Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
November 6, 2018
Intracranial DAVF with spinal venous drainage (Cognard V)
- Background
- Rare intracranial DAVF with spinal perimedullary venous drainage.
- Clinical Presentation
- Majority of patients present in the 5th and 6th decades; rare in children.
- (Cognard V) 4:1 predilection for males over females.
- Myelopathy from venous hypertension may be present with spinal perimedullary venous drainage.
- Chances of myelopathy increased when DAVF venous drainage is not just limited to the cervical cord, but descends towards the conus medullaris.
- Patient-specific symptomatology is dependent on involved cord level/s.
- Classification
- Two predominant classifications systems exist: Borden and Cognard.
- The Cognard classification system is based on venous drainage, flow pattern in the sinus, and cortical venous drainage.
- The Borden classification system is based on venous drainage site and cortical venous drainage.
- In either classification scheme, the presence of cortical venous drainage or venous ectasia is a higher-risk feature which predisposes to greater risk for resultant hemorrhage, as well as non-hemorrhagic neurologic deficit.
- This case constitutes a Cognard V, given the presence of cortical venous with spinal perimedullary venous drainage.
- Key Diagnostic Features
- CT and MRI usually provide the initial radiological basis for diagnosis.
- In the setting of spinal perimedullary venous drainage, a spinal MRI is helpful in not only delineating the presence of perimedullary flow voids, but also in evaluating for potential myelopathy.
- Conventional angiography remains the most accurate means to diagnose and accurately classify dAVFs. Note that segmental spinal angiography is normal when the fistulous connection is intracranial.
- Treatment
- Endovascular treatment is the current mainstay of modern therapy, with surgery and stereotatic radiosurgery reserved for cases in which endovascular treatment may not be possible.
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