Surgical Treatment for Scoliosis in Marfan Syndrome

Purpose: To investigate 23 patients with Marfan syndrome who underwent posterior instrumented fusion for scoliosis and were reviewed at a 7-year follow-up period (max. 18 yrs).

Methods: From 1982 to 1995, 23 patients with scoliosis in Marfan syndrome aged 15 years on average (11 yrs to 18 yrs) were treated at our Department. The diagnosis of Marfan syndrome was made using the most recent Ghent criteria. Eight patients had a double major curve pattern, whereas 15 a single curve pattern. Six cases had a concomitant thoraco-lumbar kyphosis and 4 a thoracic hypokyphosis. Long posterior instrumented fusion was performed in all patients and included 12 levels on average. The Harrington rod instrumentation (single with compressor rod in 1 case, and double in the other one) was used in the first 2 cases (1982) and it was associated with sublaminar wires and compressor rod in another 15 patients (1983-1992), using in all cases prolonged postoperative cast immobilization (7 months on average). The remaining 6 patients (1993-1995) were instrumented with either Cotrel-Dubousset or Colorado instrumentation with thoracolumbar and lumbar pedicular screws (8 on average) and many thoracic hooks; a post-operative brace was worn for 5 months.

Results: At a minimum follow-up of 7 years (max. 18 yrs), all of the 23 patients were reviewed, analyzing the clinical conditions and the radiographs. The mean age was 22.2 years (19 yrs to 33 yrs). One patient (4.3%) has been operated on recently by cardio-surgeons. All complications were encountered in the 17 patients treated using the Harrington rod instrumentation (with or without sublaminar wires) : 2 pseudoarthroses (8.6% of the total 23 cases) that required salvage surgery consisting of a new instrumented posterior fusion; 4 (17.3%) distal hook dislodgements with 2 broken wires and loss of scoliosis correction at follow-up within 10º that did not require surgery, and finally 1 (4.3%) cervico-thoracic junctional kyphosis with onset 11 years after surgery. The average pre-operative scoliosis value of 79º (60º to 110º) was initially corrected to 41º (15º to 80º), averaged 45º (20º to 82º) 1 year after surgery, and was finally equal to 49º (20º to 95º) at last follow-up. No neurological complications were seen for any of the patients.

Conclusions: Patients with Marfan syndrome can achieve solid posterior fusion, but an extended arthrodesis, a secure segmental fixation using more recent instrumentations with pedicular screws and a careful pre-operative and follow-up evaluation are required, to reduce the high risk of complications reported in literature.