Functional defecation disorders in children: Associated comorbidity and advances in management - Chapter 9: Lack of agreement on how to use antegrade enemas in children

(1)

UvA-DARE is a service provided by the library of the University of Amsterdam (https://dare.uva.nl)

UvA-DARE (Digital Academic Repository)

Functional defecation disorders in children

Associated comorbidity and advances in management Kuizenga-Wessel, S. Publication date 2017 Document Version Other version License Other Link to publication

Citation for published version (APA):

Kuizenga-Wessel, S. (2017). Functional defecation disorders in children: Associated comorbidity and advances in management.

General rights

It is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an open content license (like Creative Commons).

Disclaimer/Complaints regulations

If you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: https://uba.uva.nl/en/contact, or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible.

(2)

(3)

Chapt%r

@

[

*

LACK OF AGREEMENT

ON HOW TO USE

ANTEGRADE ENEMAS

IN CHILDREN

S. Kuizenga-Wessel, H.M. Mousa, M.A. Benninga, C. Di Lorenzo

Journal of Pediatric Gastroenterology and Nutrition 2016;62:71-79

(4)

ABSTRACT

Objectives

The aim of the present study was to provide an overview of the existing litera-ture regarding the outcomes of the antegrade continence enema (ACE) proce-dure and to assess the present practices of physicians worldwide regarding the use of the ACE.

Methods

A search of the MEDLINE database was performed using the following criteria: having a clear definition of ‘‘successful outcome,’’ published in full manuscript form, sample size >20 patients, age <25 years. We then conducted a survey among 23 pediatric gastroenterologists and surgeons worldwide who were known to use the ACE using an 18 item questionnaire.

Results

A total of 21 articles met the inclusion criteria. Successful outcomes were reported in 15% to 100%. Thirteen studies classified the outcome as full conti-nence (success) or inconticonti-nence (failure), with a mean successful outcome of 75.6%. The 23 physicians who completed the questionnaire diﬀered in their opinions about indications and mandatory preoperative testing. Constipation with (78%) or without (91%) fecal incontinence, anorectal malformations (96%), and spinal abnormalities (100%) were considered suitable indications for the ACE by the majority. There was less agreement regarding the required preop-erative diagnostic workup. Most physicians (70%) start infusions using saline solutions and do not add a stimulant laxative to the cleansing solution. Discussion

There is a wide variation in the reported outcome of the ACE procedure and in the way success is defined. The survey identifies important diﬀerences among physicians using the ACE. Consensus on optimal use of the ACE could improve outcome of this treatment option.

(5)

Tr an sa na l i rr ig at io n 9

INTRODUCTION

The antegrade continence enema (ACE) has been reported as an eﬀective treatment option for children with defecatory disorders when maximal behav-ioral, dietary, and medical therapies are not successful. Though originally recommended only for children with anorectal malformations or neuromus-cular abnormalities, its use was then extended to the management of pedi-atric patients with several disorders ranging from idiopathic constipation to Hirschsprung disease1_{. Since the original report in 1990 by Malone et al}2_{, the}

original procedure has undergone several modifications, with a trend toward

a more minimally invasive approach3-5_{. Although the ACE is described in}

liter-ature as successful in most studies, the percentages of reported success vary

widely6_{. Despite the extensive use of ACE worldwide, guidelines for when, how,}

and in whom to perform it have not been established. Treatment regimens vary among centers and are largely based on personal preference and trial and error. Differences in practice among physicians using ACE could lead to the different reported clinical outcomes, and heterogeneously reported outcomes may make it more difficult to implement changes in practice. Our aims include providing an overview of the existing literature on how treatment outcomes of the ACE procedure are defined and to pool experts to assess their present practices regarding the use of the ACE.

METHODS

Literature Search

A literature search occurred in August 2012. The MEDLINE database was searched using the following medical subject heading (MeSH) terms ‘‘cecos-tomy’’ [MeSH], combined with an all-field search of (ACE) and (appendicos-tomy). The results were limited to English language publications. The title and the abstracts of all the citations identified by the database were reviewed. Eligible studies were retrieved and read in full to verify that they satisfied the following selection criteria: patients with an upper age limit of 25 years, published in full manuscript form, sample size of ≥20 patients, and including a clearly stated definition of treatment success. To ensure a thorough review of literature, we also searched the references of relevant articles with the goal to identify other studies that may have been missed initially. We excluded studies describing treatment success in p-values, instead of absolute numbers or percent-ages. Articles solely describing surgical techniques only were also excluded.

