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Spontaneous Regression Of Extradural High-flow Vascular Malformation In Spinal Arteriovenous Metameric Syndrome (sams): A Unique Case Report

Iampreechakul, P. 2023Other/Unknown

Iampreechakul, P., Chuntaroj, S., Wattanasen, Y., Hangsapruek, S., Lertbutsayanukul, P., & Siriwimonmas, S. (2023). Spontaneous regression of extradural high-flow vascular malformation in spinal arteriovenous metameric syndrome (SAMS): A unique case report. Surgical neurology international, 14, 163. https://doi.org/10.25259/SNI_4_2023

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Abstract

BACKGROUND: Spinal arteriovenous metameric syndrome (SAMS) is a rare nonhereditary genetic vascular disorder, involving multiple layers of tissues in the same metameric level. Spontaneous regression of SAMS has never been reported in the medical literature. CASE DESCRIPTION: A 42-year-old woman presented with intermittent low back pain for 6 months. Magnetic resonance imaging of the thoracolumbar spine incidentally found clusters of spinal vascular malformations involving spinal cord, vertebral bodies, epidural space, and paraspinal muscles. There was no sign of venous congestion. Magnetic resonance angiography and spinal angiography revealed intradural spinal cord arteriovenous malformation (SCAVM) at the level of T10-11 and extradural high-flow osseous arteriovenous fistula. Due to asymptomatic SAMS and a high risk of anterior spinal arterial compromise during treatment, conservative treatment was considered in our patient. Spinal angiography obtained 8 years after initial angiography demonstrated significant regression of extradural component of SAMS and stable intradural SCAVM. CONCLUSION: We describe a unique case of SAMS with spontaneous regression of extradural component during a long-term observation period.

Case Details

Disease Location

Cns vasculature

Personal Characteristics

42-year-old woman

Clinical Characteristics

Intermittent low back pain for 6 months plain radiography of lumbar spine, revealed scoliosis and mixed lytic and sclerotic lesions at l1 and l2 vertebral bodies with collapse of left side of l1 vertebral body. MRI of the thoracolumbar spine was performed and incidentally found clusters of vascular malformations. Spinal angiography confirmed spinal cord arteriovenous malformation (scam) nidus type, at the level of t10-11 supplied by left posterior spinal arteries that receives radiculopial branches from the left t11intercostal and l1 segmental arteries, and sulco-commissural feeder arising from the anterior spinal artery of the left t9 with caudal drainage into perimedullary veins

Remission Characteristics

Spinal angiography obtained 8 years after initial angiography surprisingly showed significant regression of extradural component of sams and stable intradural scam

Treatment & Mechanisms

Proposed Remission Mechanisms

None reported

Clinical Treatment

None reported

Non-Clinical Treatment

None reported