Spinal fusion in Duchenne's muscular dystrophy

Area of Science:

• Orthopedic Surgery
• Pediatric Spine Surgery
• Neuromuscular Diseases

Background:

• Duchenne muscular dystrophy (DMD) frequently leads to progressive scoliosis, impacting patient mobility and quality of life.
• Spinal fusion is a common surgical intervention for scoliosis in DMD patients.
• Evaluating different instrumentation techniques is crucial for optimizing outcomes.

Purpose of the Study:

• To review the Women's and Children's Hospital experience with Luque spinal fusion in Duchenne muscular dystrophy.
• To assess the clinical and radiologic outcomes and safety of spinal fusion procedures.
• To compare the effectiveness of L-rod instrumentation versus Galveston constructs with pelvic fixation.

Main Methods:

• Retrospective review of 17 boys with Duchenne muscular dystrophy who underwent spinal fusion since 1983.
• Comparison of outcomes between patients treated with L-rod instrumentation (n=10) and Galveston construct with pelvic fixation (n=7).
• Evaluation of clinical parameters (sitting balance, scoliosis progression) and radiologic outcomes (pelvic obliquity correction).

Main Results:

• The Galveston construct group showed a mean pelvic obliquity correction of 63% with better maintenance compared to the L-rod group.
• No pseudoarthroses or instrument failures were reported in the Galveston group, unlike some issues in the L-rod group.
• Spinal fusion provided a mean correction of 60% and controlled pelvic obliquity without significant postoperative deterioration.

Conclusions:

• Spinal fusion with the Luque rod construct and pelvic fixation, particularly the Galveston construct, is a safe and effective procedure for DMD patients.
• The Galveston construct with pelvic fixation offers superior correction and stability for scoliosis in Duchenne muscular dystrophy.
• Surgery can be safely performed in patients with forced vital capacity (FVC) values as low as 20% predicted.