Clinical and Radiographic Outcomes Following Complex Spine Reconstruction in Neurofibromatosis with Severe Spinal...

Area of Science:

• Orthopedic Surgery
• Spine Surgery
• Neurofibromatosis Research

Background:

• Neurofibromatosis (NF) associated spinal deformities are uncommon but can be debilitating.
• Surgical correction of severe spinal deformities in NF patients presents significant challenges and high complication rates.
• Vertebral column resections (VCR) are complex procedures for extreme spinal deformities.

Purpose of the Study:

• To evaluate the outcomes of complex spine reconstruction in Neurofibromatosis (NF) patients with severe spinal deformities (>100° or treated with VCR).
• To assess the complication rates associated with these complex surgical procedures.
• To analyze the impact of surgery on functional outcomes and quality of life using SRS-22 scores.

Main Methods:

• A multi-center study enrolled twelve consecutive NF patients with severe spinal deformities (>100° or VCR).
• Data collected included demographics, pre-operative, intra-operative, and post-operative complications.
• Scoliosis Research Society (SRS)-22 scores were used to compare functional outcomes at 2-year follow-up.

Main Results:

• Complex reconstruction significantly improved coronal and sagittal Cobb angles (mean reduction from ~100° to ~45° and ~63° respectively).
• Postoperative complications were low, including 8.3% new neurological deficit (recovered within a year) and 16.7% pulmonary complications.
• Health-related quality of life, measured by SRS-22 scores, showed significant improvement at 2-year follow-up.

Conclusions:

• Complex spine reconstruction effectively corrects severe NF-associated spinal deformities.
• The surgical approach demonstrated a favorable safety profile with low complication rates.
• Significant improvements in functional outcomes and quality of life were observed post-surgery.