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: frescorl@iupui.edu

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: nmnovotn@iupui.edu

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

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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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