A 78-year-old male presented with progressive myelopathic symptoms. The clinical course and imaging findings raised a high suspicion for venous hypertensive myelopathy due to a spinal dural arteriovenous fistula (SDAVF). Magnetic resonance angiography and four complete spinal angiograms did not reveal the presence of an SDAVF. Despite multiple negative angiograms, intraoperative ultrasound revealed abnormal cord edema and arterialized pulsatile vessels, confirming the presence of an SDAVF. The fistula was found and cauterized, which resulted in a decrease in the caliber of the dilated veins and an observed reduction of spinal cord stiffness posttreatment. The patient exhibited gradual improvement in neurological function. Retrospective analysis of the multiple complete spinal angiograms failed to reveal an anomaly at the treated level or any other level. This case underscores the diagnostic utility of intraoperative Doppler ultrasound and the importance of maintaining a high index of suspicion for SDAVF in cases with consistent clinical characteristics and a lack of alternative diagnoses, even with negative spinal angiography. https://thejns.org/doi/10.3171/CASE24438.
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