ORIGINAL ARTICLE
Malone antegrade continence enema (MACE) for fecalincontinence in imperforate anus improves quality of life
Kelly D. Mattix Æ Nathan M. Novotny ÆAnita A. Shelley Æ Frederick J. Rescorla
Published online: 16 October 2007
� Springer-Verlag 2007
Abstract The MACE procedure has been used in patients
with imperforate anus (IA) to improve fecal continence. Our
aim was to assess the impact of the MACE on the quality of
life (QOL) in children with IA and fecal incontinence. A
retrospective review was performed of children with IA that
underwent the MACE procedure between 1997 and 2004.
Patients and their parents were contacted by telephone sur-
vey regarding continence and its psychosocial effects before
and after MACE. The same survey was given to the patients’
teachers. Responses to 15 questions were compiled and a
QOL score calculated and significance evaluated by t-test
(P £ 0.05). IRB approval was obtained. Thirty-two patients
were identified with a mean age at operation of 9 years (4–
19 years) and mean follow-up of 3.8 years (7 months to
8 years). Four patients had a low malformation, 8 were
intermediate, 15 were high, and 5 had a cloacal anomaly.
Twenty patients had documented sacral/spinal anomalies,
including five with tethered cord. Post-MACE complica-
tions included stenosis in 16 (50%), with 11 requiring an
operative revision at a mean of 21.7 months (2 months to
6 years), takedown in one at 4 years and volvulus in one at
18 months. Prior to the MACE, 18/25 (72%) had poor QOL
scores. Post-MACE QOL results were similar between
patients, parents and teachers. Patients’ mean QOL score
improved from 59.9 to 26.3% (P \ 0.001), with parents
from 59.7 to 26.4% (P \ 0.001). QOL score improved
[50% in nine families, 25–50% in ten and\25% in six. All
patients and parents interviewed reported an improvement in
their QOL following the MACE. This procedure should be
offered to children with IA with the expectation of signifi-
cant improvement in QOL.
Keywords Malone antegrade continence enema �MACE � Antegrade continence enema � Ace �Quality of life
Introduction
Malone first described the antegrade continence enema in
1990 [1]. The Malone Antegrade Continence Enema
(MACE) procedure has been utilized in children and adults
with fecal incontinence and constipation with a wide variety
of primary diagnoses [2–7]. The MACE procedure has been
adopted as an accepted treatment for children with imper-
forate anus (IA) and poor fecal continence. While studies
have assessed quality of life (QOL) after repair of IA, none
has addressed QOL of patients with IA after MACE [8–12].
Our aim was to assess the impact of the MACE on the QOL
in children with IA and fecal incontinence.
Materials and methods
A retrospective chart review was performed of children
with IA undergoing MACE between 1997 and 2004 at
Presented as an oral presentation at the 14th Annual International
Colorectal Club Conference and Exhibition in York, England, July
14–16, 2007.
A. A. Shelley � F. J. Rescorla (&)
Section of Pediatric Surgery,
Indiana University School of Medicine and Riley Hospital
for Children, RH 2500, Indianapolis, IN 46202, USA
e-mail: [email protected]
K. D. Mattix � N. M. Novotny
Department of Surgery, Indiana University School of Medicine
and Riley Hospital for Children, 545 N. Barnhill Dr. EH202,
Indianapolis, IN 46202, USA
e-mail: [email protected]
123
Pediatr Surg Int (2007) 23:1175–1177
DOI 10.1007/s00383-007-2026-3
Riley Hospital for Children in Indianapolis, IN, USA.
Demographic information, type of malformation and
associated comorbidities, and clinical outcomes including
need for additional procedures were collected.
A telephone survey of 15 questions was developed for
parents, patients, and school teachers of the children to
assess QOL at home and school. Patients were questioned
regarding their current situation post-MACE and retro-
spectively, prior to the MACE procedure. The questionnaire
administered to the teachers included questions specific to
post-MACE, as the teacher may or may not have known the
child prior to the procedure. The questionnaires were
administered by a surgical resident during a scripted tele-
phone conversation to confirm willingness to participate in
the study.
