eMedicine Specialties > Neurology > Neuro-vascular Diseases

Arteriovenous Malformations: Differential Diagnoses & Workup

Author: H Christian Schumacher, MD, Postdoctoral Residency Fellow, Doris and Stanley Tananbaum Stroke Center, Columbia University Presbyterian 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: May 31, 2006

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 (see Images 1-3).
    • 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 (see Image 4) but is inadequate to delineate the morphology of feeding arteries and draining veins; small aneurysms can be missed easily.
  • Cerebral angiography
    • Angiogram is required for hemodynamic assessment, which is essential for planning treatment (see Image 5).
    • 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
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References

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

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

  3. Al-Shahi R, Bhattacharya JJ, Currie DG. 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].

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

  5. 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].

  6. Flickinger JC, Kondziolka D, Lunsford LD. 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].

  7. 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].

  8. Hartmann A, Mast H, Mohr JP, et al. Determinants of staged endovascular and surgical treatment outcome of brain arteriovenous malformations. Stroke. Nov 2005;36(11):2431-5. [Medline]. [Full Text].

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

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  11. 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].

  12. 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].

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  14. Ogilvy CS, Stieg PE, Awad I. 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].

  15. Stapf C, Mast H, Sciacca RR. The New York Islands AVM Study: design, study progress, and initial results. Stroke. May 2003;34(5):e29-33. [Medline].

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Further Reading

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Keywords

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

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Contributor Information and Disclosures

Author

H Christian Schumacher, MD, Postdoctoral Residency Fellow, Doris and Stanley Tananbaum Stroke Center, Columbia University Presbyterian Medical Center
H Christian Schumacher, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, and American Medical Association
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
Randolph S Marshall, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, and American Medical Association
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: Nothing to disclose.

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; Pfizer Honoraria Speaking and teaching; Novartis Consulting fee Review panel membership

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD, Professor, Department of Neurology, Oregon Health and 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; Boston Scientific Honoraria Speaking and teaching; Concentric Medical None Review panel membership; Northstar Neuroscience  Review panel membership; ev3 Consulting fee Review panel membership

 
 
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