Transition and Reversal: Long-term Experience of the MACE Procedure in Children

Overview

Journal Pediatr Surg Int

Specialties General Surgery
Pediatrics

Date 2022 Apr 8

PMID 35394166

Authors

Riyad Peeraully

Jason Langley

Nicola Hayes

Kate Storry

Louise Richardson

Demelza Burridge

Bharat More

Affiliations

Soon will be listed here.

Abstract

Purpose: Analysis of outcomes and follow-up of children who underwent the Malone antegrade continence enema (MACE) procedure in a UK tertiary paediatric surgery unit.

Methods: Children who underwent a MACE procedure from 1998 to 2020 were identified. Demographic and clinical data were obtained from contemporaneous records. Outcomes were categorised as full (success), partial or failure.

Results: Ninety-five children were identified for inclusion (chronic idiopathic constipation (CIC, 59), anorectal malformations (ARM, 23) and Hirschsprung's disease (HD, 13)). Mean age at surgery was 9.4 years (3-19 years) and mean follow-up time was 6 years (0.3-16.8 years). Outcomes were successful in 69% of CIC patients, 78% in ARM and 69% in HD. Twenty (21%) underwent MACE reversal after developing independent continence, with a significant difference between groups (CIC 19%, ARM 9%, HD 54%, p = 0.0047). 50% of patients > 16 years old were transitioned to adult services.

Conclusion: We report a success rate of 72% for MACE procedures in our unit, with a significant difference in reversal rate between diagnostic groups. Long term, a fifth of patients no longer required their MACE. When these patients reach adolescence, those who require ongoing support outside of the paediatric surgery setting should be safely transitioned to adult services.

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References

Malone P, Ransley P, Kiely E . Preliminary report: the antegrade continence enema. Lancet. 1990; 336(8725):1217-8. DOI: 10.1016/0140-6736(90)92834-5. View

Masadeh M, Krein M, Peterson J, Bauer M, Phearman L, Pitcher G . Outcome of antegrade continent enema (ACE) procedures in children and young adults. J Pediatr Surg. 2013; 48(10):2128-33. DOI: 10.1016/j.jpedsurg.2013.04.009. View

Randall J, Coyne P, Jaffray B . Follow up of children undergoing antegrade continent enema: experience of over two hundred cases. J Pediatr Surg. 2014; 49(9):1405-8. DOI: 10.1016/j.jpedsurg.2014.02.090. View

Curry J, Osborne A, Malone P . The MACE procedure: experience in the United Kingdom. J Pediatr Surg. 1999; 34(2):338-40. DOI: 10.1016/s0022-3468(99)90204-x. View

Har A, Rescorla F, Croffie J . Quality of life in pediatric patients with unremitting constipation pre and post Malone Antegrade Continence Enema (MACE) procedure. J Pediatr Surg. 2013; 48(8):1733-7. DOI: 10.1016/j.jpedsurg.2013.01.045. View

Jones A, John M, Singh S, Williams A . Transition from paediatric surgery: how many patients do we need to plan for?. Ann R Coll Surg Engl. 2016; 98(8):586-588. PMC: 5392899. DOI: 10.1308/rcsann.2016.0275. View

Peeraully M, Lopes J, Wright A, Davies B, Stewart R, Singh S . Experience of the MACE procedure at a regional pediatric surgical unit: a 15-year retrospective review. Eur J Pediatr Surg. 2014; 24(1):113-6. DOI: 10.1055/s-0033-1357502. View

Dindo D, Demartines N, Clavien P . Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. 2004; 240(2):205-13. PMC: 1360123. DOI: 10.1097/01.sla.0000133083.54934.ae. View

Kyrklund K, Pakarinen M, Rintala R . Long-term bowel function, quality of life and sexual function in patients with anorectal malformations treated during the PSARP era. Semin Pediatr Surg. 2017; 26(5):336-342. DOI: 10.1053/j.sempedsurg.2017.09.010. View

10.

Svetanoff W, Kapalu C, Lopez J, Fraser J, Briggs K, Rentea R . Psychosocial factors affecting quality of life in patients with anorectal malformation and Hirschsprung disease-a qualitative systematic review. J Pediatr Surg. 2021; 57(3):387-393. DOI: 10.1016/j.jpedsurg.2021.05.004. View

11.

Khoo A, Askouni E, Basson S, Ng J, Cleeve S . How long will I have my ACE? The natural history of the antegrade continence enema stoma in idiopathic constipation. Pediatr Surg Int. 2017; 33(11):1159-1166. DOI: 10.1007/s00383-017-4128-x. View

12.

Rigby E, Hagell A, Davis M, Gleeson H, Mathews G, Turner G . Getting health services right for 16-25 year-olds. Arch Dis Child. 2020; 106(1):9-13. DOI: 10.1136/archdischild-2019-318648. View

13.

Chong C, Featherstone N, Sharif S, Cherian A, Cuckow P, Mushtaq I . 5 years after an ACE: what happens then?. Pediatr Surg Int. 2016; 32(4):397-401. DOI: 10.1007/s00383-016-3857-6. View

14.

Dolejs S, Smith Jr J, Sheplock J, Croffie J, Rescorla F . Contemporary short- and long-term outcomes in patients with unremitting constipation and fecal incontinence treated with an antegrade continence enema. J Pediatr Surg. 2016; 52(1):79-83. DOI: 10.1016/j.jpedsurg.2016.10.022. View

15.

King S, Sutcliffe J, Southwell B, Chait P, Hutson J . The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg. 2005; 40(12):1935-40. DOI: 10.1016/j.jpedsurg.2005.08.011. View

16.

Mugie S, Machado R, Mousa H, Punati J, Hogan M, Benninga M . Ten-year experience using antegrade enemas in children. J Pediatr. 2012; 161(4):700-4. DOI: 10.1016/j.jpeds.2012.04.042. View

17.

Davidson J, Kyrklund K, Eaton S, Pakarinen M, Thompson D, Cross K . Long-term surgical and patient-reported outcomes of Hirschsprung Disease. J Pediatr Surg. 2021; 56(9):1502-1511. DOI: 10.1016/j.jpedsurg.2021.01.043. View

18.

Meinds R, van der Steeg A, Sloots C, Witvliet M, de Blaauw I, van Gemert W . Long-term functional outcomes and quality of life in patients with Hirschsprung's disease. Br J Surg. 2019; 106(4):499-507. PMC: 6590339. DOI: 10.1002/bjs.11059. View

19.

Bongers M, van Wijk M, Reitsma J, Benninga M . Long-term prognosis for childhood constipation: clinical outcomes in adulthood. Pediatrics. 2010; 126(1):e156-62. DOI: 10.1542/peds.2009-1009. View