Skip to main content

Advertisement

SpringerLink
Log in

Assessment of long-term quality of life, bowel and voiding function outcomes in patients with anorectal malformation at a single UK centre

  • Original Article
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Aims

Assess long-term quality of life (QoL), bowel and voiding function in anorectal malformation (ARM) paediatric patients.

Method

Retrospective review of ARM patients between 2007 and 2020 was performed. QoL (all patients), bowel and voiding function (> 5 yo) were assessed using the paediatric quality of life inventory (PedsQL), paediatric incontinence and constipation score (PICS) and dysfunctional voiding scoring system (DVSS), respectively.

Results

There were 122 patients (49% female, 85 > 5 yo) with ARM. Two had died, four refused, twenty-two were non-contactable, leaving ninety-four patients (65 > 5 yo) included. Mean age was 89 months (19–183), and follow-up was 86 months (13–183). Patients had significantly poorer scores for QoL, bowel and voiding function compared to published healthy controls. 57% had poor bowel function, 32% had poor voiding function and 38% required ‘ancillary aids’ to facilitate function. Patients using ‘ancillary aids’ for voiding function had a significantly lower QoL (parent: 62 vs 77; p = 0.01, patient: 66 vs 79; p = 0.05). Bowel continence was worse in those with high vs low ARM (13 vs 20, p = 0.004) and timely vs delayed diagnosis (17 vs 24, p = 0.04).

Conclusion

Patients with ARM have significantly worse QoL, bowel and voiding function than normal healthy controls. There is a need for long-term monitoring of function and further support for these children.

Level of evidence

III.

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
Fig. 2

Similar content being viewed by others

Data availability

No datasets were generated or analysed during the current study.

References

  1. deVries PA, Peña A (1982) Posterior sagittal anorectoplasty. J Pediatr Surg 17(5):638–643. https://doi.org/10.1016/S0022-3468(82)80126-7

    Article  CAS  PubMed  Google Scholar 

  2. Levitt MA, Peña A (2007) Anorectal malformations. Orphanet J Rare Dis 2(1):33. https://doi.org/10.1186/1750-1172-2-33

    Article  PubMed  PubMed Central  Google Scholar 

  3. Giuliani S, Grano C, Aminoff D, Schwarzer N, Van De Vorle M, Cretolle C, Haanen M, Brisighelli G, Marzheuser S, Connor M (2017) Transition of care in patients with anorectal malformations: consensus by the ARM-net consortium. J Pediatr Surg 52(11):1866–1872. https://doi.org/10.1016/j.jpedsurg.2017.06.008

    Article  PubMed  Google Scholar 

  4. Zheng H, Liu G, Liang Z, Chen Y, Wen Z, Yu J, Xu X, Liang H, Wang Y (2019) Middle-term bowel function and quality of life in low-type anorectal malformation. Ital J Pediatr 45(1):98. https://doi.org/10.1186/s13052-019-0701-3

    Article  PubMed  PubMed Central  Google Scholar 

  5. Wigander H, Nisell M, Frenckner B, Wester T, Brodin U, Öjmyr-Joelsson M (2019) Quality of life and functional outcome in Swedish children with low anorectal malformations: a follow-up study. Pediatr Surg Int 35(5):583–590. https://doi.org/10.1007/s00383-018-04431-8

    Article  PubMed  PubMed Central  Google Scholar 

  6. Grano C, Fernandes M, Bucci S, Aminoff D, Lucidi F, Violani C (2018) Self-efficacy beliefs, faecal incontinence and health-related quality of life in patients born with anorectal malformations. Colorectal Dis 20(8):711–718. https://doi.org/10.1111/codi.14259

    Article  CAS  PubMed  Google Scholar 

  7. Nah SA, Ong CCP, Saffari SE, Ong LY, Yap T-L, Low Y, Jacobsen AS (2018) Anorectal malformation & Hirschsprung’s disease: a cross-sectional comparison of quality of life and bowel function to healthy controls. J Pediatr Surg 53(8):1550–1554. https://doi.org/10.1016/j.jpedsurg.2017.08.018