(6)

200

Table 1 Characteristic of included studies

St ud y Da te Lo ca tio n Sa m ple si ze _(M%) Ag e (y ea rs ) (ra ng e) Po pu la tio n A C E p la ce m en t O ut co m e v ar i-ab le s s uc ce ss fu l ou tc om e D ef in iti on o f s uc ce ss Su cc es s (% ) Pa rt ia l su cc es s (% ) Fa ilu re (% ) Mu gi e 7 20 12 US A 99 ( 57 .6 ) M ed ia n 8 (2 -2 2) Mi xe d Ce cos to m y Co nt in en ce To ta lly s ym pt om f re e 71 20 9 Si dd iq ui 8 20 11 US A 10 5 ( 43 .8 ) M ea n 11 .2 Mi xe d Mu lti pl e Co nt in en ce Sc or e s ys te m : “ ex ce lle nt ” o r “ go od ” s co re s 69 31 La w al 9 20 11 US A 44 ( 61 .4 ) M ea n 8. 6 Mi xe d Ap pe ndi co s-to m y Co nt in en ce C on tin en t b et w ee n e ne m as 10 0 0 Ho ek st ra 10 20 11 Th e Ne th er -la nd s 23 ( 60 .9 ) M ed ia n 7 (2 -1 7) Mi xe d Mu lit pl e Co nt in en ce Sa tis fa ct io n Fu ll: T ot al ly c le an o r e xp er ie nc in g o nl y m in or l ea ka ge o n th e n ig ht o f t he w as ho ut Pa rt ia l: c le an , b ut s ig ni fic an t r ec ta l l ea ka ge Ba se d o n s co re s ys te m : s co re ≥ 6 87 15 8. 7 ₇₀ 4. 3 15 Do nk ol 11 20 10 Eg yp t 21 ( 61 .9 ) M ea n 9. 5 (5 -1 6) Fe ca l in co nt i-ne nc e Ce cos to m y Co nt in en ce Sa tis fa ct io n Re so lu tio n o f c on tin uo us f ec al i nc on tin en ce Sa tis fie d w ith t he e ﬀe ct iv en es s o f t he p ro ce du re 85 .7 87 14 .3 13 Ja ﬀr ay 12 20 09 UK 80 ( 55 ) M ed ia n 9.6 (3 .4 -1 8. 7) Id io pa th ic co ns tip a-tio n Ap pe ndi co s-to m y Co nt in en ce Fu ll: A C E c lo su re d ue t o n or m al b ow el h a bi t, c ol on ic la va ge n o l on ge r n ee de d i n p re vi ou s 6 m on th s Pa rt ia l: s til l u sin g A C E w ith s at isf ac tio n 15 70 15 Na ni gi a n 13 20 08 US A 22 ( 59 .1) M ea n 7.8 (4 -1 5) Mi xe d Ap pe ndi co s-to m y Co nt in en ce Fr ee f ro m f ec al i nc on tin en ce a nd c on st ip at io n 10 0 0 Si nh a 6 20 08 UK 48 M ea n 10 Mi xe d Mu lti pl e Co nt in en ce C om pl et el y c on tin en t 92 8 Va n d en B er g 14 20 06 US A 32 ( 59 .4 ) Ra ng e 2-17 Mi xe d Ce cos to m y Co nt in en ce N o o cc as io na l f ec al i nc on tin en ce , a nd a d ef ec at io n fre qu en cy o f 5/ w ee k – 3/ da y 78 22 Ki ng 15 20 05 Au st ra lia 42 ( 73 .8 ) M ea n 13 .1 (6 .9 -2 5) Mi xe d Ap pe ndi co s-to m y Co nt in en ce Fu ll: c om pl et el y c on tin en ce Pa rt ia l: d ec re as ed s oi lin g f re qu en cy 26 50 24 Ca sci o 16 20 04 UK 49 ( 49 ) Ra ng e 3-18 Id io pa th ic co ns tip a-tio n Mu lti pl e Co nt in en ce Fu ll: c om pl et el y c on tin en ce Pa rt ia l: o cc as io na l f ec al i nc on tin en ce 79 .6 6.1 14 .3 De y 17 20 03 UK 32 M ea n 11 .5 (3 .8 -1 7.6 ) Mi xe d Mu lti pl e Co nt in en ce Fu ll: c om pl et el y c on tin en t Pa rt ia l: f ec al i nc on tin en ce o cc as io na l o r m on th ly 28 .1 56 .3 15 .6 Ch ai t 18 20 03 Ca na da 12 4 Ra ng e 2-23 Mi xe d Ce cos to m y Co nt in en ce ≤ 1 f ec al i nc on tin en ce e pi so de/ 6 m on th s 33 .3 66 .6 Table 1 Continued St ud y Da te Lo ca tio n Sa m ple si ze _(M%) Ag e (y ea rs ) (ra ng e) Po pu la tio n A C E p la ce m en t O ut co m e v ar i-ab le s s uc ce ss fu l ou tc om e D ef in iti on o f s uc ce ss Su cc es s (% ) Pa rt ia l su cc es s (% ) Fa ilu re (% ) Ma rs ha ll 19 20 01 Au st ra lia 28 Ra ng e 5-17 Sl ow t ra ns it co ns tip a-tio n Ap pe ndi co s-to m y Co nt in en ce Fu ll: n o f ec al i nc on tin en ce o r m in or r ec ta l l ea ka ge o n n ig ht of w as ho ut Pa rt ia l: c le an , b ut s ig ni fic an t r ec ta l o r s to m al l ea ka ge , a nd per ce iv ed im pr ov em en t 21 43 25 Fo nka ls ru d 20 19 98 US A 24 M ea n 5. 7 (7 -1 5) Mi xe d Ce cos to m y Co nt in en ce C om pl et el y c le an d ur in g d ay tim e, w ith in 1 w ee k p os to p-er at iv e 45 .8 54 .2 Wi lc ox 21 19 98 UK 36 M ea n 8. 3 (3 -1 4) Mi xe d Ap pe ndi co s-to m y Co nt in en ce ≤1 f ec al i nc on tin en ce e pi so de/ w ee k 78 22 Sh an ka r 22 19 98 UK 40 ( 45 ) M ed ia n 11 (6 -2 1) Mi xe d Mu lti pl e Co nt in en ce ≤1 f ec al i nc on tin en ce e pi so de/ m on th , a nd c le an w ith in 60 m in ut es a fte r t he w as ho ut 70 30 Sc he ll 23 19 97 UK 23 ( 47 .8 ) M ea n 7.4 (3 -1 4) Mi xe d Mu lti pl e Co nt in en ce Q ua lit y o f l ife C om pl et el y c on tin en t Ba se d o n s co re s ys te m . Fu ll: “ id ea l” o r “ gr ea t i m pr ov em en t” Pa rt ia l: “ de fin ite ” o r “ so m e i m pr ove m en t” 82 .6 87 13 17 .4 0 Gr ifi th s 24 19 95 UK 21 M ed ia n 12 (1 8-32 4) Mi xe d Mu lti pl e Co nt in en ce Fu ll: c om pl et el y c le an Pa rt ia l: 95 % c on tin en t o r g re at i m pr ov em en t 57 .1 14 .3 28 .6 Ko yl e 25 19 95 US A 22 ( 40 .9 ) M ea n 13 (5 -2 3) Mi xe d Mu lti pl e Co nt in en ce C om pl et el y c on tin en t ≥ 4 m on th s a fte r s ur ge ry 73 27 Sq ui re 26 19 93 UK 25 M ea n 9 (3 -1 8) Mi xe d Mu lti pl e Co nt in en ce C om pl et el y c on tin en t b et w ee n e ne m as 76 24

