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Exploring potential sex differences in Hirschsprung disease : a national cohort study of diagnostic patterns and early postoperative outcome

Söderström, Linnea ; Graneli, Christina LU ; Hagelsteen, Kristine LU orcid ; Gunnarsdottir, Anna ; Oddsberg, Jenny ; Svensson, Pär Johan ; Borg, Helena ; Bräutigam, Matilda ; Gustafson, Elisabet and Löf Granström, Anna , et al. (2025) In Pediatric Surgery International 41(1).
Abstract

Purpose: There are limited data to show how sex impacts the early clinical course of patients with Hirschsprung disease (HSCR). This study aimed to explore potential sex related disparities in the preoperative, surgical, and early postoperative course of HSCR patients. Methods: This retrospective study analyzed data of HSCR patients who underwent pull-through surgery at pediatric surgery centers in Sweden from July 1st, 2013, to June 30th, 2023. Male and female patients were compared regarding diagnostics, surgical treatment, unplanned procedures under general anesthesia or readmissions within 90 days after pull-through, and complications (Clavien-Madadi grade ≥ 3) up to 30 days after pull-through. Results: A total of 197 patients were... (More)

Purpose: There are limited data to show how sex impacts the early clinical course of patients with Hirschsprung disease (HSCR). This study aimed to explore potential sex related disparities in the preoperative, surgical, and early postoperative course of HSCR patients. Methods: This retrospective study analyzed data of HSCR patients who underwent pull-through surgery at pediatric surgery centers in Sweden from July 1st, 2013, to June 30th, 2023. Male and female patients were compared regarding diagnostics, surgical treatment, unplanned procedures under general anesthesia or readmissions within 90 days after pull-through, and complications (Clavien-Madadi grade ≥ 3) up to 30 days after pull-through. Results: A total of 197 patients were included from four treating centers (158 males, 39 females). Females had a higher prevalence of familial disease (28.2% vs. 8.2%; p < 0.01) and RET gene mutations (15.4% vs. 2.5%; p = 0.02). No differences were observed in age at biopsy, need for re-biopsy, preoperative stoma rates, or age at diagnosis. Time from diagnosis to pull-through was longer in females (median 48.5 vs. 28 days; p = 0.02), but age at pull-through did not differ. No significant differences were found in postoperative hospital stay, severe complications within 30 days, nor unplanned procedures, HAEC, or readmissions within 90 days. Conclusion: The early clinical course of HSCR patients does not appear to be sex dependent. Although females had a longer interval from diagnosis to pull-through, their age at pull-through was comparable to males. As expected, a higher proportion of females reported familial disease and had a verified RET-mutation. Level of evidence: Level III.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Hirschsprung disease, Sex, Surgical outcome
in
Pediatric Surgery International
volume
41
issue
1
article number
309
publisher
Springer
external identifiers
  • scopus:105017769318
  • pmid:41042336
ISSN
0179-0358
DOI
10.1007/s00383-025-06213-5
language
English
LU publication?
yes
id
cb0ffe9f-63f6-40a0-b542-61170f7d0d82
date added to LUP
2025-11-21 12:10:06
date last changed
2025-11-22 03:00:02
@article{cb0ffe9f-63f6-40a0-b542-61170f7d0d82,
  abstract     = {{<p>Purpose: There are limited data to show how sex impacts the early clinical course of patients with Hirschsprung disease (HSCR). This study aimed to explore potential sex related disparities in the preoperative, surgical, and early postoperative course of HSCR patients. Methods: This retrospective study analyzed data of HSCR patients who underwent pull-through surgery at pediatric surgery centers in Sweden from July 1st, 2013, to June 30th, 2023. Male and female patients were compared regarding diagnostics, surgical treatment, unplanned procedures under general anesthesia or readmissions within 90 days after pull-through, and complications (Clavien-Madadi grade ≥ 3) up to 30 days after pull-through. Results: A total of 197 patients were included from four treating centers (158 males, 39 females). Females had a higher prevalence of familial disease (28.2% vs. 8.2%; p &lt; 0.01) and RET gene mutations (15.4% vs. 2.5%; p = 0.02). No differences were observed in age at biopsy, need for re-biopsy, preoperative stoma rates, or age at diagnosis. Time from diagnosis to pull-through was longer in females (median 48.5 vs. 28 days; p = 0.02), but age at pull-through did not differ. No significant differences were found in postoperative hospital stay, severe complications within 30 days, nor unplanned procedures, HAEC, or readmissions within 90 days. Conclusion: The early clinical course of HSCR patients does not appear to be sex dependent. Although females had a longer interval from diagnosis to pull-through, their age at pull-through was comparable to males. As expected, a higher proportion of females reported familial disease and had a verified RET-mutation. Level of evidence: Level III.</p>}},
  author       = {{Söderström, Linnea and Graneli, Christina and Hagelsteen, Kristine and Gunnarsdottir, Anna and Oddsberg, Jenny and Svensson, Pär Johan and Borg, Helena and Bräutigam, Matilda and Gustafson, Elisabet and Löf Granström, Anna and Stenström, Pernilla and Wester, Tomas}},
  issn         = {{0179-0358}},
  keywords     = {{Hirschsprung disease; Sex; Surgical outcome}},
  language     = {{eng}},
  number       = {{1}},
  publisher    = {{Springer}},
  series       = {{Pediatric Surgery International}},
  title        = {{Exploring potential sex differences in Hirschsprung disease : a national cohort study of diagnostic patterns and early postoperative outcome}},
  url          = {{http://dx.doi.org/10.1007/s00383-025-06213-5}},
  doi          = {{10.1007/s00383-025-06213-5}},
  volume       = {{41}},
  year         = {{2025}},
}