    Article  PubMed  Google Scholar 

  8. Kyrklund K, Neuvonen M, Pakarinen M, Rintala R (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(06):522–528. https://doi.org/10.1055/s-0037-1607356

    Article  PubMed  Google Scholar 

  9. Danielson J, Karlbom U, Graf W, Wester T (2017) Outcome in adults with anorectal malformations in relation to modern classification–which patients do we need to follow beyond childhood? J Pediatr Surg 52(3):463–468. https://doi.org/10.1016/j.jpedsurg.2016.10.051

    Article  PubMed  Google Scholar 

  10. Tannuri ACA, Ferreira MAE, Mathias AL, Tannuri U (2016) Long-term evaluation of fecal continence and quality of life in patients operated for anorectal malformations. Rev Assoc Med Bras 62(6):544–552. https://doi.org/10.1590/1806-9282.62.06.544

    Article  PubMed  Google Scholar 

  11. Grano C, Bucci S, Aminoff D, Lucidi F, Violani C (2015) Transition from childhood to adolescence: quality of life changes 6 years later in patients born with anorectal malformations. Pediatr Surg Int 31(8):735–740. https://doi.org/10.1007/s00383-015-3736-6

    Article  PubMed  Google Scholar 

  12. Grano C, Bucci S, Aminoff D, Lucidi F, Violani C (2013) Quality of life in children and adolescents with anorectal malformation. Pediatr Surg Int 29(9):925–930. https://doi.org/10.1007/s00383-013-3359-8

    Article  PubMed  Google Scholar 

  13. Athanasakos EP, Kemal KI, Malliwal RS, Scott SM, Williams NS, Aziz Q, Ward HC, Knowles CH (2013) Clinical and psychosocial functioning in adolescents and young adults with anorectal malformations and chronic idiopathic constipation. Br J Surg 100(6):832–839. https://doi.org/10.1002/bjs.9111

    Article  CAS  PubMed  Google Scholar 

  14. Grano C, Aminoff D, Lucidi F, Violani C (2012) Long-term disease-specific quality of life in children and adolescent patients with ARM. J Pediatr Surg 47(7):1317–1322. https://doi.org/10.1016/j.jpedsurg.2012.01.068

    Article  PubMed  Google Scholar 

  15. Grano C, Aminoff D, Lucidi F, Violani C (2011) Long-term disease-specific quality of life in adult anorectal malformation patients. J Pediatr Surg 46(4):691–698. https://doi.org/10.1016/j.jpedsurg.2010.10.016

    Article  PubMed  Google Scholar 

  16. Hashish MS, Dawoud HH, Hirschl RB, Bruch SW, El Batarny AM, Mychaliska GB, Drongowski RA, Ehrlich PF, Hassaballa SZ, El-Dosuky NI, Teitelbaum DH (2010) Long-term functional outcome and quality of life in patients with high imperforate anus. J Pediatr Surg 45(1):224–230. https://doi.org/10.1016/j.jpedsurg.2009.10.041

    Article  PubMed  Google Scholar 

  17. Senel E, Akbiyik F, Atayurt H, Tiryaki HT (2010) Urological problems or fecal continence during long-term follow-up of patients with anorectal malformation. Pediatr Surg Int 26(7):683–689. https://doi.org/10.1007/s00383-010-2626-1

    Article  PubMed  Google Scholar 

  18. Hartman EE, Oort FJ, Sprangers MA, Hanneman MJ, van Heurn LE, de Langen ZJ, Madern GC, Rieu PN, van der Zee DC, Looyaard N, van Silfhout-Bezemer M, Aronson DC (2008) Factors affecting quality of life of children and adolescents with anorectal malformations or hirschsprung disease. J Pediatr Gastroenterol Nutr 47(4):463–471. https://doi.org/10.1097/MPG.0b013e31815ce545

