Correction of scoliotic deformity in Duchenne
muscular dystrophy (DMD) is performed to maintain adequate seating posture and facilitate comfort. Delaying surgery can predispose to greater morbidity as DMD exhibits progressive cardiorespiratory compromise. Early limited instrumentation may provide a solution to optimize patients with this condition.
Purpose
The aim was to assess outcomes for a cohort of DMD patients who had posterior single-rod instrumentation and bilateral spinal fusion of their neuromuscular scoliotic deformity.
Study design
This was a retrospective cohort study.
Patient sample
Forty-one consecutive patients were included.
Outcome measures
Perioperative morbidity, seating outcomes, pulmonary function, deformity correction, and instrumentation integrity were assessed.
Methods
Clinical and radiographic review was performed.
Results
No perioperative mortality or neurologic deterioration was encountered. Total surgical time was 96 minutes, mean total blood loss was 2.3l, mean intensive care unit stay was 41 hours, and overall length of stay was 11 days. Mean Cobb angle improved from 24.3° to 15.6°, pelvic obliquity improved from 7° preoperatively to 5° postoperatively. Three patients had failure of fixation at a mean of 3.5 years. Forced vital capacity was 60% preoperatively and 56% at 1 year, forced expiratory volume/1 second was 67% and 62% at 1 year postoperatively. Seating and posture was satisfactory in all these patients.
Conclusions
The authors advocate early operative intervention using a limited instrumentation technique in patients with DMD to maintain seating balance and minimize perioperative morbidity.