Abstract
Prior to the introduction of steroid management in Duchenne Muscular Dystrophy (DMD), the prevalence of scoliosis approached 100%, concomitant with progressive decreases in pulmonary function. As such, early scoliosis correction (at 20-25°) was advocated, prior to substantial pulmonary function decline. With improved pulmonary function and delayed curve progression with steroid treatment, the role of early surgery has been questioned. The purpose of this study was to compare the post-operative outcomes of early versus late scoliosis correction in DMD. We hypothesize that performing later surgery with larger curves would not lead to worse post-operative complications.
Retrospective cohort study. Patients with DMD who underwent posterior scoliosis correction, had pre-operative pulmonary function testing, and at least 1-year post-operative follow-up, were included; divided into 2 Groups by pre-operative curve angle – 1: ≤45°, 2: >45°. Primary outcome was post-operative complications by Clavien-Dindo (CD) grading. Secondary outcomes included: age at surgery, forced vital capacity (FVC), steroid utilization, fractional shortening (FS) by echocardiogram, surgery duration, blood transfusion requirements, ICU length of stay (LOS), days intubated post-operatively, hospital LOS, infection, curve correction. Two-tailed t-test and chi-square testing were used for analysis of patient factors and CD complication grade, respectively.
31 patients met the inclusion criteria, with a mean total follow-up of 8.3±3.2 years. Steroid treatment (prednisone, deflazacort) was utilized for 21 (67.7%) patients, for a mean duration of 8.2±4.0 years. Groups were comparable for steroid use, FVC, echo FS, and age at surgery (p>0.05). Primary curve angle was 31.7±10.4° and 58.3±11.1° for Groups 1 and 2, respectively (p 0.05). Surgery duration, ICU LOS, days intubated, hospital LOS, were also not different between Groups. For the entire cohort, however, the overall complication rate was higher for patients with steroid treatment [61.9% vs 10%, respectively (p=0.008)], the majority being CDII. Neither FVC nor echo FS were different between Groups at final follow-up (p=0.6; p=0.4, respectively).
Post-operative complication rates were not different for early and late scoliosis correction in DMD. In general, however, patients undergoing steroid treatment were at higher risk of blood transfusion and deep infection. Delaying scoliosis correction in DMD while PF is favourable is reasonable, but patients with prior steroid treatment should be counseled regarding the higher risk of complications.