Surgical treatment of scoliosis in patients with Marfan syndrome

ZHONG Zhao-ming; LUO Shi-gan; WANG Ji-xing; QU Dong-bin; JIANG Hui; CHEN Jian-ting

doi:10.3969/j.issn.2095-252X.2017.10.008

Abstract：Objective To explore surgical results of scoliosis in Marfan syndrome. Methods Eight consecutive patients with Marfan syndrome, including 2 males and 6 females, were treated from January 2010 to January 2013 for scoliosis by a posterior instrumented fusion with pedicle screws. The mean age at surgery was 14.3 years ( range: 11.5 - 18 years ). Surgical results and complications were retrospectively analyzed. Results All patients were followed up for a mean period of 42.2 months ( range: 22 - 65 months ). The average operation time was 365 min ( range: 300 - 540 min ). The average blood loss was 1175 ml ( range: 500 - 3000 ml ). The preoperative Cobb's angle averaged 76.8° ( range: 53° - 120° ). The average Cobb's angle was corrected to 18.6° ( range: 3° - 73° ) with a correction rate of 79.9% ( range: 39.2% - 94.3% ) immediately after the surgery, and 23.6° ( range: 4° - 77° ) with a correction rate of 72.2% ( range: 34.4% - 92.5% ) at the final follow-up. The average correction loss at follow-up was 5° ( range: 0° - 33° ). No neurological problem, pseudarthrosis or failure of instrumentation was found. Late wound infection occurred in 1 case in 15 months postoperatively. Instrumentation removal, debridement plus drainage were applied, and the infection was finally controlled. Conclusions Posterior pedicle screw instrumentation plus fusion is effective and safe for the treatment of scoliosis in patients with Marfan syndrome.