eMedicine Specialties > Radiology > Brain/Spine
Brain, Venous Vascular Malformations
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.
Surgical treatment for DVAs has been advocated, but most experts believe that the resulting risk of an iatrogenic venous infarct would far exceed the risk of irreversible damage from the DVA itself during the patient's lifetime. In fact, most patients with DVAs who become symptomatic have an associated cavernous angioma, which suggests that the symptoms are actually caused by the cavernoma.
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.
They likely result from the absence of normal venous drainage, which forces the venous outflow to find an alternative course.
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
More on Brain, Venous Vascular Malformations |
 Overview: Brain, Venous Vascular Malformations |
| Imaging: Brain, Venous Vascular Malformations |
| Follow-up: Brain, Venous Vascular Malformations |
| Multimedia: Brain, Venous Vascular Malformations |
| References |
| Further Reading |
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References
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Further Reading
Related eMedicine topics
Arteriovenous Malformations
Chiari Malformation
Intracranial Arteriovenous Malformation
Brain, Arteriovenous Malformation
Chiari I Malformation
Chiari II Malformation
Brain, Capillary Telangiectasia
Clinical guidelines
ACR Appropriateness Criteria® cerebrovascular disease. American College of Radiology - Medical Specialty Society. 1996 (revised 2006). 20 pages. NGC:005545
Clinical trials
Diagnosis of Hemangiomas and Vascular Malformations
Influence of MMP on Brain AVM Hemorrhage
Comparison of Abnormal Cortical Development in Brain Malformations on Postmortem Imaging With Autopsy
A Randomized Trial of Unruptured Brain AVMs
Brain Development Research Program
Keywords
venous vascular malformation, brain venous vascular malformation, developmental venous anomaly, DVA, venous angioma, cavernous angioma, arteriovenous malformation, capillary telangiectasia
