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Vascular malformation of the nervous system

MedGen UID:: 156014
•Concept ID:: C0752155
•: Anatomical Abnormality

Synonym:	Central Nervous System Vascular Malformations
SNOMED CT:	Vascular malformation of the nervous system (253193002)

Definition

A congenital abnormality of the arteries and veins that is located in the brain or spina cord, and which may increase the risk of bleeding, possibly resulting in impaired mental or physical function. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVVascular malformation of the nervous system

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Abnormality of the nervous system
        Vascular malformation of the nervous system
        Arteriovenous Malformation, Intracranial
        Vein of Galen malformation
        Central Nervous System Cavernous Hemangioma
        Intracranial cavernous angioma
        Central Nervous System Venous Angioma
        Cerebral arteriovenous malformation
        Cerebral cavernous malformation
        Hereditary cavernous hemangioma of brain
        Cerebral cavernous malformation 1
        Cerebral cavernous malformation 2
        Cerebral cavernous malformation 3
        Cerebral cavernous malformation 4
        Meningioangiomatosis
        Sinus pericranii

Professional guidelines

PubMed

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Alkhaibary A, Alnefaie N, Alharbi A, Alammar H, Arishy AM, Alshaya W, Khairy S
Acta Neurol Belg 2023 Apr;123(2):359-366. Epub 2022 Nov 14 doi: 10.1007/s13760-022-02133-6. PMID: 36374476

Updates in the management of cranial dural arteriovenous fistula.

Baharvahdat H, Ooi YC, Kim WJ, Mowla A, Coon AL, Colby GP
Stroke Vasc Neurol 2020;5(1):50-58. Epub 2019 Nov 21 doi: 10.1136/svn-2019-000269. PMID: 32411408 Free PMC Article

Synopsis of Guidelines for the Clinical Management of Cerebral Cavernous Malformations: Consensus Recommendations Based on Systematic Literature Review by the Angioma Alliance Scientific Advisory Board Clinical Experts Panel.

Akers A, Al-Shahi Salman R, A Awad I, Dahlem K, Flemming K, Hart B, Kim H, Jusue-Torres I, Kondziolka D, Lee C, Morrison L, Rigamonti D, Rebeiz T, Tournier-Lasserve E, Waggoner D, Whitehead K
Neurosurgery 2017 May 1;80(5):665-680. doi: 10.1093/neuros/nyx091. PMID: 28387823 Free PMC Article

See all (962)

Recent clinical studies

Etiology

Pediatric Cerebral Cavernous Malformations.

Paddock M, Lanham S, Gill K, Sinha S, Connolly DJA
Pediatr Neurol 2021 Mar;116:74-83. Epub 2020 Nov 27 doi: 10.1016/j.pediatrneurol.2020.11.004. PMID: 33494000

Updates in the management of cranial dural arteriovenous fistula.

Baharvahdat H, Ooi YC, Kim WJ, Mowla A, Coon AL, Colby GP
Stroke Vasc Neurol 2020;5(1):50-58. Epub 2019 Nov 21 doi: 10.1136/svn-2019-000269. PMID: 32411408 Free PMC Article

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

Spinal cavernous malformations.

Clark AJ, Wang DD, Lawton MT
Handb Clin Neurol 2017;143:303-308. doi: 10.1016/B978-0-444-63640-9.00030-8. PMID: 28552154

Spinal arteriovenous fistulae: surgical management.

Day AL, Turkmani AH, Chen PR
Handb Clin Neurol 2017;143:189-198. doi: 10.1016/B978-0-444-63640-9.00018-7. PMID: 28552141

See all (4874)

Diagnosis

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Alkhaibary A, Alnefaie N, Alharbi A, Alammar H, Arishy AM, Alshaya W, Khairy S
Acta Neurol Belg 2023 Apr;123(2):359-366. Epub 2022 Nov 14 doi: 10.1007/s13760-022-02133-6. PMID: 36374476

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

Spinal cavernous malformations.

Clark AJ, Wang DD, Lawton MT
Handb Clin Neurol 2017;143:303-308. doi: 10.1016/B978-0-444-63640-9.00030-8. PMID: 28552154

Intracranial arteriovenous malformations.

Barreau X, Marnat G, Gariel F, Dousset V
Diagn Interv Imaging 2014 Dec;95(12):1175-86. Epub 2014 Nov 24 doi: 10.1016/j.diii.2014.10.004. PMID: 25465120

Cavernous malformations.

Ryan D
J Neurosci Nurs 2010 Oct;42(5):294-9. doi: 10.1097/jnn.0b013e3181ecaf95. PMID: 20968226

See all (5166)

Therapy

Safety and efficacy of propranolol for treatment of familial cerebral cavernous malformations (Treat_CCM): a randomised, open-label, blinded-endpoint, phase 2 pilot trial.

Lanfranconi S, Scola E, Meessen JMTA, Pallini R, Bertani GA, Al-Shahi Salman R, Dejana E, Latini R; Treat_CCM Investigators
Lancet Neurol 2023 Jan;22(1):35-44. Epub 2022 Nov 17 doi: 10.1016/S1474-4422(22)00409-4. PMID: 36403580

Glue, Onyx, Squid or PHIL? Liquid Embolic Agents for the Embolization of Cerebral Arteriovenous Malformations and Dural Arteriovenous Fistulas.

