prevalence of 0.7% to 29.6% with no differences found fecal incontinence, and abdominal pain. Less than 5% to
stenosis, anorectal malformations), metabolic and gastroin-
Journal of Pediatric Surgery (2011) 46, 16031608 Corresponding author.between Europe, Oceania, and North America . The main 10% of children with constipation have an underlyingorganic cause, such as anatomic malformation (ie, analMethods: A retrospective analysis of 23 patients who underwent the construction of a MACE stoma wasperformed. Preoperative and postoperative data were evaluated. A specific questionnaire was used toassess patient satisfaction.Results: A significant increase was found in defecation frequency (1.0 [range, 0-4] pretreatment vs 5.5[range, 0-28] posttreatment per week; P b .006) and a significant decrease in fecal incontinencefrequency (10 [range, 0-14] pretreatment vs 0 [range, 0-14] posttreatment per week; P b .034).Postoperative complications of the MACE procedure were fecal leakage (43%), wound infection (52%),and stomal stenosis (39%). A total of 86% of the patients were satisfied with the results of the Malonestoma (n = 21).Conclusions: The MACE procedure is an effective treatment in children with intractable defecationdisorders. Postoperative complications are, however, not uncommon. Further refinement of the techniquefocused to reduce the complication rate is necessary to expand the application of this approach. 2011 Elsevier Inc. All rights reserved.
Constipation is a common problem in childhood with a symptoms of constipation are infrequent painful defecation,0dKey words:Malone antegradecontinence enema;
MACE;Intractable constipation;Fecal incontinenceLisette T. Hoekstra a,, Caroline F. Kuijper a, Roel Bakx a, Hugo A. Heij a,Daniel C. Aronson a, Marc A. Benningab
aPediatric Surgical Center of Amsterdam, Emma Children's Hospital / AMC, Academic Medical Center, Amsterdam 1105 AZ,The NetherlandsbDivision of Pediatric Gastroenterology, Emma Children's Hospital / AMC, Academic Medical Center, Amsterdam 1105 AZ,The Netherlands
Received 19 October 2010; revised 7 April 2011; accepted 7 April 2011
AbstractBackground: The Malone antegrade continence enema (MACE) procedure has been previouslydescribed as a safe and effective option for the treatment of children with chronic defecation disorderswhen maximal medical therapy and conventional treatment have failed.Purpose: To evaluate clinical success, complications, and quality of life of children with chronicdefecation disorders with a MACE stoma.The Malone antegrade continence enema procedure: theE-mail address: firstname.lastname@example.org (L.T. Hoekstra).
022-3468/$ see front matter 2011 Elsevier Inc. All rights reserved.oi:10.1016/j.jpedsurg.2011.04.050www.elsevier.com/locate/jpedsurgtestinal causes (ie, hypothyroidism, celiac disease, cystic
fibrosis), or intestinal nerve and muscle disorders (ie,
and Free University Hospital in Amsterdam, the Netherlands.
created, following the concept that the direction of theppendiceal peristalsis was toward the colon. The Ransleychnique for the VQZ skinplasty was applied in the secondart of the series, trying to prevent stomal stenosis . In casee appendix was unavailable, a neo-appendix was createdsing tubularized colon, or a button in either the ascendingolon or in the sigmoid was used. The Malone stoma wasonducted by laparotomy or, in the second half of the seriesy a laparoscopic approach, depending on the experience ofe surgeon. Surgical complications were classified as major,efined as requiring operative reintervention or minor,efined as not requiring operative reintervention.
reminded by telephone. The questionnaire was developed atour center (see Appendix) and evaluated the indication for
1604 L.T. Hoekstra et al.Of all patients, data regarding demographics, preoperativecomplaints (defecation frequency, fecal incontinence fre-quency, abdominal pain, use of laxatives and enemas, orrectal washouts), indication for MACE, postoperative effecton defecation and continence, surgical technique, complica-tions and revision surgery, and the use of antibiotics wereevaluated using patient files, operative reports, and officenotes. All patients with intractable constipation were treatedwith high dosages of oral laxatives before operation. Almostall patients used enemas preoperatively and/or requiredcolonic washouts. Colonic washouts were performed,usually in the morning with water 20 mL/kg (bodytemperature), according to the recently published Dutchguideline for children with constipation . The volume wasgradually increased until we found the right volume for theindividual child. A nonvalidated questionnaire was used toassess patient satisfaction.