    Article  PubMed  Google Scholar 

  19. Iwai N, Deguchi E, Kimura O, Kubota Y, Ono S, Shimadera S (2007) Social quality of life for adult patients with anorectal malformations. J Pediatr Surg 42(2):313–317. https://doi.org/10.1016/j.jpedsurg.2006.10.004

    Article  PubMed  Google Scholar 

  20. Goyal A, Williams JM, Kenny SE, Lwin R, Baillie CT, Lamont GL, Turnock RR (2006) Functional outcome and quality of life in anorectal malformations. J Pediatr Surg 41(2):318–322. https://doi.org/10.1016/j.jpedsurg.2005.11.006

    Article  PubMed  Google Scholar 

  21. Hartman EE, Oort FJ, Aronson DC, Hanneman MJG, van der Zee DC, Rieu PNMA, Madern GC, De Langen ZJ, van Heurn LWE, van Silfhout-Bezemer M, Looyaard N, Sprangers MAG (2004) Critical factors affecting quality of life of adult patients with anorectal malformations or hirschsprung’s disease. Am J Gastroenterol 99(5):907–913. https://doi.org/10.1111/j.1572-0241.2004.04149.x

    Article  PubMed  Google Scholar 

  22. Bai Y, Yuan Z, Wang W, Zhao Y, Wang H, Wang W (2000) Quality of life for children with fecal incontinence after surgically corrected anorectal malformation. J Pediatr Surg 35(3):462–464. https://doi.org/10.1016/S0022-3468(00)90215-X

    Article  CAS  PubMed  Google Scholar 

  23. Hassink EAM, Rieu PNMA, Brugman ATM, Festen C (1994) Quality of life after operatively corrected high anorectal malformation: a long-term follow-up study of patients aged 18 years and older. J Pediatr Surg 29(6):773–776. https://doi.org/10.1016/0022-3468(94)90367-0

    Article  CAS  PubMed  Google Scholar 

  24. Rintala R, Mildh L, Lindahl H (1992) Fecal continence and quality of life in adult patients with an operated low anorectal malformation. J Pediatr Surg 27(7):902–905. https://doi.org/10.1016/0022-3468(92)90394-M

    Article  CAS  PubMed  Google Scholar 

  25. Goossens WJH, de Blaauw I, Wijnen MH, de Gier RPE, Kortmann B, Feitz WFJ (2011) Urological anomalies in anorectal malformations in the Netherlands: effects of screening all patients on long-term outcome. Pediatr Surg Int 27(10):1091–1097. https://doi.org/10.1007/s00383-011-2959-4

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Fuchs ME, Halleran DR, Shin YJ, Sebastião Y, Weaver L, Ahmad H, Booth K, Ching CB, Levitt MA, Wood RJ, Dajusta DG (2020) Anatomic factors predict urinary continence in patient with anorectal malformation. J Pediatr Urol 16(5):545.e1-545.e7. https://doi.org/10.1016/j.jpurol.2020.06.011

    Article  PubMed  Google Scholar 

  27. Bjoersum-Meyer T, Kaalby L, Lund L, Christensen P, Jakobsen MS, Baatrup G, Qvist N, Ellebaek M (2021) Long-term functional urinary and sexual outcomes in patients with anorectal malformations–a systematic review. Eur Urol Open Sci 25:29–38. https://doi.org/10.1016/j.euros.2021.01.007

    Article  PubMed  PubMed Central  Google Scholar 

  28. Chong C, Hamza Y, Tan YW, Paul A, Garriboli M, Wright AJ, Olsburgh J, Taylor C, Sinha MD, Mishra P, Taghizadeh A (2022) Long-term urology outcomes of anorectal malformation. J Pediatr Urol 18(2):150.e1-150.e6. https://doi.org/10.1016/j.jpurol.2022.01.019

    Article  PubMed  Google Scholar 

  29. Giuliani S, Decker E, Leva E, Riccipetitoni G, Bagolan P (2016) Long term follow-up and transition of care in anorectal malformations: an international survey. J Pediatr Surg 51(9):1450–1457. https://doi.org/10.1016/j.jpedsurg.2016.03.011

