Abstract
Introduction The study aimed to compare 1-year outcomes for primary versus multiple-staged (three
operations with colostomy) repairs in Hirschsprung's disease (HD).
Materials and Methods Retrospective analysis of a large national administrative database (Hospital Episode
Statistics) including all the neonates born with HD in England between 2003 and 2015.
Main outcomes were: 1-year mortality, postoperative readmissions, and reoperations.
Secondary outcomes: cumulative length of hospital stay (cLOS) and hospital volume–outcome
relationship.
Results A total of 1,333 neonates with HD were treated in 21 specialist pediatric surgical
centers; 874 (65.5%) patients had a primary repair for HD. One-year mortality was
2.8%. The overall readmission rate was 70.2%, with a significant difference between
primary and multiple-staged repair (79.9 vs. 90.1%, p < 0.01). There was no difference in reoperation. Primary pull-through was associated
with a significantly lower probability of postoperative readmission (odds ratio [OR] = 0.08,
95% confidence interval [CI] = 0.06–0.11, p < 0.001) and cLOS (OR = 0.38, 95% CI = 0.28–0.52, p < 0.001) compared with multiple-staged repair. There were no significant difference
in outcomes between patients treated in low-volume (<37 cases/year) and high-volume
(> 55 cases/year) specialist centers.
Conclusion Whenever clinically indicated, primary repair should be used in HD as this is associated
with fewer readmissions and shorter time spent in the hospital.
Keywords
Hirschsprung's disease - outcomes - primary repair - multiple-staged - length of stay