(7)

201 Tr an sa na l i rr ig at io n 9 Table 1 Continued Tr an sa na l i rr ig at io n 9

St ud y Da te Lo ca tio n Sa m ple si ze _(M%) Ag e (y ea rs ) (ra ng e) Po pu la tio n A C E p la ce m en t O ut co m e v ar i-ab le s s uc ce ss fu l ou tc om e D ef in iti on o f s uc ce ss Su cc es s (% ) Pa rt ia l su cc es s (% ) Fa ilu re (% ) Mu gi e 7 20 12 US A 99 ( 57 .6 ) M ed ia n 8 (2 -2 2) Mi xe d Ce cos to m y Co nt in en ce To ta lly s ym pt om f re e 71 20 9 Si dd iq ui 8 20 11 US A 10 5 ( 43 .8 ) M ea n 11 .2 Mi xe d Mu lti pl e Co nt in en ce Sc or e s ys te m : “ ex ce lle nt ” o r “ go od ” s co re s 69 31 La w al 9 20 11 US A 44 ( 61 .4 ) M ea n 8. 6 Mi xe d Ap pe ndi co s-to m y Co nt in en ce C on tin en t b et w ee n e ne m as 10 0 0 Ho ek st ra 10 20 11 Th e Ne th er -la nd s 23 ( 60 .9 ) M ed ia n 7 (2 -1 7) Mi xe d Mu lit pl e Co nt in en ce Sa tis fa ct io n Fu ll: T ot al ly c le an o r e xp er ie nc in g o nl y m in or l ea ka ge o n th e n ig ht o f t he w as ho ut Pa rt ia l: c le an , b ut s ig ni fic an t r ec ta l l ea ka ge Ba se d o n s co re s ys te m : s co re ≥ 6 87 15 8. 7 ₇₀ 4. 3 15 Do nk ol 11 20 10 Eg yp t 21 ( 61 .9 ) M ea n 9. 5 (5 -1 6) Fe ca l in co nt i-ne nc e Ce cos to m y Co nt in en ce Sa tis fa ct io n Re so lu tio n o f c on tin uo us f ec al i nc on tin en ce Sa tis fie d w ith t he e ﬀe ct iv en es s o f t he p ro ce du re 85 .7 87 14 .3 13 Ja ﬀr ay 12 20 09 UK 80 ( 55 ) M ed ia n 9.6 (3 .4 -1 8. 7) Id io pa th ic co ns tip a-tio n Ap pe ndi co s-to m y Co nt in en ce Fu ll: A C E c lo su re d ue t o n or m al b ow el h a bi t, c ol on ic la va ge n o l on ge r n ee de d i n p re vi ou s 6 m on th s Pa rt ia l: s til l u sin g A C E w ith s at isf ac tio n 15 70 15 Na ni gi a n 13 20 08 US A 22 ( 59 .1) M ea n 7.8 (4 -1 5) Mi xe d Ap pe ndi co s-to m y Co nt in en ce Fr ee f ro m f ec al i nc on tin en ce a nd c on st ip at io n 10 0 0 Si nh a 6 20 08 UK 48 M ea n 10 Mi xe d Mu lti pl e Co nt in en ce C om pl et el y c on tin en t 92 8 Va n d en B er g 14 20 06 US A 32 ( 59 .4 ) Ra ng e 2-17 Mi xe d Ce cos to m y Co nt in en ce N o o cc as io na l f ec al i nc on tin en ce , a nd a d ef ec at io n fre qu en cy o f 5/ w ee k – 3/ da y 78 22 Ki ng 15 20 05 Au st ra lia 42 ( 73 .8 ) M ea n 13 .1 (6 .9 -2 5) Mi xe d Ap pe ndi co s-to m y Co nt in en ce Fu ll: c om pl et el y c on tin en ce Pa rt ia l: d ec re as ed s oi lin g f re qu en cy 26 50 24 Ca sci o 16 20 04 UK 49 ( 49 ) Ra ng e 3-18 Id io pa th ic co ns tip a-tio n Mu lti pl e Co nt in en ce Fu ll: c om pl et el y c on tin en ce Pa rt ia l: o cc as io na l f ec al i nc on tin en ce 79 .6 6.1 14 .3 De y 17 20 03 UK 32 M ea n 11 .5 (3 .8 -1 7.6 ) Mi xe d Mu lti pl e Co nt in en ce Fu ll: c om pl et el y c on tin en t Pa rt ia l: f ec al i nc on tin en ce o cc as io na l o r m on th ly 28 .1 56 .3 15 .6 Ch ai t 18 20 03 Ca na da 12 4 Ra ng e 2-23 Mi xe d Ce cos to m y Co nt in en ce ≤ 1 f ec al i nc on tin en ce e pi so de/ 6 m on th s 33 .3 66 .6 Table 1 Continued St ud y Da te Lo ca tio n Sa m ple si ze _(M%) Ag e (y ea rs ) (ra ng e) Po pu la tio n A C E p la ce m en t O ut co m e v ar i-ab le s s uc ce