other clinical syndromes (eg, the blue rubber bleb nevus syndrome) [77]. DVAs also may occur concurrently with cavernous malformations (CMs) in 13 to 40 percent, as well as with other intracranial vascular malformations and with superficial venous malformations of the head and neck [32,78,79]. Clinical presentation DVAs are considered benign lesions, although they may uncommonly present with seizures, progressive neurologic deficits, and hemorrhage [52,76,80-83]. Headache is the most common presenting complaint, followed by seizures and sensory-motor phenomena. However, a direct correlation between these symptoms and the DVA is uncertain [84,85]. In a 10-year prospective clinical and MRI study involving 80 patients, a symptomatic hemorrhage rate of 0.34 percent per year was observed [84]. In another study of 93 patients with 492 person- years of follow-up, no symptomatic hemorrhages occurred [83]. The hemorrhages were usually minor, although fatal intracranial hemorrhages have been described [82]. A retrospective case series reviewed the clinical presentations of 68 patients with imaging findings suggestive of DVAs whose symptoms could not be attributed to other pathologies [86]. Cases with associated CMs were excluded. Two major pathophysiologic mechanisms were reported: Mechanical compression of intracranial structures by a component of the vein was seen in 21 percent. The most common associated symptoms were hydrocephalus, tinnitus, brainstem deficits, hemifacial spasm, and trigeminal neuralgia. Flow-related symptoms were present in 72 percent. Increased inflow was present in 28 percent, typically related to an arteriovenous malformation (AVM) draining via dilated and ectatic medullary veins, resulting parenchymal and/or intraventricular hemorrhage, or https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 12/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate venous infarction. Symptoms included headaches, neurologic deficits, seizures, and coma. Restricted outflow, either by an anatomic obstruction (eg, stenosis or thrombosis of the vein) in 38 percent or by a physiologic obstruction (eg, increased venous pressure secondary to a distal arteriovenous shunt or AVM) was observed in 6 percent. Patients presented with variable combinations of neurologic deficits, headaches, seizures, and altered mentation, a clinical picture that resembled that of cerebral venous thrombosis, with increased intracranial pressure, venous congestive edema, and/or intraparenchymal or subarachnoid hemorrhage. No obvious alteration was found to explain symptoms in 9 percent. Diagnosis Cerebral angiography is considered the gold standard for the diagnosis of DVAs, but they are usually identified with contrast-enhanced cross-sectional imaging modalities such as CT, MRI, and magnetic resonance angiography (MRA) [78]. Computed tomography Nonenhanced CT scans do not usually demonstrate DVAs unless there is an associated cavernous malformation. After contrast administration, the enlarged vein is all that is typically identified. CT angiography (CTA) has also been used to identify DVAs ( image 6) [87]. Magnetic resonance imaging and angiography MRI with gadolinium typically shows medullary veins in "caput medusae" pattern that converge on a dilated transcerebral draining vein. A characteristic "sunburst" pattern is seen on enhanced T1-weighted images ( image 7) [88]. Gradient echo sequences should be included to increase sensitivity for detecting associated CMs [78]. White matter abnormalities and/or calcifications may be observed in the parenchyma adjacent to the DVA. MRA usually demonstrates the dilated venous channel with variable depiction of the smaller medullary veins. Catheter angiography Cerebral angiography usually is not needed for the diagnosis of DVA since MRI is often sufficient to make the diagnosis. In atypical cases, angiographic findings are pathognomonic; during the late capillary or venous phase there is a paucity of normal veins in the region of the lesion and a characteristic "caput medusae" appearance of the radially arranged small medullary veins ( image 8). The arterial phase is typically normal; however, so-called arterialized DVAs have been described with early-phase opacification on angiography and/or enlarged arterial feeders [78]. These lesions may have a bleeding risk that is more similar to an arteriovenous malformation. https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 13/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Management DVAs should be treated conservatively in the vast majority of cases, with associated symptoms such as headaches and seizures managed medically [85]. Obliteration may be considered in the rare patient with hemorrhage or uncontrolled seizures associated with a DVA [82]. However, surgical or endovascular obliteration is associated with a risk of venous infarction [76]. In patients who undergo surgery, preoperative MRI with gadolinium is required to identify an associated cavernous malformation [32]. Venous infarction has been reported with