Spine deformity correction in Marfan syndrome

Study Design. Retrospective review of records and radiographs with postoperative questionnaire of patients with Marfan syndrome receiving surgical correction for spinal deformity. Objective. To characterize and analyze the challenges of surgical correction for spinal deformity in Marfan syndrome and to present recommendations that may reduce morbidity and increase success for these procedures. Summary of Background Data. Spinal deformities are common in Marfan syndrome and usually refractory to conservative management. The few, smaller studies of surgical intervention reported on earlier surgical techniques but suggested increased complication rates. Methods. Records and radiographs of 39 patients with confirmed Marfan syndrome who underwent surgical management at either of two institutions for primary scoliosis (n = 26), kyphosis (n = 7), or deformity secondary to previous surgery elsewhere (n = 6) were reviewed. Presentation features, complications, and therapeutic results were analyzed. Low back outcome scores were generated from a questionnaire given to patients after surgery. Results. Increased blood loss and rates of infection (10%), dural tear (8%), instrumentation fixation failure (21%), pseudarthrosis (10%), and coronal (8%) and sagittal (21%) curve decompensation were noted. Infection was often associated with dural tear and decompensation with extreme correction. One patient died of valvular insufficiency 11 weeks after surgery. Conclusion. The cardiopulmonary condition of patients with Marfan syndrome should be evaluated and planned for before surgery. Preoperative computed tomograph to assess bony adequacy for fixation and magnetic resonance imaging to evaluate dural ectasia are indicated. Attention paid to the sagittal profile, extension of fusion to vertebrae neutral and stable in both planes, minimization of soft tissue dissection, and avoidance of extreme correction may prevent curve decompensation.