ss fu l ou tc om e D ef in iti on o f s uc ce ss Su cc es s (% ) Pa rt ia l su cc es s (% ) Fa ilu re (% ) Ma rs ha ll 19 20 01 Au st ra lia 28 Ra ng e 5-17 Sl ow t ra ns it co ns tip a-tio n Ap pe ndi co s-to m y Co nt in en ce Fu ll: n o f ec al i nc on tin en ce o r m in or r ec ta l l ea ka ge o n n ig ht of w as ho ut Pa rt ia l: c le an , b ut s ig ni fic an t r ec ta l o r s to m al l ea ka ge , a nd per ce iv ed im pr ov em en t 21 43 25 Fo nka ls ru d 20 19 98 US A 24 M ea n 5. 7 (7 -1 5) Mi xe d Ce cos to m y Co nt in en ce C om pl et el y c le an d ur in g d ay tim e, w ith in 1 w ee k p os to p-er at iv e 45 .8 54 .2 Wi lc ox 21 19 98 UK 36 M ea n 8. 3 (3 -1 4) Mi xe d Ap pe ndi co s-to m y Co nt in en ce ≤1 f ec al i nc on tin en ce e pi so de/ w ee k 78 22 Sh an ka r 22 19 98 UK 40 ( 45 ) M ed ia n 11 (6 -2 1) Mi xe d Mu lti pl e Co nt in en ce ≤1 f ec al i nc on tin en ce e pi so de/ m on th , a nd c le an w ith in 60 m in ut es a fte r t he w as ho ut 70 30 Sc he ll 23 19 97 UK 23 ( 47 .8 ) M ea n 7.4 (3 -1 4) Mi xe d Mu lti pl e Co nt in en ce Q ua lit y o f l ife C om pl et el y c on tin en t Ba se d o n s co re s ys te m . Fu ll: “ id ea l” o r “ gr ea t i m pr ov em en t” Pa rt ia l: “ de fin ite ” o r “ so m e i m pr ove m en t” 82 .6 87 13 17 .4 0 Gr ifi th s 24 19 95 UK 21 M ed ia n 12 (1 8-32 4) Mi xe d Mu lti pl e Co nt in en ce Fu ll: c om pl et el y c le an Pa rt ia l: 95 % c on tin en t o r g re at i m pr ov em en t 57 .1 14 .3 28 .6 Ko yl e 25 19 95 US A 22 ( 40 .9 ) M ea n 13 (5 -2 3) Mi xe d Mu lti pl e Co nt in en ce C om pl et el y c on tin en t ≥ 4 m on th s a fte r s ur ge ry 73 27 Sq ui re 26 19 93 UK 25 M ea n 9 (3 -1 8) Mi xe d Mu lti pl e Co nt in en ce C om pl et el y c on tin en t b et w ee n e ne m as 76 24

(8)

For each included study, we extracted the following data: study method-ology, location, sample size, age range, definition of successful treatment, and reported outcome. Descriptive statistics are used to illustrate the characteristics of included studies.

Survey

In an eﬀort to better understand diﬀerent clinical practices, we sent a question-naire to 12 pediatric gastroenterologists and 13 surgeons worldwide who were known to use the ACE. These physicians were selected by the authors of this article. To gain insight in all aspects of this treatment, questions about indica-tions, diagnostic workup, and treatment regimens were included in this survey. The institutional review board of the Nationwide Children’s Hospital approved the study protocol.

RESULTS

Literature Search

Our search yielded 196 relevant articles. All of the titles and/or abstracts were screened for eligibility. A total of 74 studies were found to be irrelevant to our research question. In addition, 103 articles did not meet our inclusion criteria (n=94) or were not retrievable (n=9). Five articles were excluded because they did not provide a clear definition of constipation, and 4 other articles were eliminated because data were only described in P values. Checking bibliog-raphies of relevant articles resulted in 3 additional articles. In total, 21 articles met our inclusion criteria.

