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Brain, Venous Vascular Malformations

Andrew L Wagner, MD, Assistant Professor of Radiology, Instructional Faculty, University of Virginia School of Medicine; Director of Neuroradiology, Department of Radiology, Rockingham Memorial Hospital

Updated: Apr 21, 2009

Introduction

Background

Venous vascular malformations, also known as venous angiomas or, more properly, developmental venous anomalies (DVAs), represent congenital anatomically variant pathways in the normal venous drainage of an area of the brain. Once thought to be rare, they are now considered to be the most common vascular malformation in the CNS.^1,2,3 They may occur in as many as 2% of individuals.

Although for many years DVAs were commonly called venous angiomas, the newer term DVA has been recommended as more appropriate because the involved vessels are not abnormally formed, but apparently merely dilated. The majority of DVAs are found incidentally and never cause symptoms, although there are isolated reports of patients with syndromes attributed to DVAs (eg, secondary to hemorrhage or thrombosis).

Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image obtained in a patient who had undergone surgery in the past for an arteriovenous malformation (AVM) shows bilateral developmental venous anomalies (DVAs) and the classic caput medusa appearance. Note the signal intensity abnormality in the inferior right cerebellar hemisphere due to the prior surgery.

Brain, venous vascular malformation. Axial fluid-attenuated inversion recovery shows some artifactual increased signal within the vessel, which can aid in detection of DVAs on noncontrasted studies.

DVAs are associated with cavernous angiomas or one of the other types of CNS vascular malformations (ie, arteriovenous malformation [AVM], capillary telangiectasia) in approximately 15-30% of patients. The most frequent conjunction is with cavernous angiomas; indeed, this association is so common that the two may be etiologically related,⁴ and the presence of a DVA on an image should prompt a search for a cavernoma, which is more clinically important.² DVAs are also associated with head and neck venous malformations and hemangiomas. Rarely, DVAs are associated with varices.⁵

Frequency

United States

Developmental venous anomalies occur in approximately 2% of the population.

International

Developmental venous anomalies occur in approximately 2% of the population.

Mortality/Morbidity

Although almost all developmental venous anomalies are incidentally found and never cause clinical symptoms, sporadic reports of hemorrhage, seizure, and infarcts due to spontaneous thrombosis exist.

• Hemorrhage is the most common clinical symptom associated with DVAs; however, an unknown number of these cases may actually represent hemorrhage from an associated cavernous malformation. Certainly, increased flow through the thin medullary veins that form the substance of the malformation can result in hemorrhage but this appears to be rare. Before a hemorrhage is attributed to a DVA, signs of an accompanying cavernoma must be carefully sought.
• Thrombosis of a DVA appears to result in the worst complications. By blocking normal venous drainage in the area, thrombosis often leads to a venous infarct. Hemorrhage may ensue if the DVA is then recanalized.
• Although seizures have often been associated with DVAs, to the author's knowledge, no scientific proof exists that these lesions are directly responsible for seizures. Striano and colleagues found DVAs in only 4 of 1020 (0.39%) epileptic patients examined at their institution⁶ ; this rate is less than that reported in the general population.

Race

No known race predilection exists.

Sex

Gender differences in the incidence of DVAs have not been reported.

Age

Because they are thought to be congenital, DVAs can occur in persons of any age. Most often they occur in adults, likely because adults undergo MRI examinations more frequently than pediatric patients.

Presentation

Anatomy

DVAs consist of a fine network of enlarged medullary venules that join to drain into a central venous outflow track that then drains into the superficial or deep venous system, depending on the location of the malformation (see Images below and Image 1 in Multimedia).

Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image obtained in a patient who had undergone surgery in the past for an arteriovenous malformation (AVM) shows bilateral developmental venous anomalies (DVAs) and the classic caput medusa appearance. Note the signal intensity abnormality in the inferior right cerebellar hemisphere due to the prior surgery.

Brain, venous vascular malformation. Coronal T1 postcontrast demonstrates a typical location for a DVA, here within the periventricular white matter. This malformation drained into a cortical vein along the parietal convexity.

Brain, venous vascular malformation. Axial postcontrast image from the same patient as in Image above demonstrates the fine network of feeder veins that converge into the single draining vein.

