Document Detail

Adult presentation of a familial-associated vein of galen aneurysmal malformation: case report.
MedLine Citation:
PMID:  21107153     Owner:  NLM     Status:  MEDLINE    
BACKGROUND AND IMPORTANCE: Vein of Galen aneurysmal malformations (VGAMs) arise from persistent arteriovenous shunting from primitive choroidal vessels into the median prosencephalic vein of Markowski, the embryonic precursor of the vein of Galen. VGAMs rarely present past infancy, and their natural history in adults is unknown. We report the first case of a familial-associated VGAM in an asymptomatic adult female patient. The clinical features of this case are presented alongside a systematic review of the literature on adult VGAM cases to assess the natural history, clinical management, and genetic basis of this rare neurovascular lesion.
CLINICAL PRESENTATION: A previously healthy 44-year-old woman with a family history of a VGAM in a stillborn presented with an 8-week onset of dizziness and vertigo that spontaneously resolved. Time-resolved magnetic resonance angiography identified a choroidal VGAM. No intervention was undertaken at this time because of the patient's asymptomatic status after 9 months of follow-up.
CONCLUSION: Based on our review of the literature, this is the first case report of a familial-associated VGAM in an adult patient and suggests that VGAM development can be genetically linked. Of 15 adult VGAM cases previously reported, all patients were either symptomatic or treated, thus precluding determination of VGAM natural history in adults. Patient outcomes correlated with the severity of presenting symptoms, which ranged from asymptomatic to immediately life-threatening. We hypothesize that self-selection may render VGAMs to be more benign for them to persist past childhood. Further investigation of the molecular biology underlying VGAM development is warranted.
David S Xu; Asad A Usman; Michael C Hurley; Christopher S Eddleman; Bernard R Bendok
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Neurosurgery     Volume:  67     ISSN:  1524-4040     ISO Abbreviation:  Neurosurgery     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-11-25     Completed Date:  2011-03-22     Revised Date:  2011-08-25    
Medline Journal Info:
Nlm Unique ID:  7802914     Medline TA:  Neurosurgery     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E1845-51; discussion 1851     Citation Subset:  IM    
Department of Neurological Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611, USA.
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MeSH Terms
Cerebral Angiography
Embolization, Therapeutic / methods
Intracranial Aneurysm / complications*,  radiography
Magnetic Resonance Angiography
Middle Aged
Treatment Outcome
Vein of Galen Malformations / complications*,  radiography,  therapy*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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