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Spine deformity correction in Marfan syndrome
Kevin B Jones1, Gurkan Erkula, Paul D Sponseller
1Department of Orthopaedic Surgery, Johns Hopkins Medical Institutions, Baltimore, MD 21287-0881, USA.
Spine
|March 14, 2003
Summary
Surgical correction of spinal deformities in Marfan syndrome presents significant challenges, including high rates of infection and instrumentation failure. Careful preoperative evaluation and surgical technique are crucial for successful outcomes in these complex cases.
Area of Science:
- Orthopedic Surgery
- Spinal Deformity Correction
- Marfan Syndrome Management
Background:
- Spinal deformities are prevalent and often resistant to conservative treatment in Marfan syndrome.
- Previous studies on surgical intervention for Marfan syndrome spinal deformities reported higher complication rates.
Purpose of the Study:
- To analyze the challenges associated with surgical correction of spinal deformities in Marfan syndrome patients.
- To provide recommendations for reducing morbidity and improving surgical success rates.
Main Methods:
- Retrospective review of records and radiographs of 39 Marfan syndrome patients undergoing spinal surgery.
- Analysis of presentation features, complications, and outcomes, including postoperative low back outcome scores.
Main Results:
- High rates of complications including infection (10%), dural tear (8%), fixation failure (21%), and curve decompensation (coronal 8%, sagittal 21%).
- Infection correlated with dural tears; decompensation with extreme correction.
- One perioperative death due to valvular insufficiency.
Conclusions:
- Thorough cardiopulmonary evaluation is essential before surgery.
- Preoperative imaging (CT, MRI) is recommended to assess bone and dural status.
- Attention to sagittal alignment, fusion levels, minimal dissection, and avoiding overcorrection can improve outcomes.