✓ This 7-year-old boy with Hajdu—Cheney syndrome presented with cervical syringomyelia related to rapidly progressing platybasia. Decompressive craniectomy provided temporary improvement, and his clinical status was eventually stabilized after external immobilization, according to findings at 2.5 years of follow up. In a review of the literature the authors found 57 cases of the syndrome, only three of which were associated with syringomyelia. The youth of the patient, the severe form and rapid course of the disease, and the very specific anatomical conditions related to cranial and facial deformities raised various therapeutic problems.
Background/Objectives: Over the last 20 years, spinal cord stimulation (SCS) has seen the development of various paresthesia-free paradigms. Recently, a novel modality has emerged (Fast-Acting Sub-perception Therapy, FAST) that engages the surrounding inhibition mechanism of action. We evaluated long-term, real-world outcomes of preferential FAST-SCS use in patients with chronic pain. Methods: In this multi-center, observational, consecutive case series, medical chart data from chronic pain patients preferentially using FAST-SCS (no exclusions) were retrospectively reviewed. Results: Data from 167 patients in 13 European centers were analyzed; 74% of patients suffered from persistent spine pain syndrome type 2 and 87% presented with low back and/or leg pain. At the last follow-up (mean 1.6 years), the numerical rating scale (NRS) overall pain score decreased by 5.1 ± 2.5 points versus baseline, from 8.0 ± 1.2 to 2.9 ± 2.2 (n = 167, p < 0.0001). 87% of patients reported ≥50% pain relief, and 55% were “high responders” with overall NRS pain scores ≤2/10. At the last follow-up, functional disability improved significantly (the Oswestry Disability Index reduced by 29.2 ± 21.5 points, n = 65, p < 0.0001) and patients had a significant gain in quality of life (EQ-5D-5L visual analog scale increased by 52.0 ± 26.9 points, n = 86, p < 0.0001). Results at the 2-year follow-up showed a sustained, substantial reduction in pain; 67% of patients were high responders and the NRS overall pain score decreased by 5.6 ± 2.4 versus baseline (n = 52, p < 0.0001). Conclusions: Our real-world outcomes suggest that in patients with chronic low back and/or leg pain, FAST-SCS therapy provided durable and profound pain relief and led to significant improvements in disability and quality of life.