脊椎および脳神経外科ジャーナル

Respiratory Arrest in a Patient with severe Cervical Dystrophic Kyphosis Secondary to Neurofibromatosis Type 1: A Case Report

Kudo D, Miyakoshi N, Abe E, Kobayashi T, Hongo M, Kasukawa Y, Ishikawa Y and Shimada Y

Background: Cases of severe cervical kyphosis due to neurofibromatosis type 1 (NF1) and requiring tracheal intubation are rare. Dystrophic-type NF1 needs a combination of anterior and posterior spinal fusion, and use of sublaminar tape with rods appears reasonable and safe for posterior corrective spinal surgery in patients with this pathology. Case presentation: A 61-year-old woman with severe cervical kyphosis of 139 presented with sudden respiratory arrest before planned spinal surgery. Two-stage posterior and anterior corrective instrumented fusion using multiple segmental sublaminar tape and rods was performed under spinal cord monitoring. Cervical kyphosis was corrected from 104° to 83° and upper thoracic lordosis was corrected from 70° to 37°. Finally, she underwent additional posterior C1-C2 fusion with transarticular screwing for postoperative atlantoaxial subluxation. Solid bone fusion was demonstrated on 2-year follow-up Computed tomography, correction was maintained for 4 years after first surgery, and respiratory function was improved. Conclusion: Rib cage hypoplasia with upper thoracic lordosis secondary to severe cervical kyphosis can lead to acute-on-chronic respiratory failure. Planned corrective posterior spinal fusion with sublaminar tape, followed by anterior spinal fusion with a fibular strut bone resulted in successful solid bone fusion and improvement of respiratory function.