Evidence map›Paper›PMID 41189484›Full record

SynthesisBJS open2025

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

Marta de Andres Crespo, Cornelia Byström, Athanasios Tyraskis, Annika Mutanen, Pernilla Stenström, Esther Hartman, Johan Danielsson, Simon Eaton, Paolo De Coppi, Anna Löf Granström and 5 more

Abstract readMeta-AnalysisSystematic Review
PubMed Full text at PMC Publisher
In one paragraph

Synthesis in BJS open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors.

Marta de Andres CrespoInstitute of Child Health, University College London, London, UK.
Cornelia ByströmDepartment of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.
Athanasios TyraskisSpecialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital, London, UK.
Annika MutanenDepartment of Pediatric Surgery, New Children's Hospital, Helsinki, Finland.
Pernilla StenströmDepartment of Pediatric Surgery, Lund University, Lund, Sweden.ORCID 0000-0002-5052-9944
Esther HartmanDepartment of Medical and Clinical Psychology, Tilburg University, Tilburg, the Netherlands.
Johan DanielssonInstitution of Women's and Children's Health, Uppsala University, Uppsala, Sweden.
Simon EatonInstitute of Child Health, University College London, London, UK.ORCID 0000-0003-0892-9204
Paolo De CoppiInstitute of Child Health, University College London, London, UK.
Anna Löf GranströmDepartment of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.
Tomas WesterDepartment of Pediatric Surgery, Karolinska Institutet, Stockholm, Sweden.
Mikko PakarinenDepartment of Pediatric Surgery, New Children's Hospital, Helsinki, Finland.ORCID 0000-0001-6016-6334
Joe CurrySpecialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital, London, UK.
Stavros LoukogeorgakisInstitute of Child Health, University College London, London, UK.
Joseph DavidsonInstitute of Child Health, University College London, London, UK.ORCID 0000-0002-3303-6320

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThere 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.

methodsA 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.

resultsFifty-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).

conclusionAmong 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.

Indexed as

Hirschsprung DiseaseQuality of LifeAdultConstipationFemaleHumansMalebowel functionfertilityHSCRquality of lifesexual functionurological function

Identifiers

PMID41189484
PMCPMC12586849

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.