Growing rods for infantile scoliosis in marfan syndrome

Study Design. Retrospective review. Objective. To evaluate the effectiveness of a new growing rod technique in controlling infantile scoliosis in patients with Marfan syndrome. Summary of Background Data. Infantile scoliosis in patients with Marfan syndrome is nearly always progressive and poorly controlled by bracing, yet previous studies have shown poor Results with first-generation extensible spinal rod techniques. Methods. Ten patients with Marfan syndrome and scoliosis developing before 3 years of age were treated with growing rods (3 single, 7 dual). Mean age at initial surgery was 5.3 years (SD, 2.7 years). Before surgery, the mean curve was 77.2° (SD, 15.6°) and the mean thoracolumbar kyphosis was 56° (SD, 21°). Patients on warfarin sodium were lengthened at yearly intervals; others, more frequently. Mean follow-up was 87 months (SD, 30.5 months). Results. Mean curve correction was 51% (SD, 23%) overall, 31% (SD, 23%) for single rods, and 60% (SD, 19%) for dual rods. Mean coronal and sagittal imbalance improved from 56 to 18 mm and from 31 to 21 mm, respectively. The mean length obtained was 11.5 cm (SD, 3.6 cm) overall and 11.2 cm (SD, 3.60 cm) for the 5 patients with final fusion. Complications included 2 rod breakages and 3 intraoperative dural leaks. There was 1 anchor dislodgement and no postoperative dural leak. No patient developed clinically noteworthy junctional kyphosis. One patient died of unrelated causes 3 months after surgery. Conclusion. As life expectancy improves for patients with neonatal Marfan syndrome, spinal deformity becomes an important issue. Extensible spinal growing rods are an effective solution to the problem. Dual rods appear to be more corrective than single rods. Substantial spinal length can be obtained to minimize trunk disproportion. Growing rods may help prevent large infantile curves from becoming severe in Marfan syndrome, allowing definitive spinal fusion closer to skeletal maturity.