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Eur J Pediatr Surg 2020; 30(01): 104-110
DOI: 10.1055/s-0039-3402712
Original Article
Georg Thieme Verlag KG Stuttgart · New York

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

Stefano Giuliani    *
1   Division of Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom
,
Kate Honeyford   *
2   Dr Foster Unit, Imperial College London, London, United Kingdom
,
Chieh-Yu Chang
2   Dr Foster Unit, Imperial College London, London, United Kingdom
,
Alex Bottle
2   Dr Foster Unit, Imperial College London, London, United Kingdom
,
Paul Aylin
2   Dr Foster Unit, Imperial College London, London, United Kingdom
› Author Affiliations
Further Information

Publication History

14 May 2019

03 December 2019

Publication Date:
07 January 2020 (online)

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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

* Stefano Giuliani and Kate Honeyford share the first authorship.


Supplementary Material

 

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