Clinical Manifestations

Symptoms from DVAs are thought to be uncommon.⁷  Although headaches, dizziness, and ataxia⁸  have been associated with DVAs, confidently attributing such generalized symptoms to this common lesion is difficult. Symptoms that are directly related to a DVA most often involve DVA thrombosis and/or adjacent hemorrhage.^9,10

While some believe that DVAs can hemorrhage on their own, most notably after venous infarction from spontaneous DVA thrombosis, most instances of hemorrhage with DVAs have been in patients with combined vascular malformations. In the vast majority of these cases, the hemorrhage probably originated from the accompanying vascular malformation rather than from the DVA.

Preferred Examination

Although contrast-enhanced CT and nonenhanced MRI can reveal a DVA, the preferred imaging technique is contrast-enhanced MRI because of its excellent depiction of the small venules and draining vein. The multiplanar capabilities of MRI are especially useful because the typical configuration of a DVA is often best recognized in the coronal plane (see Images below and Images 1-2 in Multimedia).

Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image obtained in a patient who had undergone surgery in the past for an arteriovenous malformation (AVM) shows bilateral developmental venous anomalies (DVAs) and the classic caput medusa appearance. Note the signal intensity abnormality in the inferior right cerebellar hemisphere due to the prior surgery.

Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image clearly shows the draining vein and associated venous network of a developmental venous anomaly (DVA).

Limitations of Techniques

Although standard contrast-enhanced MRI is excellent in depicting DVAs, adjacent hemosiderin from associated cavernomas may not be appreciated without the use of gradient-echo or echo-planar imaging, especially with fast spin-echo techniques.

Differential Diagnoses

Brain, Arteriovenous Malformation
Brain, Capillary Telangiectasia
Brain, Cavernous Angiomas
Brain, MRI Appearance of Hemorrhage
Brain, Stroke

Computed Tomography

Findings

Developmental venous anomalies are typically not visible on nonenhanced CT scans but they can be visualized after the administration of contrast medium. They appear as a large vascular structure in the brain parenchyma that drains into the deep or superficial venous system. The smaller surrounding veins are usually arranged in a radial pattern around the central vein. DVAs do not have a surrounding mass effect or edema and the adjacent brain is typically normal.

Degree of Confidence

The typical appearance of a DVA on CT scans is often diagnostic but MRI may be needed in atypical cases, particularly those involving the posterior fossa where CT is limited because of streak artifacts.

False Positives/Negatives

Although arteriovenous malformations can occasionally be mistaken for DVAs on CT scan and vice versa, differentiation between the two is usually not a problem because AVMs have large feeding arteries, tortuous vessels, and abnormal adjacent brain parenchyma that are not observed in DVAs.

Magnetic Resonance Imaging

Brain, venous vascular malformation. Axial T2 image from same patient as Images 5 and 6 shows that the DVA can be subtle. In this patient, the draining vein is large enough to have a flow void on the image. The parenchymal abnormality is typically not visible.

Brain, venous vascular malformation. Axial fluid-attenuated inversion recovery shows some artifactual increased signal within the vessel, which can aid in detection of DVAs on noncontrasted studies.

Brain, venous vascular malformation. On fast low-angle shot images, both the venous cluster and the draining vein may have mild susceptibility artifact (although not as much as hemosiderin) secondary to the deoxyhemoglobin within the slow-flowing veins (arrows).

Brain, venous vascular malformation. Axial T1 postcontrast demonstrates a large DVA originating from the frontal lobe white matter. Note the cluster of small vessels that form the large draining vein.

Brain, venous vascular malformation. Slightly higher image in the same patient as Image 10. The large draining vein is noted to drain into the superior sagittal sinus.

Findings

On contrast-enhanced MRI, the cluster of veins in developmental venous anomalies has a spoke-wheel appearance (see Image 6); the veins are small at the periphery and gradually enlarge as they approach a central draining vein (see Image 1, Image 2). This appearance has been referred to as caput medusa, or the head of Medusa, because of the serpentine appearance of the curvilinear peripheral draining veins. The intervening brain parenchyma is normal; this is a distinguishing characteristic of a DVA. However, two recent studies report parenchymal abnormalities within the drainage territory of most DVAs^11,12

Brain, venous vascular malformation. Axial postcontrast image from the same patient as in Image above demonstrates the fine network of feeder veins that converge into the single draining vein.

Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image obtained in a patient who had undergone surgery in the past for an arteriovenous malformation (AVM) shows bilateral developmental venous anomalies (DVAs) and the classic caput medusa appearance. Note the signal intensity abnormality in the inferior right cerebellar hemisphere due to the prior surgery.

Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image clearly shows the draining vein and associated venous network of a developmental venous anomaly (DVA).

