Case ReportRemote Thoracic Myelopathy From a Spinal Dural Arteriovenous Fistula at the Craniocervical Junction: Case Report and Review of Literature
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.
References (16)
- et al.
Natural history, current concepts, classification, factors impacting endovascular therapy, and pathophysiology of cerebral and spinal dural arteriovenous fistulas
Clin Neurol Neurosurg
(2014) - et al.
The variable presentations of craniocervical and cervical dural arteriovenous malformations
Surg Neurol
(1990) - et al.
Intracranial dural arteriovenous fistula associated with progressive cervical myelopathy and normal venous drainage of the thoracolumbar cord: case report and review of the literature
Surg Neurol
(2006) - et al.
Microvascular anatomy of spinal dural arteriovenous fistulas: arteriovenous connections and their relationships with the dura mater
J Neurosurg Spine
(2015) - et al.
Three-dimensional angioarchitecture of spinal dural arteriovenous fistulas, with special reference to the intradural retrograde venous drainage system
J Neurosurg Spine
(2013) - et al.
Successful treatment of a group of spinal cord arteriovenous malformations by interruption of dural fistula
J Neurosurg
(1983) - et al.
Spinal vascular malformations
- et al.
Arteriovenous fistulas of the cervicomedullary junction as a cause of myelopathy: radiographic findings in two cases
AJNR Am J Neuroradiol
(1990)
Cited by (6)
Thoracic Dural Arteriovenous Fistula Presenting with Isolated Pseudobulbar Palsy Mimicking Brainstem Lesion
2020, World NeurosurgeryCitation 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.
Treatment of high cervical arteriovenous fistulas in the craniocervical junction region
2023, Frontiers in NeurologyThoracolumbar spinal dural arteriovenous fistulae present with longer arteriovenous transit compared to cranial and cervical dural fistulae
2023, Interventional NeuroradiologyClinical analysis of three cases of infratentorial dural arteriovenous fistula
2020, Chinese Journal of NeurologyAsymptomatic 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.