eMedicine Specialties > Neurology > Neuro-vascular Diseases

Arteriovenous Malformations: Differential Diagnoses & Workup

Author: H Christian Weston-Schumacher, MD, Assistant Professor of Neurology (Vascular Neurology), Division of Vascular Neurology and Neurocritical Care, The Saul R Korey Department of Neurology, Albert Einstein College of Medicine; Director, Stroke Service, Jack D Weiler Hospital, Montefiore Medical Center
Coauthor(s): Randolph S Marshall, MD, Co-Director, Levine Cerebral Localization Lab, Associate Professor, Department of Neurology, Division of Cerebrovascular Diseases, Columbia University Presbyterian Medical Center
Contributor Information and Disclosures

Updated: Jan 28, 2010

Differential Diagnoses

Amyloid Angiopathy
Headache: Pediatric Perspective
Anterior Circulation Stroke
Intracranial Hemorrhage
Cardioembolic Stroke
Migraine Headache
Cavernous Sinus Syndromes
Migraine Headache: Neuro-Ophthalmic Perspective
Cerebral Aneurysms
Migraine Headache: Pediatric Perspective
Cerebral Venous Thrombosis
Migraine Variants
Chronic Paroxysmal Hemicrania
Moyamoya Disease
Cluster Headache
Posterior Cerebral Artery Stroke
Dissection Syndromes
Subarachnoid Hemorrhage
Fibromuscular Dysplasia
Vein of Galen Malformation

Other Problems to Be Considered

Arteriovenous fistula (traumatic)
Cavernous hemangioma
Dural arteriovenous fistulas
Venous malformation
Neuroimaging of vascular malformations and hematomas of the brain

Workup

Imaging Studies

  • High-quality imaging studies are the key to diagnosis of AVMs.
  • CT scan
    • CT scanning easily identifies an intracerebral hemorrhage, raising suspicion of AVM in a younger person or a patient without clear risk factors for hemorrhage.
    • CT scan can identify only large AVMs.
  • MRI
    • MRI is essential for initial diagnosis of AVMs.
    • AVMs appear as irregular or globoid masses anywhere within the hemispheres or brain stem, as shown in the images below.

      • Axial T2 MRI showing an arteriovenous malformatio...

        Axial T2 MRI showing an arteriovenous malformation, with hemorrhage, in the territory of the left posterior cerebral artery.

        Axial T2 MRI showing an arteriovenous malformatio...

        Axial T2 MRI showing an arteriovenous malformation, with hemorrhage, in the territory of the left posterior cerebral artery.


      • T1 axial MRI showing a small subcortical arteriov...

        T1 axial MRI showing a small subcortical arteriovenous malformation in the right frontal lobe.

        T1 axial MRI showing a small subcortical arteriov...

        T1 axial MRI showing a small subcortical arteriovenous malformation in the right frontal lobe.


      • T2 coronal MRI showing an arteriovenous malformat...

        T2 coronal MRI showing an arteriovenous malformation in the left medial temporal lobe.

        T2 coronal MRI showing an arteriovenous malformat...

        T2 coronal MRI showing an arteriovenous malformation in the left medial temporal lobe.

    • AVMs may be cortical, subcortical, or in deep gray or white matter.
    • Small, round, low-signal spots within or around the mass on T1, T2, or fluid-attenuated inversion recovery (FLAIR) sequences are the "flow voids" of feeding arteries, intranidal aneurysms, or draining veins.
    • If hemorrhage has occurred, the mass of blood may obscure other diagnostic features, requiring angiogram or follow-up MRI.
    • Low signal of extracellular hemosiderin may be seen around or within the AVM mass, indicating prior symptomatic or asymptomatic hemorrhage.
    • Larger aneurysms within the AVM or on feeding arteries may be identified occasionally.
    • Magnetic resonance angiography (MRA) may identify AVMs greater than 1 cm in size, as in the image below, but is inadequate to delineate the morphology of feeding arteries and draining veins; small aneurysms can be missed easily.

      • Magnetic resonance angiography showing a left med...

        Magnetic resonance angiography showing a left medial temporal arteriovenous malformation.

        Magnetic resonance angiography showing a left med...

        Magnetic resonance angiography showing a left medial temporal arteriovenous malformation.

  • Cerebral angiography
    • Angiogram, shown below, is required for hemodynamic assessment, which is essential for planning treatment.

      • Angiogram (anteroposterior view) showing an arter...

