Skip to main content

Advertisement

SpringerLink
Log in

Bowel function at preschool and early childhood age in children with long-segment Hirschsprung disease

  • RESEARCH
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Bowel dysfunction has been proven to be the most typical complication of long-segment Hirschsprung disease (LSHD). Additionally, bowel dysfunction in preschool and early childhood should be properly assessed, because it may result in persistent bowel dysfunction, social issues, and depression in adolescence and adulthood. This study evaluated bowel function during preschool and early childhood in patients with LSHD. A cross-sectional bowel function score (BFS) questionnaire survey was conducted on 270 infants with short-segment Hirschsprung disease (SSHD) and LSHD who underwent pull-through (PT) between January 2014 and December 2019 at the Children’s Hospital of Nanjing Medical University. One hundred ninety patients who consented to the survey were asked to complete the questionnaire and were divided into two groups: the LSHD group (n = 42) and the SSHD group (n = 148). Bowel function outcomes were assessed by using a questionnaire. The total BFS score in the LSHD group was significantly lower than that in the SSHD group (15.0 [14.0, 17.0] vs. 18.0 [16.0, 19.0], p < 0.05) and did not improve with age (p > 0.05). Independent BFS items with lower scores were discovered in LSHD than in SSHD, including the capacity to hold back defecation, feeling/reporting the urge to defecate, frequency, soiling, and accidents (all p < 0.05). After subgroup analysis of follow-up age (3–5 years, 5 ~ 7 years, and > 7 years), there was no difference between the score of ability to hold back defecation over 7 years old and feeling/reporting the urge to defecate over 5 years old (all p > 0.05). The frequency and soiling scores were lower in the LSHD group than in the SSHD group over 5 years of age (all p < 0.05). Fortunately, there was no difference in constipation scores and social problems between the LSHD and SSHD groups in every sub-follow-up group (all p > 0.05).

  Conclusion: Overall, bowel function at preschool and early childhood age was poorer in LSHD than in SSHD and did not improve with age. However, the ability to hold back defecation and feeling/reporting the urge to defecate with age in LSHD may be similar to those in SSHD. Simultaneously, the frequency, soiling, and number of accidents were consistently worse in the LSHD group.

  Trial registration: This study was retrospectively registered in the ClinicalTrials database.gov (NCT05461924) in August 2022.

What is Known:

• Bowel function in SSHD is satisfactory or near-normal, and the general consensus is that the longer the aganglionic segment, the worse the bowel function after surgery.

• Bowel functional outcomes of LSHD were not defined, and the limited reports of bowel functional outcomes compared with SSHD were extensively varied. Bowel dysfunction at preschool and early childhood may lead to persistent bowel dysfunction, social problems, and depression in adolescence or adulthood.

What is New:

• Bowel function in preschool and early childhood in patients with LSHD has not been defined and should be thoroughly assessed in a larger group of patients with precise definitions of incontinence or soiling.

• The present study was primarily designed to evaluate bowel function at preschool and early childhood age in LSHD compared with SSHD in a relatively large number of HD cases using BFS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Data availability

All data generated or analyzed during this study are available from the corresponding authors upon reasonable request.

References

  1. Lu C, Hou G, Liu C (2017) Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates: a multicenter study. J Pediatr Surg 52(7):1102–1107

    Article  PubMed  Google Scholar 

  2. Fang Y, Bai J, Zhang B (2020) Laparoscopic Soave procedure for long-segment Hirschsprung’s disease - single-center experience. Videosurgery and Other Miniinvasive Techniques 15(1):234–238

    Article  PubMed  Google Scholar 

  3. Kawaguchi AL, Guner YS, Somme S (2021) Management and outcomes for long-segment Hirschsprung disease: a systematic review from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg 56(9):1513–1523

    Article  PubMed  PubMed Central  Google Scholar 

  4. Kim SH, Cho YH, Kim HY (2021) Assessment of defecation function beyond infantile period for transanal single-stage endorectal pull-through in Hirschsprung disease. Annals of Surgical Treatment and Research 101(4):231–239

    Article  PubMed  PubMed Central  Google Scholar 

  5. Bystrom C, Ostlund S, Hoff N (2021) Evaluation of bowel function, urinary tract function, and quality of life after transanal endorectal pull-through surgery for Hirschsprung’s disease. Eur J Pediatr Surg 31(1):40–48

    Article  PubMed  Google Scholar 

  6. Verkuijl SJ, Meinds RJ, van der Steeg A (2022) Functional outcomes after surgery for total colonic, long-segment, versus rectosigmoid segment Hirschsprung disease. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION 74(3):348–354

    Article  CAS  Google Scholar 

  7. Stenstrom P, Brautigam M, Borg H (2017) Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis. J Pediatr Surg 52(8):1302–1307

