This case series examines the experience with Malone antegrade continence enema (ACE) procedure in 11 patients at a pediatric hospital from 2003 to 2016. The ACE procedure improved bowel control and quality of life in most patients. However, over 40% discontinued use of the ACE after a median of 11 years, often due to symptom resolution or non-compliance. Complications included stomal stenosis requiring revision in some cases. Overall, the ACE provided satisfactory initial results for managing severe constipation and incontinence in selected pediatric patients.
1. 葉奕廷 王國強 蔡昕霖 劉君恕 錢大維
台北榮總兒童外科
The Experience of
Malone
Antegrade
Continence
Enema
in VGHTPE
2. Case Series of ACE at VGHTPE (‘03-’16)
# Etiology
Age
at OP
Type of
OP
Known
Complications
F/U
period
Documented
improve
Current ACE Use
1 ARM 10 Malone nil 2 year yes n/a
2 Syringomyelia 21 Malone nil 12 years yes
No (symptoms
resolved)
3 ARM 20 Malone nil 1 year yes n/a
4 ARM 8 Malone Wound infection 12 years yes Yes
5 Spinal bifida 7 Malone Stomal stricture 1 year yes n/a
6 ARM 19 Monti Stomal stricture 7 years yes Yes
7 Spinal bifida 13 Malone Stomal stricture 11 years yes
No (rectal
irrigation)
8 Spinal bifida 12 Malone Stomal stricture 10 years yes
No (symptoms
resolved)
9 ARM 28 Monti Stomal stricture 9 years yes Yes
10
Sacral
meningocele
14 Malone nil 1 year yes No (not effective)
11 ARM 23 Malone nil 1 year yes Yes
n/a: not available for chart/telephone interview
3. Indication for ACE
ARM:
High type, or low type s/p multiple
operations with severe to total
incontinence with constipation
Neurogenic incontinence:
Severe constipation with total
incontinence
Exclusion: idiopathic constipation
4. Surgical Technique
Appendix present: Malone's method
Non-reverse,in-situ appendicostomy
Cecal flap (seromuscular) wraping around appendix
Fashioning of stoma by V-shape skin flap or direct
anastomosis
Appendix not present: Monti's method
One segment of terminal ileum(about 40-60 cm from
ileocecalvalve) was used as conduit
Tapering of the conduit at anti-mesenteric side
Irrigation began 5-7 days post-op
Stent removed 2 weeks post-op
5. Results of Procedure: Use of ACE
Patient Number Irrigant Frequency Time Spent on Enema
2 Tap water 1/day 45 min
4 Saline + glycerin 2-3/week* 60 min
6 Tap water 1/day 50 min
7 Saline + glycerin 1/day 60 min
8 Saline + glycerin 1/day 60 min
9 Tap water 1/day 20 min
10 GB 1/day Variable
11 Saline 2/day 60 min
* The patient is completely clean if ACE is performed once daily, but
modified to 2-3/week due to lifestyle
6. St. Mark’s Hospital Incontinence Score
Item Never Rarely Sometimes Weekly Daily
Incontinence for solid 0 1 2 3 4
Incontinence for liquid 0 1 2 3 4
Incontinence for gas 0 1 2 3 4
Alteration in lifestyle 0 1 2 3 4
No Yes
Need to wear pad 0 2
Taking constipating medicine 0 2
Lack of ability to defer defecation
for 15 minutes
0 4
* Never: no episode in recent 4 weeks; rarely: 1 episode in recent 4
weeks; sometimes, > 1 episode in recent 4 weeks but not weekly
Vaizey CJ, Gut 1999
7. Rating of Outcome: Continence
St. Mark’s Hospital Incontinence Score
Patient Number Continence Score (0 – 24)
2 10
4 18
6 9
7 15
8 20
9 15
10
11 14
Median 15
* Worse performing items: incontinence of gas, need to wear pads,
lack of ability to defer defecation
8. Rating of Outcome: Confidence and
Satisfaction
Item #2 #4 #6 #7 #8 #9 #10 #11 Median
Confidence in
one’s ability to
manage the
irrigation
8 10 8 7 7 10 7 6 7.5
Confidence that
ACE will give
control to one’s
bowel
9 9 8 6 6 10 7 7 7.5
Confidence to go