        Angiogram (anteroposterior view) showing an arteriovenous malformation in the deep left middle cerebral artery territory measuring approximately 3 cm in diameter, with a deep draining vein (arrow).

        Angiogram (anteroposterior view) showing an arter...

        Angiogram (anteroposterior view) showing an arteriovenous malformation in the deep left middle cerebral artery territory measuring approximately 3 cm in diameter, with a deep draining vein (arrow).

    • The morphology of the AVM determines the treatment algorithm. Important features include feeding arteries, venous drainage pattern, and arterial and venous aneurysms.
    • Ten to fifty-eight percent of patients with AVM have aneurysms located in vessels remote from the AVM, in arteries feeding the AVM, or within the nidus of the AVM itself.
    • Intranidal aneurysms may have a higher risk of rupture than those outside the bounds of the AVM.
    • Other important angiographic features may include kinking or ectasia of draining veins, which can cause venous congestion, thrombosis, or rupture; and stenosis of feeding arteries due to angiopathy caused by high-velocity, turbulent flow into the fistula.
    • Special expertise is required to perform superselective catheterization into AVM feeding arteries, which allows both pressure measurements and superselective anesthetic injections to map neurological function in and around the AVM (see Superselective angiography in Procedures).
  • Functional MRI
    • Use of functional MRI (fMRI) is becoming more common to map brain function during treatment planning for AVMs.
    • Localization of language, memory, vision, motor, or sensory function may be obtained to help identify "eloquent" brain regions in and around the AVM prior to treatment by embolization, radiation, or surgery.

Procedures

  • Superselective angiography
    • Superselective angiography is performed with standard cerebral angiography, with access via a femoral artery puncture.
    • A special, flexible, directable catheter is threaded up into one of the main cerebral arteries (carotid or vertebral), then into sequentially smaller branch arteries, until the catheter tip is near or within the AVM nidus.
    • Pressure measurements can be obtained via a coaxial catheter. Higher feeding pressures increase the risk of subsequent hemorrhage.
    • Sodium amytal, an anesthetic agent, can be injected to produce temporary anesthesia of the area perfused by the artery. In this so-called "superselective Wada testing," language, memory, visual-spatial, sensory, and motor function can be tested during 5 minutes of anesthetic effect to determine whether "eloquent" function originates in that region, which would therefore be at risk for neurological deficits should that brain area be injured during embolization or surgery. Arteries directly feeding the AVM or "en passage" vessels that feed the AVM but continue past the AVM to feed normal brain tissue can be studied.

More on Arteriovenous Malformations

Overview: Arteriovenous Malformations
Differential Diagnoses & Workup: Arteriovenous Malformations
Treatment & Medication: Arteriovenous Malformations
Follow-up: Arteriovenous Malformations
Multimedia: Arteriovenous Malformations
References

References

  1. Weinsheimer S, Kim H, Pawlikowska L, Chen Y, Lawton MT, Sidney S, et al. EPHB4 gene polymorphisms and risk of intracranial hemorrhage in patients with brain arteriovenous malformations. Circ Cardiovasc Genet. Oct 2009;2(5):476-82. [Medline].

  2. Laakso A, Dashti R, Juvela S, Niemelä M, Hernesniemi J. Natural history of arteriovenous malformations: presentation, risk of hemorrhage and mortality. Acta Neurochir Suppl. 2010;107:65-9. [Medline].

  3. Stapf C, Mast H, Sciacca RR, Berenstein A, Nelson PK, Gobin YP, et al. The New York Islands AVM Study: design, study progress, and initial results. Stroke. May 2003;34(5):e29-33. [Medline].

  4. Mast H, Young WL, Koennecke HC. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. Oct 11 1997;350(9084):1065-8. [Medline].

  5. Halim AX, Johnston SC, Singh V. Longitudinal risk of intracranial hemorrhage in patients with arteriovenous malformation of the brain within a defined population. Stroke. Jul 2004;35(7):1697-702. [Medline].

  6. ARUBA Investigators. Unruptured brain arteriovenous malformation trial. [The Internet Stroke Center]. Feb 2006;[Full Text].

  7. ARUBA Study. Unruptured brain arteriovenous malformation trial. [ARUBA Study Site]. Feb 2006;[Full Text].

  8. Ogilvy CS, Stieg PE, Awad I, Brown RD Jr, Kondziolka D, Rosenwasser R. AHA Scientific Statement: Recommendations for the management of intracranial arteriovenous malformations: a statement for healthcare professionals from a special writing group of the Stroke Council, American Stroke Association. Stroke. Jun 2001;32(6):1458-71. [Medline].

