide surveillance unit for rare pediatric diseases. Patients aged upto 18 years with a new diagnosis of VGM were prospectively included between January 1, 2014 and December 31, 2015. Results In the two-year study period, 30 individuals with newly diagnosed VGM were identified, resulting in an estimated annual incidence rate of VGM in Germany of at least 1: 58,100 live births. The main symptoms were age dependent. Neonates presented with mainly cardiac manifestations of high output failure; infants and older children mainly suffered from neurological complications. Of the patients with endovascular treatment 95% survived, but only 41% of these patients were discharged home without any complications. The Bicêtre Neonatal Evaluation Score was a good predictor for poor outcome but could have led to palliative care in some patients with good outcome. Conclusions The incidence of VGM in Germany is estimated to be 1:58,100 live births. The majority of patients were eligible for endovascular treatment. Nevertheless, due to side effects and long-term sequelae in…
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“this vascular pathology is exceptionally rare (less than 1 in 50,000 live births [41]).”
From Rapid patient-specific neural networks for intraoperative X-ray to volume registration (2025) · cites this paper as [41]
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Cited by
Rapid patient-specific neural networks for intraoperative X-ray to volume registration
Gopalakrishnan, V., Chlorogiannis, D.-D., Abumoussa, A., Larson, A. M., Haouchine, N., Orbach, D. B., Frisken, S., Dey, N., & Golland, P. (2025) · arXiv · doi:10.48550/arxiv.2503.16309 · Cited by 13
Metadata
- Authors
- Brevis Nuñez, F., & Dohna-Schwake, C.
- Venue
- Pediatric Neurology
- Publisher
- Elsevier BV
- Published
- 2021-06
- Volume
- 119
- Pages
- 50-55
- ISSN
- 0887-8994 (ISSN-L)
- OpenAlex
- W3133772163
- Cited by
- 35
- References
- 38
- Citations by year
- 2026: 8 · 2025: 7 · 2024: 7 · 2023: 9 · 2022: 4
- FWCI
- 2.41
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- www.elsevier.com/legal/tdmrep-license (tdm)
- doi.org/10.15223/policy-017 (stm-asf)
- doi.org/10.15223/policy-037 (stm-asf)
- doi.org/10.15223/policy-012 (stm-asf)
- doi.org/10.15223/policy-029 (stm-asf)
- doi.org/10.15223/policy-004 (stm-asf)
- Locations
- Pediatric Neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Vascular Malformations Diagnosis and Treatment
- Health Sciences › Medicine › Surgery › Vascular Malformations and Hemangiomas
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
- Keywords
- vein of Galen malformation, incidence, epidemiology, endovascular treatment, neurological complications, high-output heart failure, neonatal outcome prediction, palliative care, pediatric rare diseases, Germany
- MeSH terms
- Child, Preschool, Female, Humans, Infant, Infant, Newborn, Male, Prospective Studies, Vein of Galen Malformations, Endovascular Procedures
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