A 30-year-old female presented with a 10-year history of pain in the lumbosacral spine; she had had casual radiation to both lower limbs. After her first delivery, she developed back pain. The MRI demonstrated a tethered cord at the L4 level and a filum terminale lipoma. The MRI also showed tortuous veins on the spinal cord surface (Figure ). Nine years later, during the second pregnancy, she noted weakness and sensory loss, imbalance with urinary and fecal incontinence. On admission, she had bilateral plantar flexion weakness (grade 4 according to modified Medical Research Council system) and reduced sensation in the gluteal regions and legs, and plantar response. The Babinski and Rossolimo signs were present bilaterally. A repeat MRI revealed intraspinal T2 hyperintensive changes in the thoracic spine and conus (Figures ). Those changes were consistent with neurologic deficit and, after exclusion of inflammatory demyelinating diseases, based on a brain MRI and an aquaporin-4 antibody test, diagnosed as myelopathy. Due to described torturous veins on the spinal cord surface (Figure ), the patient underwent spinal digital subtraction angiography (DSA). The range of DSA was from the Th6 level to the coccygeal artery. The results did not reveal any vascular malformation. Therefore, the preliminary diagnosis was symptomatic TCS and filum terminale lipoma. In view of weakness and neurological deficit surgical spinal cord de-tethering, without lipoma resection was planned.\nThe sacral canal was opened with a median incision. The dura was thin and transparent. After a midline dura and arachnoid incision, a tumor was visualized that appeared to be a lipoma. It engulfed the filum terminale and spinal nerve roots caudally, to the S2 level. The S1 and S2 nerve roots were positioned lateral to the tumor. A thickened, tortuous, bright-red vessel was also noted on the filum terminale, running in the cranial direction. The external surface of the dura was explored bilaterally at the sacral level in search for a SDAVF. A clearly visible nidus was identified at the S3 level on the left side. An empirical test was performed to support this finding. A clip was placed on the arterialized vein, intradurally, for a few minutes. Then, the vessel on the filum terminale clearly changed color, from bright red into bluish gray, and its tension decreased. The SDAVF was then intradurally disconnected, and the nidus was extradurally coagulated. In addition, the filum terminale was cut, and samples of lipoma were taken for histology. The dura was sutured in a watertight fashion, and the wound was closed in layers.\nThe postoperative course was uneventful. A few days later, the patient was discharged. A follow-up MRI showed resolution of the myelopathic changes (Figures –). At the 1-year follow-up, the patient had considerably improved, with no incontinence or motor deficit.
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).