Abstract
PURPOSE: Spinal deformity in neurofibromatosis, when associated with dystrophic change, is a major treatment challenge. Early anterior and posterior spinal fusion is the accepted treatment plan for severe progressive kyphoscoliosis. Resection of the vertebral body as an additional step in correction and fusion has not been previously investigated. Information on the functional status of the neurofibromatous curves is also scarce in the literature. The purpose of this study is to report an experience with corpectomy and circumferential fusion in dystrophic spines of neurofibromatosis. METHODS: Among 16 patients with dystrophic spinal curves, nine cases who had undergone anterior and posterior fusion with 6.7 years (range 2-11.9) average follow-up were evaluated clinically, radiographically, and functionally (SRS-22). RESULTS: Nine cases with a mean age of 11.8 years (range 7.8-17) at surgery consisted of seven kyphoscoliotic patients, who had received one or two levels of cord decompression and corpectomy, and two cases of scoliosis with hypokyphosis. Surgery improved the mean preoperative scoliosis of 87 (range 60-110) and local kyphosis of 69.3° (range 50-100) to 49 (range 15-85) and 49° (range 35-70), respectively. Loss of correction of 5° in the scoliosis and 13° in the kyphosis angles was observed in the final follow-up. One surgery resulted in an immediate postoperative mortality. Fusion was achieved in all cases. The average SRS-22 score of 4.1 (range 3-4.6) was obtained. CONCLUSIONS: Simultaneous anterior and posterior fusion with corpectomy for dystrophic neurofibromatous spinal deformities is associated with a high fusion rate, good correction, and also good functional outcome.