to social event 8 9 10 8 6 10 7 6 8
Confidence to
undertake daily
activities
10 10 10 10 7 7 7 6 8.5
Confidence to
attend full-day
activities
7 10 8 10 6 8 7 6 7.5
Overall satisfaction
of life 8 10 8 8 8 6 8 6 8
Overall satisfaction
with the procedure 9 10 8 6 6 9 6 6 7
9. Reason for Discontinuation of ACE
(Patients #2, #7, #8, #10)
Patient #2 discontinued ACE 4 years after the
procedure because of resolution of symptoms
Patient #7 discontinued ACE 6 years after the
procedure because she felt that the effectiveness
decreased and shift to colonic hydrotherapy machine
at home to manage her bowel
Patient #8 discontinued ACE 2 years after the
procedure because she felt that the effectiveness
decreased. In addition, she regained continence
gradually over the next 4 years and is currently partially
continent without intervention
Patient #10 discontinued ACE 3 months after the
procedure because the time interval to rectal
emptying is highly variable (irrigant: GB only)
10. Reason for Lower Satisfaction
(Patients #7, #8, # 11)
Patient #7: weekly incontinence for solid stool,
alteration of lifestyle and personal preference
of rectal irrigation with good result
Patient #8: weekly incontinence for solid stool
Patient #10: highly variable time interval to
rectal emptying (irrigant: GB only)
Patient #11: comorbidity of schizophrenia,
poor self-care, weak family support, totally
rely on caretaker to administer the irrigations
11. Conclusions
ACE can provide satisfactory initial result
(improvement of bowel control and quality
of life) for most patients
Early “failure” may be related to poor
compliance/self-care/family support
Late “failure” may be related to gradual
loss of effect or symptom relieve
13. Fecal Incontinence in the Pediatric
Population
Prevalence of fecal incontinence among children: 0.8 – 7.8%
Chronic constipation is the underlying cause in 90% of the cases
Causes of chronic constipation:
Idiopathic constipation
Anorectal malformation
Neuropathic
Hirschsprung disease
Gastrointestinal dysmotility
Fecal incontinence significant decreases quality of life and causes
behavioral and emotional problems
One-third of patients have persistent incontinence into adulthood
Basson S, Pediatr Surg Int 2014
14. Management of Severe Constipation
Large-volume colonic enema
Route of administration
Retrograde: limited by the patients ability to
resist the urge and the ability of the sphincter,
thus only clears the distal colon
Antegrade: via an appendicostomy or
cecostomy
Initially described in 1990 by Malone
More effective in evacuation of both sides of the colon
15. Success Rate of the Procedure
High success rates (78-94%) have been reported
Basson S, Pediatr Surg Int 2014; Randall J, J Pediatr Surg 2014
However, more than 40% of patients discontinuing use of their ACE
after a median of 11 years in one study
Yardley ID, J Pediatr Surg 2009
Reason for discontinuation:
Successful resolution of symptoms
Disuse due to non-compliance or ineffectiveness
Predictor of poor outcome:
Underlying etiology: worse prognosis in idiopathic constipation or GI
dysmotility patients
Age at operation: younger patients (<5 y/o) may have decreased
compliance and more complications (controversial)
Basson S, Pediatr Surg Int 2014
16. Common Complications of ACE
Stenosis of the appendicostomy requiring surgical
revision (usually at or near the skin level) is the
most common complication
Randall J, J Pediatr Surg 2014
Other complications include: stomal granulation,
stomal leakage, stomal prolapse, local infection
and incisional hernia