Brain, venous vascular malformation. Axial proton density–weighted image in the same patient as image 2 demonstrates the high signal intensity of the draining vein, which is typical on images obtained with this sequence. Note the yin-yang appearance of the vessel with an area of decreased signal intensity adjacent to the area with increased signal intensity.

Gradient-echo images often show decreased signal intensity in the venous angioma that is not due to hemosiderin but is secondary to the paramagnetic effects of deoxyhemoglobin in the venous blood (see Image 9). Findings on diffusion images are usually normal.

Magnetic resonance (MR) venography is almost never necessary. If obtained, venograms show the draining vein with some of the surrounding radially arranged veins. Because the DVA provides the venous drainage for a section of brain, anatomically normal venous drainage is not present in that area.

Because DVAs are often associated with other CNS vascular lesions (particularly cavernous angiomas), when a DVA is identified, carefully evaluate the brain and obtain gradient-echo (GRE) images (see Image 4). Cavernomas typically appear as focal areas of blood products that often show different stages of evolution (ie, hemosiderin with extracellular methemoglobin). Capillary telangiectasias are small areas of lacelike enhancement that are dark on GRE images without signal intensity abnormality on T2-weighted images. AVMs have enlarged feeding arteries and tortuous vessels with surrounding gliosis.

Degree of Confidence

MRI findings are diagnostic in almost all instances. However, in cases with questionable findings, MR venography usually suggests the diagnosis.

Angiography

Findings

Developmental venous anomalies found on angiograms are almost invariably incidental findings, as they are with newer MRIs, and the diagnosis can be made in almost every instance. When observed, the DVA appears as a blush of contrast enhancement during the venous phase of the study and drains into a large anatomically anomalous vein. In its most frequent location (adjacent to the lateral ventricles), the vein usually drains into a subependymal vein, although superficial drainage also occurs.

Degree of Confidence

A DVA has a characteristic angiographic appearance and should not be confused with an AVM; no early filling occurs with a DVA.

Intervention

Medicolegal Pitfalls

• AVMs should not be identified as DVAs. The difference is usually obvious because a DVA does not have abnormally enlarged feeding arteries or the tortuous vessels observed in an AVM. Because DVAs are considered to be clinically silent lesions, misdiagnosing a DVA as an AVM can lead to catastrophic consequences if a surgeon removes it. Removal of a DVA likely leads to venous infarction in that part of the brain.

Multimedia

Media file 1: Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image obtained in a patient who had undergone surgery in the past for an arteriovenous malformation (AVM) shows bilateral developmental venous anomalies (DVAs) and the classic caput medusa appearance. Note the signal intensity abnormality in the inferior right cerebellar hemisphere due to the prior surgery.

Media file 2: Brain, venous vascular malformation. Coronal T1-weighted contrast-enhanced image clearly shows the draining vein and associated venous network of a developmental venous anomaly (DVA).

Media file 3: Brain, venous vascular malformation. Axial proton density–weighted image in the same patient as image 2 demonstrates the high signal intensity of the draining vein, which is typical on images obtained with this sequence. Note the yin-yang appearance of the vessel with an area of decreased signal intensity adjacent to the area with increased signal intensity.

Media file 4: Brain, venous vascular malformation. Axial proton density–weighted image in the same patient as Image 2 and Image 3 shows an area of marked signal intensity loss in the right cerebellum adjacent to the developmental venous anomaly (DVA). This finding is consistent with a coexistent cavernous angioma.

Media file 5: Brain, venous vascular malformation. Coronal T1 postcontrast demonstrates a typical location for a DVA, here within the periventricular white matter. This malformation drained into a cortical vein along the parietal convexity.

Media file 6: Brain, venous vascular malformation. Axial postcontrast image from the same patient as in Image above demonstrates the fine network of feeder veins that converge into the single draining vein.

Media file 7: Brain, venous vascular malformation. Axial T2 image from same patient as Images 5 and 6 shows that the DVA can be subtle. In this patient, the draining vein is large enough to have a flow void on the image. The parenchymal abnormality is typically not visible.

Media file 8: Brain, venous vascular malformation. Axial fluid-attenuated inversion recovery shows some artifactual increased signal within the vessel, which can aid in detection of DVAs on noncontrasted studies.

Media file 9: Brain, venous vascular malformation. On fast low-angle shot images, both the venous cluster and the draining vein may have mild susceptibility artifact (although not as much as hemosiderin) secondary to the deoxyhemoglobin within the slow-flowing veins (arrows).

