Hemispheric developmental venous anomaly draining into a vein of Galen varix: a case report and literature review
© The Author(s) 2017
Received: 26 May 2016
Accepted: 8 December 2016
Published: 6 March 2017
Developmental venous anomaly (DVA), previously known as “venous angioma”, is the most common cerebral vascular malformation, but hemispheric DVA is extremely rare.
In this article we present a 21-year-old female with a large DVA draining the whole left hemisphere, combined with a vein of Galen varix and falcine sinus. To our knowledge, only 4 cases of hemispheric DVA have been reported before, however, none with a vein of Galen varix. This patient’s digital subtraction angiography also revealed double straight sinus with severe stenosis of one of the sinuses, which was considered to be the possible reason of the venous varix. The patient was managed conservatively, and her clinical status was stable for 1 year.
Because brain DVA drains the normal cerebral parenchyma and the hemorrhage risk is relatively low, hemispheric DVA is considered to be a benign anomaly, and conservative treatment is recommended.
KeywordsCerebral hemisphere Developmental venous anomaly Vein of Galen varix Venous malformation Digital subtraction angiography
Developmental venous anomaly (DVA), also known as “venous angioma” in the past, is the most common cerebral vascular malformation . Most DVAs drain into a dilated collecting vein, and only a few DVAs drain into a cerebral varix . Hemispheric DVA draining into a vein of Galen varix is extremely rare. We present a case of a female with hemispheric DVA draining into a vein of Galen varix and then draining into a falcine sinus; the patient also had double straight sinuses, with severe stenosis of one of them. The imaging features of this patient are presented, and the literature is reviewed. The aim of this case report is to help us further understand the formation of DVA and the process of venous development.
Summary of the literature on patients with hemispheric developmental venous anomaly
Site of the DVA
Aagaard, 1999 
Seizures, Hemangiomas throughout the face
Enlargement of VG, straight sinus and torcular herophili
Uchino, 2001 
Cerebral hemiatrophy, hypoplastic straight sinus
Casey, 2011 
Tortuous draining vein terminating in straight sinus
Jung, 2013 
Venous varix, as Lasjaunias described in the 1980s, “does not exist without an obstruction in the cerebral venous system, and the location varies from the proximal straight sinus to the jugular foramen” . However, as an increasing number of cases are currently being described, “venous hypertension” is becoming the new cause of these types of anomalies. In the cases without collecting vein stenosis, venous hypertension may also exist because of volume overload as the result of histopathological changes in the vessel wall, including wall thickening and hyalinization . In this case, stenosis of the inferior straight sinus is considered to be the reason for vein of Galen varix. To compensate for the increased pressure of the venous system, the falcine sinus did not close, and another straight sinus occurred, as well as a vein of Galen varix and a dilated duro-venous system. The key point in the differential diagnosis of vein of Galen varix from a true vein of Galen aneurysmal malformation (VGAM) was the absence of arteriovenous malformation (AVM) or arteriovenous fistula (AVF).
As this special system drained the normal cerebral parenchyma, these changes are considered to be a “venous anomaly” and not a “venous malformation”. As the hemorrhage risk of DVA is very low (the retrospective risk is 0.22% per year , and the prospective risk is 0.68% per year ), and an inappropriate surgical or interventional therapy may cause a horrible consequence, conservative treatment is recommended for patients with endurable clinical symptoms.
Hemispheric developmental venous anomaly is an extreme type of DVA that drains the blood of the entire hemisphere, and it tends to combine with nonspecific clinical symptoms such as headache or dizziness. In this report, we presented the imaging results of a special case of hemispheric DVA combined with a vein of Galen varix. The hemorrhagic risk is relatively low, which made it a benign anomaly, and conservative management is recommended.
Digital subtraction angiography
Developmental venous anomaly
Internal cerebral vein
Magnetic resonance imaging
Vein of galen
Vein of galen aneurysmal malformation
Availability of data and materials
HX has evaluated the patient, and drafted and revised the manuscript. JZ evaluated the patient, carried out the literature review. GC conceived of the study, and helped to draft the manuscript. JC Collected data and carried out the follow-up. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
We obtained written permission from the patient to publish this case report.
Ethics approval and consent to participate
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