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Spinal fusion in Duchenne muscular dystrophy
F Miller1, C F Moseley, J Koreska
1Department of Orthopaedics, Alfred I. duPont Institute, Wilmington, DE 19899.
Abstract:
Sixty-eight patients from a clinical population of 183 patients with Duchenne muscular dystrophy underwent posterior spinal fusion with instrumentation for progressive spinal deformity. Pulmonary complications were the most common postoperative problem, occurring in 17 per cent of the patients. A 35 per cent normal forced vital capacity was a reliable indicator of pulmonary complication risk. The patients with surgically stabilized spines were more comfortable in the later years of life and easier to care for, but deteriorating pulmonary function was not affected by the spinal fusion. The average age at death for the 29 boys who underwent spinal fusion was 18.3 years, which was similar to that of the 58 boys with scoliosis. Factors that improved the patients' quality of life included segmental instrumentation, fusion from T2 to the pelvis, correcting or balancing scoliosis, creating normal sagittal plane alignment and correcting pelvic obliquity.
Insights
Posterior spinal fusion in Duchenne muscular dystrophy (DMD) patients improves comfort and care but does not halt pulmonary decline. Preoperative lung function is key for predicting postoperative complications in DMD scoliosis surgery.
Area of Science:
- Orthopedic Surgery
- Pediatric Neurology
- Pulmonology
Background:
- Duchenne muscular dystrophy (DMD) frequently leads to progressive spinal deformity (scoliosis).
- Spinal fusion is a common surgical intervention for managing scoliosis in DMD patients.
- Pulmonary complications are a significant concern following spinal fusion in this population.
Purpose of the Study:
- To evaluate the outcomes of posterior spinal fusion with instrumentation in DMD patients with progressive spinal deformity.
- To identify risk factors for postoperative pulmonary complications.
- To assess the impact of spinal fusion on quality of life and survival in DMD.
Main Methods:
- Retrospective review of 183 DMD patients, with 68 undergoing posterior spinal fusion.
- Analysis of postoperative complications, focusing on pulmonary issues.
- Assessment of factors influencing quality of life, including surgical technique and spinal alignment.
- Comparison of survival rates with historical data.
Main Results:
- Pulmonary complications occurred in 17% of patients post-surgery.
- A preoperative forced vital capacity (FVC) of 35% predicted pulmonary complication risk.
- Spinal stabilization improved patient comfort and ease of care.
- Pulmonary function deterioration was not altered by spinal fusion; survival age was similar to DMD patients without fusion.
Conclusions:
- Posterior spinal fusion offers significant quality of life benefits for DMD patients with scoliosis.
- Careful patient selection and monitoring are crucial due to the risk of pulmonary complications.
- Surgical techniques like segmental instrumentation and achieving normal sagittal alignment enhance outcomes.