Abstract
BACKGROUND: A sharply angular thoracic deformity, commonly seen in dystrophic scoliosis associated with neurofibromatosis type 1, can compress the spinal cord and potentially cause neurological impairment. However, managing paraplegia due to severe kyphoscoliosis associated with neurofibromatosis type 1, coupled with low body mass index and extremely severe kyphoscoliosis, presents a significant challenge. CASE PRESENTATION: A 13-year-old girl of Mongolian ethnicity with severe dystrophic kyphoscoliosis associated with neurofibromatosis type 1 presented with paraplegia and dyspnea. Preoperative radiograph imaging revealed the presence of a thoracic kyphosis and scoliosis, with a Cobb angle of 150° and 130°, respectively. A two-stage strategy was devised, comprising halo-pelvic traction and spinal fusion with pedicle screws. The neurological deficit showed gradual improvement and ultimately complete recovery during the distraction phase. The curve decreased to an acceptable level, and posterior pedicle screws were implanted and fused without osteotomy. Postoperatively, the hunchback was no longer visible. There were no complications associated with halo-pelvic traction. At the 3-year follow-up, the correction angle and trunk balance were well maintained. CONCLUSION: It is possible that neurological deficit resulting from severe scoliosis may be reversed following the correction of the spinal curvature. The application of halo-pelvic traction generates substantial corrective forces, facilitating the correction of severe spinal deformities in a gradual and secure manner. A two-stage treatment strategy for patients with severe kyphoscoliosis in neurofibromatosis type 1 may offer an alternative approach to correcting the severe curve while avoiding the potential complications associated with a rapid, one-stage correction.