A 46-year-old male was admitted to our institute due to a history of progressive paraparesis for 2 weeks. Eight months earlier, he experienced severe left buttock pain radiating to left leg for 2 weeks. He was admitted to other private hospital and underwent discectomy L5-S1. Five months later, he suffered from right leg pain and urinary retention. He went back to the same private hospital and underwent revised surgery with spinal fusion L5-S1. Two weeks before hospitalization, he developed progressive paraparesis and constipation. He was unable to walk more than about 100 m. The patient went to another private hospital and was transferred to Prasat Neurological Institute for further investigation and proper treatment after performing MRI of the lumbosacral and thoracic spine. The neurological examination revealed the evidence of spastic paraparesis (muscle strength 3/5) and the lack of pinprick sensation below T10 level, impairment of proprioception, hyperreflexia, and presence of Babinski's sign in the lower extremities. The previous series of MRI of the lumbosacral and thoracic spine were retrospectively reviewed and revealed spinal canal stenosis and bilateral lateral recesses stenosis at L5-S1 level due to moderate diffuse annular disc bulging with moderate enlargement of facet joints and thickening of the ligamentum flavum []. There was an abnormal hyperintense T2 signal with patchy enhancement representing spinal cord congestion extending from the conus medullaris to the level of T4 with subtle perimedullary flow voids along ventral and dorsal cord surfaces [Figures and ]. Magnetic resonance (MR) myelography also showed “worm-like” appearance of the area from thoracic level to the conus medullaris []. MR angiography (MRA) of the thoracolumbar spine demonstrated early opacification of perimedullary venous plexus along anterior and posterior surfaces of spinal cord []. Furthermore, there was a curvilinear flow void, probably the dilated vein of the filum terminale, running from lower lumbar level to the conus medullaris []. Spinal angiography demonstrated SDAVF at the level of L5-S1, probably located on the left S1 nerve root sleeve, which is supplied by the left lateral sacral artery (LSA) originating from the internal iliac artery with venous drainage through the dilated radicular vein into the dilated vein of the filum terminale [Figure and ]. The artery of Adamkiewicz arose from the left L2 lumbar artery and left T9 intercostal artery. Transarterial embolization with N-butyl-cyanoacrylate (NBCA) through the left LSA was successfully performed with NBCA filling across the fistula into the proximal draining vein []. The patient had gradually improved until being the ability to walk independently 3 months later. His bowel and bladder controls had completely regained. Six months after the embolization, follow-up MRI of the thoracolumbar spine also confirmed completely regression of spinal cord congestion [].
A 58-year-old woman was referred for neurosurgical evaluation because of a 2-year history of rapid decline in bilateral motor functioning. Ten years earlier, she underwent an L4 and L5 laminectomy for cord detethering at an outside facility but had no meaningful improvement. Since then, she had experienced a gradual deterioration in her ambulation. Magnetic resonance imaging (MRI) demonstrated persistent evidence of cord tethering, with an L4/L5 lipomyelomeningocele extending to the sacral hiatus. Notably, prominent flow voids were also seen within the ventral subarachnoid space, with the most caudal draining vessel extending below the S2 sacral body. She had 3/5 strength in her left leg, 4/5 strength in her right, globally diminished sensation to light touch, and pin prick, as well as some bladder urgency. The patient did endorse some lower back pain but also has a history of chronic lower back pain. A diagnostic spinal angiogram showed evidence of a DAVF with arterial feeders from the bilateral lateral sacral arteries and right internal iliac artery with venous drainage to a dilated perimedullary vein. The patient underwent Onyx embolization of both feeding right and left lateral sacral arteries.\nThree months later, the patient exhibited marked improvement in her leg strength and sensation, improved 4-5/5 strength throughout her lower extremities, improved walking with assistance, and marked improvement in balance. At her 6-month follow-up, however, she reported stagnant functional capacity, with worsening of her left leg function that began after a ground-level fall. Physical examination revealed stable to improved right leg strength with subtle worsening of left leg strength (3-4/5). MRI revealed decreased flow voids compared with those seen on the prior study with no evidence of a new abnormality; however, one month later, evaluation revealed globally decreased lower extremity strength (3/5). Repeat spinal angiogram and lumbar MRI revealed new collateralized supply from the right internal iliac artery and lateral sacral branches. The DAVF now extended into the lipomyelomeningocele with dilated draining veins rostrally and spinal canal involvement.\nThe findings of redeveloping DAVF and the patient's clinical condition prompted us to recommend lipomyelomeningocele exploration/resection, DAVF ligation, and cord detethering. A standard midline approach was utilized, and a grossly abnormal fatty myelomeningocele was circumferentially dissected (). Intradural exploration demonstrated diffuse blood supply tracking through the lipomyelomeningocele with a large draining vein ventral to a tethered filum. The filum was sectioned, and the DAVF was clipped and ligated well into the arterialized vein. The remaining lipomyelomeningocele was mobilized and resected ().\nSix months after surgery, the patient regained full motor function of her proximal lower extremities with 5/5 strength throughout and 5/5 and 4/5 strength in her distal right and left lower extremities, respectively. MRI of the thoracic spine demonstrated marked improvement in the appearance of the spinal DAVF, with the tortuous vessels no longer evident. Follow-up spinal angiogram showed no evidence of residual DAVF or early draining vein ().