    Article  PubMed  Google Scholar 

  30. Varni JW, Burwinkle TM, Seid M, Skarr D (2003) The PedsQLTM* 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Peditr 3(6):329–341

    Article  Google Scholar 

  31. Fichtner-Feigl S, Sailer M, Höcht B, Thiede A (2003) Development of a new scoring system for the evaluation of incontinence and constipation in children. Colo-Proctol 25(1):10–15. https://doi.org/10.1007/s00053-003-5084-6

    Article  Google Scholar 

  32. Akbal C, Genc Y, Burgu B, Ozden E, Tekgul S (2005) Dysfunctional voiding and incontinence scoring system: quantitative evaluation of incontinence symptoms in pediatric population. J Urol 173(3):969–973. https://doi.org/10.1097/01.ju.0000152183.91888.f6

    Article  PubMed  Google Scholar 

  33. Varni JW, Junger KF, Kellermann T, Grossman LB, Wagner J, Mucci GA, Guilfoyle SM, Smith G, Zupanc ML, Modi AC (2020) PedsQL™ cognitive functioning scale in youth with epilepsy: reliability and validity. Epilepsy Behav 103:106850. https://doi.org/10.1016/j.yebeh.2019.106850

    Article  PubMed  Google Scholar 

  34. Akoglu H (2018) User’s guide to correlation coefficients. Turk J Emerg Med 18(3):91–93. https://doi.org/10.1016/j.tjem.2018.08.001

    Article  PubMed  PubMed Central  Google Scholar 

  35. Chan YH (2003) Biostatistics 104: correlational analysis. Singap Med J 44(12):614–619

    CAS  Google Scholar 

  36. Raman VS, Agarwala S, Bhatnagar V (2017) Correlation between quality of life and functional outcomes in operated children with anorectal malformations using the krickenbeck consensus. Ind J Pediatr 84(3):177–182. https://doi.org/10.1007/s12098-016-2269-x

    Article  Google Scholar 

  37. Borg HC, Holmdahl G, Gustavsson K, Doroszkiewicz M, Sillén U (2013) Longitudinal study of bowel function in children with anorectal malformations. J Pediatr Surg 48(3):597–606. https://doi.org/10.1016/j.jpedsurg.2012.10.056

    Article  PubMed  Google Scholar 

  38. van der Steeg HJJ, van Rooij IALM, Iacobelli BD, Sloots CEJ, Morandi A, Broens PMA, Makedonsky I, Leon FF, Schmiedeke E, Vázquez AG, Miserez M, Lisi G, Midrio P, Amerstorfer EE, Fanjul M, Ludwiczek J, Stenström P, van der Steeg AFW, de Blaauw I (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. https://doi.org/10.1016/j.jpedsurg.2022.02.015

    Article  PubMed  Google Scholar 

  39. Witvliet MJ, Slaar A, Heij HA, van der Steeg AFW (2013) Qualitative analysis of studies concerning quality of life in children and adults with anorectal malformations. J Pediatr Surg 48(2):372–379. https://doi.org/10.1016/j.jpedsurg.2012.11.018

    Article  PubMed  Google Scholar 

  40. Hartman EE, Oort FJ, Aronson DC, Sprangers MA (2011) Quality of life and disease-specific functioning of patients with anorectal malformations or hirschsprung’s disease: a review. Arch Dis Child 96(4):398–406. https://doi.org/10.1136/adc.2007.118133

    Article  CAS  PubMed  Google Scholar 

  41. Oltean I, Hayawi L, Larocca V, Bijelić V, Beveridge E, Kaur M, Grandpierre V, Kanyinda J, Nasr A (2022) Quality of life outcomes in children after surgery for hirschsprung disease and anorectal malformations: a systematic review and meta-analysis. World J Pediatr Surg 5(4):e000447. https://doi.org/10.1136/wjps-2022-000447