The 21 articles included a total of 940 patients, 276 were female patients and 295 were male patients, and in 7 articles the patients’ sex was not mentioned. The studies were published between 1993 and 2012. Most of the studies had a retrospective design; only 1 was prospective. None of the studies included a control group, 18 studies were conducted in Europe or North America, 2 in Australia, and 1 in Egypt. The study population of 11 studies consisted of a combination of patients with functional and organic causes, 6 studies only included patients with functional constipation, and 4 studies had only patients with organic etiologies. To provide access for administration of the antegrade enemas, 5 studies only used cecostomies, 6 solely appendicostomies, and 10 studies used a combination of both techniques (Table 1).

Various successful outcomes were reported, ranging between 15% and 100%. Successful outcome was measured in multiple ways: 13 articles classified the

(9)

Table 2 Outcome variables used in literature

Outcome variable of “successful outcome” Number

of articles Successful outcomemean % (range) Continence Success/failure Success/partial success/failure 13 8 75.6 (33.3-100) 48.1 (15-87) Patient satisfaction with the procedure 2 86-87 Quality of life improvement 1 87

outcome as full fecal continence (success) or fecal incontinence (failure), with a mean successful outcome of 75.6% (range 33.3%–100%); 8 articles described continence in 3 categories: full success, partial success, or failure, with a mean full successful outcome of 48.1% (range 15%–87%). Two articles used ‘‘satisfaction with the procedure’’ with a mean successful outcome of 86.5%, and 1 article used quality of life (QoL) (successful outcome 87%) (Table 2). Among the 11 studies including both patients with functional and organic causes, only 6 reported data regarding diﬀerences in outcome between these patient groups (Table 2).

Survey

All 12 pediatric gastroenterologists and 11 of 13 pediatric surgeons completed the questionnaire (nonresponders practice in the United States and England). In total, 23 surveys were included. The majority of the respondents practiced in theUnited States 16 of 23 (70%), 1 pediatric gastroenterologist in Canada, and 1 in the Netherlands. Four pediatric surgeons practiced in Europe, 1 in Australia, and 6 in the United States. Three of the pediatric surgeons also worked in pediatric urology. The pediatric gastroenterologists had a mean experience in pediatric gastroenterology of 17 years (range 5–32 years), and they had recommended a mean of 51 ACE procedures each (range 6–150). The pediatric surgeons performed a mean of 81 ACE procedures each (range 20–250) and had a mean experience in their field of 18 years (5–31 years) (Table 3).

Indications for ACE

Constipation (78%), constipation associated with fecal incontinence (91%), anorectal malformations (96%), Hirschsprung disease (83%), and spinal abnor-malities (100%) were considered suitable indications for the ACE procedure by the vast majority of respondents. There was less agreement regarding func-tional nonretentive fecal incontinence (FNRFI) (35%) as an indication for the ACE. Only 22% of the participants did not require a minimal age for this

(10)

Table 3 Survey results

Questions survey Answered Yes (n) Answered Yes (%) Indications for ACE

Constipation (11/12) 91.7 Constipation + FI (10/12) 83.3 FNRFI (4/12) 33,3 Hirschsprung disease (11/12) 91.7 Anorectal malformations (11/12) 91,7 Spinal abnormalities (12/12) 100 Mandatory pre-operative tests

Rectal biopsies (5/12) 41.7

Anorectal manometry (10/12) 83,3

Barium enema (6/12) 50

Colon transit time study (10/12) 83,3

MRI (4/12) 33.3

Colonic manometry (6/12) 50

Requiring a minimal age

No minimum age (3/12) 25

1-3 years (4/12) 33.3

4-6 years (4/12) 33.3

> 6 years (1/12) 8.3

ACE placement

Intervention Radiology (IR) (2/12) 16.7

Lap cecostomy (8/12) 66,7 Open ceocstomy (2/12) 16,7 Appendicostomy (3/12) 25 LAPEC (1/12) 8,3 Antibiotics preoperative (10/12) 83,3 Antibiotics postoperative (11/12) 91.7 Hospital stay between 2-5 days (12/12) 100

procedure. A total of 30% of the respondents required a minimal age of ≥1 year, another 44% of ≥4 years. One respondent answered that the minimal age for this procedure should be 7 years of age. Anatomical or significant behav-ioral problems were mentioned by a third of the participants and abnormal colonic motility by 20% as contraindications for this procedure. Other contra-indications mentioned were the inability to sit on the toilet and a history of unsuccessful rectal irrigation.

