Skip to main content Skip to main navigation menu Skip to site footer

Long-term assessment of functional outcome in operated cases of Hirschsprung disease

  • Hery Poerwosusanta ,
  • Nathan Aditya Willyanto ,


Background: Hirschsprung disease (HD) is a rare congenital anomaly (1 in 5000 live births) characterized by varying levels of aganglionosis. The evolution of surgical options and outcomes for the successful management of HD has been driven by the aspiration of pediatric surgeons. Hirschsprung's disease postoperative results of pull-through procedure could be influenced by surgical and pathological proficiency and the quality of perioperative care. Controversies persist as to which technique offers the best outcome. The absence of incontinence and constipation remains the most important marker of a good outcome. 

Methods: Medical records of HD children who underwent temporary colostomy procedures were analyzed retrospectively from July 2017 to June 2021. Risk factors were evaluated to determine the outcome of HD procedures.

Results: Ninety-two HD patients were included. Based on the univariate analysis, most patients in the study (58.7%) were between the ages of 2 and 12 months. The study population predominantly consisted of males (68.5%) who had low vegetable intake (65.2%) and occasionally exhibited stool stains (66.3%), but their stool consistency was mainly normal (47.8%). Further examination through bivariate analysis revealed a significant negative correlation between stool stains and SOAVE (name of the condition). Similar findings were observed for stool consistency, indicating a significant correlation. Consequently, both variables were subjected to multivariate analysis. The multivariate analysis demonstrated a significant association between stool consistency and SOAVE among patients aged 2-12 months (p=0.000, OR =2.044E-7) and those aged 1-5 years (p=0.000, OR =2.044E-7). An odds ratio (OR) value of less than 1 implies that individuals with normal stool consistency have a lower likelihood of experiencing SOAVE at a young age.

Conclusions: This study showed that children with HD correlated with low daily vegetable intake. Normal feces were associated with higher SOAVE ages and a lower possibility of experiencing SOAVE at a young age.


