Vertebral column resection (VCR) at the subapical vertebra for correction of angular kyphosis associated with neurofibromatosis type 1(NF1): a case report

Purpose To describe the process and outcome of vertebral column resection (VCR) at the subapical vertebra for correction of angular kyphosis associated with neurofibromatosis type 1(NF1). Methods A review and summary of the medical history, radiographs, operative procedure, and complications of a 16-year-old male presenting with severe angular kyphosis associated with NF1 with dyspnea. Results A 16-year-old male presented with severe angular kyphosis associated with NF1 with dyspnea. Preoperative radiographs demonstrated multiple vertebrae were rotated in the vicinity of the apical vertebra, with a wedge-shaped deformity, dysplasia, T10-T12 kyphotic angle of 160 degrees, and T2-L2 kyphotic angle of 95 degrees. VCR at the L1 vertebra (distal end of the apical vertebra) with bone grafting and internal fixation was performed. Postoperative imaging revealed that the T2-L2 Cobb angle was 20 degrees, denoting a correction rate of 79%. The patient's height increased from 130 to 150 cm. The position of internal fixation was not displaced, and the correction angle was maintained at 2-year follow-up. Conclusions The novel strategy of performing VCR at the subapical vertebra, with posterior displacement of the distal end, and remodeling of the spinal canal is potentially a safe and efficacious option to correct sharp angular kyphosis.

Automated summary

This study describes a novel strategy for correcting angular kyphosis associated with neurofibromatosis type 1 (NF1) by performing vertebral column resection (VCR) at the subapical vertebra, with posterior displacement of the distal end and remodeling of the spinal canal. The surgery resulted in significant correction of the kyphosis, which was maintained at the 2-year follow-up.