(11)

Questions survey Answered Yes (n) Answered Yes (%) Start using ACE after the procedure

On day 1 (3/12) 25 On day 2-7 (4/12) 33,3 On day 8-13 (1/12) 8,3 On day 14 (4/12) 33,3 Enema solution Tap water (3/12) 25 Saline (NaCl) (10/12) 83,3 Polyethylene glycol (2/12) 16,7 Sodium-phosphate (1/12) 8,3 Soap-suds (0/12) 0 Enema volume 10-20 mg/kg (9/12) 75 0-249 ml (1/12) 8,3 250-500 ml (2/12) 16,7

Enema: adding of laxatives

At beginning (2/12) 16,7

Only if not eﬀective (10/12) 83,3 Name of laxative

Bisacodyl only (4/12) 33,3

Glycerine or Bisacodyl (7/12) 58,3 Combination of Glycerine and Bisacodyl (1/12) 8,3 Enema frequency

Daily (12/12) 100

Duration of infusion enema solution

0-15 min (6/11) 54,5

16-30 min (5/11) 45,5

Toilet sitting time

<30 min (5/12) 41,7 30-60 min (7/12) 58,3 Wean ACE Yes No (9/11) (2/11) 81,8 18,2 Table 3 Continued

(12)

Preoperative Diagnostic Workup

Respondents had diﬀerent opinions on which investigations should be part of the preoperative diagnostic workup. A small minority of the respondents believed that anorectal manometry (57%), colonic transit studies (65%), and barium enema (52%) are mandatory preoperative tests. A minority (22%) considers MRI of the spine or rectal biopsies (30%) necessary before surgery. Only 26% of the participants require a preoperative colonic manometry in their diagnostic workup. These 6 physicians were all pediatric gastroenterolo-gists. Most physicians, however, who do not consider colonic manometry as a mandatory preoperative test required a colonic transit study in their preoper-ative testing.

Type of Procedure

The ACE was most often done as a laparoscopic cecostomy (57%). Appen-dicostomies (39%), open cecostomies (22%), or placement of a cecostomy by interventional radiology (17%) were used less frequently. Only 1 respondent used laparoscopic-assisted percutaneous endoscopic cecostomy placement. In most hospitals >1 technique was used to create an ACE. In 19 hospitals, patients were generally able to leave the hospital within 2 to 5 days postoperatively, in 3 hospitals 1 day after the procedure, and in 1 hospital after 1 week.

Antibiotics Regimen

All of the physicians prescribed antibiotics for the placement of an ACE. The majority of the respondents (83%) used antibiotics both before and after the placement of the ACE, regardless of the technique used to perform it. The specific type of antibiotics given and the duration of the treatment varied, ranging from just the immediate perioperative period to until 7 days after the procedure.

Cleansing Solutions

There was a wide variability in the number of days (1–15 days) to wait before starting to use the ACE after the procedure, with 35%of the respondents starting to use it on day 1, 35%between days 2 and 7, 4% between days 8 and 13, and 26% after day 13. Most respondents (70%) started the infusions using NaCl solution as the antegrade enema solution. Tap water, sodium phosphate, and polyethylene glycol were used less often. None of the physicians used soapsuds as enema solution. Only 2 respondents added a stimulant laxative (glycerin or bisacodyl) to the cleansing solution from the beginning. All the other physicians only added stimulant laxatives if the initial antegrade enemais

(13)

not eﬀective. All but 1 respondent started by using the antegrade enemas on a daily basis, 62% preferred a duration of infusion between 0 and 15 minutes, and 38% between 16 and 30 minutes. A total of 35%believed that the time to be spent on the toilet after administration of the enema solution should be <30 minutes, and 65%instructed the patients to have a toilet sitting time between 30 and 60 minutes.

Weaning from ACE

Only 4 respondents do not consider weaning patients from the ACE because they believe that patients will most likely revert back to constipation and/or incontinence. The majority of the physicians consider weaning patients from ACE after they do well for a certain number of months.

DISCUSSION

The ACE procedure has been widely accepted as a treatment option for chil-dren with chronic defecation disorders. There is a variation in both the reported outcome of the ACE procedure and the way such outcome is measured. The first part of this study focused on how the success of ACE is reported in the literature. Our review shows a variation in the outcome measures used and their definitions. In the 21 articles selected, the 3 main different primary outcomes that were used to describe the success of ACE included fecal conti-nence, QoL, and patient/family satisfaction. The mean success was much higher when treatment success was measured on a 2 instead of a 3-point scale (Table 2). To compare results from clinical trials, it is important to use uniform definitions and outcome measures. The use of inappropriate outcome measures and their definitions can compromise the generalizability of a study and may lead to overestimating or underestimating the benefit of an inter-vention. Thus, it would be important to achieve consensus on these domains. Differences in reported outcomes of the ACE procedure may be because of differences in how the ACE is used. Thus, in the second part of this study, the present clinical practice of experts in the field of functional and motility disor-ders regarding their use of the ACE was assessed. Results of the survey identi-fied important differences among physicians when using the ACE.

The findings from our survey regarding the indications for ACE placement are consistent with the published literature. It was, however, interesting to learn that

(14)

some physicians consider FNRFI a suitable indication for ACE. To our knowl-edge, there are no studies evaluating the eﬀicacy of the ACE in children with FNRFI, a condition that in the majority of patients very challenging to manage and has no well-established treatment strategies.