1.1. Surgical technique
In the original technique, Malone used a reversedappendix as a conduit . In the present series, the appendixwas neither reversed nor was a primary antireflux valveHirschsprung's disease, spina bifida, visceral myopathies).Acute simple constipation is traditionally treated with a
high-fiber diet and sufficient fluid intake, and combined withthe request to fill out a bowel diary and toilet training,whereas the treatment of chronic constipation includes 4phases: (1) education, (2) disimpaction, (3) prevention of re-accumulation of feces and 4) follow-up (naspghan guidelines2006). Despite this intensive medical and behavioraltreatment approach, follow-up studies have shown thatafter 5 years, approximately 50% of the children still havecomplaints of constipation [2,3].
The Malone antegrade continence enema (MACE) hasfirst been described in 1990 by Malone et al  for childrenwith chronic, severe defecation disorders. This surgicalintervention has been suggested as a safe and satisfactorytreatment option when long-lasting oral and rectal treatmentin combination with conservative measures has failed. Todate, insufficient knowledge exists on the quality of life andcomplications in children with a MACE stoma or colonicbutton. Therefore, the aim of this study was to evaluateclinical success, complications, and quality of life of childrenwith chronic defecation disorders treated with a MACEstoma or button.
1. Patients and methods
A retrospective analysis was performed in 23 patients (14boys and 9 girls) with intractable constipation and/or fecalincontinence who received a MACE stoma between July2002 and May 2008 in the Emma Children's Hospital/AMCthe MACE procedure, complications, reoperation(s), use ofMACE, social functioning, patient satisfaction, choice forsurgery in retrospect, and the patients' recommendation toother patients. The patients' satisfaction was evaluated on ascale from 1 to 10. In this scale, a higher score represents ahigher level of satisfaction. We reported a number of 6 orhigher as satisfactory.
1.3. Statistical analysis
Statistical analysis was performed using SPSS software(Statistical Package of the Social Sciences 14.0 forWindows; SPSS, Chicago, Ill). Demographic characteristicswere compared using the paired Student's t test forcontinuous outcomes. A P value of less than .05 wasconsidered statistically significant.
A MACE procedure was performed in 23 children withsevere defecation disorders, not manageable with conserva-
Table 1 Baseline characteristics
Sex (n)Male 14Female 9Median age at surgery (y) 7.3 (2-17)Symptom duration (y) 4.7 (1-11)Defecation frequency/wk 1.0 (0-4)Fecal incontinence/wk 10 (0-140)Abdominal pain (%) 57
Results are median (range).Participating patients received a nonvalidated question-naire by mail to determine the effect of the surgicalprocedure. In case of no response, the patient was kindlyatepthuccbthdd
lasted longer (105 minutes compared to 42-80 minutes). Anincrease in the median operation time was seen hereafter,when the use of the VQZ-plasty was introduced. Between2007 and 2008, the operations lasted an average of 130minutes (SD, 19.87 minutes). Most of them were performedusing the laparoscope.
After surgical intervention, the (median) defecationfrequency for all children with constipation increased from1.0 (range, 0-4) to 5.5 (range, 0-28) per week (P b .006).A significant decrease in the (median) fecal incontinencefrequency from 10 (range, 0-140) to 0 (range, 0-14) perweek was found (P b .034). In the group of children withconstipation without fecal incontinence (n = 4), themedian defecation frequency increased from 1.0 (range,0-3) to 3.0 (range, 0-5) per week (P b 1.00) after surgery.Children initially presenting complaints of constipation
Table 2 Complications
Minor complications n Major complications n
Stomal stenosis 6 Stomal stenosis,persistent complaints
Prolaps 5 Stenosis, anastomticleakage, abcesses
Fecal leakage 10 Stenosis, wound infection 1Pain 7 Fistula 1Wound infection 11Catheter problems 7Persistent constipation 3Stomal bleeding 2
Surgical complications were classified as major, defined as requiringoperative reintervention, or minor, defined as not requiring operativereintervention.
1605MACE proceduretive means. The demographics and complaints are shown inTable 1. The underlying diagnosis was idiopathic constipa-tion with and without fecal incontinence in 15 children, ofwhich 11 constipated patients were fecally incontinent and 4constipated patients without fecal incontinence. Otherunderlying diseases were spina bifida in 5 children andHirschsprung's disease in 3 children. In the children withspina bifida, 40% presented with constipation and fecalincontinence, whereas the remaining patients had constipa-tion as the only symptom. One of the children withHirschsprung's disease had complaints of constipation andfecal incontinence; the other 2 presented with severecons