Treatment of Spinal Deformities with Neurofibromatosis

Chang, Bong Soon; Hwang, Il Ung; Han, Il Kyu; Kim, Dong Han; Lee, Choon Ki

Abstract

STUDY DESIGN: A retrospective study.
OBJECTIVES
By analyzing the patients of neurofibromatosis with spinal deformities, to identify presence of dystrophic changes, progression of deformity and associated factors, and treatment results according for sagittal curve pattern and operative methods. SUMMARY OF LITERATURE REVIEW: A single thoracic curve involving four, five, or six vertebrae is recognized as the most common pattern. Risk factors for progression of curve were anterior vertebral scalloping, particularly in younger patients, three or more penciled ribs, abnormal kyphosis, etc. It has been stated that the most effective management for dystrophic curves is early and aggressive surgery.
MATERIALS AND METHODS
Thirty nine patients with neurofibromatosis and spinal deformities were reviewed with chart and radi-ographic review from 1977 to 1999.
RESULTS
Four of thirty nine patients were nondystrophic type, and all patients were treated nonoperatively. Thirty five of thirty nine patients were dystrophic type, and twenty seven patients were treated operatively. Eight of these patients had been in progress till operation with 7.9 degrees/year progression rate, and their commonest pattern of deformity is a single curve in lower thoracic area with dystrophic changes such as vertebral scalloping, wedging, pencilling of average four ribs, particularly. Forty three percent of dystrophic type has sagittal plane deformities. The pedicle screw system was most excellent among the instrumentations. The complications of surgery were 6 progression of curve, 2 metal failure. Reoperation was done in 5 of 27 operated patients.
CONCLUSIONS
Nondystrophic type had good results with nonoperative treatment, but dystrophic type mostly required surgical intervention and had rapid progression. The treatment should be done by rigid fixation after considering sagittal plane deformi-ties and long term follow-up was needed for progressions of curve.