Study design: Retrospective review of a case series.
Objective: To present the radiologic and surgical characteristics of scoliosis treatment in patients with Marfan syndrome (MFS).
Summary of background data: The treatment of scoliosis in MFS has been reported to pose unique challenges. However, the information on surgical outcomes is sparse. In clinical practice, surgery for scoliosis in MFS is reported to confer higher perioperative risks and instrumentation-related complications compared with adolescent idiopathic scoliosis because of atypical and rigid curve patterns and the underlying desmogenic disorder.
Methods: Database research identified 26 MFS patients treated surgically during 7 years at a single spine center. Three patients presented with previous failed surgeries and were excluded. The medical records, charts, and radiographs of 23 patients were analyzed focusing on curve characteristics, surgical outcomes including complications, and curve correction using modern third-generation hybrid or pedicle screw systems, and the behavior of junctional segments and compensatory curves.
Results: The sample included 18 female and 5 male patients with an average age of 18.2±9.2 years (13-52 y) at index surgery and 21.2±9.2 years (14-53 y) at follow-up, averaging 35.8±23.5 months (6-95 mo). According to the Lenke classification, 30% presented as type 1, 9% as type 2, 22% as type 3, 9% as type 4, 17% as type 5, and 13% of patients as type 6. Seventy-four percent of patients had a type C lumbar modifier. In total, 48% of patients underwent a posterior spinal fusion (PSF). Thirty percent had instrumented anterior spinal fusion (AISF), whereas 22% had a combined anterior release and staged PSF. Ninety-one percent of patients achieved solid fusion; there was 1 asymptomatic nonunion and 1 recalcitrant nonunion. Add-on phenomena were identified in 13% of patients (n=3) treated with AISF, indicating staged PSF once. In total, complications were encountered in 30% of patients, indicating redo surgery in 17% of patients. The cause for revision included nonunion (2x), liquor leakage (1x), and wound infection at the iliac crest (1x). We judged the outcome as excellent/good if the patient had no major redo surgery and was very satisfied/satisfied. Overall, excellent/good outcome was noted in 78% of the patients. Blood loss averaged 659 mL in AISF and 1748 mL in PSF. The surgical time was 193 minutes in AISF and 229 minutes in PSF. Preoperative, postoperative, and follow-up Cobb T4-T12 was 13, 13, and 16 degrees, respectively; the mean thoracic curves measured 66 (23-106), 36 (0-58), and 38 degrees (0-58), respectively. Lumbar curves measured 63 (23-110), 27 (0-80), and 24 degrees (0-68), respectively. Coronal plumb line measured 2.2, 2.6, and 1.2 cm, respectively, indicating good trunk balance in most patients. The flexibility rates of thoracic curves and lumbar curves were 38% and 47%, respectively. Thoracic curve correction in PSF and combined anterior release/PSF was 44%, and in AISF, it reached 57%.
Conclusions: The current study highlights the potential pitfalls in scoliosis surgery for patients with MFS. Surgery was performed using third-generation pedicle screw-based and hook-based systems for PSF and second-generation and third-generation implants for AISF. We illustrated that the treatment of scoliosis in MFS, taking into account the individual challenges of the underlying desmogenic disorder, can be performed with a moderately increased risk for surgical complications compared with adolescent idiopathic scoliosis.