  9. Hernesniemi J, Romani R, Lehecka M, Isarakul P, Dashti R, Celik O, et al. Present state of microneurosurgery of cerebral arteriovenous malformations. Acta Neurochir Suppl. 2010;107:71-6. [Medline].

  10. Castel JP, Kantor G. [Postoperative morbidity and mortality after microsurgical exclusion of cerebral arteriovenous malformations. Current data and analysis of recent literature]. Neurochirurgie. May 2001;47(2-3 Pt 2):369-83. [Medline].

  11. Al-Shahi R, Bhattacharya JJ, Currie DG, Papanastassiou V, Ritchie V, Roberts RC, et al. Prospective, population-based detection of intracranial vascular malformations in adults: the Scottish Intracranial Vascular Malformation Study (SIVMS). Stroke. May 2003;34(5):1163-9. [Medline].

  12. ApSimon HT, Reef H, Phadke RV, Popovic EA. A population-based study of brain arteriovenous malformation: long-term treatment outcomes. Stroke. Dec 2002;33(12):2794-800. [Medline].

  13. Flickinger JC, Kondziolka D, Lunsford LD, Pollock BE, Yamamoto M, Gorman DA. A multi-institutional analysis of complication outcomes after arteriovenous malformation radiosurgery. Int J Radiat Oncol Biol Phys. Apr 1 1999;44(1):67-74. [Medline].

  14. Hartmann A, Mast H, Mohr JP, Pile-Spellman J, Connolly ES, Sciacca RR. Determinants of staged endovascular and surgical treatment outcome of brain arteriovenous malformations. Stroke. Nov 2005;36(11):2431-5. [Medline][Full Text].

  15. Hillman J. Population-based analysis of arteriovenous malformation treatment. J Neurosurg. Oct 2001;95(4):633-7. [Medline].

  16. Hofmeister C, Stapf C, Hartmann A, et al. Demographic, morphological, and clinical characteristics of 1289 patients with brain arteriovenous malformation. Stroke. Jun 2000;31(6):1307-10. [Medline][Full Text].

  17. Maruyama K, Kawahara N, Shin M. The risk of hemorrhage after radiosurgery for cerebral arteriovenous malformations. N Engl J Med. Jan 13 2005;352(2):146-53. [Medline].

  18. Nataf F, Ghossoub M, Schlienger M. Bleeding after radiosurgery for cerebral arteriovenous malformations. Neurosurgery. Aug 2004;55(2):298-305; discussion 305-6. [Medline].

Further Reading

Keywords

cerebrovascular malformation, vascular malformation, AVM, cerebral AVM, stroke, cerebral hemorrhage, intracranial hemorrhage, arteriovenous malformations, cerebral arteriovenous malformations, AVMs, hemorrhagic stroke

Contributor Information and Disclosures

Author

H Christian Weston-Schumacher, MD, Assistant Professor of Neurology (Vascular Neurology), Division of Vascular Neurology and Neurocritical Care, The Saul R Korey Department of Neurology, Albert Einstein College of Medicine; Director, Stroke Service, Jack D Weiler Hospital, Montefiore Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

Randolph S Marshall, MD, Co-Director, Levine Cerebral Localization Lab, Associate Professor, Department of Neurology, Division of Cerebrovascular Diseases, Columbia University Presbyterian Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Edward L Hogan, MD, Professor, Department of Neurology, Medical College of Georgia; Emeritus Professor and Chair, Department of Neurology, Medical University of South Carolina
Edward L Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Neurological Association, American Society for Biochemistry and Molecular Biology, Phi Beta Kappa, Sigma Xi, Society for Neuroscience, and Southern Clinical Neurological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association
Disclosure: Boehringer Ingelheim Honoraria Speaking and teaching; BMS/Sanofi Honoraria Speaking and teaching; Novartis Honoraria Speaking and teaching

Chief Editor

Helmi L Lutsep, MD, Professor, Department of Neurology, Oregon Health & Science University; Associate Director, Oregon Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association
Disclosure: Co-Axia Consulting fee Review panel membership; Talecris Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Concentric Medical Consulting fee Review panel membership; Abbott Consulting fee Consulting; Sanofi  Consulting

 
 
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