Surgical treatment for scoliosis in Marfan syndrome

Review of results of patients with Marfan syndrome treated with instrumented posterior fusion alone for scoliosis.

To analyze the results of surgical treatment for scoliosis in Marfan syndrome.

Few studies have been reported in the literature on surgical treatment for scoliosis in Marfan syndrome, analyzing long-term results of posterior instrumented fusion.

Twenty-three patients with Marfan syndrome with a mean age of 17 years (range, 11-31 years) were treated surgically from 1982 to 1995 for scoliosis, using a posterior instrumented fusion alone (Harrington rod with sublaminar wires in the first 16 cases, and a more recent hybrid instrumentation in the remaining 7 cases). All of the patients received a long posterior instrumented fusion, including 12.3 levels on average (range, 9-17), extending the fusion area to vertebrae that were neutral and stable in both coronal and sagittal planes before surgery. Patients were analyzed as two different groups (Group 1 and Group 2) according to the different posterior instrumentations employed: Group 1 included 16 patients treated by the Harrington distraction rod technique with sublaminar wires, while Group 2 included 7 patients treated using more recent hybrid instrumentations. Presentation features, complications, and results were analyzed.

At a minimum follow-up of 7 years (maximum, 18 years), all 23 patients were reviewed. The mean age was 26.8 years (range, 20-38 years). The average preoperative scoliosis value of 69.91 degrees was initially corrected to 38.17 degrees, averaged 40.89 degrees 1 year after surgery, and was finally equal to 44.09 degrees at the last follow-up. Differences in terms of scoliosis correction achieved with different instrumentations (Groups 1 and 2) did not reach statistical significance. In Group 2 patients, the percentage of postoperative correction was slightly lower (44.23%) than that of Group 1 (46.55%) but remained more stable at the last follow-up (40.97% vs. 36.38% of Group 1). There were 11 complications in 10 of the 23 patients (43.4%); two complications occurred in 1 patient. Intraoperatively, dural tears occurred in 2 cases (8.6%). Pseudarthrosis with instrumentation failure in 2 cases (8.6%) required revision surgery. Five (21.7%) distal hook dislodgements with moderate loss of scoliosis correction, 1 (4.3%) mild loss of correction without instrumentation failure, and 1 asymptomatic cervicothoracic junctional kyphosis. did not require surgery. All complications occurred among the 16 Group 1 patients, treated using the Harrington rod instrumentation with sublaminar wires.

These results seemed to demonstrate that a satisfactory stabilization of scoliosis can be achieved by posterior instrumentation alone in patients with Marfan syndrome. Instrumented posterior fusion should be extended to include vertebrae that are neutral and stable in both coronal and sagittal planes before surgery, in order to ensure stabilization of the deformity and reduce the risks of decompensation of the spine.