Media file 10: Brain, venous vascular malformation. Axial T1 postcontrast demonstrates a large DVA originating from the frontal lobe white matter. Note the cluster of small vessels that form the large draining vein.

Media file 11: Brain, venous vascular malformation. Slightly higher image in the same patient as Image 10. The large draining vein is noted to drain into the superior sagittal sinus.

References

1. Cheong WY, Tan KP. Cerebral venous angioma--a misnomer?. Ann Acad Med Singapore. Sep 1993;22(5):736-41. [Medline].

2. Zimmer A, Hagen T, Ahlhelm F, Viera J, Reith W, Schulte-Altedorneburg G. [Developmental venous anomaly (DVA)]. Radiologe. Oct 2007;47(10):868, 870-4. [Medline].

3. Zimmer A, Hagen T, Ahlhelm F, Viera J, Reith W, Schulte-Altedorneburg G. [Developmental venous anomaly (DVA)]. Radiologe. Oct 2007;47(10):868, 870-4. [Medline].

4. Perrini P, Lanzino G. The association of venous developmental anomalies and cavernous malformations: pathophysiological, diagnostic, and surgical considerations. Neurosurg Focus. Jul 15 2006;21(1):e5. [Medline].

5. Sirin S, Kahraman S, Gocmen S, Erdogan E. A rare combination of a developmental venous anomaly with a varix. Case report. J Neurosurg Pediatrics. Feb 2008;1(2):156-9. [Medline].

6. Striano S, Nocerino C, Striano P. Venous angiomas and epilepsy. Neurol Sci. Jun 2000;21(3):151-5. [Medline].

7. Kovács T, Osztie E, Bodrogi L, Pajor P, Farsang M, Juhász C, et al. Cerebellar developmental venous anomalies with associated vascular pathology. Br J Neurosurg. Apr 2007;21(2):217-23. [Medline].

8. Fenzi F, Rizzuto N. Ataxia and migraine-like headache in a girl with a cerebellar developmental venous anomaly. J Neurol Sci. Oct 15 2008;273(1-2):127-9. [Medline].

9. Brasse G, Stammel O, Siemens P, Töpper R. [Thrombosis of developmental venous anomaly and consecutive venous infarction]. Nervenarzt. Jun 2008;79(6):703-5. [Medline].

10. Vieira Santos A, Saraiva P. Spontaneous isolated non-haemorrhagic thrombosis in a child with development venous anomaly: case report and review of the literature. Childs Nerv Syst. Dec 2006;22(12):1631-3. [Medline].

11. San Millán Ruíz D, Delavelle J, Yilmaz H, Gailloud P, Piovan E, Bertramello A, et al. Parenchymal abnormalities associated with developmental venous anomalies. Neuroradiology. Dec 2007;49(12):987-95. [Medline].

12. Santucci GM, Leach JL, Ying J, Leach SD, Tomsick TA. Brain parenchymal signal abnormalities associated with developmental venous anomalies: detailed MR imaging assessment. AJNR Am J Neuroradiol. Aug 2008;29(7):1317-23. [Medline].

13. Aagaard BD, Song JK, Eskridge JM. Complex right hemisphere developmental venous anomaly associated with multiple facial hemangiomas. Case report. J Neurosurg. Apr 1999;90(4):766-9. [Medline].

14. Aksoy FG, Gomori JM, Tuchner Z. Association of intracerebral venous angioma and true arteriovenous malformation: a rare, distinct entity. Neuroradiology. Jun 2000;42(6):455-7. [Medline].

15. Augustyn GT, Scott JA, Olson E. Cerebral venous angiomas: MR imaging. Radiology. Aug 1985;156(2):391-5. [Medline].

16. Borden NM, Khayata MH, Dean BL. Unusual pattern of a deep developmental venous anomaly on CT and MR studies. J Comput Assist Tomogr. Nov-Dec 1995;19(6):885-9. [Medline].

17. Boukobza M, Enjolras O, Guichard JP. Cerebral developmental venous anomalies associated with head and neck venous malformations. AJNR Am J Neuroradiol. May 1996;17(5):987-94. [Medline].

18. Damiano TR, Truwit CL, Dowd CF. Posterior fossa venous angiomas with drainage through the brain stem. AJNR Am J Neuroradiol. Apr 1994;15(4):643-52. [Medline].

19. Field LR, Russell EJ. Spontaneous hemorrhage from a cerebral venous malformation related to thrombosis of the central draining vein: demonstration with angiography and serial MR. AJNR Am J Neuroradiol. Oct 1995;16(9):1885-8. [Medline].

