Remote Thoracic Myelopathy From a Spinal Dural Arteriovenous Fistula at the Craniocervical Junction: Case Report and Review of Literature

doi:10.1016/j.wneu.2017.08.158

World Neurosurgery

Volume 108, December 2017, Pages 992.e1-992.e4

https://doi.org/10.1016/j.wneu.2017.08.158 Get rights and content

Background

The craniocervical junction is a rare location for spinal dural arteriovenous fistulas (dAVFs). Typically, fistulas at this location present with findings related to intracranial cortical venous reflux or cervical myelopathy. We present a case of craniocervical junction dAVF with isolated clinical and radiographic findings of thoracolumbar myelopathy.

Case Description

A 54-year-old man presented with subacute onset of lower extremity weakness, paresthesias, and gait dysfunction. Routine spine magnetic resonance imaging demonstrated edema of the conus medullaris and distal thoracic spinal cord and prominent dorsal venous flow voids, suggestive of a thoracolumbar dAVF. Spinal angiography performed at an outside institution failed to demonstrate a fistula. Noninvasive spinal angiographic imaging with time-resolved magnetic resonance angiography (TR-MRA) performed at our institution was able to demonstrate presence of the fistula at the craniocervical junction. Subsequent cerebral angiography identified feeding vessels arising from the posterior meningeal artery and ascending pharyngeal artery on the right side. The fistula was successfully embolized with onyx embolic material, with rapid resolution of his clinical symptoms.

Conclusions

Isolated lower extremity myelopathic symptoms are a rarely reported finding in patients harboring craniocervical junction dAVFs. At our institution, noninvasive imaging with TR-MRA is routinely used to aid localization of dAVFs. This results in decreased contrast dose and radiation exposure, and inclusion of the cervical spine should be performed when thoracolumbar imaging fails to identify a dAVF prior to proceeding to invasive angiography.

Introduction

Spinal dural arteriovenous fistulas (dAVFs) are rare causes of myelopathy. Given the slow, progressive nature of symptoms, these lesions can present a significant diagnostic dilemma. The classic presentation of including progressive, ascending myelopathic symptoms is typically associated with thoracolumbar dAVFs or conus medullaris perimedullary arteriovenous fistulas.¹ In contrast, cervical or cervicomedullary dAVFs are rare variants, and are usually present with both upper and lower extremity symptoms.2, 3 We present a case of cervicomedullary dAVF presenting exclusively with subacute onset of thoracic myelopathy (Foix-Alajouanine syndrome), which mimicked a thoracic dAVF both clinically and radiographically.

Section snippets

Case Description

A 54-year-old man without significant history of spinal disease or trauma was initially evaluated at an outside institution for a 10-day history of bilateral lower extremity weakness, decreased sensation to light touch below the T11 dermatome, and urinary retention. Routine spinal magnetic resonance imaging demonstrated the presence of prominent dorsal flow voids along the thoracolumbar spinal cord, and cord edema extending from T8 to the conus medullaris. The patient had neither clinical

Discussion

This case represents a unique combination of classic dAVF presentation, suggesting a thoracic fistula location on routine magnetic resonance imaging and clinical examination, with a very rare anatomic configuration.⁴ Since the advent of selective spinal arteriography by Doppman and Djindjian in the 1960s,⁵ substantial experience with the clinical and radiographic features of these lesions has been accumulated worldwide. Despite the absence of true natural history studies of untreated dAVFs, the

Conclusions

Despite increased awareness of the pathologic, clinical, and radiographic findings of dAVFs in the workup for myelopathy, successful diagnosis can still be elusive. The presented case demonstrates that the craniocervical location can be a cause of classic findings of the vastly more common thoracolumbar variant. Use of TR-MRA can help identify fistulas in atypical locations noninvasively, improving the efficiency and safety of formal diagnostic spinal angiography.