Vollherbst DF, Chapot R, Bendszus M, Möhlenbruch MA
Clin Neuroradiol 2022 Mar;32(1):25-38. Epub 2021 Jul 29 doi: 10.1007/s00062-021-01066-6. PMID: 34324005 Free PMC Article

Updates in the management of cranial dural arteriovenous fistula.

Baharvahdat H, Ooi YC, Kim WJ, Mowla A, Coon AL, Colby GP
Stroke Vasc Neurol 2020;5(1):50-58. Epub 2019 Nov 21 doi: 10.1136/svn-2019-000269. PMID: 32411408 Free PMC Article

Pharmacotherapy for cavernous malformations.

Rudy RF, Du R
Handb Clin Neurol 2017;143:309-316. doi: 10.1016/B978-0-444-63640-9.00031-X. PMID: 28552155

Etiologies of intracerebral hematomas.

Wang QT, Tuhrim S
Curr Atheroscler Rep 2012 Aug;14(4):314-21. doi: 10.1007/s11883-012-0253-0. PMID: 22562528

See all (2232)

Prognosis

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Alkhaibary A, Alnefaie N, Alharbi A, Alammar H, Arishy AM, Alshaya W, Khairy S
Acta Neurol Belg 2023 Apr;123(2):359-366. Epub 2022 Nov 14 doi: 10.1007/s13760-022-02133-6. PMID: 36374476

Updates in the management of cranial dural arteriovenous fistula.

Baharvahdat H, Ooi YC, Kim WJ, Mowla A, Coon AL, Colby GP
Stroke Vasc Neurol 2020;5(1):50-58. Epub 2019 Nov 21 doi: 10.1136/svn-2019-000269. PMID: 32411408 Free PMC Article

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

Epidemiology of cavernous malformations.

Goldstein HE, Solomon RA
Handb Clin Neurol 2017;143:241-247. doi: 10.1016/B978-0-444-63640-9.00023-0. PMID: 28552146

Intracranial arteriovenous malformations.

Barreau X, Marnat G, Gariel F, Dousset V
Diagn Interv Imaging 2014 Dec;95(12):1175-86. Epub 2014 Nov 24 doi: 10.1016/j.diii.2014.10.004. PMID: 25465120

See all (3046)

Clinical prediction guides

Dural Arteriovenous Fistula.

El Naamani K, Tjoumakaris SI, Gooch MR, Jabbour P
Neurosurg Clin N Am 2024 Jul;35(3):331-342. Epub 2024 Mar 19 doi: 10.1016/j.nec.2024.02.005. PMID: 38782526

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Alkhaibary A, Alnefaie N, Alharbi A, Alammar H, Arishy AM, Alshaya W, Khairy S
Acta Neurol Belg 2023 Apr;123(2):359-366. Epub 2022 Nov 14 doi: 10.1007/s13760-022-02133-6. PMID: 36374476

The use of contrast-enhanced, time-resolved magnetic resonance angiography in cerebrovascular pathology.

Grossberg JA, Howard BM, Saindane AM
Neurosurg Focus 2019 Dec 1;47(6):E3. doi: 10.3171/2019.9.FOCUS19627. PMID: 31786556

Brainstem and cerebellar cavernous malformations.

Atwal GS, Sarris CE, Spetzler RF
Handb Clin Neurol 2017;143:291-295. doi: 10.1016/B978-0-444-63640-9.00028-X. PMID: 28552152

Emerging techniques for evaluation of the hemodynamics of intracranial vascular pathology.

Chang W, Huang M, Chien A
Neuroradiol J 2015 Feb;28(1):19-27. doi: 10.15274/NRJ-2014-10115. PMID: 25924168 Free PMC Article

See all (2654)

Recent systematic reviews

Intracranial Arterial Fenestration and Risk of Aneurysm: A Systematic Review and Meta-Analysis.

Guo X, Gao L, Shi Z, Liu D, Wang Y, Sun Z, Chen Y, Chen W, Yang Y
World Neurosurg 2018 Jul;115:e592-e598. Epub 2018 Apr 24 doi: 10.1016/j.wneu.2018.04.106. PMID: 29702312

Natural history of brain arteriovenous malformations: systematic review.

Goldberg J, Raabe A, Bervini D
J Neurosurg Sci 2018 Aug;62(4):437-443. Epub 2018 Mar 28 doi: 10.23736/S0390-5616.18.04452-1. PMID: 29595047

Clinical importance of the middle meningeal artery: A review of the literature.

Yu J, Guo Y, Xu B, Xu K
Int J Med Sci 2016;13(10):790-799. Epub 2016 Oct 17 doi: 10.7150/ijms.16489. PMID: 27766029 Free PMC Article

Brainstem cavernous malformations.

Gross BA, Batjer HH, Awad IA, Bendok BR
Neurosurgery 2009 May;64(5):E805-18; discussion E818. doi: 10.1227/01.NEU.0000343668.44288.18. PMID: 19404127

See all (209)

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Vascular malformation of the nervous system

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

Recent activity