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Clinical characteristics and influence of postoperative Hirschsprung-associated enterocolitis: retrospective study at a tertiary children’s hospital

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To explore the influence of postoperative Hirschsprung-associated enterocolitis (post-HAEC) on long-term outcomes and to identify risk factors of post-HAEC.


The medical records of 304 eligible patients diagnosed with Hirschsprung’s disease (HSCR) were reviewed. We analyzed the clinical characteristics of post-HAEC and its influence on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were identified separately.


The overall incidence of post-HAEC was 29.9% (91/304). We categorized early HAEC as occurring within postoperative 3 months (n = 39) and recurrent HAEC as occurring ≥ 3 episodes within postoperative 6 months (n = 25). Patients with early HAEC were more likely to experience worse nutritional status, defecation function, and quality of life compared to those with late or no episodes (P < 0.05). Similarly, the adverse influences of recurrent HAEC on these outcomes were also significant (P < 0.05). The risk factors for early HAEC included preoperative undernutrition, long-segment HSCR, and postoperative Grade 3–4 complications within 30 days. For recurrent HAEC, risk factors were preoperative malnutrition, non-parental caregivers, long-segment HSCR, and postoperative Grade 3–4 complications within 30 days.


Classification of post-HAEC based on the first episode time and frequency was necessary. The earlier or more frequent episodes of post-HAEC have detrimental influences on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were different.

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Data availability

The data presented in this study are available on request from the corresponding author.


  1. Montalva L, Cheng LS, Kapur R et al (2023) Hirschsprung disease. Nat Rev Dis Primers 9(1):54.

    Article  PubMed  Google Scholar 

  2. Beltman L, Labib H, Ahmed H et al (2023) Transition zone pull-through in patients with hirschsprung disease: is redo surgery beneficial for the long-term outcomes? J Pediatr Surg 58(10):1903–1909.

    Article  PubMed  Google Scholar 

  3. More K, Rao S, McMichael J et al (2014) Growth and developmental outcomes of infants with hirschsprung disease presenting in the neonatal period: a retrospective study. J Pediatr 165(1):73-77.e2.

    Article  PubMed  Google Scholar 

  4. Roorda D, Oosterlaan J, van Heurn E et al (2021) Risk factors for enterocolitis in patients with Hirschsprung disease: a retrospective observational study. J Pediatr Surg 56(10):1791–1798.

    Article  PubMed  Google Scholar 

  5. Yan J, Sun J, Wu R et al (2020) Barium enema findings in total colonic aganglionosis: a single-center, retrospective study. BMC Pediatr 20(1):499.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Kyrklund K, Sloots C, de Blaauw I et al (2020) ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease. Orphanet J Rare Dis 15(1):164.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Wood RJ, Garrison AP (2022) Total colonic aganglionosis in hirschsprung disease. Semin Pediatr Surg 31(2):151165.

    Article  PubMed  Google Scholar 

  8. Li S, Zhang Y, Li K et al (2023) Update on the pathogenesis of the Hirschsprung-associated enterocolitis. Int J Mol Sci 24(5):4602.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Chantakhow S, Tepmalai K, Singhavejsakul J et al (2023) Prognostic factors of postoperative Hirschsprung-associated enterocolitis: a cohort study. Pediatr Surg Int 39(1):77.

    Article  PubMed  Google Scholar 

  10. Liu J, Dong R, Chen G et al (2019) Risk factors and prognostic effects of cholangitis after kasai procedure in biliary atresia patients: a retrospective clinical study. J Pediatr Surg 54(12):2559–2564.

    Article  PubMed  Google Scholar 

  11. Li Z, Bai B, Ji G et al (2018) Relationship between Clavien-Dindo classification and long-term survival outcomes after curative resection for gastric cancer: a propensity score-matched analysis. Int J Surg 60:67–73.

    Article  PubMed  Google Scholar 

  12. Chung P, Yu M, Wong K et al (2019) Risk factors for the development of post-operative enterocolitis in short segment Hirschsprung’s disease. Pediatr Surg Int 35(2):187–191.

    Article  PubMed  Google Scholar 

  13. Huang SC, Chen JS, Cheng CN et al (2012) Hypoalbuminaemia is an independent predictor for hemophagocytic lymphohistiocytosis in childhood Epstein-Barr virus-associated infectious mononucleosis. Eur J Haematol 89(5):417–422.

    Article  PubMed  Google Scholar 

  14. McLean TW, Stewart RM, Curley TP et al (2020) Hypoalbuminemia in children with cancer treated with chemotherapy. Pediatr Blood Cancer 67(2):e28065.

    Article  PubMed  Google Scholar 

  15. Mertens A, Benjamin-Chung J, Colford JM Jr et al (2023) Child wasting and concurrent stunting in low- and middle-income countries. Nature 621(7979):558–567.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Monica Carissa T et al (2022) Long-term functional outcomes of patients with Hirschsprung disease following pull-through. BMC Pediatr 22(1):246.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Liu Z, Zhang Y, Li S et al (2023) Long-term bowel function after single-stage transanal endorectal pull-through in neonatal patients with Hirschsprung disease. Pediatr Surg Int 39(1):255.

    Article  PubMed  Google Scholar 

  18. Koo F, Chan M, King SK et al (2023) The early years: Hirschsprung disease and health-related quality of life. Qual Life Res 32(12):3327–3337.

