Single posterior skip instrumented fusion for the treatment of dystrophic type-1 neurofibromatosis scoliosis: A case report and review of the literature

Tai-Jung Hsieh ； Shih-Chieh Yang ； Yu-Hsien Kao ； Yuan-Kun Tu

《Formosan Journal of Musculoskeletal Disorders》 10卷2期 (2019/05) Pp. 83-88

https://doi.org/10.6492/FJMD.201905_10(2).0007

摘要

We report a 14-year-old adolescent boy with type-1 neurofibromatosis (NF1), severe thoracolumbar dystrophic kyphoscoliosis, and radiculopathy. Vertebral deformity, namely, thoracolumbar scoliosis, is the predominant orthopedic manifestation of NF1. Patients may present with debilitating pain and, rarely, myelopathy. The commonly dystrophic nature of these deformities makes them particularly recalcitrant to surgical correction. Traditionally, circumferential arthrodesis through combined anterior and posterior approaches has been recommended. This patient underwent skip posterior instrumentation and tumor debulking surgery. Local autograft mixed with artificial bone graft was used as fusion substrate. After the operation, the center of gravity was shifted back to the midline. The patient could walk without aid the day after surgery. Solid fusion was radiologically evident after 6 months. We suggest that skip posterior instrumentation and tumor debulking surgery represents a safer, but efficacious method for the treatment of dystrophic scoliosis spinal deformity in patients with NF1.

關鍵字

kyphoscoliosis ； neurofibromatosis ； spinal deformity