• We are sorry, but NCBI web applications do not support your browser and may not function properly. More information

Surgical Interventions for Maintenance: Effectiveness of the ACE procedure in Children with Chronic Idiopathic Constipation

Bibliographic InformationStudy Type & Evidence LevelNumber of PatientsPatient CharacteristicsIntervention & ComparisonFollow-up & Outcome MeasuresEffect SizeReviewer Comments
King et al. The antegrade continence enema successfully treats idiopathic slow-transit constipation. 2005. Journal of Pediatric Surgery 40[12], 1935-1940Study Type:
Retrospective cohort

Evidence level:
2+

Study aim:
to determine whether ACE are successful for idiopathic paediatric slow transit constipation (STC)
56 children

Inclusion criteria:
patients with appendicostomy for idiopathic constipation formed between Jan/95 and Oct/04, who satisfied Rome II criteria for functional constipation, with/without faecal incontinence and had undergone a prolonged period of unsuccessful medical management

Exclusion criteria:
not stated
42 children

31 boys

mean age at interview: 13.1 years (median 12.4; range 6.9 to 25.0)

mean age at procedure: 9.1 years (median 7.8, range 3.1 to 18.5)
-

recurrent soiling: 29/42 (69%)

-

inability to adequately pass stool: 7/42 (17%)

-

recurrent hospital admissions for nasogatric washouts: 6/42 (14%)

Country:
Australia
Intervention:
appendicostomy (ACE):
laparoscopy or mini-laparotomy

Comparison:
none

Enemas:
-

median initial regimes used:

  • Golytely (PEG 3350 and electrolytes): 250 to 500 ml every second day, infused over 20 to 30 mins for 1 to 3 months
    Liquorice , 250 to 500 ml daily, infused over 10 to 20 mins infused over 10 to 20 mins for 1 to 3 months
-

median regime at time of interview:

  • Golytely (PEG 3350 and electrolytes): 500 to 750 ml every second day, infused over 10 to 20 mins with no need for disimpaction
Follow-up period:
Mean: 48 months (median 39, range 3 to 118)

Outcome Measures:
-

ACE usage

-

ACE efficacy

-

ACE complications

ACE usage
  1. ACE regimes
    -

    median initial regimes used (% children):

    • Golytely (79)
      Liquorice (12)
      Water (2)
      Other (7)
    -

    outcome (% children):

    • Excellent (29)
      Good (36)
      Average (7)
      Poor (28)
    -

    median regime at time of interview:

    • Golytely: (how many children?): Defecation occurred 20 to 30 mins after ACE finished, with 20 to 30 mins spent on toilet
    • Majority of patients (25/42, 60%) either using the initial regime or had tried one regimen change. No correlation between numbers of ACE regimens tried, patient satisfaction or length of ACE usage. Many families believed regimes changes were a necessary response to increased tolerance to a particular ACE solution
  2. patient input into ACE regimen (n children)
    -

    completely independent: 7 (all older 10 years)

    -

    requiring supervision only: 5

    -

    needing help setting up and cleaning up: 15

    -

    completely dependent: 15

  3. patients satisfaction with ACE (n children)
    -

    very satisfied or satisfied: 37 (88%)

    -

    families would recommend ACE to other children: 41 (98%)

    -

    families felt significant improvement in quality of child's life: 39 (93%)

    -

    mean optimal age for appendicostomy formation, as felt by families: 4.9 years (median 4, range 2 to 12)

  4. effectiveness
    -

    effective: 41 (98%)

  5. symptoms resolution (n patients)
    -

    ceased ACE: 15 (36%): in 7 symptoms resolved, in 4 a colostomy was formed, in 2 an ileostomy was formed and 2 patients returned to conservative management

    -

    successful ACE: 34 (81%)

