Utility of the spinal instability neoplastic score to identify patients with Gorham-Stout disease requiring spine surgery

Abstract

Background: Gorham-Stout disease (GSD) is a rare syndrome presenting with progressive osteolysis which in the spine can lead to cord injury, instability, and deformity. Here, the early spine surgery may prevent catastrophic outcomes. Case Description: A 25-year-old male with GSD involving the T2 to T6 levels presented with acute traumatic kyphoscoliosis at T3 and T4 and left lower extremity paraparesis. The CT scan 4 weeks before this showed progressing osteolysis versus the CT 5 years ago. Unfortunately, the patient underwent delayed treatment resulting in permanent neurological sequelae. Surgery included a laminectomy and vertebrectomy of T3/T4 with instrumented fusion from T1-10. The use of the spinal instability neoplastic score (SINS) is a useful tool to prompt early referral to spine surgeons. Conclusion: We recommend using the SINS score in GSD patients who develop spinal lesions to prompt early referral for consideration of surgery.