  1. Loganathan AK, Mathew AS, Kurian JJ. Assessment of Quality of Life and Functional Outcomes of Operated Cases of Hirschsprung Disease in a Developing Country. Pediatr Gastroenterol Hepatol Nutr. 2021;24(2):145–53.
  2. Apfeld JC, Wood RJ, Halleran DR, Deans KJ, Minneci PC, Cooper JN. Relationships Between Hospital and Surgeon Operative Volumes and Surgical Outcomes in Hirschsprungs Disease. Journal of Surgical Research. 2021;257:379–88.
  3. Wester T, Granström AL. Hirschsprung disease—Bowel function beyond childhood. Semin Pediatr Surg. 2017;26(5):322–7.
  4. Diposarosa R, Bustam NA, Sahiratmadja E, Susanto PS, Sribudiani Y. Literature review: enteric nervous system development, genetic and epigenetic regulation in the etiology of Hirschsprung’s disease. Vol. 7, Heliyon. Elsevier Ltd; 2021.
  5. Gunadi, Monica Carissa T, Stevie, Daulay EF, Yulianda D, Iskandar K, et al. Long-term functional outcomes of patients with Hirschsprung disease following pull-through. BMC Pediatr. 2022;22(1).
  6. Haikal Z, Dwihantoro A, Gunarti H, Gunadi. Accuracy of transition zone in contrast enema to predict intraoperative aganglionosis level in patients with Hirschsprung disease. BMC Res Notes. 2020;13(1).
  7. Peters N, Menon P, N. Rao K, Samujh R. Modified duhamel’s two-staged procedure for hirschsprung’s disease: Further modifications for improved outcomes. J Indian Assoc Pediatr Surg. 2020;25(5):269.
  8. Aworanti OM, Mcdowell DT, Martin IM, Hung J, Quinn F. Comparative review of functional outcomes post surgery for Hirschsprung’s disease utilizing the paediatric incontinence and constipation scoring system. Vol. 28, Pediatric Surgery International. 2012. p. 1071–8.
  9. Verkuijl SJ, Meinds RJ, Van Der Steeg AFW, Van Gemert WG, De Blaauw I, Witvliet MJ, et al. Functional Outcomes after Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung Disease. J Pediatr Gastroenterol Nutr. 2022;74(3):348–54.
  10. Aworanti OM, McDowell DT, Martin IM, Quinn F. Does Functional Outcome Improve with Time Postsurgery for Hirschsprung Disease? European Journal of Pediatric Surgery. 2016;26(2):192–9.
  11. Dai Y, Deng Y, Lin Y, Ouyang R, Li L. Long-term outcomes and quality of life of patients with Hirschsprung disease: A systematic review and meta-analysis. BMC Gastroenterol. 2020;20(1).
  12. Zakaria OM. Bowel function and fecal continence after Soave’s trans-anal endorectal pull-through for Hirschsprung’s disease: A local experience. Updates Surg. 2012;64(2):113–8.
  13. Gunadi, Sukarelawanto AVR, Ritana A, Balela N, Putri WJK, Sirait DN, et al. Postoperative enterocolitis assessment using two different cut-off values in the HAEC score in Hirschsprung patients undergoing Duhamel and Soave pull-through. BMC Pediatr. 2020;20(1).
  14. Drissi F, Meurette G, Baayen C, Wyart V, Cretolle C, Guinot A, et al. Long-term Outcome of Hirschsprung Disease: Impact on Quality of Life and Social Condition at Adult Age. In: Diseases of the Colon and Rectum. Lippincott Williams and Wilkins; 2019. p. 727–32.
  15. Bhama AR, Steele SR. Anal Incontinence: Etiology and Clinical Treatment. In: Anorectal Physiology. Springer International Publishing; 2020. p. 175–83.
  16. Collins L, Collis B, Trajanovska M, Khanal R, Hutson JM, Teague WJ, et al. Quality of life outcomes in children with Hirschsprung disease. J Pediatr Surg. 2017;52(12):2006–10.
  17. Saysoo MR, Dewi FST, Gunadi. Quality of life of patients with Hirschsprung disease after Duhamel and Soave pull-through procedures: A mixed-methods sequential explanatory cohort study. Annals of Medicine and Surgery. 2020;56:34–7.
  18. Westfal ML, Okiemy O, Chung PHY, Feng J, Lu C, Miyano G, et al. Optimal timing for Soave primary pull-through in short-segment Hirschsprung disease: A meta-analysis. J Pediatr Surg. 2022;57(4):719–25.
  19. Vrsansky P, Bourdelat D, Pagès R. Principal modifications of the Duhamel procedure in the treatment of Hirschsprung’s disease. Analysis based on results of an international retrospective study of 2,430 patients. Pediatr Surg Int. 1998;13(2):125–32. Available from:
  20. Lu C, Hou G, Liu C, Geng Q, Xu X, Zhang J, et al. Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates: A multicenter study. J Pediatr Surg. 2017;52(7):1102–7.
  21. Heikkinen M, Rintala RJ, Louhimo I. Bowel function and quality of life in adult patients with operated Hirschsprung’s disease. Pediatr Surg Int. 1995;10(5):342–4. Available from:
  22. Drissi F, Meurette G, Baayen C, Wyart V, Cretolle C, Guinot A, et al. Long-term Outcome of Hirschsprung Disease: Impact on Quality of Life and Social Condition at Adult Age. Dis Colon Rectum. 2019;62(6). Available from:
  23. Davidson JR, Kyrklund K, Eaton S, Pakarinen MP, Thompson DS, Cross K, et al. Long-term surgical and patient-reported outcomes of Hirschsprung Disease. J Pediatr Surg. 2021;56(9):1502–11.

How to Cite

Poerwosusanta, H., & Willyanto, N. A. (2024). Long-term assessment of functional outcome in operated cases of Hirschsprung disease. Intisari Sains Medis, 15(1), 476–481.




Search Panel

Hery Poerwosusanta
Google Scholar
ISM Journal

Nathan Aditya Willyanto
Google Scholar
ISM Journal