    Article  PubMed  Google Scholar 

  8. Chung P, Wong K, Tam P (2018) Are all patients with short segment Hirschsprung’s disease equal? A retrospective multicenter study. Pediatr Surg Int 34(1):47–53

    Article  PubMed  Google Scholar 

  9. Chan K, Lee KH, Wong H (2021) Long-term results of one-stage laparoscopic-assisted endorectal pull-through for rectosigmoid Hirschsprung’s disease in patients aged above 5 years. J Laparoendosc Adv Surg Tech 31(2):225–229

    Article  Google Scholar 

  10. Townley OG, Lindley RM, Cohen MC (2020) Functional outcome, quality of life, and ‘failures’ following pull-through surgery for Hirschsprung’s disease: a review of practice at a single-center. J Pediatr Surg 55(2):273–277

    Article  PubMed  Google Scholar 

  11. Fosby MV, Stensrud KJ, Bjornland K (2020) Bowel function after transanal endorectal pull-through for Hirschsprung disease - does outcome improve over time? J Pediatr Surg 55(11):2375–2378

    Article  PubMed  Google Scholar 

  12. Dai Y, Deng Y, Lin Y (2020) Long-term outcomes and quality of life of patients with Hirschsprung disease: a systematic review and meta-analysis. BMC Gastroenterol 20(1):67

    Article  PubMed  PubMed Central  Google Scholar 

  13. Drissi F, Meurette G, Baayen C (2019) Long-term outcome of Hirschsprung disease: impact on quality of life and social condition at adult age. Dis Colon Rectum 62(6):727–732

    Article  PubMed  Google Scholar 

  14. Meinds RJ, van der Steeg A, Sloots C (2019) Long-term functional outcomes and quality of life in patients with Hirschsprung’s disease. Br J Surg 106(4):499–507

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Tran VQ, Mahler T, Dassonville M (2018) Long-term outcomes and quality of life in patients after soave pull-through operation for Hirschsprung’s disease: an observational retrospective study. Eur J Pediatr Surg 28(5):445–454

    Article  PubMed  Google Scholar 

  16. Kyrklund K, Neuvonen MI, Pakarinen MP (2018) Social morbidity in relation to bowel functional outcomes and quality of life in anorectal malformations and Hirschsprung’s disease. Eur J Pediatr Surg 28(6):522–528

    Article  PubMed  Google Scholar 

  17. Peng C, Tan SS, Pang W (2021) Rectourethral and rectovesical fistula as serious and rare complications after Hirschsprung disease operation: experience in seven patients. J Pediatr Surg 56(2):263–268

    Article  PubMed  Google Scholar 

  18. Jiao C, Zhuansun D, He Y (2022) Transanal full-thickness pull-through approach in the treatment of anastomotic leakage after operation for Hirschsprung disease. Pediatr Surg Int 38(9):1263–1271

    Article  PubMed  Google Scholar 

  19. Oyania F, Ogwal A, Nimanya S (2020) Long term bowel function after repair of anorectal malformations in Uganda. J Pediatr Surg 55(7):1400–1404

    Article  PubMed  Google Scholar 

  20. van der Steeg H, van Rooij I, Iacobelli BD (2022) Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study. J Pediatr Surg 57(9):89–96

    Article  PubMed  Google Scholar 

  21. Verkuijl SJ, Jonker JE, Trzpis M (2021) Functional outcomes of surgery for colon cancer: a systematic review and meta-analysis. EJSO 47(5):960–969

    Article  PubMed  Google Scholar 

  22. Magdeburg J, Glatz N, Post S (2016) Long-term functional outcome of colonic resections: how much does faecal impairment influence quality of life? Colorectal Dis 18(11):O405–O413

    Article  CAS  PubMed  Google Scholar 

  23. Hoel AT, Tofft L, Bjornland K (2021) Reaching adulthood with Hirschsprung’s disease: patient experiences and recommendations for transitional care. J Pediatr Surg 56(2):257–262

    Article  PubMed  Google Scholar 

  24. Roorda D, Verkuij SJ, Derikx J (2022) Did age at surgery influence outcome in patients with Hirschsprung disease? A nationwide cohort study in the Netherlands. Journal Of Pediatric Gastroentology And Nutrition 75(4):431–437

    Article  Google Scholar 

  25. Sakurai T, Tanaka H, Endo N (2021) Predictive factors for the development of postoperative Hirschsprung-associated enterocolitis in children operated during infancy. Pediatr Surg Int 37(2):275–280

    Article  PubMed  Google Scholar 

  26. Gunadi M, C.T., Stevie, (2022) Long-term functional outcomes of patients with Hirschsprung disease following pull-through. BMC Pediatr 22(1):246

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Yuan Y, Xu M, Yang H (2021) The efficacy of biofeedback therapy for the treatment of fecal incontinence after soave procedure in children for Hirschsprung’s disease. Front Pediatr 9:638120

