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Vein of Galen malformation

MedGen UID:
287069
•Concept ID:
C1562503
•
Congenital Abnormality
Synonyms: Galen Malformations Veins; Malformations Vein, Galen; Malformations Veins, Galen; Vein Of Galen Malformation; Vein of Galen Malformations
SNOMED CT: Vein of Galen malformation (416792008)

Definition

An arteriovenous malformation in the vein of Galen that is located at the base of the brain. The malformation may result in developmental delays, hydrocephalus, seizures, and congestive heart failure. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVein of Galen malformation

Professional guidelines

PubMed

Epidemiology, Diagnostics, and Management of Vein of Galen Malformation.
Brevis Nuñez F, Dohna-Schwake C
Pediatr Neurol 2021 Jun;119:50-55. Epub 2021 Mar 3 doi: 10.1016/j.pediatrneurol.2021.02.007. PMID: 33895584
Multimodality Management of Vein of Galen Malformations-An Institutional Experience.
Hosmann A, El-Garci A, Gatterbauer B, Bavinzski G, Knosp E, Gruber A
World Neurosurg 2018 Apr;112:e837-e847. Epub 2018 Feb 3 doi: 10.1016/j.wneu.2018.01.172. PMID: 29408495
Evolution of treatment options for vein of Galen malformations.
Khullar D, Andeejani AM, Bulsara KR
J Neurosurg Pediatr 2010 Nov;6(5):444-51. doi: 10.3171/2010.8.PEDS10231. PMID: 21039167

Recent clinical studies

Etiology

Evolution of transvenous embolization in vein of Galen malformation: case series and review of the literature.
Fifi JT, Bazil MJ, Matsoukas S, Shigematsu T, Sorscher M, Berenstein A
J Neurointerv Surg 2023 Jun;15(6):579-583. Epub 2022 Jul 14 doi: 10.1136/jnis-2022-019121. PMID: 35835461
Epidemiology, Diagnostics, and Management of Vein of Galen Malformation.
Brevis Nuñez F, Dohna-Schwake C
Pediatr Neurol 2021 Jun;119:50-55. Epub 2021 Mar 3 doi: 10.1016/j.pediatrneurol.2021.02.007. PMID: 33895584
Cerebral vascular malformations and their imaging modalities.
Zafar A, Fiani B, Hadi H, Arshad M, Cathel A, Naeem M, Parsons MS, Farooqui M, Bucklin AA, Leone MJ, Baig A, Quadri SA
Neurol Sci 2020 Sep;41(9):2407-2421. Epub 2020 Apr 25 doi: 10.1007/s10072-020-04415-4. PMID: 32335778
Long-term outcome of vein of Galen malformation.
Taffin H, Maurey H, Ozanne A, Durand P, Husson B, Knebel JF, Adamsbaum C, Deiva K, Saliou G
Dev Med Child Neurol 2020 Jun;62(6):729-734. Epub 2019 Nov 12 doi: 10.1111/dmcn.14392. PMID: 31713850
Pediatric Congenital Cerebrovascular Anomalies.
Goyal P, Mangla R, Gupta S, Malhotra A, Almast J, Sapire J, Kolar B
J Neuroimaging 2019 Mar;29(2):165-181. Epub 2018 Oct 31 doi: 10.1111/jon.12575. PMID: 30378722

Diagnosis

Neurovascular disease and syndromes: Diagnosis and therapy in children.
Nesbit GM
Handb Clin Neurol 2021;176:305-323. doi: 10.1016/B978-0-444-64034-5.00015-8. PMID: 33272401
Cerebral arteriovenous fistulae.
Alexander MD, Darflinger R, Cooke DL, Halbach VV
Handb Clin Neurol 2021;176:179-198. doi: 10.1016/B978-0-444-64034-5.00004-3. PMID: 33272395
Vein of Galen Malformation.
Cordova EG, Levy P, Kheir JN, Orbach DB, Barnewolt C, Estroff JA
Neoreviews 2020 Oct;21(10):e678-e686. Epub 2020 Oct 1 doi: 10.1542/neo.21-10-e678. PMID: 33004561
Human genetics and molecular mechanisms of vein of Galen malformation.
Duran D, Karschnia P, Gaillard JR, Karimy JK, Youngblood MW, DiLuna ML, Matouk CC, Aagaard-Kienitz B, Smith ER, Orbach DB, Rodesch G, Berenstein A, Gunel M, Kahle KT
J Neurosurg Pediatr 2018 Apr;21(4):367-374. Epub 2018 Jan 19 doi: 10.3171/2017.9.PEDS17365. PMID: 29350590
Vein of Galen malformation.
Hoang S, Choudhri O, Edwards M, Guzman R
Neurosurg Focus 2009 Nov;27(5):E8. doi: 10.3171/2009.8.FOCUS09168. PMID: 19877798

