Introduction: Neural tube defects, such as tethered cord, intradural lipoma or myelomeningocele may coexist with spinal arteriovenous malformations. The coexistence of these two rare entities is suggestive of a causal relationship between the two which may lead to further understanding of their pathogenesis. We present a series of six patients with epidural spinal arteriovenous fistula associated with neural tube defects.

Methods: We retrieved cases of spinal vascular malformations associated with neural tube defects seen at our Institution. The clinical presentation, MRI/MRA and angiographic imaging, treatment outcomes, and long-term neurological outcomes were analyzed. Descriptive statistical analyses are reported.

Results: Six patients with an epidural arteriovenous fistula and neural tube defects were included in this study. Mean age at presentation was 42 years and the most common presenting symptoms were lower extremity weakness followed by sensory disturbances and bladder bowel dysfunction. In the majority of cases (5/6) the fistulae were located at the sacral level. All cases were fed by the lateral sacral artery (6/6). Four patients had prior spine surgery but the fistula was located in the operative bed in two cases. All fistulae were extradural with secondary intradural venous drainage. Five patients underwent transarterial embolization with Onyx and one patient suffered a treatment related complication.

Conclusions: It is conceivable that there is a pathophysiological link between neural tube defects and development of spinal vascular malformations. Delayed neurological deterioration or high conus signal in a patient with neural tube defect should raise the possibility of such an association.

Patient Care: The rare concurrence of neural tube defects with spinal vascular malformations is something that should be kept in consideration when evaluating a patient with a neural tube defect and myelopathy

Learning Objectives: By the conclusion of this session, participants should be able to: 1)be aware that the rare concurrence of neural tube defects with spinal vascular malformations its not just speculative, 2) keep that in consideration when evaluating a patient with a neural tube defect and myelopathy 3)paid attention in identifying the anterior spinal artery arising from a low lying artery such as the lateral sacral artery .

References: 1. Chanthanaphak E, Pongpech S, Jiarakongmun P, et al. Filum terminale arteriovenous fistulas: the role of endovascular treatment. Journal of neurosurgery Spine 2013;19:49-56 2. Chatkupt S, Ruzicka PO, Lastra CR. Myelomeningocele, spinal arteriovenous malformations and epidermal nevi syndrome: a possible rare association? Developmental medicine and child neurology 1993;35:737-741 3. Cheung AC, Kalkanis SN, Ogilvy CS. Paraplegia after tethered cord surgery: an uncommon combined anomaly of spinal arteriovenous fistula and sacral lipoma--case report. Neurosurgery 2005;57:E598; discussion E598 4. Djindjian M, Ayache P, Brugieres P, et al. Sacral lipoma of the filum terminale with dural arteriovenous fistula. Case report. Journal of neurosurgery 1989;71:768-771 5. Erdogan C, Hakyemez B, Arat A, et al. Spinal dural arteriovenous fistula in a case with lipomyelodysplasia. The British journal of radiology 2007;80:e98-e100 6. Gross BA, Du R. Spinal pial (type IV) arteriovenous fistulae: a systematic pooled analysis of demographics, hemorrhage risk, and treatment results. Neurosurgery 2013;73:141-151; discussion 151 7. Hurst RW, Bagley LJ, Marcotte P, et al. Spinal cord arteriovenous fistulas involving the conus medullaris: presentation, management, and embryologic considerations. Surgical neurology 1999;52:95-99 8. Kendall BE, Logue V. Spinal epidural angiomatous malformations draining into intrathecal veins. Neuroradiology 1977;13:181-189 9. Konig M, Hentsch A, Schmieder K, et al. Extraspinal dural arteriovenous fistula in a patient with lipomyelodysplasia: value of MRI and MRA. Neuroradiology 1999;41:579-583 10. Krisht KM, Karsy M, Ray WZ, et al. Extraspinal type I dural arteriovenous fistula with a lumbosacral lipomyelomeningocele: a case report and review of the literature. Case reports in neurological medicine 2015;2015:526321 11. Lee JH, Chung CK, Choe G, et al. Combined anomaly of intramedullary arteriovenous malformation and lipomyelomeningocele. AJNR American journal of neuroradiology 2000;21:595-600 12. Lim SM, Choi IS, David CA. Spinal arteriovenous fistulas of the filum terminale. AJNR American journal of neuroradiology 2011;32:1846-1850 13. Macht S, Chapot R, Bieniek F, et al. Unique sacral location of an arteriovenous fistula of the filum terminale associated with diastematomyelia and lowered spinal cords. Neuroradiology 2012;54:517-519 14. Mavani SB, Nadkarni TD. Tethered cord due to caudal lipomeningocele associated with a lumbar dural arteriovenous fistula. Journal of neurosurgery Spine 2014;21:489-493 15. Rajeev K, Panikar D. Dural arteriovenous fistula coexisting with a lumbar lipomeningocele. Case report. Journal of neurosurgery Spine 2005;3:386-389 16. Rice JF, Jelsma RK. Combined arteriovenous malformation and intramedullary lipoma: surgery and embolotherapy. AJNR American journal of neuroradiology 1986;7:171-173 17. Sato M, Takigawa T, Shiigai M, et al. Spinal dural arteriovenous fistula with lipomyelodysplasia. Neurologia medico-chirurgica 2013;53:107-109 18. Srinivasan A, Roy NK, Ghuman MS, et al. Spinal dysraphism masking the presentation of spinal dural arteriovenous fistula: A serendipitous development or a congenital predisposition to an early-onset variant of spinal dural arteriovenous fistula? Neurology India 2016;64:1380-1381 19. Tubbs RS, Oakes WJ. Lipomyelomeningocele and arteriovenous malformation: case reports and a review of the literature. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2006;22:628-631 20. Weon YC, Chung JI, Roh HG, et al. Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele. Neuroradiology 2005;47:774-779 21. Wortsman J, Chui M, Gray T, et al. Spinal meningeal AVM supplied from the internal iliac artery associated with tethered cord syndrome in an adult: case report. Journal of the Canadian Association of Radiologists 1983;34:323-325 22. Nasr DM, Brinjikji W, Clarke MJ, et al. Clinical presentation and treatment outcomes of spinal epidural arteriovenous fistulas. Journal of neurosurgery Spine 2017;26:613-620 23. Kroszczynski AC, Kohan K, Kurowski M, et al. Intraforaminal location of thoracolumbar anterior medullary arteries. Pain medicine (Malden, Mass) 2013;14:808-812