Spinal Dural Arteriovenous Fistula: A Review

Abstract

Spinal dural arteriovenous fistula (SDAVF) is a rare disease, the etiology of which is not entirely clear. It is the most common vascular malformation of the spinal cord, comprising 60-80 % of the cases. The clinical presentation and imaging findings may be nonspecific and misleading, often mistaking it for other entities like demyelinating or degenerative diseases of the spine.This chapter describes the imaging findings, clinical signs, and symptoms of this disease and also the available treatment options according to the current literature.Angiography is still considered the gold standard for diagnosis; however, MRI/MRA is increasingly used as a screening tool. Modern endovascular techniques are becoming increasingly more effective in treating SDAVF offering a less invasive treatment option; however, they still lag behind surgical success rates which approach 100 %. The outcome of both treatment options is similar if complete obliteration of the fistula is obtained and depends mainly on the severity of neurological dysfunction before treatment.Heightened awareness by radiologists and clinicians to this rare entity is essential to make a timely diagnosis of this treatable disease. A multidisciplinary treatment approach is required in order to make appropriate treatment decisions.

Progressive Paraplegia Due to Spinal Dural Arteriovenous Fistula: A Rare but Treatable Diagnosis That Should Not Be Missed

“…The spinal dural arteriovenous fistula was first described in 1926 by Foix and Alajouanine [1]. It is the most common vascular malformation of the spinal cord, albeit still rare, with an annual incidence of only five to ten cases per million [2].…”

“…More than 75% of the SDAVFs are located from the mid-thoracic to the upper lumbar spine. The presence of a fistula leads to arterialization of the spinal veins resulting in venous hypertension, venous engorgement, and if untreated will lead to cord ischemia and infarction [1].…”

“…To our surprise, the literature reveals an average delay of around 15-24 months from the symptom-onset to the confirmation of the diagnosis of SDAVF [3-4]. This delay in diagnosis results in an increase in the severity of motor and sensory symptoms, with 10-30% of the cases are wheel-chair bound at the time of diagnosis [1]. Apart from the motor weakness, worsening sphincter dysfunction and sexual problems adversely affect the quality of life.…”

“…These include (1) T2 hyperintense signals within the cord, (2) spinal cord expansion, and (3) vessel flow voids on the dorsal and/or ventral aspect of the cord. In chronic cases of SDAVF, there may be some T1 postcontrast enhancement or spinal cord atrophy [1]. When these MRI features are missed on initial MRI spine and radiologists do not suspect a vascular etiology, a mean diagnostic delay of 281 days from the initial MRI scan was seen in the diagnosis of SDAVF as compared to only 22 days when a vascular etiology was suspected in the initial MRI reporting [5].…”

Cerebrospinal fluid cannot be used to distinguish inflammatory myelitis from congestive myelopathy due to spinal dural arteriovenous fistula: case series

“…Raised CSF protein and pleocytosis in SDAVF may be related to venous hypertension from an arterialised venous drainage system resulting in raised intravenule pressure,4 5 with transudative flow of protein and cells into the extravenule space and CSF. Kilic et al reported presence of mature lymphocytes in a patient with SDAVF also treated initially with steroids, but detailed CSF analysis was not described 1.…”