    Article  PubMed  Google Scholar 

  19. Yamashita Y, Shirabe K, Tsuijita E et al (2013) Third or more repeat hepatectomy for recurrent hepatocellular carcinoma. Surgery 154(5):1038–1045.

    Article  PubMed  Google Scholar 

  20. Zhang X, Sun D, Xu Q et al (2023) Risk factors for Hirschsprung disease-associated enterocolitis: a systematic review and meta-analysis. Int J Surg 109(8):2509–2524.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Rosso M, Fremion E, Santoro SL et al (2020) Down syndrome disintegrative disorder: a clinical regression syndrome of increasing importance. Pediatrics 145(6):e20192939.

    Article  PubMed  Google Scholar 

  22. Wang D, Zhu T, Zhu L et al (2023) Screening of undernutrition in children with Hirschsprung disease using preoperative anthropometric parameters: a multicenter cross-sectional study. JPEN J Parenter Enteral Nutr 47(1):151–158.

    Article  CAS  PubMed  Google Scholar 

  23. Bechard LJ, Rothpletz-Puglia P, Touger-Decker R et al (2013) Influence of obesity on clinical outcomes in hospitalized children: a systematic review. JAMA Pediatr 167(5):476–482.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Conrey PE, Denu L, O’Boyle KC et al (2023) IgA deficiency destabilizes homeostasis toward intestinal microbes and increases systemic immune dysregulation. Sci Immunol.

    Article  PubMed  Google Scholar 

  25. Sun H, Bi J, Lei Q et al (2018) Partial enteral nutrition increases intestinal sIgA levels in mice undergoing parenteral nutrition in a dose-dependent manner. Int J Surg 49:74–79.

    Article  PubMed  Google Scholar 

  26. Jiao CL, Chen XY, Feng JX (2016) Novel insights into the pathogenesis of Hirschsprung’s-associated enterocolitis. Chin Med J (Engl) 129(12):1491–1497.

    Article  PubMed  Google Scholar 

  27. Parahita IG, Makhmudi A, Gunadi. (2018) Comparison of Hirschsprung-associated enterocolitis following soave and Duhamel procedures. J Pediatr Surg.

    Article  PubMed  Google Scholar 

  28. Xie C, Yan J, Zhang Z et al (2022) Risk factors for Hirschsprung-associated enterocolitis following soave: a retrospective study over a decade. BMC Pediatr 22(1):654.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Taylor MA, Bucher BT, Reeder RW et al (2021) Comparison of Hirschsprung disease characteristics between those with a history of postoperative enterocolitis and those without: results from the pediatric colorectal and pelvic learning consortium. Eur J Pediatr Surg 31(3):207–213.

    Article  PubMed  Google Scholar 

  30. Le-Nguyen A, Righini-Grunder F, Piché N et al (2019) Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC). J Pediatr Surg 54(5):959–963.

    Article  PubMed  Google Scholar 

  31. Zhang GW, Lin JH, Qian JP et al (2014) Analyzing risk factors for early postoperative bile leakage based on Clavien classification in bile duct stones. Int J Surg 12(8):757–761.

    Article  PubMed  Google Scholar 

  32. Rohit A, Kirkham R, McCarthy L et al (2021) Exploring differences in perceptions of child feeding practices between parents and health care professionals: a qualitative study. BMC Public Health 21(1):1449.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Harvey L, Bryant-Waugh R, Watkins B et al (2015) Parental perceptions of childhood feeding problems. J Child Health Care 19(3):392–401.

    Article  PubMed  Google Scholar 

  34. Knaus ME, Pendola G, Srinivas S et al (2023) Social determinants of health and Hirschsprung-associated enterocolitis. J Pediatr Surg 58(8):1458–1462.

    Article  PubMed  Google Scholar 

  35. Lardner DR, Dick BD, Crawford S (2010) The effects of parental presence in the postanesthetic care unit on children’s postoperative behavior: a prospective, randomized, controlled study. Anesth Analg 110(4):1102–1108.

    Article  PubMed  Google Scholar 

  36. Pastor AC, Osman F, Teitelbaum DH et al (2009) Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg 44(1):251–256.

    Article  PubMed  Google Scholar 

  37. Frykman PK, Kim S, Wester T et al (2018) Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: a multicenter study of 116 children with Hirschsprung disease. J Pediatr Surg 53(4):708–717.

    Article  PubMed  Google Scholar 

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This study was supported by research grants from the National Clinical Research Center for Child Health and Disorders, Children’s Hospital of Chongqing Medical University (No.NCRCCHD-2022-GP-03) and Program for Youth Innovation in Future Medicine, Chongqing Medical University (No.W0125).

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Authors and Affiliations



All the authors contributed to the study’s conception and design. Data collection and analysis were performed by Wei Feng and Yi Wang. The first draft of the manuscript was written by Wei Feng and Linxiao Fan, and all the authors commented on previous versions of the manuscript. All the authors approved the final manuscript as submitted.

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Correspondence to Zhenhua Guo.

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The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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The study was approved by the Institutional Research Ethics Board of Children’s Hospital affiliated Chongqing Medical University (Date: 2021/No: 391).

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Feng, W., Zhang, B., Fan, L. et al. Clinical characteristics and influence of postoperative Hirschsprung-associated enterocolitis: retrospective study at a tertiary children’s hospital. Pediatr Surg Int 40, 106 (2024).

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