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General Considerations
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Arteriovenous malformations are congenital vascular malformations that result from a localized maldevelopment of part of the primitive vascular plexus and consist of abnormal arteriovenous communications without intervening capillaries They vary in size, ranging from massive lesions that are fed by multiple vessels and involve a large part of the brain to lesions so small that they are hard to identify at arteriography, surgery, or autopsy In approximately 10% of cases, there is an associated arterial aneurysm, while 1 2% of patients presenting with aneurysms have associated arteriovenous malformations Clinical presentation may relate to hemorrhage from the malformation or an associated aneurysm or may relate to cerebral ischemia due to diversion of blood by the anomalous arteriovenous shunt or due to venous stagnation Regional maldevelopment of the brain, compression or distortion of adjacent cerebral tissue by enlarged anomalous vessels, and progressive gliosis due to mechanical and ischemic factors may also be contributory In addition, communicating or obstructive hydrocephalus may occur and lead to symptoms
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Clinical Findings
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A Symptoms and Signs
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1 Supratentorial lesions Most cerebral arteriovenous malformations are supratentorial, usually lying in the territory of the middle cerebral artery Initial symptoms consist of hemorrhage in 30 60% of cases, recurrent seizures in 20 40%, headache in 5 25%, and miscellaneous complaints (including focal deficits) in 10 15% Up to 70% of arteriovenous malformations bleed at some point in their natural history, most commonly before the patient reaches the age of 40 years This tendency to bleed is unrelated to the lesion site or to the patient s sex, but small arteriovenous malformations are more likely to bleed than large ones Arteriovenous malformations that have bled once are more likely to bleed again Hemorrhage is commonly intracerebral as well as into the subarachnoid space, and it has a fatal outcome in about 10% of cases Focal or generalized seizures may accompany or follow hemorrhage, or they may be the initial presentation, especially with frontal or parietal arteriovenous malformations Headaches are especially likely when the external carotid arteries are involved in the malformation These sometimes simulate migraine but more commonly are nonspecific in character, with nothing about them to suggest an underlying structural lesion In patients presenting with subarachnoid hemorrhage, examination may reveal an abnormal mental status and signs of meningeal irritation Additional findings may help localize the lesion and sometimes indicate that intracranial pressure is increased A cranial bruit always suggests the possibility of a cerebral arteriovenous malformation, but bruits may also be found with aneurysms, meningiomas, acquired arteriovenous fistulas, and arteriovenous malformations involving the scalp, calvarium, or orbit Bruits are best heard over the ipsilateral eye or mastoid region and are of some help in lateralization but of no help in localization Absence of a bruit in no way excludes the possibility of arteriovenous malformation
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6 Arteriovenous Malformations
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ESSENTIALS OF DIAGNOSIS
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Sudden onset of subarachnoid and intracerebral hemorrhage Distinctive neurologic signs reflect the region of the brain involved Signs of meningeal irritation in patients presenting with subarachnoid hemorrhage Seizures or focal deficits may occur
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2 Infratentorial lesions Brainstem arteriovenous malformations are often clinically silent, but they may hemorrhage, cause obstructive hydrocephalus, or lead to progressive or relapsing brainstem deficits Cerebellar arteriovenous malformations may also be clinically inconspicuous but sometimes lead to cerebellar hemorrhage
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B Imaging
In patients presenting with suspected hemorrhage, CT scanning indicates whether subarachnoid or intracerebral bleeding has recently occurred, helps localize its source, and may reveal the arteriovenous malformation If the CT scan shows no evidence of bleeding but subarachnoid hemorrhage is diagnosed clinically, the cerebrospinal fluid should be examined When intracranial hemorrhage is confirmed but the source of hemorrhage is not evident on the CT scan, arteriography is necessary to exclude aneurysm or arteriovenous malformation MR angiography is not sensitive enough for this purpose Even if the findings on CT scan suggest arteriovenous malformation, arteriography is required to establish the nature of the lesion with certainty and to determine its anatomic features so that treatment can be planned The examination must generally include bilateral opacification of the internal and external carotid arteries and the vertebral arteries Arteriovenous malformations typically appear as a tangled vascular mass with distended tortuous afferent and efferent vessels, a rapid circulation time, and arteriovenous shunting Findings on plain radiographs of the skull are often normal unless an intracerebral hematoma is present, in which case there may be changes suggestive of raised intracranial pressure and displacement of a calcified pineal gland In patients presenting without hemorrhage, CT scan or MRI usually reveals the underlying abnormality, and MRI frequently also shows evidence of old or recent hemorrhage that may have been asymptomatic The nature and detailed anatomy of any focal lesion identified by these means are delineated by angiography, especially if operative treatment is under consideration
their location are sometimes treated solely by embolization; although the risk of hemorrhage is not reduced, neurologic deficits may be stabilized or even reversed by this procedure Two other techniques for the treatment of intracerebral arteriovenous malformations are injection of a vascular occlusive polymer through a flow-guided microcatheter and permanent occlusion of feeding vessels by positioning detachable balloon catheters in the desired sites and then inflating them with quickly solidifying contrast material Stereotactic radiosurgery with the gamma knife or related approaches is also useful in the management of inoperable cerebral arteriovenous malformations
Choi JH et al Brain arteriovenous malformations in adults Lancet Neurol 2005 May;4(5):299 308 [PMID: 15847843] Hartmann A et al Treatment of arteriovenous malformations of the brain Curr Neurol Neurosci Rep 2007 Jan;7(1):28 34 [PMID: 17217851]
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