Anal canal and sphincter function in children with Hirschsprung disease after definitive surgery


      • Some bowel dysfunction remains after pullthrough in the majority of patients with hirschsprung disease (HD).
      • Recto-anal inhibitory reflex (RAIR) is present in 37.5% patients after pullthrough.
      • Anal resting pressures (ARP) and reappearance of RAIR do not predict bowel continence or control during follow-up.



      Anorectal manometry may be useful to objectively evaluate anorectal function following definitive pullthrough for Hirschsprung Disease (HD) but there is little published data. Our study aims to investigate anorectal manometry findings and their association with bowel function.


      This was a prospective observational study. Convenience sampling method was used to recruit all HD patients who had definitive pullthrough from January 2019 to December2020 in our institution. High-resolution anorectal manometry (HRAM) was used to record anal resting pressure (ARP), length of high-pressure zone (HPZ), and presence/absence of recto-anal inhibitory reflex (RAIR). The Paediatric Incontinence/Constipation Scoring System (PICSS) was scored for all participants. PICSS is a validated questionnaire with scores mapped to an age-specific normogram to denote constipation, incontinence, and their combinations. Non-parametric and chi-square tests at significance p<0.05 were conducted to examine the relationship between PICSS categories and manometry findings. Ethical approval was obtained.


      There were 32 participants (30 boys). Median age at participation was 26.5 months (range: 13.8–156). Twenty-four (75%) had transanal pullthrough, 8(25%) underwent Duhamel procedure. PICSS scored 10(31.3%) as normal, 8(25%) as constipation, 10(31.3%) as incontinent, and 4(12.5%) as mixed. RAIR was present in 12 patients (37.5%). HPZ, maximum ARP, mean ARP were comparable across all PICSS groups without statistically significant differences. Presence of RAIR was not significantly associated with any PICSS groups (p = 0.13).


      Bowel function does not appear to be significantly associated with HRAM findings after definitive pullthrough for HD, but our study is limited by small sample size. RAIR was present in 37.5% patients after pullthrough.

      Level of Evidence

      Level II


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Gfroerer S.
        • Rolle U.
        Pediatric intestinal motility disorders.
        World J Gastroenterol. 2015; 21: 9683-9687
        • Engum S.A.
        • Grosfeld J.L.
        Long-term results of treatment of Hirschsprung's disease.
        Semin Pediatr Surg. 2004; 13: 273-285
        • Hadzic N.
        • Quaglia A.
        • Portmann B.
        • et al.
        Hepatocellular carcinoma in biliary atresia: King's College Hospital experience.
        J Pediatr. 2011; 159 (e1): 617-622
        • Marty T.L.
        • Seo T.
        • Matlak M.E.
        • et al.
        Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 135 patients.
        J Pediatr Surg. 1995; 30: 655-658
        • Rescorla F.J.
        • Morrison A.M.
        • Engles D.
        • et al.
        Hirschsprung's disease. Evaluation of mortality and long-term function in 260 cases.
        Arch Surg. 1992; 127 (discussion 41-2): 934-941
        • Rintala R.J.
        • Pakarinen M.P.
        Long-term outcomes of Hirschsprung's disease.
        Semin Pediatr Surg. 2012; 21: 336-343
        • Athanasakos E.
        • Cleeve S.
        • Thapar N.
        • et al.
        Anorectal manometry in children with defecation disorders.
        BSPGHAN Motility Working Group Consensus Statement. 2020; 32: e13797
        • Fichtner-Feigl S.
        • Sailer M.
        • Höcht B.
        • et al.
        Development of a new scoring system for the evaluation of incontinence and constipation in children.
        Coloproctology. 2003; 25: 10-15
        • Aworanti O.M.
        • McDowell D.T.
        • Martin I.M.
        • et al.
        Comparative review of functional outcomes post surgery for Hirschsprung's disease utilizing the paediatric incontinence and constipation scoring system.
        Pediatr Surg Int. 2012; 28: 1071-1078
        • Evans-Barns H.M.E.
        • Swannjo J.B.
        • Trajanovska M.
        • et al.
        Post-operative anorectal manometry in children with Hirschsprung disease: a systematic review.
        Neurogastroenterol Motil. 2021; : e14311
        • Banasiuk M.
        • Banaszkiewicz A.
        • Dziekiewicz M.
        • et al.
        Values from three-dimensional high-resolution anorectal manometry analysis of children without lower gastrointestinal symptoms.
        Clin Gastroenterol Hepatol. 2016; 14 (e3): 993-1000
        • Demirbag S.
        • Tiryaki T.
        • Purtuloglu T.
        Importance of anorectal manometry after definitive surgery for Hirschsprung's disease in children. 2013; 10: 1-4
        • Gad El-Hak N.A.
        • El-Hemaly M.M.
        • Negm E.H.
        • et al.
        Functional outcome after Swenson's operation for Hirshsprung's disease.
        Saudi J Gastroenterol. 2010; 16: 30-34
        • Tran V.Q.
        • Mahler T.
        • Bontems P.
        • et al.
        Interest of anorectal manometry during long-term follow-up of patients operated on for Hirschsprung's disease.
        J Neurogastroenterol Motil. 2018; 24: 70-78
        • Bjornland K.
        • Diseth T.H.
        • Emblem R.
        Long-term functional, manometric, and endosonographic evaluation of patients operated upon with the Duhamel technique.
        Pediatr Surg Int. 1998; 13: 24-28
        • Heikkinen M.
        • Rintala R.
        • Luukkonen P.
        Long-term anal sphincter performance after surgery for Hirschsprung's disease.
        J Pediatr Surg. 1997; 32: 1443-1446
        • Guinet A.
        • Verollet D.
        • Deffontaines Rufin S.
        • et al.
        Qualitative and quantitative analysis of rectoanal inhibitory reflex (RAIR) modulation in functional bowel disorders.
        Int J Colorectal Dis. 2011; 26: 501-505
        • Meunier P.
        • Mollard P.
        Control of the internal anal sphincter (manometric study with human subjects).
        Pflügers Archiv. 1977; 370: 233-239
        • Sangwan Y.P.
        • Solla J.A.
        Internal anal sphincter: advances and insights.
        Dis Colon Rectum. 1998; 41: 1297-1311
        • Cortesini C.
        • Paparozzi C.
        • Carassale G.
        • et al.
        Rectoanal reflex in healthy subject.
        Boll Soc Ital Biol Sper. 1979; 55: 877-883