A 51-year-old male, with a medical history of hypertension and diabetes mellitus, was admitted to the local hospital due to intermittent acute exacerbation of myelopathy for 3 months. One year earlier, he had developed gait disturbance and difficulty in balancing. There was no history of trauma. Nine months later, the patient had acute onset of paraparesis after waking up in the morning. He collapsed on the ground and could not stand up for a while. After 30 min, he could raise his legs and walk as baseline. He complained of minimal back pain and denied numbness. Two months before hospitalization, he could not stand up after having dinner in the evening. After 30 min later, his symptoms improved. He had occasionally numbness on his left leg. After this event, he had a limitation on ambulation and was unable to walk more than about 50–100 m without stopping to rest. No bowel or bladder involvement was complained. Three weeks before admission, the patient had the same symptoms and rest for about 30 min then improved. MRI of whole spine was performed and was interpreted of a long segment T2 hyperintense cord lesion extending from the level of T4 to the conus medullaris. Lumbar puncture was performed due to the putative diagnosis of transverse myelitis. Cerebrospinal fluid pressure, cell count, and chemistries were within normal limits. The patient was transferred to Prasat Neurological Institute for definite diagnosis and proper management. The physical examination revealed bilateral lower extremity weakness (muscle strength 4/5) without paresthesia. Hyperreflexia was noted in the lower extremities. Babinski's sign was also positive. By counting vertebrae from whole spine screening, there was a degenerative bulging disc at the level of L4–L5 with spinal canal stenosis and compression of bilateral L5 traversing roots []. MRI of the thoracolumbar spine showed the spinal cord congestion at the level of T4 to the conus medullaris with subtle perimedullary flow voids along ventral and dorsal cord surfaces [Figure and ]. Furthermore, there was a curvilinear intradural flow void on T2-weighted image, identified below the conus medullaris. Contrast-enhanced MRA of the thoracolumbar spine demonstrated an enlarged intradural vessel in the midline location extending from the lower lumbar level to the conus medullaris, probably the vein of the filum terminale [], corresponding with the curvilinear flow void on MRI. Spinal angiography demonstrated SDAVF at the level of L4–L5, probably located on the right L5 nerve root sleeve, which is supplied by the right iliolumbar artery originating from the internal iliac artery with cranial drainage into the dilated vein of the filum terminale. The artery of Adamkiewicz arose from the right T8 intercostal artery. Endovascular treatment with NBCA through the right iliolumbar artery was successfully performed to fill in the arterial feeder and fistulous site but fail to reach the proximal vein []. Postembolization angiography of the internal iliac and iliolumbar arteries revealed complete obliteration of the fistula. The patient had gradually improved and was sent back to the local hospital for further physiotherapy. Four months later, he returned and was readmitted to our institution with gait disturbance with the deterioration of clinical symptoms. Follow-up MRI and MRA of the lumbosacral spine revealed unchanged long segment of spinal cord congestion with the presence of SDAVF at the same level. One week later, the patient underwent decompressive laminectomy with obliteration of the intradural draining vein. The patient had significantly improved until being the ability to walk independently 2 weeks later. Spinal angiography, obtained 2 weeks after surgery, confirmed complete obliteration of the fistula []. MRI of the thoracolumbar spine, obtained 4 months after surgery, confirmed the resolution of spinal cord congestion [].
A 33-year-old male presented with a 2-year history of pain in the lumbosacral spine with progressive lower-limb weakness, urinary frequency, and incontinence. Symptoms intensified after exercise and prolonged standing. Lumbar spine MRI demonstrated a tethered cord at the L3 level, a filum terminale lipoma, and edema in the spinal cord. It also showed tortuous veins on the posterior surface of the spinal cord (Figures ).\nThe neurological examination revealed bilateral hip-joint flexion weakness (grade 4 according to modified Medical Research Council system), plantar flexion weakness (grade 3 according to modified Medical Research Council system), and reduced sensation in the S2–S3 dermatomes. The Babinski and Rossolimo signs were present bilaterally. Based on the clinical and radiological signs, a SDAVF was suspected. A DSA confirmed the SDAVF at the S2-S3 level, in the dura supplied by lateral sacral arteries and branches from the internal iliac arteries. Tortuous draining veins were present on the posterior surface of the entire spinal cord and terminated intracranially (Figures ).\nThe patient underwent three endovascular embolizations, each with a different agent. First, embolization was performed with Onyx™ 18, administered in two microcatheters. This treatment resulted in an occlusion of the fistula. The patient significantly improved, but due to poor penetration of the draining vein, after a few weeks, the fistula recanalized and symptoms returned.\nA second embolization was performed 4 months later. The vascular access to the fistula was more complicated. A 25% Phil™ injection was feasible only through the small sacral branches of the left internal iliac artery. This procedure achieved a significant reduction of flow through the fistula. Eighteen months later embolization of the fistula was attempted with diluted glue. Despite good occlusion of the fistula observed in a DSA, the tortuous vessels remained visible in the follow-up MRI (Figures ). The symptoms persisted, and the patient was qualified for surgical treatment.\nThe goal of surgery was to occlude the draining vein and the arterial feeder and to reduce the lipoma. With a median incision, the sacral canal was opened on the right side. The dura was thick, and there were multiple vessels filled with embolic agents. There was one large, bright-red vessel extradurally and one intradurally at the S2-S3 level. A clip was placed upon the arterialized vein intradurally for a couple of minutes. After that, the vessel changed color and the tension decreased. Both intra- and extradural vessels were coagulated. Afterwards, the lipoma was visualized and partially removed. The dura was sutured in a watertight fashion, and the wound was closed in layers.\nThe postoperative course was uneventful, and a few days later, the patient was discharged. After 6 months, a follow up MRI showed the absence of tortuous vessels on the posterior surface of the spinal cord and a significant reduction in the myelopathic changes (Figure ). On follow-up, the patient considerably improved, with no incontinence, some urinary frequency and mild weakness (grade 4 according to modified Medical Research Council system).