Operative treatment of spinal deformities in patients with neurofibromatosis

Introduction. Neurofibromatosis is a hereditary disorder, in which scoliosis is the most common orthopedic problem. The goal of this study is a retrospective analysis of results of scoliosis surgery in patients with neurofibromatosis, depending on the type of spinal deformity, and with assessment of spinal balance both in coronal and sagittal planes. Material consists of 28 consecutive patients with von Recklinghausen disease, treated surgically due to scoliosis between 1986 and 2008. Dystrophic scoliosis was observed in 21 cases, “normal” scoliosis – in 7. At the surgery, average patients’ age was 13.5 yrs. The mean follow up period was 5.4yrs. Methods. Radiologic data before and after treatment were analyzed as well as at last control examination. Type of surgery and intra- and postoperative complications were noted. Posterior fusion was performed in 11 patients, anterior fusion (with or without instrumentation) and posterior fusion with implants in second stage – 8 cases, anterior release + posterior fusion (one operation) – 8 patients, anterior fusion with implants – 1 case. Results. Prior to surgery, average Cobb angle was 85.3o, and it decreased postoperatively to 53.8o. At follow up mean Cobb angle was 60.8o, so mean final correction was 28.2%. Preoperatively, the mean kyphosis angle was 63o and lordosis angle was 41o. After surgery kyphosis equaled 56.4o and after follow up period 68.9o. After surgery the mean lumbar lordosis was 39.7o and 48o at last examination. Before surgery mean coronal balance was 5.3mm, after treatment -3.3mm, and after follow up -11.3mm. Sagittal imbalance was reported in 16 cases before treatment, in 10 cases after surgery and in 13 cases after follow up. Conclusion. Correction achieved in scoliosis with neurofibromatosis is smaller than in idiopathic scoliosis, and surgery is connected with greater risk of loss of correction. Correction of pre-existing sagittal imbalance is very difficult. Wide anterior release and posterior fusion seems to be the best surgical solution.