ACE efficacy (mean, median and range):
-

continence score:

pre-ACE: 2.5 ( 2; 0 to 8)

post-ACE:: 5.2 (5; 1 to 12)

p<0.0001

-

quality of life score:

pre-ACE: 1.4 (1.5; 0.5 to 3.0)

post-ACE: 2.2 (2.5; 0.5 to 3.0)

p<0.0001

-

soiling frequency score:

pre-ACE: 5.7 (6; 0 to 6)

post-ACE: 3.0 ( 3; 0 to 6)

p<0.0001

-

abdominal pain severity score:

pre-ACE: 7.4 (8; 0 to 10)

post-ACE: 3.0 (3; 0 to 8)

p<0.0001

-

abdominal pain frequency score:

pre-ACE: 5 (6; 0-6 to 3-6 d/week)

post-ACE: 2.5 (2.5; 0-6 to 1-2 d/month)

p<0.0001

ACE complications:
  1. symptoms at some stage of treatment:
    • Total: 30/42 (71%)
      cramping: 18/30
      nausea: 17/30
      vomiting: 7/30
      sweating: 14/30
      dizziness: 10/30
      pallor: 10/30
      (3 or more symptoms present in 12/30 patients)
  2. Long-term complications (n, %), N=42:
    -

    granulation tissue: 33 (79), unresolved: 15%

    -

    anxiety about ACE: 21 (50), unresolved: 29%

    -

    stomal infection: 18 (43), unresolved: 11%

    -

    stomal leakage (ACE days): 16 (38), unresolved:13%

    -

    embarrassment about device: 16 (36), unresolved: 87%

    -

    dislikes device: 12 (29), unresolved: 58%

    -

    stomal leakage (non ACE days): 12 (29), unresolved: 8%

    -

    stomal pain: 11 (26), unresolved: 45%

    -

    stomal stenosis: 8 (19), unresolved: 0

    -

    new behavioural disturbance: 7 (17), unresolved: 72%

    -

    stomal prolapse: 6 (14), unresolved: 33%

    -

    stomal bleeding: 6 (14), unresolved: 0

    -

    limited activity: 4 (10), unresolved: 75%

    -

    weight loss: 2 (5), unresolved: 0

    -

    perforation: 2 (5), unresolved: 0

Additional information from study:
Independent investigator conducted confidential telephone interviews using a modified questionnaire

Continence score: modified Holschneider (maximum score 12). Modification required because the criterion of “frequency of defecation” not appropriate for the cohort

Quality of life score: modified Templeton and Toogood

Frequency score used for all frequency measures: daily=6, 3 to 6 d/wk=5, 1 to 2 d/wk=4, 1 to 2 d/fortnight=3, 1 to 2 d/mo=2, once every 2 to 3 months=1 and never=0)

Reviewer comments:
Originally 56 children met the inclusion criteria, but only 42 (75% of the families) were interviewed without a clear explanation for that

Source of funding: Dr. King funded by scholarships from the NHMRC (Australia) and the Royal Australian College of Surgeons
Youssef et al. Management of intractable constipation with antegrade enemas in neurologically intact children. 2002. Journal of Pediatric Gastroenterology and Nutrition 34[4], 402-405Study Type:
Retrospective case series

Evidence level:
3

Study aim:
to assess the benefit of antegrade colonic enemas through caecostomy catheters in children with severe constipation who were referred to a tertiary care centre
12 children

Inclusion criteria:
children referred to a tertiary care motility centre for further evaluation of intractable constipation, who had undergone caecostomy placement for administration of antegrade enemas

Exclusion criteria:
neurologic handicap and other organic causes of constipation
12 children
9 boys
mean age: 8.7 ± 4.4 years

Country:
USA
Intervention:
Caecostomy (surgically and by interventional radiology)

Comparison:
none

Choice of irrigation solution used after caecostomy varied, based on preference of treating physician. Most patients began with low volume infusions of solution, which were increased according to therapeutic response. 67% of patients used 200 mL to 1,000 mL (mean 478 mL ± 262 mL) polyethylene glycol irrigation solution, daily to every other day. 25% of patients used a combination of saline and glycerin, mixing 60 mL to 75 mL of glycerin in 240 mL to 300 mL of saline. 1 patient received 90 mL phosphate soda solution followed by 300 mL of saline. Evacuation occurred within 1 hour of enema administration in 7 children and occurred within 3 hours in the other 5 children.
Follow-up period:
13.5 ± 8.5 months

