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Growing rods for infantile scoliosis in Marfan syndrome
Paul D Sponseller1, George H Thompson, Behrooz A Akbarnia
1Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland 21224, USA.
Insights
Growing rods effectively control infantile scoliosis in Marfan syndrome patients, offering significant curve correction and spinal lengthening. Dual rods showed superior results compared to single rods in this study.
Area of Science:
- Pediatric Orthopedics
- Spinal Surgery
- Genetics and Rare Diseases
Background:
- Infantile scoliosis in Marfan syndrome is typically progressive and resistant to bracing.
- Previous growing rod techniques showed limited success in managing this condition.
- Improved life expectancy in neonatal Marfan syndrome highlights the need for effective spinal deformity management.
Purpose of the Study:
- To assess the efficacy of a novel growing rod technique for infantile scoliosis in Marfan syndrome.
- To compare the corrective potential of single versus dual growing rod constructs.
- To evaluate the capacity of growing rods to achieve spinal lengthening and minimize trunk disproportion.
Main Methods:
- Retrospective review of ten patients with Marfan syndrome and early-onset scoliosis (<3 years).
- Treatment involved growing rods (3 single, 7 dual) with follow-up averaging 87 months.
- Pre-operative curves averaged 77.2 degrees; thoracolumbar kyphosis averaged 56 degrees.
Main Results:
- Overall curve correction averaged 51%, with dual rods achieving 60% correction versus 31% for single rods.
- Significant improvements were observed in coronal (56mm to 18mm) and sagittal (31mm to 21mm) imbalance.
- Mean spinal lengthening of 11.5 cm was achieved; complications included rod breakages and dural leaks.
Conclusions:
- Extensible spinal growing rods represent an effective treatment for spinal deformities in Marfan syndrome.
- Dual growing rod constructs offer superior curve correction compared to single rods.
- Growing rods facilitate substantial spinal growth, potentially preventing severe curves and allowing later definitive fusion.
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 degrees (SD, 15.6 degrees ) and the mean thoracolumbar kyphosis was 56 degrees (SD, 21 degrees ). 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.
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