Surgical Treatment of Dystrophic Scoliosis in Neurofibromatosis Type 1

Abstract

STUDY DESIGN: This is a retrospective cohort study. OBJECTIVE: To investigate surgical outcomes and instrumentation-related complications (IRCs) of dystrophic scoliosis associated with neurofibromatosis type 1 (NF-1). SUMMARY OF BACKGROUND DATA: Surgical management, including the growing rod technique and early definitive fusion, has been recommended to avoid progression of NF-1 scoliosis. However, no study has investigated the outcomes and complications of different surgical interventions. MATERIALS AND METHODS: We performed a retrospective review of a cohort of 59 patients diagnosed with NF-1 dystrophic scoliosis and treated surgically. All clinical and radiographic data within a 3-year follow-up period were collected. The patients were divided into 2 groups according to the surgical procedure used: those who underwent initial fusion surgery were assigned to group A (n=32) and those who underwent growing rod surgery to group B (n=27). RESULTS: Patients in group A were older than those in group B at the initial surgery (10.4 vs. 5.8 y; P<0.001). There was no difference in the sex ratio, preoperative Cobb angle, or preoperative kyphosis angle between the 2 groups (P>0.05). The correction rate of the main curve Cobb angle was higher in group A than B (55.1% vs. 42.4%; P<0.05). The incidence of IRC was higher in group B than A (48.1% vs. 12.5%; P<0.05). Complications in group A comprised 1 case of screw pullout, 1 case of rod breakage, 1 case of adding-on phenomenon, and 1 case of proximal junctional kyphosis. Complications in group B comprised 5 cases of adding-on phenomenon, 4 cases of trunk shift, 3 cases of curve progression, 1 case of rod breakage, and 1 case of cap loosening. CONCLUSIONS: The use of growing rod effectively controls the spinal deformity and facilitates growth of the spine. Compared with fusion surgery, however, growing rod surgery yields a higher incidence of IRCs and lower corrective rate for scoliosis associated with NF-1.