DMD – CLINICAL CARE: EP.111 Evolution of scoliosis management in Duchenne muscular dystrophy

Abstract

The long-term effects of scoliosis surgery on respiratory function and longevity in Duchenne muscular dystrophy (DMD) have been debated. Corticosteroids, respiratory care and cardioprotection have altered the natural history of DMD, making decisions regarding scoliosis management even more challenging. The aim of this study was to present the evolution of scoliosis management over time and its impact on disease-related outcomes, with focus on life expectancy and causes of death. All male patients born since 1st January 1970 who died by 31st December 2019 and were followed at a medical clinic in Sweden with a confirmed diagnosis of DMD were included in the study. Patients were identified via the National Quality Registry for Neuromuscular Diseases (NMiS), the Swedish National Registry for Respiratory Failure (Swedevox) and the pathology laboratories performing skeletal muscle biopsies in Sweden. Data was collected from the patients’ medical records in a systematic manner. We identified 129 patients, of whom approximately 80% had confirmed scoliosis by last follow-up or death at a median age of 30 years. 28 patients were treated solely with thoracolumbosacral orthosis (TLSO). Scoliosis surgery was performed in almost half of scoliosis patients, at a median age of 15.4 years. Median time from loss of ambulation to scoliosis surgery was 5.4 years. The evolution of scoliosis management over time, disease-related outcomes before and after scoliosis surgery, as well as correlation to other therapies, such as corticosteroids and non-invasive ventilation, will be presented. Scoliosis management in DMD is complex, necessitating multidisciplinary evaluation of comorbidities and close monitoring. Its evolution and impact on life expectancy and causes of death will be discussed.