Outcome Measures:
-

Bowel movements/week

-

Soiling episodes/week

-

Number of medications used for constipation

-

Episodes of abdominal pain/week

-

Missed school days/month

-

Emotional health

-

Overall health

-

Physician office visits/year

Bowel movements/week
before: 1.4 ± 0.7
after: 7.1 ± 3.8
p<0.005

Soiling episodes/week
before: 4.7 ± 3.2
after: 1.0 ± 1.4
p<0.01

Number of medications used for constipation
before: 4.0 ± 1.0
after: 0.8 ± 0.6
p<0.005

Abdominal pain score:
before: 2.9 ± 1.6
after: 0.9 ± 1.0
p<0.005

Missed school (days/month)
before: 7.5 ± 6.9
after: 1.5 ± 2.5
p<0.02

Emotional health score
before: 1.9 ± 0.8
after: 3.6 ±1.1
p<0.005

Overall health score:
before: 1.7 ± 0.9
after: 3.6 ± 0.9
p<0.005

Physician office visits/year
before: 24.0 ± 19.1
after: 9.2 ± 14.2
p<0.05
No acute adverse events

Postoperative adverse events (n children):
-

skin breakdown and development of granulation tissue: 1

-

leakage of irrigation solution: 1

-

accidental removal of the catheter with subsequent easy catheter replacement by the interventional radiologist: 2

No adverse event led to discontinuation of antegrade enema use.
No child has required admission to a hospital because of faecal impaction since starting antegrade enemas. 5 patients discontinued antegrade enemas with removal of the caecostomy at a mean of 14.6 ± 9.1 months after beginning treatment. None has redeveloped problems with constipation or faecal soiling.
Additional information from study:
A questionnaire used to interview caregivers 13.5 ± 8.5 months after caecostomy placement. No caregiver refused to participate in interview

Scoring for episodes of abdominal pain: 0 = none, 1=once or twice, 2=a few times, 3=fairly often, 4=very often, 5= everyday

Scoring for overall health and emotional state: 1=poor, 2=fair, 3=good, 4=very good, 5=excellent

Reviewer comments:
Very small sample

Not clear who performed the review of the clinical records

Not clear who interviewed the parents

Researchers not reported blinded

Questionnaire not reported piloted/validated

Source of funding: not stated
Cascio et al. MACE or caecostomy button for idiopathic constipation in children: a comparison of complications and outcomes. 2004. Pediatric Surgery International 20[7], 484-487Study Type:
Retrospective cohort

Evidence level:
2+

Study aim:
to compare the results complications and outcomes of the Malone antegrade enema (MACE) with the caecostomy button (CB) in children with intractable constipation
49 children

Inclusion criteria:
children who underwent MACE or CB between June 1998 and August 2002 for intractable idiopathic constipation and faecal soiling that had failed conventional treatment

Exclusion criteria: not clearly stated, but all rectal biopsies were aganglionic.
49 children
15 boys
-

MACE:

37 children

15 boys

-

CB:

12 children

9 boys

Country:
UK
Intervention:
Malone antegrade enema (MACE)

Antegrade enemas started on the 4th postoperative day and Foley catheter left in appendicostomy for 6 weeks

Comparison: Caecostomy button (CB)

Enemas started on 4th postoperative day and MIC-KEY gastrostomy tube changed to standard gastrostomy button after 6 weeks

Enemas performed by administering saline (20ml/kg) to empty the entire colon at a convenient time for patient. Children not responding to saline wash-out used Klean-Prep. Frequency and volume of enemas individualised to each patient to achieve cleanliness and stop soiling
Follow-up period:
Mean, 18 months

Outcome Measures:
-

Soiling

-

Failure

-

Surgical complications

Soiling (n children in which stopped completely)