    Article  PubMed  PubMed Central  Google Scholar 

  28. Kyrklund K, Koivusalo A, Rintala RJ (2012) Evaluation of bowel function and fecal continence in 594 Finnish individuals aged 4 to 26 years. Dis Colon Rectum 55(6):671–676

    Article  PubMed  Google Scholar 

  29. Wood RJ, Levitt MA (2018) Anorectal malformations. Clin Colon Rectal Surg 31(2):61–70

    Article  PubMed  PubMed Central  Google Scholar 

  30. Choi BJ, Kwon W, Baek SH (2020) Single-port laparoscopic Deloyers procedure for tension-free anastomosis after extended left colectomy or subtotal colectomy: a 6-patient case series. Medicine 99(31):e21421

    Article  PubMed  PubMed Central  Google Scholar 

  31. Saadai P, Trappey AF, Goldstein AM (2019) Guidelines for the management of postoperative soiling in children with Hirschsprung disease. Pediatr Surg Int 35(8):829–834

    Article  CAS  PubMed  Google Scholar 

  32. Saad SA, Elseed M, AbouZeid AA (2020) Histopathological perspective of the pulled-through colon in Hirschsprung disease: impact on clinical outcome. J Pediatr Surg 55(9):1829–1833

    Article  PubMed  Google Scholar 

  33. Brooks LA, Fowler KL, Veras LV (2020) Resection margin histology may predict intermediate-term outcomes in children with rectosigmoid Hirschsprung disease. Pediatr Surg Int 36(8):875–882

    Article  PubMed  PubMed Central  Google Scholar 

  34. Coyle D, O’Donnell AM, Tomuschat C (2019) The extent of the transition zone in Hirschsprung disease. J Pediatr Surg 54(11):2318–2324

    Article  PubMed  Google Scholar 

  35. Kapur RP, Smith C, Ambartsumyan L (2020) Postoperative pullthrough obstruction in Hirschsprung disease: etiologies and diagnosis. Pediatric Aand Developmental Pathology 23(1):40–59

    Article  Google Scholar 

  36. Langer JC, Rollins MD, Levitt M (2017) Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 33(5):523–526

    Article  CAS  PubMed  Google Scholar 

  37. Ghosh DN, Liu Y, Cass DT (2017) Transition zone pull-through in Hirschsprung’s disease: a tertiary hospital experience. ANZ J Surg 87(10):780–783

    Article  PubMed  Google Scholar 

  38. Fernandez IM, Sanchez MJ, Martinez CI (2014) Quality of life and long term results in Hirschsprung’s disease]. Cir Pediatr 27(3):117–124

    Google Scholar 

  39. Xiong X, Chen X, Wang G (2015) Long term quality of life in patients with Hirschsprung’s disease who underwent heart-shaped anastomosis during childhood: a twenty-year follow-up in China. J Pediatr Surg 50(12):2044–2047

    Article  PubMed  Google Scholar 

Download references

Funding

This study was funded by the Top of Jiangsu Provincial Health Committee’s Six One Project, China (LGY2020019); Jiangsu Provincial Key Research and Development Program, China (BE2017609); and the General Project of the Nanjing Health Commission (YKK20121).

Author information

Author notes

  1. Changgui Lu, Shiwen Pan and Xinyi Hua contributed equally.

Authors and Affiliations

  1. Department of Pediatric Surgery, Children’s Hospital of Nanjing Medical University, Nanjing, 210008, China

    Changgui Lu, Weiwei Jiang & Weibing Tang

  2. Department of Anesthesia and Operation, Children’s Hospital of Nanjing Medical University, Nanjing, 210008, China

    Shiwen Pan

  3. Nanjing Medical University, Nanjing, 210008, China

    Xinyi Hua

Authors
  1. Changgui Lu
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Shiwen Pan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Xinyi Hua
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Weiwei Jiang
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Weibing Tang
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Weibing Tang and Weiwei Jiang contributed to the conception and design of the study and were the corresponding authors. Shiwen Pan, Xinyi Hua, and Changgui Lu organized the database and performed statistical analyses. Changgui Lu wrote the first draft of the manuscript. Xinyi Hua, Shiwen Pan, and Changgui Lu contributed equally to the study. All authors contributed to the manuscript revision and read and approved the submitted version.

Corresponding authors

Correspondence to Weiwei Jiang or Weibing Tang.

Ethics declarations

Ethical approval

All procedures involving human participants were performed in accordance with the ethical standards of the institutional and/or national research committee and the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all the participants included in the study.

Competing interests

The authors declare no competing interests.

Additional information

Communicated by Peter de Winter.

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Lu, C., Pan, S., Hua, X. et al. Bowel function at preschool and early childhood age in children with long-segment Hirschsprung disease. Eur J Pediatr 182, 1251–1259 (2023). https://doi.org/10.1007/s00431-023-04814-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-023-04814-7

Keywords

Search

Navigation