Correction of Severe Dystrophic Scoliosis in Neurofibromatosis 1 With Posterior Vertebral Column Resection in Multiple Levels

Abstract

Objective To evaluate the results after multiple posterior vertebral column resection (PVCR) in patients with severe kyphoscoliosis secondary to neurofibromatosis type 1 (NF-1). Methods Retrospective study of 4 adult male patients, mean age of 26.5 years, with severe rigid deformity, mean kyphosis of 122º and scoliosis of 88º, through clinical analysis and complementary exams. Results The mean postoperative follow-up was 27 months. Three vertebrae were resected on average. Interbody consolidation of arthrodesis was confirmed by CT in all cases. The correction rates of sagittal and coronal deformities were 54.09% and 70.45%, respectively. Prior to surgery, all patients were neurologically intact and as a post-surgical complication one patient had motor deficit (ASIA D) and pneumothorax also occurred in two patients. Conclusions The correction of severe kyphoscoliosis in adult patients with NF-1 is technically demanding and is not free of major complications. However, in view of the limited possibility of correction of these deformities, multiple-level PVCR has proved to be a safe and effective technique. Level of evidence IV; Case Series.