20. Herbreteau O, Auffray-Calvier E, Desal H. [Symptomatic venous angioma. Report of a case]. J Neuroradiol. Jun 1999;26(2):126-31. [Medline].

21. Huber G, Henkes H, Hermes M. Regional association of developmental venous anomalies with angiographically occult vascular malformations. Eur Radiol. 1996;6(1):30-7. [Medline].

22. Kesava PP, Turski PA. MR angiography of vascular malformations. Neuroimaging Clin N Am. May 1998;8(2):349-70. [Medline].

23. Konan AV, Raymond J, Bourgouin P. Cerebellar infarct caused by spontaneous thrombosis of a developmental venous anomaly of the posterior fossa. AJNR Am J Neuroradiol. Feb 1999;20(2):256-8. [Medline].

24. Kuroiwa T, Ohta T. Cavernous angioma associated with venous angioma--two case reports. Neurol Med Chir (Tokyo). Oct 1998;38(10):648-53. [Medline].

25. Lai PH, Chen PC, Pan HB. Venous infarction from a venous angioma occurring after thrombosis of a drainage vein. AJR Am J Roentgenol. Jun 1999;172(6):1698-9. [Medline].

26. Lasjaunias P, Burrows P, Planet C. Developmental venous anomalies (DVA): the so-called venous angioma. Neurosurg Rev. 1986;9(3):233-42. [Medline].

27. Lee BC, Vo KD, Kido DK. MR high-resolution blood oxygenation level-dependent venography of occult (low-flow) vascular lesions. AJNR Am J Neuroradiol. Aug 1999;20(7):1239-42. [Medline].

28. Lupret V, Negovetic L, Smiljanic D. Cerebral venous angiomas: surgery as a mode of treatment for selected cases. Acta Neurochir (Wien). 1993;120(1-2):33-9. [Medline].

29. Merten CL, Knitelius HO, Hedde JP. Intracerebral haemorrhage from a venous angioma following thrombosis of a draining vein. Neuroradiology. Jan 1998;40(1):15-8. [Medline].

30. Nussbaum ES, Heros RC, Madison MT. The pathogenesis of arteriovenous malformations: insights provided by a case of multiple arteriovenous malformations developing in relation to a developmental venous anomaly. Neurosurgery. Aug 1998;43(2):347-51; discussion 351-2. [Medline].

31. Rafalowska J, Drac H, Dziewulska D. Coexistence of various vascular malformations within the brain. Folia Neuropathol. 1995;33(4):247-50. [Medline].

32. Sarwar M, McCormick WF. Intracerebral venous angioma. Case report and review. Arch Neurol. May 1978;35(5):323-5. [Medline].

33. Scamoni C, Dario A, Basile L. The association of cavernous and venous angioma. Case report and review of the literature. Br J Neurosurg. Aug 1997;11(4):346-9. [Medline].

34. Truwit CL. Venous angioma of the brain: history, significance, and imaging findings. AJR Am J Roentgenol. Dec 1992;159(6):1299-307. [Medline].

35. Uchino A, Hasuo K, Matsumoto S. Cerebral venous angiomas associated with hemorrhagic lesions. Their MRI manifestations. Clin Imaging. Jul-Sep 1996;20(3):157-63. [Medline].

36. Wilms G, Bleus E, Demaerel P. Simultaneous occurrence of developmental venous anomalies and cavernous angiomas. AJNR Am J Neuroradiol. Aug 1994;15(7):1247-54; discussion 1255-7. [Medline].

Keywords

venous vascular malformation, brain venous vascular malformation, developmental venous anomaly, DVA, venous angioma, cavernous angioma, arteriovenous malformation, capillary telangiectasia

Contributor Information and Disclosures

Author

Andrew L Wagner, MD, Assistant Professor of Radiology, Instructional Faculty, University of Virginia School of Medicine; Director of Neuroradiology, Department of Radiology, Rockingham Memorial Hospital
Andrew L Wagner, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Robert A Koenigsberg, DO, MSc, FAOCR, Professor, Director of Neuroradiology, Program Director, Diagnostic Radiology and Neuroradiology Training Programs, Department of Radiology, Hahnemann University Hospital, Drexel University College of Medicine
Robert A Koenigsberg, DO, MSc, FAOCR is a member of the following medical societies: American Osteopathic Association, American Society of Neuroradiology, Radiological Society of North America, and Society of NeuroInterventional Surgery
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

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