Posterior Vertebral Column Resection for the Treatment of Dystrophic Kyphosis Associated With Type-1 Neurofibromatosis

Abstract

Case report. To describe the use of posterior-only vertebral column resection and postoperative traction for spinal deformity associated with type-1 neurofibromatosis (NF1). Vertebral deformity, namely, thoracic scoliosis, is the predominant orthopedic manifestation of NF1. Patients may present with debilitating pain and rarely, myelopathy. The commonly dystrophic nature of these deformities makes them particularly recalcitrant to surgical correction. Traditionally, circumferential arthrodesis via combined anterior and posterior approaches has been recommended. Clinical and radiographical case review. A 14-year-old adolescent boy with NF1, severe cervicothoracic angular kyphosis, thoracic dislocation, and myelopathy presented status postmultiple anterior and posterior spinal fusions. The patient underwent posterior-only vertebral column resection after 6 weeks of halo-gravity traction. The surgery consisted of thoracic laminectomies, total corpectomies of T3 and T4, circumferential fusion, and posterior instrumentation from the occiput to T11. Autologous rib and iliac crest grafts were used as fusion substrate. Postoperatively, a halo vest was worn for 4 months to support the correction of his chin-on-chest deformity. The patient's neurological status returned to normal by 6 weeks postoperatively, and solid fusion was radiologically evident after 1 year. We think that posterior-only vertebral column resection represents a safe and efficacious but technically challenging option for the treatment of angular kyphotic spinal deformity and associated neurological deficit in patients with NF1.