Management of severe scoliosis in patients with Turner's syndrome: A case series

The prevalence and treatment of severe scoliosis and other spinal anomalies in patients with Turner's syndrome (TS) is not well reported. This is the largest case series to date regarding the treatment course and outcomes of severely scoliotic TS patients. A retrospective chart review was performed to identify all patients with TS seen at a single center academic pediatric institution from 2007 to 2021. Of these, the presence of concomitant severe scoliosis or other spinal anomalies was determined, defined by a major coronal curve measuring 45° or greater. Demographic, clinical, surgical, and radiologic data was collected at both pre- and post-intervention time points. A retrospective chart review identified 306 patients with TS. Of those, six were identified to have severe scoliosis or other severe spinal anomalies requiring fusion. All four posterior spinal fusion (PSF) patients demonstrated improvement of their spinal curvature. One patient who electively pursued only bracing demonstrated minimal improvement and surgery was subsequently recommended, but not pursued. One patient expired from a pre-existing heart condition prior to intervention. All postoperative complications resolved with no further complications. The only brace-related complication was an allergic rash related to the brace material. All four patients who underwent PSF demonstrated significant improvement of their spinal curvature with few post-surgical complications. None of the patients in the bracing cohort demonstrated stabilization of their spinal curvature. Therefore, these data corroborate with prior studies, suggesting that operative management consisting of spinal fusion with instrumentation provides optimal clinical outcomes, compared to bracing only.