DVA resection [46]; 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 these lesions. In rare patients who present with symptomatic thrombosis of a DVA, there is anecdotal support for considering the use of systematic anticoagulation [78]. (See "Cerebral venous thrombosis: Treatment and prognosis".) CAPILLARY TELANGIECTASIAS Capillary telangiectasias are small lesions most commonly found in the pons, middle cerebellar peduncles, and cerebellar 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 or gliosis. A common histopathological feature of these lesions is a dilated efferent system, probably representing 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 [26,89]. Although not proven, angiogenesis is believed to play a role in lesion evolution. Most telangiectasias represent an angiodysplastic phenomenon resulting from faulty embryogenesis of the vascular wall and have been associated with angiomatous phacomatoses such as Osler- Weber-Rendu (hereditary hemorrhagic telangiectasia), Louis-Bar (ataxia-telangiectasia), and Wyburn-Mason (unilateral retinocephalic vascular malformation) syndromes [90]. (See "Clinical manifestations and diagnosis of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)", section on 'Cerebral vascular abnormalities' and "Ataxia-telangiectasia", section on 'Neuroimaging features'.) Clinical presentation Capillary telangiectasias are usually clinically silent, found incidentally on neuroimaging studies or at postmortem examination. In a systematic review of 10 series and https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 14/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate 203 patients with capillary telangiectasias, symptomatic cases accounted for 6 percent [91]; headache, nausea, and seizures have been described in patients with these lesions, although a causal relationship is unclear. Diagnosis MRI with gadolinium contrast is the most sensitive imaging modality for the identification of brain capillary telangiectasias, but the lesions may be inconspicuous on precontrast MRI. Small lesions that are hypo- to isointense on T1-weighted sequences and iso- to slightly hyperintense on T2-weighted sequences with faint enhancement on postcontrast T1- weghted sequences are suggestive, although not diagnostic, of these lesions [92]. Compared with other precontrast MRI sequences, sensitivity for the detection of capillary telangiectasias is increased with the use of gradient echo and especially with susceptibility-weighted imaging (SWI) ( image 9) [92,93]. Telangiectasias can be identified in the late arterial/early capillary phase of angiography as a faint blush with an associated venous channel. Thus, these lesions can be distinguished from developmental venous anomalies that are visualized during the venous phase of the study. Management Capillary telangiectasias are nonoperable lesions. Management is conservative, particularly since most lesions are asymptomatic. The routine care and additional screening for patients with capillary telangiectasias associated with other conditions are discussed separately. (See "Ataxia-telangiectasia", section on 'Management' and "Hereditary hemorrhagic telangiectasia (HHT): Routine care including screening for asymptomatic AVMs" and "Hereditary hemorrhagic telangiectasia (HHT): Evaluation and therapy for specific vascular lesions".) SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Stroke in adults" and "Society guideline links: Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)".) INFORMATION FOR PATIENTS UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." th th The Basics patient education pieces are written in plain language, at the 5 to 6 grade reading level, and they answer the four or five key questions a patient might have about a given https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 15/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more th th sophisticated, and more detailed. These articles are written at the 10 to 12 grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.) Basics topic (see "Patient education: Arteriovenous malformations in the brain (The Basics)") SUMMARY AND RECOMMENDATIONS Brain arteriovenous malformations Arteriovenous malformations are the most dangerous cerebral vascular malformation and can cause hemorrhage, seizures, headaches, and focal neurologic deficits. These lesions are discussed in detail separately. (See "Brain arteriovenous malformations".) Cavernous malformations Cavernous malformations are thin-walled dilated capillaries with a simple endothelial lining ( image 1). They may occur as a sporadic or familial condition and may be associated with developmental venous anomalies in about 25 percent. (See 'Cavernous malformations' above.) Cavernous malformations may be found incidentally on neuroimaging or may present with neurologic symptoms such as hemorrhage, seizures, and/or progressive neurologic deficits ( image 3 and image 2). Recurrent hemorrhage is more common after an initial bleed and may be as high as 5 percent per year for