Spinal vascular malformations: Early interventions and importance of proper diagnosis

Oct. 02, 2020

W. Oliver Tobin, M.B., B.Ch., B.A.O., Ph.D., a neurologist, and Giuseppe Lanzino, M.D., a neurosurgeon, at Mayo Clinic in Rochester, Minnesota, discuss spinal vascular malformations.

What makes a spinal vascular malformation unique and difficult to treat?

Spinal vascular malformations encompass a range of disorders that often result in an intermittent or progressive myelopathy. Standard magnetic resonance imaging (MRI) may not identify the vascular abnormality and can be normal in the early stages of the disorder. The myelopathy can be exercise dependent initially and can spontaneously remit. However, if not identified, it can lead to a progressive disabling myelopathy, which is difficult to reverse even after correction of the spinal vascular abnormality. A negative formal spinal angiogram does not completely exclude a spinal vascular malformation, as there is a large range of abnormalities that extend throughout the length of the spine and beyond into the cerebral vasculature and pelvic vasculature.

What is Mayo Clinic's approach to the diagnosis and treatment of spinal vascular malformations?

We use a multidisciplinary approach to the evaluation of myelopathy and of suspected spinal vascular malformations. A carefully coordinated evaluation is completed by experts in neurology, neuroradiology, neurosurgery, physical medicine and rehabilitation, urology, and other fields to identify the cause for the myelopathy and determine a treatment plan.

As noted in a 2018 article in Neurology, almost 1 in 5 patients who are referred to Mayo Clinic for evaluation of an idiopathic myelopathy are identified as having a vascular myelopathy.

Why is early intervention or treatment important?

In a 2016 American Journal of Neuroradiology (AJNR) article, Mayo Clinic researchers noted that early identification and treatment of vascular myelopathy is critical for good patient outcomes. We also know that people with a longstanding dural arteriovenous fistula with an associated myelopathy have a poor prognosis for recovery.

Have there been any recent technological advancements or other innovations that have helped further enhance the treatment of this malformation?

Mayo Clinic noted, in research published in the Journal of Neurosurgery in 2017 and the Journal of NeuroInterventional Surgery in 2016, that enhanced diagnostic tools allow us to see distinct patterns on MRI that can identify dural arteriovenous fistula. Also, some spinal vascular malformations once considered extremely rare, such as epidural spinal arteriovenous fistulas, are now being recognized in an increasing number of patients due to improved diagnostic tools and enhanced knowledge and better understanding.

When should a physician refer a patient for treatment or a second opinion for spinal vascular malformation treatment?

Any patient with a myelopathy of uncertain origin should be referred for secondary evaluation. After evaluation at Mayo Clinic, only a very small proportion of patients initially referred with a diagnosis of idiopathic transverse myelitis were thought to be idiopathic following the evaluation. Many of these patients underwent treatment, including 1 in 5 patients who underwent treatment for a vascular myelopathy. We also know from our research published in AJNR in 2016, that even after a negative spinal angiogram, patients should be referred for an evaluation, as we have found that of all patients with a delayed diagnosis, approximately 20% had a nonrevealing spinal angiogram in the course of their initial evaluation.

At Mayo Clinic, who would be part of a care team for a patient with spinal vascular malformation? Why is this distinction important?

We have a close collaboration among all the teams at Mayo Clinic. This enables us to be very coordinated on all necessary appointments and have test results available quickly. Since we work together as a multidisciplinary team, we are able to provide that expert, individualized care to every person who seeks our expertise. We have the experience, knowledge, state-of-the-art research and laboratory facilities, and advanced technology, and we are constantly innovating to continually best meet the needs of the patients.

For more information

Zalewski NL, et al. Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses. Neurology. 2018;90:e96.

Brinjikji W, et al. Clinical outcomes of patients with delayed diagnosis of spinal dural arteriovenous fistulas. American Journal of Neuroradiology. 2016;37:380.

Nasr DM, et al. Clinical presentation and treatment outcomes of spinal epidural arteriovenous fistulas. Journal of Neurosurgery. 2017;26:613.

Brinjikji W, et al. Spinal epidural arteriovenous fistulas. Journal of NeuroInterventional Surgery. 2016;8:1305.