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Vascular malformations of the central nervous system
AuthorsRobert J Singer, MDChristopher S Ogilvy, MDGuy Rordorf, MD Section EditorJose Biller, MD, FACP, FAAN, FAHA Deputy EditorJohn F Dashe, MD, PhDLast literature review version 17.1: January 2009 | This topic last updated: January 24,2009 (More)
INTRODUCTION
 
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Cerebral vascular malformations occur in 0.1 to 4.0 percent of the generalpopulation [1,2] . Four general subtypes of congenital malformations have been described:Developmental venous anomaliesCapillary telangiectasiasCavernous malformationsArteriovenous malformationsDevelopmental venous anomalies are most common in autopsy series, with an incidence of 2percent [3,4] . This is followed by arteriovenous malformations (1 percent), capillarymalformations (telangiectasias, 0.7 percent), and cavernous malformations (0.4 percent).Developmental venous anomalies and capillary telangiectasia are usually benign, while cavernousmalformations and arteriovenous malformations have a greater tendency toward neurologicsequelae.This topic will review our understanding of the natural history and treatment of these threelesions, which continues to evolve with our burgeoning imaging capabilities and clinicalexperience.Cerebral and spinal cord arteriovenous malformations are discussed separately. (See "Brainarteriovenous malformations" and see "Disorders affecting the spinal cord", section on Vascularmalformations).
DEVELOPMENTAL VENOUS ANOMALIES
 
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Developmental venous anomalies (DVAs), also knownas venous angiomas, are composed of a radially arranged configuration of medullary veinsseparated by normal brain parenchyma (most commonly white matter). These small venous
 
conduits empty into a central, dilated superficial or deep vein that drains the normal brain.Microscopically, the venous structures appear largely normal with rare degenerative changesconsisting of thickening and hyalinization. The lesions do not occur in the diencephalon,brainstem, or spinal cord.DVAs most often are solitary, although multiple lesions have been described in association withother clinical syndromes (eg, the blue rubber bleb nevus syndrome) [5] . DVAs also may occurconcurrently with cavernous malformations [6] . A second cerebrovascular anomaly has beenreported in approximately 19 percent of this patient population [7] .Clinical presentation
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DVAs are considered benign lesions, although they may uncommonlypresent with seizures, progressive neurologic deficits, and hemorrhage [8-10] . Headache is themost common presenting complaint, followed by seizures and sensory-motor phenomena.However, a direct correlation between these symptoms and the existence of a DVA has not beenfirmly established [11,12] . In a ten-year prospective clinical and magnetic resonance imagingstudy, a symptomatic hemorrhage rate of 0.34 percent per year was observed [11] . Thehemorrhages were usually benign, although fatal intracranial hemorrhages have been described[10] .The most informative study retrospectively reviewed patients with presentations that could bedirectly linked to vascular complications of DVAs but not to other pathologies [13] . Cases withcavernous malformations were excluded because of the known association with DVAs. Seventeenpatients were identified, and a review of the literature published after the introduction of MRIidentified another 51 cases fulfilling the same criteria.From these 68 cases, two major pathophysiologic categories of symptomatic DVAs were identified[13] : Mechanical compression of intracranial structures by a component of the DVA was seen in14 patients (21 percent). The most common associated symptoms were hydrocephalus, tinnitus,brainstem deficits, hemifacial spasm, and trigeminal neuralgia Flow-related symptoms werepresent in 49 patients (72 percent), with two subcategories:- Increased inflow in 19 patients (28 percent), typically related to an arteriovenousmalformation (AVM) draining via dilated and ectatic medullary veins through a DVA, resulting inheadaches, neurologic deficits, seizures, and coma, usually secondary to parenchymal and/orintraventricular hemorrhage- Restricted outflow, either by an anatomic obstruction (eg, stenosis or thrombosis of the DVAor its draining vein) in 26 patients (38 percent) or by a physiologic obstruction (eg, increasedvenous pressure secondary to a distal arteriovenous shunt or AVM) in four patients (6 percent).These mechanisms were associated with variable combinations of neurologic deficits, headaches,seizures, and altered mentation. The clinical picture in several cases resembled that caused bycerebral venous thrombosis, with increased intracranial pressure, venous congestive edema,and/or intraparenchymal or subarachnoid hemorrhage No obvious alteration attributable to theDVA was found to explain symptoms in six cases (9 percent)
 
 Diagnosis
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Cerebral angiography is considered the gold standard for the diagnosis of DVAs, butthey are usually identified with contrast enhanced cross-sectional imaging modalities such ascomputed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonanceangiography (MRA).Computed tomography
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Nonenhanced CT scans do not usually demonstrate DVAs unless there isan associated cavernous malformation. After contrast administration, the enlarged vein is all thatis typically identified. CT angiography (CTA) has also been used to identify DVAs [14] .Magnetic resonance imaging and angiography
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MRI typically shows medullary veins convergingon the dilated transcerebral vein. A characteristic "sunburst" pattern is seen on enhanced T1-weighted images. MRA usually demonstrates the dilated venous channel with variable depiction of the smaller medullary veins.Angiography
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As mentioned, cerebral angiography usually is not needed for the diagnosis of DVAsince axial imaging sequences on MRI often suffice to make the diagnosis. In atypical cases,angiographic findings are pathognomonic; during the late capillary or venous phase there is apaucity of normal veins in the region of the lesion and a characteristic "caput medusae"appearance of the radially arranged small medullary veins (show radiograph 1). The arterial phaseis typically normal.Treatment
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DVAs should be treated conservatively in the vast majority of cases, with associatedsymptoms such as headaches and seizures managed medically [12] . Surgery may be required inthe rare patient with hemorrhage associated with a DVA or with uncontrolled seizures [10] .In patients who undergo surgery, preoperative contrast enhanced imaging is required to identifyan associated cavernous malformation [6] . Venous infarction has been reported with DVAresection [15] ; thus, it is reasonable to simply evacuate the hematoma and leave the DVA in situ.Radiosurgical and endovascular techniques do not have a defined role in the management of theselesions.
CAPILLARY TELANGIECTASIAS
 
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Capillary telangiectasias are small lesions most commonly foundin the pons, middle cerebellar peduncles, and dentate nuclei. Multiple lesions are common.The lesions are composed of small, dilated capillaries devoid of smooth muscle or elastic fibers.The intervening brain is often normal; it may also demonstrate areas of microhemorrhage orgliosis. A common histopathological feature of these lesions is a dilated efferent system, probablyrepresenting a venous channel.An argument has been made for these lesions representing the early stage in the spectrum of development of cavernous malformations and other "mixed" vascular malformations [16,17] .Although not proven, angiogenesis is believed to play a role in lesion evolution. Mosttelangiectasias represent an angiodysplastic phenomenon resulting from faulty embryogenesis of 
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