supratentorial lesions and 21 percent for brainstem lesions. Cavernous malformations are typically identified on MRI and are often angiographically occult. They can occur throughout the brain but are most common in the subcortical rolandic and temporal areas. Asymptomatic cavernous malformations are generally followed without intervention. Surgical resection may be indicated for accessible symptomatic lesions associated with progressive neurologic deficits, intractable epilepsy, and/or hemorrhage. Stereotactic radiosurgery is an option for nonoperable lesions, but long-term safety is uncertain. https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 16/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Developmental venous anomalies Developmental venous anomalies (DVAs) consist of a radially arranged configuration of medullary veins separated by normal brain parenchyma ( image 7). The lesions are usually solitary but can be multiple and occur with cavernous malformations. (See 'Developmental venous anomalies' above.) DVAs are usually an incidental finding but may rarely present with seizures or hemorrhage. They are usually identified on MRI. Cerebral angiography is considered the gold standard for diagnosis of a DVA. After diagnosis, hemorrhage is unusual. Most patients with DVAs are followed without intervention; rarely, surgery is required for hemorrhage or intractable epilepsy. Capillary telangiectasias Capillary telangiectasias are small, dilated capillaries devoid of smooth muscle or elastic fibers. (See 'Capillary telangiectasias' above.) MRI is the most sensitive imaging modality for the identification of brain capillary telangiectasias ( image 9). They are most commonly found in the pons, middle cerebellar peduncles, and dentate nuclei. Multiple lesions are common. Capillary telangiectasias are usually clinically silent, found incidentally on neuroimaging studies. They are not associated with morbidity, and intervention is not required. Use of UpToDate is subject to the Terms of Use. REFERENCES 1. el-Gohary EG, Tomita T, Gutierrez FA, McLone DG. Angiographically occult vascular malformations in childhood. Neurosurgery 1987; 20:759. 2. McCormick WF. The pathology of vascular ("arteriovenous") malformations. J Neurosurg 1966; 24:807. 3. Al-Shahi R, Bhattacharya JJ, Currie DG, et al. 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AJNR Am J Neuroradiol 2014; 35:2302. https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 23/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Topic 1129 Version 24.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 24/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate GRAPHICS MRI of a cerebral cavernous malformation Cavernous malformations appear characteristically on MRI as a "popcorn" pattern of variable image intensities. Courtesy of Guy Rordorf, MD. Graphic 80348 Version 4.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 25/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Cavernous malformation of the spinal cord on MRI (A) Sagittal T2-weighted MRI sequence shows target-like lesion (thick arrow) with hypointense core consistent with CM. Note both rostral and caudal ill-defined T2-hypointense acute hemorrhage (arrows), as well as more rostral cord edema (bracket). https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 26/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate (B) Magnified pre-contrast sagittal T1-weighted sequence shows hyperdensity consistent with hemorrhage (thick arrow). (C) Magnified sagittal and (D) axial post-contrast T1-weighted MRI sequences show mild contrast enhancement of CM (thick arrows). MRI: magnetic resonance imaging; CM: cavernous malformation. Courtesy of Glenn A Tung, MD, FACR. Graphic 139103 Version 1.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 27/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate MRI of a cerebral cavernous malformation with old hemorrhage A dark hemosiderin ring around the cavernous malformation is evident on this T-2 weighted MR image, suggestive of old hemorrhage. MRI: magnetic resonance imaging. Courtesy of Guy Rordorf, MD. Graphic 68000 Version 5.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 28/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Cerebral cavernous malformations (CCM) on CT and MRI sequences SWI is very sensitive to slow-flow vascular malformations such as CCM, as in this child with a large left temporal CCM (dashed arrows), with a characteristic "popcorn" appearance on CT and MRI. There are typical internal calcifications on CT (A), hyperintense subacute blood products on T1-weighted imaging (B), mild internal enhancement on postcontrast T1-weighted imaging (C), and peripheral hypointense hemosiderin on FLAIR (D) and T2-weighted imaging (E). The lesion appears larger on SWI (F) due to blooming artifact. More important, additional smaller CCMs are detected (arrows), which indicate that the patient likely has multiple familial CCM syndrome (which can occur in as many as one-third of patients with CCM). SWI parameters