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Cited by (6)

The role of venous anatomy in guiding treatment approach for dural arteriovenous fistulas of the craniocervical junction; case series & systematic review
2023, Journal of Clinical Neuroscience
Dural arteriovenous fistulas (DAVF) of the craniocervical junction (CCF) are an uncommon entity with the following venous drainage pattern: inferior, superior and mixed. Patients may present with subarachnoid hemorrhage, myelopathy or brainstem dysfunction. CCJ DAVF can be treated with microsurgery or with transarterial and transvenous embolization, depending on the venous drainage pattern. We present our institutional experience of treating CCJ DAVFs along with a systematic review of the literature.
Six patients with CCJ DAVF were treated at our institution over five years. Data was collected using electronic medical record review. Systematic review was performed on CCJ DAVF using the PubMed database from 1990 to 2021. We characterized venous drainage patterns, treatment choices, and outcomes to create a classification system.
50 case reports, consisting of 115 patients, were included in our review. 61 (53.0 %) patients had inferior drainage while 32 (27.8 %) patients had superior drainage and 22 (19.2 %) patients had mixed venous drainage. Patients with inferior drainage had the fistulous connection at the foramen magnum while patients with superior drainage had a fistulous connection at C1-C2 (p value = 0.026). Patients with inferior drainage were more likely to present with myelopathy while patients with superior drainage presented with hemorrhage (p value = 0.000).
Classifying the venous drainage pattern is essential in making treatment decision. Transvenous embolization works best with large superior venous drainage. If endovascular treatment is not an option, then surgical clipping can achieve successful cure. Transarterial embolization is a reasonable option in cases with a large arterial feeder.
Thoracic Dural Arteriovenous Fistula Presenting with Isolated Pseudobulbar Palsy Mimicking Brainstem Lesion
2020, World Neurosurgery
Citation Excerpt :
Borghei-Razavi et al.4 described a case of sacral DAVF presenting as acute isolated thoracic myelopathy. Do et al.5 and Lang et al.6 also each reported a case of remote thoracic myelopathy from DAVF at the craniocervical junction. Because of the rarity of this isolated lesion, congestion in the brainstem cannot prevent the diagnosis of thoracic DAVF.
Spinal dural arteriovenous fistulas (DAVFs) are usually associated with neurologic dysfunction adjacent to the shunt point; however, the symptoms are uncommon far from the site of the fistula. To our knowledge, this is the first report of a patient with rapidly progressive isolated pseudobulbar palsy because of thoracic DAVF.
We report a patient with thoracic DAVF presenting with remote symptoms of brainstem congestion. The patient was a 36-year-old man who presented with a sudden history of vomiting, dysphagia, and flaccid weakness in the 4 limbs. Intracranial magnetic resonance (MR) imaging at a local hospital demonstrated T2 signal hyperintensity within the medulla, and he was referred to our hospital for a suspected brainstem lesion. However, cervical MR imaging revealed a dilated and tortuous perimedullary venous plexus, and spinal angiography revealed DAVF in T5-6 with a feeding artery from the intercostal artery. After obliteration of the fistula, the progression of the disease was stopped and the symptoms improved.
Although rare, thoracic DAVFs may present symptoms resembling brainstem infarction. Prompt surgical intervention is necessary for patients with thoracic DAVF presenting with rapidly progressive pseudobulbar palsy.
Treatment of high cervical arteriovenous fistulas in the craniocervical junction region
2023, Frontiers in Neurology
Thoracolumbar spinal dural arteriovenous fistulae present with longer arteriovenous transit compared to cranial and cervical dural fistulae
2023, Interventional Neuroradiology
Clinical analysis of three cases of infratentorial dural arteriovenous fistula
2020, Chinese Journal of Neurology
Asymptomatic spinal dural arteriovenous fistula: Case series and systematic review
2019, Journal of Neurosurgery: Spine

: Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Case ReportRemote Thoracic Myelopathy From a Spinal Dural Arteriovenous Fistula at the Craniocervical Junction: Case Report and Review of Literature

Background

Case Description

Conclusions

Introduction

Section snippets

Case Description

Discussion

Conclusions

Clin Neurol Neurosurg

Surg Neurol

Surg Neurol

Microvascular anatomy of spinal dural arteriovenous fistulas: arteriovenous connections and their relationships with the dura mater

J Neurosurg Spine

Three-dimensional angioarchitecture of spinal dural arteriovenous fistulas, with special reference to the intradural retrograde venous drainage system

J Neurosurg Spine

Successful treatment of a group of spinal cord arteriovenous malformations by interruption of dural fistula

J Neurosurg

Spinal vascular malformations

Arteriovenous fistulas of the cervicomedullary junction as a cause of myelopathy: radiographic findings in two cases

AJNR Am J Neuroradiol

Case Report
Remote Thoracic Myelopathy From a Spinal Dural Arteriovenous Fistula at the Craniocervical Junction: Case Report and Review of Literature