Elsevier

Mayo Clinic Proceedings

Volume 80, Issue 2, February 2005, Pages 269-281
Mayo Clinic Proceedings

Symposium on Cerebrovascular Diseases
Natural History, Evaluation, and Management of Intracranial Vascular Malformations

https://doi.org/10.4065/80.2.269Get rights and content

Intracranial vascular malformations are seen increasingly in clinical practice, primarily because of advances in cross-sectional brain and spinal cord imaging. Commonly encountered lesion types include arteriovenous malformations, cavernous malformations, venous malformations, dural arteriovenous fistulas, and capillary telangiectasias. Patients can experience various symptoms and signs at presentation. The natural history of vascular malformations depends on lesion type, location, size, and overall hemodynamics. The natural history for each lesion subtype is reviewed, with special consideration of the risk of hemorrhage or other adverse outcomes after the lesion is detected and any known predictors of hemorrhage or other outcomes. In practice, these data are compared with the risk of available treatment options as the optimal management is clarified. A multidisciplinary approach including neurosurgery, radiosurgery, interventional neuroradiology, and vascular neurology is most useful in determining the best management strategy.

Section snippets

INCIDENCE

Population data are scant regarding the overall incidence of intracranial vascular malformations; however, they are apparently about 1/7 as common as intracranial saccular aneurysms.3 In a large autopsy series, the overall frequency of detection of AVMs was 1.4% (46 AVMs among 3200 brain tumor cases).4 In that series, 12.2% of patients were symptomatic. A population-based study from Olmsted County, Minnesota, reported that the age- and sex-adjusted incidence for intracranial vascular

ARTERIOVENOUS MALFORMATIONS

Arteriovenous malformations are congenital lesions that occur most commonly in the supratentorial region. These space-occupying vascular lesions consist of multiple arteries and veins, connecting as a fistula without an intervening normal capillary bed. Typically, AVMs are single lesions, except when associated with hereditary hemorrhagic telangiectasia.6 The histology includes clusters of normal or dilated arteries and abnormal veins with calcification and occasionally some prior hemorrhage.

CAVERNOUS MALFORMATIONS

A CM is typically a small mulberry or “popcornlike” lesion in the brain, spinal cord, or nerve roots that consists of a tangle of intertwined clusters of sinusoidal vascular channels. The overall frequency of CMs, also called cavernous angiomas, cavernomas, or cavernous hemangiomas, was approximately 0.4% in a large autopsy study of 4069 people.66, 67 A retrospective review of MRI scans reported a detection rate of 0.4% to 0.9%.68, 69 Cavernous malformations are uncommon in children,70 with

VENOUS MALFORMATIONS

A VM is physiologically important to brain tissue because it provides normal venous drainage. Venous malformations are thin-walled venous channels with normal intervening neural tissue. Computed tomography with contrast enhancement shows a large draining vein, although non- contrast-enhanced CT results are usually normal. Magnetic resonance imaging is sensitive; it shows a flow void and may show a radial pattern, the so-called classic caput medusae pattern.96 Arteriography is required rarely

DURAL ARTERIOVENOUS FISTULAS

Dural arteriovenous fistulas (DAVFs), also called dural AVMs, involve a vascular malformation of the wall of one of the major venous sinuses.1, 103 Venous outflow obstruction, sometimes associated with sinus thrombosis, can precede formation of some DAVFs. Head CT typically does not detect DAVFs, although dilated veins may suggest their presence; MRI also may detect these lesions, showing the diagnostic dilated veins and feeding arteries in an appropriate location, but is not sensitive.

CAPILLARY TELANGIECTASIAS

On pathological examination, capillary telangiectasias are somewhat enlarged ectatic capillaries found typically with other vascular malformations.1, 77, 80 They are detected frequently and were the second most common intracranial vascular malformation in one series.1 Capillary telangiectasias are noted often in the posterior fossa or spinal cord and have an overall frequency of about 0.1%.1 They are extremely unlikely to cause symptoms, although there are case reports of patients with

CONCLUSION

Intracranial vascular malformations are seen commonly in clinical practice, particularly as CT and MRI are performed increasingly. The range of symptoms and signs at presentation is myriad. Natural history depends on lesion type, location, size, and overall hemodynamics. Management decisions are often complex. A multidisciplinary approach including neurosurgery, radiosurgery, interventional neuroradiology, and vascular neurology is most useful in determining the best management strategy.

REFERENCES (146)

  • K Jellinger

    Vascular malformations of the central nervous system: a morphological overview

    Neurosurg Rev

    (1986)
  • WJ Michelson

    Natural history and pathophysiology of arteriovenous malformations

    Clin Neurosurg

    (1978)
  • H Olivecrona et al.

    Arteriovenous aneurysms of the brain: their diagnosis and treatment

    Arch Neurol Psychiatry

    (1948)
  • RD Brown et al.

    Incidence and prevalence of intracranial vascular malformations in Olmsted County, Minnesota, 1965 to 1992

    Neurology

    (1996)
  • W Kucharczyk et al.

    Intracranial vascular malformations: MR and CT imaging

    Radiology

    (1985)
  • RD Brown et al.

