Surgical and long-term functional outcomes of patients with Duchenne muscular dystrophy following spinal deformity correction.

Abstract

Background: Life expectancy in patients with Duchenne muscular dystrophy (DMD) has improved due to advances in medical care. DMD patients develop progressive spinal deformity after loss of ambulatory function and onset of wheelchair dependence for mobility. There is limited published data on the effect of spinal deformity correction on long-term functional outcomes, quality of life (QoL), and satisfaction in DMD patients.

Aim: To investigate the long-term functional outcomes following spinal deformity correction in DMD patients.

Methods: This was a retrospective cohort study from 2000-2022. Data was collected from hospital records and radiographs. At follow-up, patients completed the muscular dystrophy spine questionnaire (MDSQ). Statistical analysis was performed by linear regression analysis and ANOVA to analyse clinical and radiographic factors significantly associated with MDSQ scores.

Results: Forty-three patients were included with mean age 14.4 years at surgery. Spino-pelvic fusion was performed in 41.9% of patients. Mean surgical time was 352.1 min and mean blood loss was 36% of estimated total blood volume. Mean hospital stay was 14.1 d. Postoperative complications occurred in 25.6% of patients. Mean preoperative scoliosis was 58°, pelvic obliquity 16.4°, thoracic kyphosis 55.8°, lumbar lordosis 11.1°, coronal balance 3.8 cm, and sagittal balance + 6.1 cm. Mean surgical correction of scoliosis was 79.2% and of pelvic obliquity was 80.8%. Mean follow-up was 10.9 years (range: 2-22.5). Twenty-four patients had died at follow-up. Sixteen patients completed the MDSQ at mean age 25.4 years (range 15.2-37.3). Two patients were bed-ridden and 7 were on ventilatory support. Mean MDSQ total score was 38.1. All 16 patients were satisfied with the results of spinal surgery and would choose surgery again if offered. Most patients (87.5%) reported no severe back pain at follow-up. Factors significantly associated with functional outcomes (MDSQ total score) included greater duration of post-operative follow-up, age, scoliosis postoperatively, correction of scoliosis, increased lumbar lordosis postoperatively, and greater age at loss of independent ambulation.

Conclusion: Spinal deformity correction in DMD patients leads to positive long-term effects on QoL and high patient satisfaction. These results support spinal deformity correction to improve long-term QoL in DMD patients.