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

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.Spinopelvic 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.

Single rod instrumentation in patients with scoliosis and comorbidities: Indications and outcomes

AIM To present our results on the use of a single rod instrumentation correction technique in a small number of patients with major medical co-morbidities.METHODS This study was a prospective single surgeon series. Patients were treated with single rod hybrid constructs and had a minimum 2-year follow-up. Indications included complex underlying co-morbidities, conversion of growing rods to definitive fusion, and moderate adolescent idiopathic primarily thoracic scoliosis with severe eczema and low body mass index(BMI).RESULTS We included 99 consecutive patients. Mean age at surgery was 12.8 years(SD 3.5 years). Mean scoliosis correction was 62%(SD 15%) from 73°(SD 22°) to 28°(SD 15°). Mean surgical time was 153 min(SD 34 min), and blood loss was 530 mL(SD 327 mL); 20% BV(SD 13%). Mean clinical and radiological follow-up was 3.2 years(range: 2-12) post-operatively. Complications included rod failure, which occurred in three of our complex patients with severe syndromic or congenital kyphoscoliosis(3%). Only one of these three patientsrequired revision surgery to address a non-union. Our revision rate was 2%(including a distal junctional kyphosis in a Marfan's syndrome patient).CONCLUSION The single rod technique has achieved satisfactory deformity correction and a low rate of complications in patients with specific indications and severe underlying medical conditions. In these children with significant co-morbidities, where the risks of scoliosis surgery are significantly increased, this technique has achieved low operative time, blood loss, and associated surgical morbidity.