In clinical practice, in the majority of the children presenting with constipa-tion and fecal incontinence, no organic diseases are found, and the diagnosis of functional constipation is made based on a thorough history and physical examination. When conventional treatment, however, fails or there are alarm symptoms and signs, diagnostic tests are usually recommended. More exten-sive testing is especially used when surgical procedures are being

contem-plated27_{. In our survey, respondents had diﬀerent opinions on the mandatory}

preoperative diagnostic workup, for example whether to perform a colonic manometry. The split opinion of the pediatric gastroenterologists in our survey reflects the literature regarding colonic manometry and ACE. A study by Van

den Berg et al14_{showed that colonic manometry is a reliable predictor of the}

success of ACE. In that study, patients with generalized colonic dysmotility were less likely to benefit from using the ACE1,4_{. Therefore, Aspirot et al.}28

recom-mended that the ACE should be placed only in children who do not have colonic inertia and who demonstrate an identifiable colonic motor response

to bisacodyl. On the contrary, Rodriguez et al29_{found that the absence of}

high-amplitude-propagated contractions (HAPCs) was not associated with a therapeutic failure of the ACE. In their study, all of the 18 patients with no HAPC at baseline had a satisfactory long-term response, and 6 patients were able to eventually completely discontinue the use of the ACE.

Irrigation regimens vary among centers, and diﬀerent solutions have been described, including saline, tap water, polyethylene glycol, and mineral oil. Some studies have warned of the risk for severe hyperphosphatemia and electrolyte abnormalities when administering hypertonic phosphate and high colonic tap water enemas30_{. The findings from our survey suggest that this}

knowledge has impacted clinical practice because most physicians use a saline solution. Few studies have suggested that an irrigation regimen, which includes stimulant laxatives, could significantly improve the fecal continence rate in patients who were initially struggling with the ACE31,32_{. In accordance}

with the literature, the majority of the respondents add a stimulant laxative after an initial failure of the ACE. Indeed, Mugie et al7_{reported that subjects}

who use stimulants from the very beginning had significantly better outcomes than subjects who started without a stimulant.

(15)

The success of the ACE varies: some patients show a lack of response, others have to use it on a chronic basis, and others are able to decrease and even discontinue the ACE completely29_{. Youssef et al}33_{reported that antegrade}

enemas can be discontinued in selected patients after a mean of 14.6 months.

Similar findings were reported by Rodriguez et al29_{who found that 64% of}

their patients were able to be weaned from the irrigations, and 28% were able to discontinue them. They also demonstrated a significant improvement in the colonic motility and in some patients a complete recovery of abnormal colonic function after the use of the ACE. Important predictors of successful weaning were normalization of the HAPCs and older age of the patients.

It should be emphasized that the aim of this study was not to determine what the best treatment strategies were. We only attempted to evaluate and better understand differences in the present clinical practice regarding the use of ACE. There are some limitations to this study. First, although our search strategy was certainly thorough and comprehensive, it is possible we did not include all rele-vant studies. We, however, did not aim to include every clinical trial regarding ACE because our objective was not to perform a meta-analysis on the effi-cacy of this treatment option. Instead, we aimed to acquire a comprehensive sample of trials for the evaluation of definitions and outcome measures used to describe treatment success. Moreover, we identified >20 studies fulfilling our criteria, and it is unlikely that the addition of few more studies would have modified our conclusions. Second, the number of physicians who completed the survey was relatively small and included an overrepresentation of physi-cians from North America, leading to a possible selection bias. The ACE proce-dure, however, is a treatment option reserved for children with chronic defe-cation disorders that fail conventional treatment. Therefore, this procedure is only performed in specialized hospitals. By selecting the experts, we identified the physicians having the most experience in using the ACE. Results, however, should be interpreted with caution, giving the underrepresentation of European physicians. Another limitation of this study includes the use of multiple choice questions in the survey. As with all multiple-choice questions, respondents are required to choose a response that may not exactly reflect the nuances of their opinions. When necessary, the respondents were able to further elabo-rate on their answers. Furthermore, the questions did not differentiate between functional and organic etiologies of defecation disorders. It may be worth it to explore the opinions of physicians regarding these different causes in future research because diagnostic workup and decision whether or not to wean patients of ACE could differ among patients with different underlying diseases.

(16)

In conclusion, we showed that there is a substantial variation in both the reported outcome of the ACE procedure and in the way it is measured. To compare results from clinical trials, uniform definitions and outcome measures must be developed. Studies with unambiguous and clear-defined outcome measures are necessary. Results of the survey identify important diﬀerences among physicians when using the ACE. Consensus on the optimal use of ACE could lead to an improved outcome of this treatment option for children with chronic defecation disorders.

(17)

REFERENCES

1 Rodriguez L, Flores A, Gilchrist BF, et al. Laparoscopic-assisted percutaneous endo-scopic cecostomy in children with defeca-tion disorders (with video). Gastrointest Endosc 2011;73:98–102.

2 Malone PS, Ransley PG, Kiely EM. Preliminary report: the antegrade continence enema. Lancet 1990;336:1217–8.

3 Casale P, Grady RW, Feng WC, et al. A novel approach to the laparoscopic antegrade continence enema procedure: intracorpo-real and extracorpointracorpo-real techniques. J Urol 2004;171:817–9.

4 Yagmurlu A, Harmon CM, Georgeson KE. Laparoscopic cecostomy button place-ment for the manageplace-ment of fecal inconti-nence in children with Hirschsprung’s disease and anorectal anomalies. Surg Endosc 2006;20:624–7.