MACE (n=37): 30 (81%)

CB (12): 9 (75%)

Occasional soiling still present in 1 child with MACE and 2 with CB. 1 child with CB resumed regular activity and CB was removed

Failure
-

MACE (n=37): 6 (16.2%)

4 patients' colonic washouts ineffective. 1 patient: colonic washout associated with abdominal pain during enema. 1 patient required revision for perforation of appendicostomy and the fibrotic-ischaemic appendix was replaced with a CB
-

CB (12): 1 (8.3%) Reason for failure was leaking faecal content around the button, converted to MACE after 20 months P >0.05

Surgical complications %):
  1. requiring operative intervention MACE (n=37)
    -

    total: 9 (24%)

    -

    stoma stenosis: 11%

    -

    iatrogenic perforation appendicostomy: 5%

    -

    difficult catheterization: 5%

    -

    adhesive obstruction: 3%

    CB (n=12)
    -

    total: 0

    -

    adhesive obstruction: 0

    Others N.A

    P=0.009 for total
  2. not requiring operative intervention
    MACE (n=37)
    -

    total: 7 (19%)

    -

    pain/difficult catheterisation: 11%

    -

    stoma granulosa: 5%

    -

    stoma stenosis: 3%

    -

    faecal leakage: 0

    -

    pain around button: N.A

    CB (n=12)
    -

    total: 11 (92%)

    -

    pain/difficult catheterisation: N.A

    -

    stoma granulosa: (33%)

    -

    stoma stenosis: N.A

    -

    faecal leakage: 42%

    -

    pain around button: 92%

    p<0.001 for total
Additional information from study:
One patient with CB and one with MACE moved to another region and were lost to follow-up

Success criteria:
-

full: totally clean or minor or minor rectal leakage on the night of the washout;

-

partial: clean, but significant stomal or rectal leakage, occasional major leak, still wearing protection but perceived by the child or parent to be an improvement

-

failure: regular soiling or constipation persisted , no perceived improvements, procedure abandoned usually to a colostomy

Source of funding: not stated
Curry et al. The MACE procedure: experience in the United Kingdom. 1999. Journal of Pediatric Surgery 34[2], 338-340Study Type:
Retrospective survey

Evidence level:
3

Study aim:
to find out the current status of the Malone Antegrade Continence Enema (MACE)
273 children

Inclusion criteria:
MACE procedures performed by UK members of the British Association of Paediatric Surgeons (or their units) up to the end of 1996

Exclusion criteria:
not clearly stated
273 children

Mean age: 12.3 years

Country:
UK
Intervention:
Malone Antegrade Continence Enema (MACE)

Comparison: None
Follow-up period:
Mean 2.4 years (range 0.3 to 6)

Outcome Measures:
-

children diagnoses

-

success rate

-

complications encountered

Overall success rate Including both full and partial):
79%

Success rate based on diagnosis (%):

Constipation (n=23)

Full: 52
Partial: 10
Failure: 38
Unknown: 1
Additional information from study:
Results included figures from authors' previous study, reported figures from one other UK centre and replies to proformas sent by authors to BAPS members

102 proformas sent, 58 returned

Success criteria:
-

full: totally clean or minor or minor rectal leakage on the night of the washout;

-

partial: clean, but significant stomal or rectal leakage, occasional major leak, still wearing protection but perceived by the child or parent to be an improvement

-

failure: regular soiling or constipation persisted , no perceived improvements, procedure abandoned usually to a colostomy

Reviewer comments:
Retrospective study

Low response rate to the proforma

Results for patients with diagnoses other than constipation not reported here because they are outside the remit of this review.

