The surgical treatment of scoliosis in neurofibromatosis Ⅰ / 中华骨科杂志

ABSTRACT

Objective To investigate the surgical management of scoliosis in neurofibromatosis Ⅰ type. Methods Since 1983, the results of surgical intervention of 31 consecutive patients for scoliosis in neurofibromatosis were reviewed. There were 19 males and 12 females. The mean age of the patients was 15.4 years (range, 5 to 25 years). The type of curve in scoliosis included thoracic curve in 23 patients, lumbar curve in 3 and thoracolumbar curve in 5. There were 6 cases of thoracic curves associated with kyphosis. The mean Cobb angle of scoliosis was 85.9? ranging from 45? to 145?, and the mean Cobb angle of kyphosis was 81.3? ranging from 43? to 120? before operation. The surgical methods included posterior operation in 17 patients, anterior operation in 2, and combination of anterior and posterior in 12. The instrumentation used for internal fixation included Harrington rod in 7 patients,Luque rod in 3, combination of Harrington and Luque rod in 5 patients, CD in 4 patients, CDH in 3 patients,TSRH in 7, and PRSS in 2, 10 cases of whom only single rod fixation were used. Results The patients were followed up for an average of 9.4 years (range, 1-18 years). The correction rate of Cobb angle in scoliosis was 33.4% in scoliosis, and 39.6% in kyphosis postoperatively. The complications of the group consisted of Luque rod breakage in 1 patient, Harrington rod breakage in 1, Harrington rod and Luque wire breakage in 1,nonunion of bone graft and pseudarthrosis in 4, curve aggravation in 1. One patient underwent revision for three times because of recurrent hook dislodgement. Conclusion Scoliosis in neurofibromatosis should be operated early, sufficient bone graft and the solid instrumentation for internal fixation are required.