5 Biyani D, Barrow E, Hodson P, et al. Endo-scopically placed caecostomy buttons: a trial ACE procedure. Colorectal Dis 2007;9:373–6.

6 Sinha CK, Grewal A, Ward HC. Antegrade continence enema (ACE): current practice. Pediatr Surg Int 2008;24:685–8.

7 Mugie SM, Machado RS, Mousa HM, et al. Ten-year experience using antegrade enemas in children. J Pediatr 2012;161:700–4.

8 Siddiqui AA, Fishman SJ, Bauer SB, et al. Long-term follow-up of patients after ante-grade continence enema procedure. J Pediatr Gastroenterol Nutr 2011;52:574–80.

9 Lawal TA, Rangel SJ, Bischoﬀ A, et al. Lapa-roscopic-assisted Malone appendicostomy in the management of fecal incontinence in children. J Laparoendosc Adv Surg Tech A 2011;21:455–9.

10 Hoekstra LT, Kuijper CF, Bakx R, et al. The Malone antegrade continence enema proce-dure: the Amsterdam experience. J Pediatr Surg 2011;46:1603–8.

11 Donkol RH, Al-Nammi A. Percutaneous cecostomy in the management of organic

fecal incontinence in children. World J Radiol 2010;2:463–7.

12 Jaﬀray B. What happens to children with idiopathic constipation who receive an ante-grade continent enema? An actuarial anal-ysis of 80 consecutive cases. J Pediatr Surg 2009;44:404–7.

13 Nanigian DK, Kurzrock EA. Intermediate-term outcome of the simplified laparoscopic ante-grade continence enema procedure: less is better. J Urol 2008;179:299–303.

14 Van den Berg MM, Hogan M, Caniano DA, et al. Colonic manometry as predictor of cecostomy success in children with defeca-tion disorders. J Pediatr Surg 2006;41:730–6.

15 King SK, Sutcliﬀe JR, Southwell BR, et al. The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg 2005;40:1935 40.

16 Cascio S, Flett ME, De la Hunt M, et al. MACE or caecostomy button for idiopathic consti-pation in children: a comparison of compli-cations and outcomes. Pediatr Surg Int 2004;20:484–7.

17 Dey R, Ferguson C, Kenny SE, et al. After the honeymoon—mediumterm outcome of antegrade continence enema procedure. J Pediatr Surg 2003;38:65–8.

18 Chait PG, Shlomovitz E, Connolly BL, et al. Percutaneous cecostomy: updates in technique and patient care. Radiology 2003;227:246–50.

19 Marshall J, Hutson JM, Anticich N, et al. Ante-grade continence enemas in the treatment of slow-transit constipation. J Pediatr Surg 2001; 36:1227–30.

20 Fonkalsrud EW, Dunn JCY, Kawaguchi AI. Simplified technique for antegrade conti-nence enemas for fecal retention and incon-tinence. J Am Coll Surg 1998;7515:457–60.

21 Wilcox D, Kiely EM. The Malone (antegrade colonic enema) procedure: early experience. J Pediatr Surg 1998;33:204–6.

(18)

22 Shankar KR, Losty PD, Kenny SE, et al. Functional results following the antegrade continence enema procedure. Br J Surg 1998;85:980–2.

23 Schell SR, Toogood GJ, Dudley NE. Control of fecal incontinence: continued success with the Malone procedure. Surgery 1997;122:626–31.

24 Griﬀiths DM, Malone PS. The Malone ante-grade continence enema. J Pediatr Surg 1995;30:68–71.

25 Koyle MA, Kaji DM, Duque M, et al. The Malone antegrade continence enema for neurogenic and structural fecal incontinence and constipation. J Urol 1995;154:759–61.

26 Squire BR, Kiely EM, Carr B, et al. The clinical application of the Malone antegrade conti-nence enema. J Pediatr Surg 1993;28:1012–5.

27 Tabbers MM, Dilorenzo C, Berger MY, et al. Evaluation and treatment of func-tional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastro-enterol Nutr 2014;58:265–81.

28 Aspirot A, Fernandez S, Di Lorenzo C, et al. Antegrade enemas for defecation disor-ders: do they improve the colonic motility? J Pediatr Surg 2009;44:1575–80.

29 Rodriguez L, Nurko S, Flores A. Factors asso-ciated with successful decrease and discon-tinuation of antegrade continence enemas (ACE) in children with defecation disor-ders: a study evaluating the eﬀect of ACE on colon motility. Neurogastroenterol Motil 2013;25:140–81.

30 Yerkes EB, Rink RC, King S, et al. Tap water and the Malone antegrade conti-nence enema: a safe combination? J Urol 2001;166:1476–8.

31 Bani-Hani AH, Cain MP, King S, et al. Tap

water irrigation and additives to optimize success with the Malone antegrade conti-nence enema: the Indiana University algo-rithm. J Urol 2008;180:1757–60.

32 Chu DI, Balsara ZR, Routh JC, et al. Experi-ence with glycerin for antegrade continExperi-ence enema in patients with neurogenic bowel. J Urol 2013;189:690–3.

33 Youssef NN, Barksdale E Jr, Griﬀiths JM, et al. Management of intractable constipation with antegrade enemas in neurologically intact children. J Pediatr Gastroenterol Nutr 2002;34:402–5