Main complications not related in paper to the clinical diagnosis and therefore not reported here

Source of funding: not stated
Mousa et al. Cecostomy in children with defecation disorders. 2006. Digestive Diseases and Sciences 51[1], 154-160Study Type:
Retrospective cohort

Evidence level:
2+

Study aim:
To report authors' 4-year experience with 2 different techniques of the caecostomy procedure and to compare the clinical outcome of caecostomy in children with defection disorders secondary to functional constipation, imperforate anus and spinal abnormalities
31 children

Inclusion criteria:
Children who received a caecostomy for constipation, faecal soiling or a combination of both. Underlying conditions included functional constipation, Hirschsprung's disease, imperforate anus, imperforated anus combined with tethered spinal cord syndrome and spinal abnormalities

Exclusion criteria:
Not stated
-

total population 31 children 58% boys

-

9 children with functional constipation

median age at time of caecostomy: 12 years old (range 3 to 16)

Country:
USA
Intervention:
Caecostomy performed percutaneously by interventional radiologist

Comparison:
Caecostomy performed by open surgical approach
Duration of study period:
4 years

Follow-up period:
Median 11 months (range 1 to 45) after caecostomy

Outcome Measures:
-

type of antegrade enemas used

-

bowel movement frequency

-

soiling frequency

-

number of medications

-

number of physician visits related to defecation problems

-

number of hospital admissions for disimpaction

-

number of missed school days per month

-

quality of life

-

complications

(all values are median)

Type of antegrade enemas used
No subgroup analysis performed

Bowel movement frequency (n=9)
Pre: <5/week
Post: 5/week to 3/day
P<0.01

Soiling frequency (n=9)
Pre: constant
Post: none
P=0.01

Number of medications (n=9)
Pre: 4
Post: 1
P=0.01

Number of physician visits related to defecation problems (n=9)
Pre: 6
Post: 2 P<0.01

Number of hospital admissions for disimpaction (n=9)
Pre: 4
Post: 0
P<0.01

Number of missed school days per month (n=9)
NS

Global health score (n=9)
Pre: poor
Post: good
P=0.01

Global emotional score (n=9)
Pre: poor
Post: good
P=0.01

Limitations of activity (n=9)
Pre: moderate
Post: mild
P<0.01

Complications
No major complications like perforation, stoma stenosis, or stoma prolapse. No difference found in occurrence of number of complications between different procedures/techniques

Other outcomes not reported here as no subgroup analysis performed
Additional information from study:
Standardised questionnaire used to obtain data on outcomes measured

Frequency of bowel movements scored as: 1, <5 bowel movements/week; 2, 5/week to 3/day; 3, 3/day

Soling frequency scoring: 1, none; 2, occasional, 3, few episodes/week; 4. few episodes/week to daily; 5, constantly

Quality of life assessed by scoring limitations of activity (none, mild, moderate and severe), global health score, and global emotional score (poor, fair, good, very good and excellent)

Reviewer comments:
Not clear who interviewed the parents

Source of funding: study supported in part by the Ter Meulen Fund, Royal Netherlands Academy of Arts and Sciences
Jaffray. What happens to children with idiopathic constipation who receive an antegrade continent enema?. An actuarial analysis of 80 consecutive cases. 2009. Journal of Pediatric Surgery 44[2], 404-407United States.Study Type:
Prospective case series

Evidence level:
3

Study aim:
to perform an actuarial analysis of the outcomes of antegrade continent enema (ACE) procedure in children who have idiopathic constipation and who did not respond to 3 years of medically supervised conservative management
80 children

Inclusion criteria:
All children with idiopathic constipation undergoing ACE surgery by 1 surgeon. In all children symptoms had persisted despite medical management supervised by paediatrician for at least 3 years

Exclusion criteria:
Hirschsprung's disease (excluded by rectal biopsy in all cases)
80 children

44 boys

median age at surgery: 9.6 years (range 3.4 to 18.7 years)

Country:
UK
Intervention:
Antegrade continent enema (ACE) procedure

Children followed up in a nurse-led continence clinic

Lavage regime was supervised by specialist nurses and used a solution of saline prepared by parents at a volume of 20mL/kg body weight

Comparison:
N.A
Follow-up period:
6 months to 10 years (median 6.2 years)

Outcome Measures:
-

Ongoing lavage

-

Failure: either the parents have stopped using the technique because colonic lavage has not been found to improve the child's bowel habit or the child's colon had not proved to be lavageable and symptoms had deteriorated