The data were subjected to statistical analysis with the
Student’s t-test. P values of \0.05 were considered statis-
tically significant. IRB approval was obtained from the
participating institution.
Results
Patients
Thirty-two patients were identified with IA who underwent
MACE procedures. (Table 1) Fifty-three percent (17/32) of
the children were females. Mean age at MACE was 9 years
(range 4–19 years). Four children had low, 8 had inter-
mediate, 15 had high malformations, and 5 had cloacal
anomaly. Associated anomolies included sacral/spinal
(n = 20), genitourinary (n = 28), cardiac (n = 7), gastro-
intestinal (gastroschisis n = 1, trachoesophageal fistula
n = 2, duodenal atresia n = 1), and chromosomal (n = 1).
Mean follow up was 3.8 years (range 7 months to 8 years).
Complications associated with the MACE procedure are
summarized in Table 2. They included: stenosis in 16/32
patients (50%) with 11 requiring operative revision, rang-
ing from 2 months to 6 years after their MACE (average
21.7 months); takedown at 4 years in one (secondary to
a dysmotile colonic segment); and volvulus in one at
18 months.
QOL
Twenty-five (78% of the total number of patients with IA
who underwent MACE) patients or parents participated in
the QOL survey. Twelve teachers participated. Prior to the
MACE, 18/25 (72%) had poor QOL scores. Answers related
to post-MACE questions were similar among patients, par-
ents, and teachers. Patients’ QOL scores significantly
improved (P \ 0.001) from 59.9 (range 29–76 %) to 26.3%
(range 6–50%). Parents, also reported a significant increase
in QOL (P \ 0.001) from 59.7 (range 18–94%) to 26.4%
(range 4–53%). All patients reported an improvement in
QOL. QOL improved[50% for nine families, 25–50% for
ten, and\25% for six.
Discussion
The MACE procedure has been utilized in patients with IA
as an adjunct treatment for treating continence issues. Few
studies have assessed the relationship to the patients’ QOL
following this procedure [7, 13, 14]. Others have studied
both the complications related to the procedure including
reoperation, stomal stenosis and volvulus, and the devel-
opment of continence [5, 6, 14–18]. Our study focused
specifically on patients with IA who have been treated with
the MACE procedure.
The MACE has been shown to significantly improve
continence in varied patient populations, including children
with fecal incontinence [6, 13], adults with idiopathic
constipation [5] and patients with myelomeningocele [7].
Likewise, we saw a significant improvement in continence
in our patient population with 22 of 25 having staining less
than once per week with no need for underwear changes.
Similar to prior studies, our patient population had a high
stomal stenosis rate. Malone initially described frequent
catheterizations, at least daily, to prevent this complication
[15]. Other studies have seen similar results in stomal stenosis
rates of 18–39% [6, 7, 16, 17, 19]. This complication appears
to be decreased with frequent dilations/catheterizations and
the inlay of skin at the cutaneous stomal anastomsosis.
Table 1 Patient demographics
No. of patients
n 32
Male 15
Female 17
Low malformation 4
Intermediate malformation 8
High malformation 15
Cloacal anomaly 5
Age (years) at MACE 9 (4–19)
Table 2 Complications experienced after MACE procedure
Complication No. of patients
Stenosis 16 (50%)
Operative reintervention 11
Volvulus 1 (3%)
Takedown 1 (3%)
1176 Pediatr Surg Int (2007) 23:1175–1177
123
Assessment of QOL has been performed by many
methods. Our data demonstrated a significant improvement
in QOL by both the patients and their parents. This was
confirmed by a third party, in that the QOL score given by
the teachers was similar to that of the patient and parent.
The limitations to this method include the need for retro-
spective data by requiring the patients to answer questions
regarding their lifestyle pre-MACE. Additionally, the data
would be strengthened by annual measurements of QOL as
performed by Ditesheim et al. [10].
Patients with IA have significantly improved QOL fol-
lowing the Malone antegrade continence enema. They
report improved continence with the most frequent com-
plication of stomal stenosis able to be treated without
surgical intervention in the majority of cases. This proce-
dure can be offered to patients with IA suffering from
incontinence with the expectation of improvement in their
QOL.
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