Therapy

Factors Contributing to Major Neurological Complications From Vein of Galen Malformation Embolization.
Bhatia K, Mendes Pereira V, Krings T, Ter Brugge K, Kortman H, Dirks P, Armstrong D, Shroff M, Muthusami P
JAMA Neurol 2020 Aug 1;77(8):992-999. doi: 10.1001/jamaneurol.2020.0825. PMID: 32338714Free PMC Article
Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications.
Meila D, Grieb D, Melber K, Jacobs C, Maslehaty H, Petridis A, El Habony R, Lanfermann H, Scholz M, Brassel F
Acta Neurochir (Wien) 2016 Jul;158(7):1279-84. Epub 2016 May 13 doi: 10.1007/s00701-016-2836-y. PMID: 27173099
Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis.
Yan J, Wen J, Gopaul R, Zhang CY, Xiao SW
J Neurosurg 2015 Oct;123(4):872-90. Epub 2015 Jul 31 doi: 10.3171/2014.12.JNS141249. PMID: 26230476
Management and outcome of vein of Galen malformation.
McSweeney N, Brew S, Bhate S, Cox T, Roebuck DJ, Ganesan V
Arch Dis Child 2010 Nov;95(11):903-9. Epub 2010 Jul 6 doi: 10.1136/adc.2009.177584. PMID: 20605862
Anesthesia and intracranial arteriovenous malformation.
Sinha PK, Neema PK, Rathod RC
Neurol India 2004 Jun;52(2):163-70. PMID: 15269462

Prognosis

The composition of landmark vein of Galen malformation research: the emergence of endovascular treatments.
Lu VM, Luther EM, Silva MA, Rangwala SD, Starke RM, Smith ER, See AP
Childs Nerv Syst 2023 Mar;39(3):733-741. Epub 2022 Sep 23 doi: 10.1007/s00381-022-05687-1. PMID: 36149485
Hydrocephalus in Vein of Galen Malformations.
Paramasivam S
Neurol India 2021 Nov-Dec;69(Supplement):S376-S379. doi: 10.4103/0028-3886.332279. PMID: 35102992
Long-term outcome of vein of Galen malformation.
Taffin H, Maurey H, Ozanne A, Durand P, Husson B, Knebel JF, Adamsbaum C, Deiva K, Saliou G
Dev Med Child Neurol 2020 Jun;62(6):729-734. Epub 2019 Nov 12 doi: 10.1111/dmcn.14392. PMID: 31713850
Pediatric Congenital Cerebrovascular Anomalies.
Goyal P, Mangla R, Gupta S, Malhotra A, Almast J, Sapire J, Kolar B
J Neuroimaging 2019 Mar;29(2):165-181. Epub 2018 Oct 31 doi: 10.1111/jon.12575. PMID: 30378722
Vein of Galen malformation.
Hoang S, Choudhri O, Edwards M, Guzman R
Neurosurg Focus 2009 Nov;27(5):E8. doi: 10.3171/2009.8.FOCUS09168. PMID: 19877798

Clinical prediction guides

Epidemiology, Diagnostics, and Management of Vein of Galen Malformation.
Brevis Nuñez F, Dohna-Schwake C
Pediatr Neurol 2021 Jun;119:50-55. Epub 2021 Mar 3 doi: 10.1016/j.pediatrneurol.2021.02.007. PMID: 33895584
Long-term outcome of vein of Galen malformation.
Taffin H, Maurey H, Ozanne A, Durand P, Husson B, Knebel JF, Adamsbaum C, Deiva K, Saliou G
Dev Med Child Neurol 2020 Jun;62(6):729-734. Epub 2019 Nov 12 doi: 10.1111/dmcn.14392. PMID: 31713850
EphrinB2-EphB4-RASA1 Signaling in Human Cerebrovascular Development and Disease.
Zeng X, Hunt A, Jin SC, Duran D, Gaillard J, Kahle KT
Trends Mol Med 2019 Apr;25(4):265-286. Epub 2019 Feb 25 doi: 10.1016/j.molmed.2019.01.009. PMID: 30819650Free PMC Article
Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series.
Gopalan V, Rennie A, Robertson F, Kanagarajah L, Toolis C, Bhate S, Ganesan V
Dev Med Child Neurol 2018 Apr;60(4):424-429. Epub 2018 Jan 23 doi: 10.1111/dmcn.13676. PMID: 29359331
Management and outcome of vein of Galen malformation.
McSweeney N, Brew S, Bhate S, Cox T, Roebuck DJ, Ganesan V
Arch Dis Child 2010 Nov;95(11):903-9. Epub 2010 Jul 6 doi: 10.1136/adc.2009.177584. PMID: 20605862

Recent systematic reviews

Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis.
Yan J, Wen J, Gopaul R, Zhang CY, Xiao SW
J Neurosurg 2015 Oct;123(4):872-90. Epub 2015 Jul 31 doi: 10.3171/2014.12.JNS141249. PMID: 26230476

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