Vertebral Column Subluxation in Neurofibromatosis Type 1-Associated Dystrophic Scoliosis: A Report of Two Cases and Narrative Review.

Abstract

Background and objective: Neurofibromatosis-1 (NF1) dystrophic scoliosis is a challenging disease to manage surgically, with multiplanar curves progressing rapidly and unpredictably. Conservative management with bracing is often unsuccessful, and many patients necessitate instrumented fusion to halt progression of their curves. In rare cases, patients can present with spontaneous vertebral subluxation, significantly complicating the surgical management of this already complex disease process. The objective here was to describe 2 cases of vertebral subluxation in NF1-associated dystrophic scoliosis along with their surgical corrections and clinical courses.

Methods: A retrospective review of 2 cases at the authors' institution was performed to describe their preoperative symptom complexes, surgical corrections, and postoperative courses. A narrative review of the literature surrounding NF1-associated dystrophic scoliosis and subluxation is also presented.

Results: Two cases of vertebral subluxation at T4-5 and C7-T1 are presented. Both patients had significant dystrophic features throughout their spines, and halo-gravity traction was unsuccessful in 1 patient and led to vertebral and subclavian artery injuries in the other. One patient underwent an uncomplicated deformity correction with partial vertebral column resection to facilitate his deformity correction. The other patient, after her vascular injuries, ultimately suffered a spinal cord injury after a fall and underwent emergent instrumentation, decompression, and partial vertebral column resection at the site of subluxation, with improvement in her neurological function afterward.

Conclusion: Dystrophic scoliosis in NF1 remains a difficult disease to treat, and deformity correction in patients with subluxation is particularly complex. These cases here highlight the unpredictability and possible complications of halo-gravity traction, need for good fixation to facilitate subluxation reduction, high chance of hardware complications and proximal or distal failure, and importance of cooperative management of these patients in conjunction with other surgical services.