Outcomes of Primary versus Multiple-Staged Repair in Hirschsprung's Disease in England

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.

Childhood colostomies: patterns, indications and outcomes in a Nigerian University Teaching Hospital

“…The congenital indications are mainly anorectal malformation (ARM) and Hirschsprung's disease (HD), but the relative contribution of each as indication varies among various publications with HD more in some 1 , 4 , 5 and ARM more in others 2 , 3 , 6 , 7 . Incidence of colostomy for HD is dropping in some countries due to early presentation and performance of more single stage transanal pull-through procedures 8 , 9 , 10 but initial colostomy is still high in our environment 11 and other Low and Middle Income Countries 12 , 13 , 14 where late presentation is still rife. In the past there used to be many transverse 1 , 2 and loop colostomies 1 , 2 but the current trend seems to be more towards construction of defunctioning and sigmoid colostomies 3 , 14 , 15 , 16 .…”