    Frequency of intracranial hemorrhage as a presenting symptom and subtype analysis: a population-based study of intracranial vascular malformations in Olmsted County, Minnesota

    J Neurosurg

    (1996)
  • RD Brown et al.

    The natural history of unruptured intracranial arteriovenous malformations

    J Neurosurg

    (1988)
  • RH Wilkins

    Natural history of intracranial vascular malformations: a review

    Neurosurgery

    (1985)
  • PM Crawford et al.

    Arteriovenous malformations of the brain: natural history in unoperated patients

    J Neurol Neurosurg Psychiatry

    (1986)
  • CJ Graf et al.

    Bleeding from cerebral arteriovenous malformations as part of their natural history

    J Neurosurg

    (1983)
  • SL Ondra et al.

    The natural history of symptomatic arteriovenous malformations of the brain: a 24-year follow-up assessment

    J Neurosurg

    (1990)
  • D Kondziolka et al.

    Simple risk predictions for arteriovenous malformation hemorrhage

    Neurosurgery

    (1995)
  • RD Brown

    Simple risk predictions for arteriovenous malformation hemorrhage [letter]

    Neurosurgery

    (2000)
  • DM Forster et al.

    Arteriovenous malformations of the brain: a long-term clinical study

    J Neurosurg

    (1972)
  • A Hartmann et al.

    Morbidity of intracranial hemorrhage in patients with cerebral arteriovenous malformation

    Stroke

    (1998)
  • DH Duong et al.

    Feeding artery pressure and venous drainage pattern are primary determinants of hemorrhage from cerebral arteriovenous malformations

    Stroke

    (1998)
  • Y Miyasaka et al.

    An analysis of the venous drainage system as a factor in hemorrhage from arteriovenous malformations

    J Neurosurg

    (1992)
  • A Kader et al.

    The influence of hemodynamic and anatomic factors on hemorrhage from cerebral arteriovenous malformations

    Neurosurgery

    (1994)
  • JC Drummond et al.

    Cerebral physiology and the effects of anesthetics and techniques

  • RD Brown et al.

    Unruptured intracranial aneurysms and arteriovenous malformations: frequency of intracranial hemorrhage and relationship of lesions

    J Neurosurg

    (1990)
  • AG Amias

    Cerebral vascular disease in pregnancy, I: haemorrhage

    J Obstet Gynaecol Br Commonw

    (1970)
  • JL Robinson et al.

    Arteriovenous malformations, aneurysms, and pregnancy

    J Neurosurg

    (1974)
  • JL Robinson et al.

    Subarrachnoid hemorrhage in pregnancy

    J Neurosurg

    (1972)
  • JC Horton et al.

    Pregnancy and the risk of hemorrhage from cerebral arteriovenous malformations

    Neurosurgery

    (1990)
  • H Olivecrona et al.

    Congenital Arteriovenous Aneurysms of the Carotid and Vertebral Arterial System

    (1957)
  • RA Solomon et al.

    Management of arteriovenous malformations of the brain stem

    J Neurosurg

    (1986)
  • MG Yaçsargil

    Microneurosurgery. Volume 3B: AVM of the Brain, Clinical Considerations, General and Special Operative Techniques, Surgical Results, Nonoperated Cases, Cavernous and Venous Angiomas, Neuroanaesthesia

    (1988)
  • MG Yaçsargil

    Microneurosurgery. Volume 3A: AVM of the Brain, History, Embryology, Pathological Considerations, Hemodynamics, Diagnostic Studies, Microsurgical Anatomy

    (1987)
  • H Batjer et al.

    Arteriovenous malformations of the posterior fossa: clinical presentation, diagnostic evaluation, and surgical treatment

    J Neurosurg

    (1986)
  • CG Drake

    Cerebral arteriovenous malformations: considerations for and experience with surgical treatment in 166 cases

    Clin Neurosurg

    (1979)
  • RC Heros et al.

    Surgical excision of cerebral arteriovenous malformations: late results

    Neurosurgery

    (1990)
  • DG Piepgras et al.

    Seizure outcome in patients with surgically treated cerebral arteriovenous malformations

    J Neurosurg

    (1993)
  • TM Sundt et al.

    Surgery for supratentorial arteriovenous malformations

    Clin Neurosurg

    (1991)
  • RF Spetzler et al.

    A proposed grading system for arteriovenous malformations

    J Neurosurg

    (1986)
  • BF Schneider et al.

    Histopathology of arteriovenous malformations after gamma knife radiosurgery

    J Neurosurg

    (1997)
  • BE Pollock et al.

    Factors associated with successful arteriovenous malformation radiosurgery

    Neurosurgery

    (1998)
  • CG Hadjipanayis et al.

    Stereotactic radiosurgery for motor cortex region arteriovenous malformations

    Neurosurgery

    (2001)
  • BE Pollock et al.

    Stereotactic radiosurgery for postgeniculate visual pathway arteriovenous malformations

    J Neurosurg

    (1996)
  • TL Ellis et al.

    Analysis of treatment failure after radiosurgery for arteriovenous malformations

    J Neurosurg

    (1998)
  • BE Pollock et al.

    Repeat stereotactic radiosurgery of arteriovenous malformations: factors associated with incomplete obliteration

    Neurosurgery

    (1996)
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