    Article  PubMed  PubMed Central  Google Scholar 

  42. van Tilburg MAL, Murphy TB (2015) Quality of life paradox in gastrointestinal disorders. J Pediatr 166(1):11–14. https://doi.org/10.1016/j.jpeds.2014.09.043

    Article  PubMed  Google Scholar 

  43. Oyania F, Ogwal A, Nimanya S, Muzira A, Kakembo N, Kisa P, Sekabira J (2020) Long term bowel function after repair of anorectal malformations in Uganda. J Pediatr Surg 55(7):1400–1404. https://doi.org/10.1016/j.jpedsurg.2019.11.015

    Article  PubMed  Google Scholar 

  44. Rice-Townsend SE, Nicassio L, Glazer D, Avansino J, Durham MM, Frischer J, Calkins C, Rentea RM, Ralls M, Fuller M, Wood RJ, Rollins M, Lee J, Lewis KE, Reeder RW, Smith CA (2023) Fecal continence outcomes and potential disparities for patients with anorectal malformations treated at referral institutions for pediatric colorectal surgery. Pediatr Surg Int 39(1):157. https://doi.org/10.1007/s00383-023-05447-5

    Article  PubMed  Google Scholar 

  45. Totonelli G, Catania VD, Morini F, Fusaro F, Mosiello G, Iacobelli BD, Bagolan P (2015) VACTERL association in anorectal malformation: effect on the outcome. Pediatr Surg Int 31(9):805–808. https://doi.org/10.1007/s00383-015-3745-5

    Article  PubMed  Google Scholar 

  46. Davies MC, Liao L-M, Wilcox DT, Woodhouse CRJ, Creighton SM (2010) Anorectal malformations: what happens in adulthood? BJU Int 106(3):398–404. https://doi.org/10.1111/j.1464-410X.2009.09031.x

    Article  PubMed  Google Scholar 

  47. Har AF, Rescorla FJ, Croffie JM (2013) Quality of life in pediatric patients with unremitting constipation pre and post malone antegrade continence enema (MACE) procedure. J Pediatr Surg 48(8):1733–1737. https://doi.org/10.1016/j.jpedsurg.2013.01.045

    Article  PubMed  Google Scholar 

  48. Ng J, Ford K, Dalton S, McDowell S, Charlesworth P, Cleeve S (2015) Transanal irrigation for intractable faecal incontinence and constipation: outcomes, quality of life and predicting non-adopters. Pediatr Surg Int 31(8):729–734. https://doi.org/10.1007/s00383-015-3735-7

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We wish to thank all the parents and children who responded to the questionnaires for this study. We wish to thank Mrs Kathleen Baster, Principal Consultant, Statistical Services Unit, University of Sheffield, for her guidance in analysing the data for this study.

Funding

Financial support statement: this research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.

Author information

Authors and Affiliations

  1. Paediatric Surgical Unit, Sheffield Children’s NHS Foundation Trust, Sheffield, S10 2TH, UK

    Harriet Beattie, Thejasvi Subramanian, Elizabeth Scudamore, Thomas Middleton, Caroline MacDonald, Richard Lindley & Govind Murthi

  2. The Medical School, University of Sheffield, Sheffield, S10 2RX, UK

    Harriet Beattie

Authors
  1. Harriet Beattie
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Thejasvi Subramanian
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Elizabeth Scudamore
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Thomas Middleton
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Caroline MacDonald
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Richard Lindley
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Govind Murthi
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. Data collection and analysis were performed by H.D., T.S., E.S. and T.M. The main manuscript was written by H.B., and all authors reviewed and commented on versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Govind Murthi.

Ethics declarations

Conflict of interest

The authors have no relevant financial or non-financial interests to disclose.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Beattie, H., Subramanian, T., Scudamore, E. et al. Assessment of long-term quality of life, bowel and voiding function outcomes in patients with anorectal malformation at a single UK centre. Pediatr Surg Int 40, 95 (2024). https://doi.org/10.1007/s00383-024-05684-2

Download citation

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00383-024-05684-2

Keywords

Search

Navigation