Survival of patients with Duchenne muscular dystrophy who underwent spinal deformity correction.

AIM

We investigated survival rates after scoliosis correction in individuals with Duchenne muscular dystrophy (DMD) and evaluated factors that can affect them.

METHOD

This was a retrospective cohort study from 2000 to 2022 with a minimum 2-year postoperative follow-up. We reviewed the hospital records/spinal radiographs and analysed data with XLSTAT. Kaplan-Meier and multivariate Cox regression survival analysis was performed.

RESULTS

Forty-three patients had a mean age at surgery of 14 years 5 months. Mean postoperative follow-up was 10 years 10 months. There was no operative or 30-day postoperative mortality in this group. Twenty-four patients died because of cardiorespiratory failure. Median survivorship was 14 years 2 months, with the longest observed survival being 22 years 6 months given the limitation of the length of postoperative follow-up. The degree of preoperative coronal imbalance and pelvic obliquity, as well as intraoperative blood loss, were factors that significantly affected survival. The impact of preoperative sagittal imbalance and extension of the fusion to the sacrum/pelvis trended towards significance. In contrast, age at surgery, preoperative/postoperative scoliosis, thoracic kyphosis, lumbar lordosis, scoliosis and pelvic obliquity flexibility or correction indices, postoperative coronal/sagittal balance, need of preoperative non-invasive ventilation, preoperative feeding disorders, development of surgical complications, and length of hospital and intensive care unit stay were exposure variables that did not affect postsurgical survival in patients with DMD.

INTERPRETATION

Survival of up to two decades or more was possible among young patients with DMD after scoliosis correction. This was affected by factors that related to disease and deformity severity, as well as surgical morbidity.

WHAT THIS PAPER ADDS

There was no operative or 30-day postoperative mortality in this group of patients with Duchenne muscular dystrophy (DMD) undergoing scoliosis correction. Survival probabilities at 5-year, 10-year, 15-year, and 20-year intervals post-surgery were 92%, 80%, 33%, and 12% respectively. Scoliosis surgery achieved good deformity correction and a balanced spine that was maintained at follow-up. Respiratory failure, severe pneumonia, and left ventricular failure were the leading causes of death in the study participants. Preoperative global coronal imbalance, pelvic obliquity, and intraoperative blood loss significantly predicted survival. Factors that affected survival after scoliosis surgery were associated with perioperative morbidity and disease or deformity severity in the DMD group.