Results of operative treatment for scoliosis in Marfan syndrome

Background. Scoliosis in patients with Marfan syndrome often has atypical shape and may be accompanied by disturbed sagittal profile of spine. Conservative treatment is unsuccessful, so surgery is treatment of choice. The goal of this study is a retrospective analysis of treatment results of patients with Marfan sydrome, operated in one hospital between 1987 and 2006. Material. Material consists of 15 patients, 7 females and 8 males with Marfan syndrome and scoliosis. Mean age at surgery was 15.3 years. The mean follow up period was 4.7 years. Methods. Radiographic data before surgery, after surgery and after follow up period were analyzed. The type of surgery and intra- and post-operative complications were assessed. Anterior fusion was performed in 3 cases, posterior fusion – in 9 cases, combined anterior release and posterior fusion was performed in 3 cases. The mean extent of fusion was 10.9 segments. Results. The mean preoperative thoracic and lumbar curve were 80.3o and 66.4o respectively. The mean postoperative thoracic and lumbar curve improved to 47o and 40.2 o, after follow up they equaled 56o and 56,7o. Mean final correction was 41.1% in thoracic spine and 46.5% in lumbar spine. Before surgery, the mean thoracic kyphosis was 29.1o, and lordosis was 39.9o. After surgery mean thoracic kyphosis was 28,0o and at control examination: 38.4o. Mean lordosis was 35.1o after surgery and 37.1o during control examination. After follow up period, coronal imbalance was in 4 cases, and sagittal imbalance – in 5 cases. In 2 cases progression of sagittal imbalance was observed after implants removal. Conclusions. Correction achieved in Marfan syndrome is smaller than in patients with idiopathic scoliosis, and operative treatment in connected with increased risk of decompensation. Saggittal imbalance connected with hyperkyphosis and flat back has essential influence on final result of scoliosis surgery. Implants removal in these patients may cause progression of decompensation.