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Long-term effects of Hirschsprung disease in adults : meta-analysis and patient-level regression study

De Andres Crespo, Marta ; Byström, Cornelia ; Tyraskis, Athanasios ; Mutanen, Annika ; Stenström, Pernilla LU orcid ; Hartman, Esther ; Danielsson, Johan ; Eaton, Simon ; De Coppi, Paolo and Löf Granström, Anna , et al. (2025) In BJS Open 9(6).
Abstract

Background: There has been an increasing number of single-centre studies describing the long-term outcomes of patients with Hirschsprung disease. This study aimed to systematically review the literature on long-term bowel, urological, and sexual functional outcomes, fertility and quality of life in adults with Hirschsprung disease. Methods: A PROSPERO-registered systematic review of the English literature was conducted for studies published up to July 2025 that reported functional outcomes beyond childhood (≥16 years) for patients who had undergone surgery for Hirschsprung disease. Centres were contacted individually for secondary analyses of patient-level data on bowel function score, Gastrointestinal Quality of Life Index, and Short... (More)

Background: There has been an increasing number of single-centre studies describing the long-term outcomes of patients with Hirschsprung disease. This study aimed to systematically review the literature on long-term bowel, urological, and sexual functional outcomes, fertility and quality of life in adults with Hirschsprung disease. Methods: A PROSPERO-registered systematic review of the English literature was conducted for studies published up to July 2025 that reported functional outcomes beyond childhood (≥16 years) for patients who had undergone surgery for Hirschsprung disease. Centres were contacted individually for secondary analyses of patient-level data on bowel function score, Gastrointestinal Quality of Life Index, and Short Form 36 questionnaire. Data were analysed and compared with those from healthy controls in the studies retrieved and from a reference healthy population. Hirschsprung disease clinical and surgical variables were correlated with these outcomes of interest in a patient-level analysis. Results: Fifty-three manuscripts fulfilled the inclusion criteria of 4277 papers retrieved. Patients with Hirschsprung disease had a greater likelihood of constipation (odds ratio 9.27, 95% confidence interval (c.i.) 4.78 to 18.06) and soiling (odds ratio 2.76, 1.96 to 3.89) compared with healthy controls. They scored lower on the Gastrointestinal Quality of Life Index (mean difference −5.21, 95% c.i. −9.53 to −0.89; P = 0.020). There were no significant differences in Short Form 36 domain scores except for physical functioning (mean difference −6.30, −8.74 to −3.87; P < 0.001). At a patient level, longer-segment disease (P < 0.001) and redo pull-through surgery (P = 0.002) were associated with a poorer bowel function score. Short form 36 scores were lower in women across six of eight domains; similarly, Gastrointestinal Quality of Life Index scores were lower in women (P < 0.001) and in patients with longer-segment disease (P < 0.001). Conclusion: Among patients with Hirschsprung disease, women, those with longer-segment disease, and patients who underwent redo surgery may be at risk of poorer quality of life.

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publishing date
type
Contribution to journal
publication status
published
subject
keywords
bowel function, fertility, HSCR, quality of life, sexual function, urological function
in
BJS Open
volume
9
issue
6
article number
zraf107
publisher
Wiley
external identifiers
  • pmid:41189484
  • scopus:105020893796
ISSN
2474-9842
DOI
10.1093/bjsopen/zraf107
language
English
LU publication?
yes
id
190ba2eb-5d8b-416f-9630-730553697ef7
date added to LUP
2025-12-12 14:57:41
date last changed
2025-12-13 03:00:04
@article{190ba2eb-5d8b-416f-9630-730553697ef7,
  abstract     = {{<p>Background: There has been an increasing number of single-centre studies describing the long-term outcomes of patients with Hirschsprung disease. This study aimed to systematically review the literature on long-term bowel, urological, and sexual functional outcomes, fertility and quality of life in adults with Hirschsprung disease. Methods: A PROSPERO-registered systematic review of the English literature was conducted for studies published up to July 2025 that reported functional outcomes beyond childhood (≥16 years) for patients who had undergone surgery for Hirschsprung disease. Centres were contacted individually for secondary analyses of patient-level data on bowel function score, Gastrointestinal Quality of Life Index, and Short Form 36 questionnaire. Data were analysed and compared with those from healthy controls in the studies retrieved and from a reference healthy population. Hirschsprung disease clinical and surgical variables were correlated with these outcomes of interest in a patient-level analysis. Results: Fifty-three manuscripts fulfilled the inclusion criteria of 4277 papers retrieved. Patients with Hirschsprung disease had a greater likelihood of constipation (odds ratio 9.27, 95% confidence interval (c.i.) 4.78 to 18.06) and soiling (odds ratio 2.76, 1.96 to 3.89) compared with healthy controls. They scored lower on the Gastrointestinal Quality of Life Index (mean difference −5.21, 95% c.i. −9.53 to −0.89; P = 0.020). There were no significant differences in Short Form 36 domain scores except for physical functioning (mean difference −6.30, −8.74 to −3.87; P &lt; 0.001). At a patient level, longer-segment disease (P &lt; 0.001) and redo pull-through surgery (P = 0.002) were associated with a poorer bowel function score. Short form 36 scores were lower in women across six of eight domains; similarly, Gastrointestinal Quality of Life Index scores were lower in women (P &lt; 0.001) and in patients with longer-segment disease (P &lt; 0.001). Conclusion: Among patients with Hirschsprung disease, women, those with longer-segment disease, and patients who underwent redo surgery may be at risk of poorer quality of life.</p>}},
  author       = {{De Andres Crespo, Marta and Byström, Cornelia and Tyraskis, Athanasios and Mutanen, Annika and Stenström, Pernilla and Hartman, Esther and Danielsson, Johan and Eaton, Simon and De Coppi, Paolo and Löf Granström, Anna and Wester, Tomas and Pakarinen, Mikko and Curry, Joe and Loukogeorgakis, Stavros and Davidson, Joseph}},
  issn         = {{2474-9842}},
  keywords     = {{bowel function; fertility; HSCR; quality of life; sexual function; urological function}},
  language     = {{eng}},
  number       = {{6}},
  publisher    = {{Wiley}},
  series       = {{BJS Open}},
  title        = {{Long-term effects of Hirschsprung disease in adults : meta-analysis and patient-level regression study}},
  url          = {{http://dx.doi.org/10.1093/bjsopen/zraf107}},
  doi          = {{10.1093/bjsopen/zraf107}},
  volume       = {{9}},
  year         = {{2025}},
}