are: 3 Tesla, TE = 20 milliseconds, TR = 29 milliseconds, FA = 15 , field of vision 250 mm 188 mm, matrix 448 336, 2 mm thick acquisition displayed with 16 mm minimum intensity projection. SWI: Susceptibility-weighted imaging; CCM: cerebral cavernous malformations; CT: computed tomography; MRI: magnetic resonance imaging; FLAIR: fluid-attenuated inversion recovery; TE: echo time; TR: repetition time; FA: flip angle. https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 29/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate From: Liu C, Li W, Tong KA, et al. Susceptibility-weighted imaging and quantitative susceptibility mapping in the brain. J Magn Reson Imaging 2015; 42(1):23-41. https://onlinelibrary.wiley.com/doi/abs/10.1002/jmri.24768. Copyright 2014 Wiley Periodicals, Inc. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/). Graphic 122720 Version 1.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 30/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Sporadic CCM with a DVA Sporadic CCM with a very large DVA. (A) Axial T2 SE of a 26-year-old female patient shows a CCM near the left lateral ventricle. (B) Postgadolinium T1 shows a large DVA involving much of the left frontal lobe. (C and D) SWI demonstrates very clearly the CCM and DVA without gadolinium administration. https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 31/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate CCM: cerebral cavernous malformation; DVA: developmental venous anomaly; SE: spin echo; SWI: susceptibility-weighted imaging. Republished with permission of the American Society of Neuroradiology, from: Petersen TA, Morrison LA, Schrader RM, Hart BL. Familial versus sporadic cavernous malformations: di erences in developmental venous anomaly association and lesion phenotype. AJNR Am J Neuroradiol 2010; 31:377; permission conveyed through Copyright Clearance Center, Inc. Copyright 2010. Graphic 122722 Version 2.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 32/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Developmental venous anomaly with dystrophic calcification (A) Axial and (B) coronal non-contrast CT images show hyperdense linear calcification in left lateral putamen. (C) Axial and (D) sagittal images from CT angiography show classic caput medusae and dilated vein (circles) characteristic of DVA. https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 33/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate CT: computed tomography; DVA: developmental venous anomaly. Courtesy of Glenn A Tung, MD, FACR. Graphic 139104 Version 1.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 34/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Left frontal DVA on MRI A DVA accompanied by a small cyst and loco-regional atrophy localized in the left frontal deep and subcortica white matter of a 45-year-old patient with headache. (A) Thin venous stems and veins in the center, leading to the caput medusae imaging on axial contrast- enhanced 3D SPGR maximum intensity projection image, are visualized. (B) Loco-regional atrophy in the frontal lobe and a millimetric cyst (arrow) adjacent to the DVA caput on a coronal contrast-enhanced 3D SPGR image are visualized. 3D SPRG: three-dimensional spoiled gradient recalled echo; DVA: developmental venous anomaly; MRI: magnetic resonance imaging. Reprinted by permission from Springer: Clinical Neuroradiology. G k e E, Acu B, Beyhan M, et al. Magnetic resonance imaging ndings of developmental venous anomalies. Clin Neuroradiol 2014; 24:135. Copyright 2014. https://link.springer.com/journal/62. Graphic 122040 Version 5.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 35/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Cerebral angiography of a developmental venous anomaly During the late capillary or venous phase of angiography, there is a paucity of normal veins in the region of a developmental venous anomaly and a characteristic "caput medusae" appearance of the radially arranged small medullary veins (arrow). Courtesy of Guy Rordorf, MD. Graphic 56038 Version 4.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 36/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate MRI of brain capillary telangiectasia A brain capillary telangiectasia (circle) seen in the pons on both GRE (A) and SWI (B) and confirmed on postcontrast T1-weighted image (C). GRE: gradient echo; MRI: magnetic resonance imaging; SWI: susceptibility-weighted imaging. Republished with permission of the American Society of Neuroradiology, from: Chaudhry US, De Bruin DE, Policeni BA. Susceptibility- weighted MR imaging: a better technique in the detection of capillary telangiectasia compared with T2* gradient-echo. AJNR Am J Neuroradiol 2014; 35:2302; permission conveyed through Copyright Clearance Center, Inc. Copyright 2014. Graphic 122043 Version 1.0 https://www.uptodate.com/contents/vascular-malformations-of-the-central-nervous-system/print 37/38 7/5/23, 12:27 PM Vascular malformations of the central nervous system - UpToDate Contributor Disclosures