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 53-year-old man with no significant past medical history was admitted to our hospital because of pain in the lower-extremities and gait disturbance. His gait disturbance noticed by his family when he was 30 years of age. At age 51, he was found to have a spinal lipoma at a neighboring hospital and was referred to our institution. His lower extremity pain and gait disturbance worsened while he was under observation and he was admitted for further examination. Manual muscle testing revealed lower limb paresis that was more pronounced on the right side. The patellar tendon reflex was not exaggerated on either side, but the Achilles tendon reflex was hyperactive and the Babinski sign was positive on both sides. There was no evidence of bladder or bowel disturbance, and the lumbar JOA score was 10/29. MRI of the spine revealed a spinal lipoma at the L2/3 level and a flow void dorsal to the spinal cord at the level of the conus medullaris (Fig. ). MRA revealed tortuous blood vessels within the spinal lipoma, as in Case 1 (Fig. ). Subsequent angiography revealed an arteriovenous shunt from the right second lumbar artery. The radiculopial artery entered the spinal canal from its ventral aspect to form a arterio-venous shunt within the lipoma. A vein from the shunt region drained into the juxtamedullary vein within the spinal canal, and formed a congestion of the venous circulation (Fig. ).\nDiagnosis was sAVF arising within the spinal lipoma, and embolization and possible surgery was scheduled. First, embolization was performed for the second lumbar artery, the feeding artery for the shunt. A xylocaine test at the feeding artery showed transient depression of MEP (as in Case 1) in the right leg muscles. However, we carried out embolization therapy as we judged the risk would be low as long as embolization was confined solely within the shunt. Because of the presence of multiple feeding arteries, the minor feeder was embolized with a coil first and then N-butyl-cyanoacrylate was injected through one of the main feeders into the shunt zone to occlude it. This did not achieve complete embolization, and surgical excision was deemed necessary. The shunt-containing lipoma was resected as far as possible. An abundance of blood vessels was confirmed within the lipoma. Intraoperative angiography confirmed there was no remaining shunt after the resection.\nAfter surgery, lower limb pain and muscle strength were improved on both sides. At 1month after surgery, lumbar JOA score was 14/29. At 1 year, angiographic examination showed no vascular abnormalities. Although the patient’s low back pain and leg numbness persisted, muscle strength and ADL score were improved. His lumbar JOA score was further improved to 17/29.