-

Cure: the appendicostomy was closed/reversed because the child achieved normal bowel habit

53 children: conventional ACE

27 children: laparoscopic ACE
-

ACE lavage failed in 12 children:

  • 4 children were identified where the appendicostomy was not being used. Although these children could be lavaged, parent's had not found it to be of help in the child's bowel management and had ceased use
    In 8 children, deterioration of symptoms occurred despite ACE lavage and required alternative treatment of symptoms. These children could not be lavaged.
  • Kaplan Meier probability of an ACE failing:
    0.3 at 8.5 years; estimated mean failure time: 8.6 years (95% CI 7.9 to 9.2)
-

12 children had normal bowel habit, no longer performed colonic lavage and underwent closure of appendicostomy. The Kaplan Meier probability of an ACE being reversed was 0.2 at 6.2 years, estimated mean time to reversal (9.1 years (95% CI: 8.4 to 9.7)

-

56 children currently performing colonic lavage.

Colonic transit time (CTT), age at surgery and duration of follow-up were not significantly associated with ACE failure, but sex was (p=0.04) the higher failure rate amongst girls was significant (p=0.02)

CTT significant factor in predicting failure in children who accommodated very large volume of lavage fluid (>10 L) in their colon without bowel evacuation. Median CTT for this subset significantly longer than for children who could be lavaged (141 h (SD 30) vs. 73 h (SD 17); 95% CI difference 9 to 74 h; p=0.01)

Additional information from study:

In the first 32 cases the diagnosis was confirmed by the use of marker studies using an established protocol. However because the marker studies did not alter treatment decisions and to avoid unnecessary radiation exposure, this practice was stopped

Previous treatment was heterogeneous and had always included prolonged treatment with laxatives, usually with periods of in-patient administration of surgical bowel cleansing solutions, frequent manual disimpaction and often involvement of a clinical psychology service

In calculating the Kaplan Meier probability of an ACE being reversed or failing, the following times were calculated:
-

ongoing lavage: length of follow up calculated as time from the date of formation of ACE to current date

-

time to failure calculated as the time from creation of the ACE to the clinic letter stating that the parents had ceased using the ACE, or the date of commencement of alternative treatment.

-

cure: the date of the operation to reverse the ACE was used as the censoring time

A minimum of 6 months follow-up judged to be appropriate because a decision regarding “cure” would take no less than 6 months to determine

Children who could not be lavaged defined as those having failed to have a bowel evacuation despite an appropriate volume of lavage fluid. These children were assessed by performing continuous lavage though the appendicostomy over several days while in hospital. Typically such children accommodate very large volumes of fluid in their colon, often in excess of 10 L without bowel evacuation

Criteria for ACE reversal: for at least the previous 6 months, child had stopped using their ACE, was stooling spontaneously at least every other day, was not requiring laxative therapy and was not soiling. ACE reversed by dissecting the appendix to the caecal wall and ligating and removing it

No patient was discharged, and none was lost to follow up

Source of funding: Not stated

From: Appendix J, Evidence tables

Cover of Constipation in Children and Young People
Constipation in Children and Young People: Diagnosis and Management of Idiopathic Childhood Constipation in Primary and Secondary Care.
NICE Clinical Guidelines, No. 99.
National Collaborating Centre for Women's and Children's Health (UK).
London: RCOG Press; 2010.
Copyright © 2010, National Collaborating Centre for Women's and Children's Health.

No part of this publication may be reproduced, stored or transmitted in any form or by any means, without the prior written permission of the publisher or, in the case of reprographic reproduction, in accordance with the terms of licences issued by the Copyright Licensing Agency in the UK [www.cla.co.uk]. Enquiries concerning reproduction outside the terms stated here should be sent to the publisher at the UK address printed on this page.

The use of registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant laws and regulations and therefore for general use.

PubMed Health. A service of the National Library of Medicine, National Institutes of Health.