Surgical correction of severe dystrophic neurofibromatosis scoliosis: an experience of 32 cases

The surgical management of severe rigid dystrophic neurofibromatosis curves is a demanding procedure with uncertain results. Several difficulties are present in such patients including a poor bone stock, sharp angulation of these dystrophic curves and dural thinning or ectasia. The aim of this work was to review the clinical and radiographic outcomes of three-dimensional correction of severe rigid neurofibromatosis curves analyzing its efficacy, safety and possible complications. Thirty-two patients were followed up for an average of 6.5 years (range 3–9 years). The average age at surgery was 14 years (range 11–19 years). All patients had typical dystrophic curves, and the apex of the deformity was thoracic (n = 13), thoracolumbar (n = 14) and lumbar (n = 5). All patients had a two-staged procedure; an anterior release followed latter by posterior hybrid instrumentation augmented by sublaminar wires. Two wires were usually placed immediately below the proximal anchor, and several sublaminar wires were always passed at the apex of the deformity. There were a total of 142 wires with an average of 6.5 wires/patient (range 5–8 wires). The mean preoperative Cobb angle of the scoliotic curve was 102.2° (range 71°–114°) corrected to an average of 39° (range 16°–49°), and the loss of correction had an average of 4°. The mean preoperative sagittal plane deformity was 49° corrected by an average of 61%, and rotation was corrected by an average of 34%. There were no dural tears during passage of the sublaminar wires, no implant-related complications and no permanent neurologic deficits. The use of extensive and vigorous anterior release with posterior hybrid instrumentation has proved useful and effective in the treatment of these severe deformities; sublaminar wires allow safe gradual correction and even distribution of forces over multiple anchor points improving the correction achieved and decreasing implant-related complications.