Published online Jun 18, 2023. doi: 10.5312/wjo.v14.i6.411
Peer-review started: December 27, 2022
First decision: March 14, 2023
Revised: March 29, 2023
Accepted: May 15, 2023
Article in press: May 15, 2023
Published online: June 18, 2023
Life expectancy in patients with Duchenne muscular dystrophy (DMD) has improved due to advances in medical care. DMD patients developed progressive spinal deformity after loss of ambulatory function and onset of wheelchair dependence for mobility. Surgical correction of scoliosis in patients with DMD aims to improve sitting balance, wheelchair mobility and quality of life (QoL). DMD patients undergoing surgical correction of spinal deformity are at high risk of perioperative morbidity and mortality.
There is limited published data on the effect of spinal deformity correction on long-term functional outcomes, QoL, and satisfaction in DMD patients.
The aim of this study was to investigate the clinical, surgical, and long-term functional results following surgical correction of scoliosis in patients with DMD, and to investigate clinical and radiological factors that predict long-term functional outcomes following surgery.
This was a retrospective cohort study, reviewing data from 113 consecutive patients with DMD who were seen in a National Spinal Deformity Service between January 2000 and August 2022. Data were collected from hospital records and radiographs. All patients had minimum data available including pre-operative clinical and demographic details, comorbidities, operative details including complications, pre- and post-operative radiographic parameters, and minimum post-surgical follow-up of two years. Functional outcomes were assessed by the muscular dystrophy spine questionnaire (MDSQ).
Forty-three patients underwent correction of spinal deformity by posterior instrumented fusion, comprising 38% of the whole cohort (43/113 patients); spinopelvic fusion was performed in 41.9% of patients, the mean surgical time was 351 min, mean blood loss was 36% of estimated total blood volume, mean hospital stay was 14.1 d, and postoperative complications occurred in 25.6% of patients. The mean correction of scoliosis was 79.2%, mean correction of pelvic obliquity was 80.8%, and mean post-surgical follow-up was 10.9 years. Mean MDSQ total score at follow-up was 38.1; all 16 patients completing the MDSQ were satisfied with the results of spinal surgery and would choose surgery again if offered, and 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.
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.
Posterior instrumented spinal fusion is indicated for treatment of progressive scoliosis in patients with DMD; surgical correction of spinal deformity is recommended before respiratory and cardiac function deteriorate to the extent that general anaesthesia and complex major surgery are unsafe and whilst spinal deformity remains flexible. DMD patients in our cohort had a low incidence of complications compared to other cohorts in the published literature. Surgical management of scoliosis in DMD patients has been associated with a significantly lower mortality rate at 6.4 years compared to those managed without surgery, and patients and parents were highly satisfied and believed scoliosis surgery improved their function, sitting balance, and QoL. Surgical correction of spinal deformity can improve QoL in DMD patients and is, therefore, indicated in the presence of a severe/progressive deformity despite an increased risk of complications compared to patients with no underlying neurodisability.