Objective: To evaluate the early and midterm outcomes of surgical correction for severe dystrophic cervical kyphosis in patients with neurofibromatosis type 1 (NF-1) and analyze the pathomechanics and the influence on surgical efficacy of related systemic skeletal dystrophy.

Methods: Ten patients who underwent surgical correction for NF-1-related severe dystrophic cervical kyphosis were reviewed. Radiographic parameters, including local and global Cobb angle, sagittal vertical axis, and T-1 slope, were measured. The visual analog scale score, Japanese Orthopaedic Association score, Neck Disability Index, Patient Satisfaction Index, and complications were evaluated.

Results: The average follow-up was 50.6 months. The local and global Cobb angle improved from the preoperative average of 82.0° and 54.9° to an average of 35.6° and 29.8°, respectively, at the time of final follow-up. The C2-7 sagittal vertical axis averaged 5.8 mm before surgery and 8.9 mm at the final follow-up. The average T1 slope was -12.3° before surgery and -1.6° at the final follow-up. The visual analog scale score, Japanese Orthopaedic Association score, and Neck Disability Index improved significantly, and the overall satisfaction rate was 90.0%. One death and 4 instrumentation failures occurred, 3 patients showed progression of the kyphosis, and 2 fusion failures were observed.

Conclusions: Surgical correction, specifically the combined anteroposterior procedure, is essential and effective for management of NF-1-related severe dystrophic cervical kyphosis. However, high incidences of instrumentation failure, kyphosis progression, and fusion failure were observed. NF-1-related continuous skeletal dystrophy caused by multiple metabolic factors remarkably affected the midterm outcomes. Early prevention and targeted pharmacotherapy may be necessary.