Journal of Pediatric Surgery (2013) 48, 17331737Quality of life in pediatric patients with unremittingconstipation pre and post Malone Antegrade ContinenceEnema (MACE) procedureAileen F. Har, Frederick J. Rescorla, Joseph M. Croffie
Riley Hospital for Children at Indiana University Health, Indianapolis, IN 46202, USA
Received 13 August 2012; revised 22 December 2012; accepted 27 January 20130h
Key words:Functional constipation;Quality of life;Malone antegradecontinence enemaAbstractObjective: The primary aim of this study was to determine if there is a change in the quality of life inpediatric patients with unremitting functional constipation and/or encopresis after undergoing aMACE procedure.Methods: Patients, ages 5 to 18 years with unremitting constipation and a normal evaluation,including anorectal manometry and colonic manometry, who opted to undergo a MACE procedurewere contacted to participate in the study. Patients with congenital anorectal malformations as well asspinal cord disorders were excluded from the study. The patient's parent/guardian completed thePedsQLTM Generic Core Scales QOL survey prior to the operation, 6 months, and 12 months afterthe procedure.Results: A total of 15 consecutive patients meeting protocol criteria were recruited within a period of20 months. Themean age at theMACE procedure was 9.8 years (range 7.011.1). 5 patients were female.The mean QOL score pre-MACE was 64.1. At 6 months post-MACE the mean overall QOL score was90.2, and it was 92.0 at 12 months. All 15 patients at the 6 month follow up had significant improvementin their QOL (p=1.9107) and all subcategories of QOL were significantly improved as well.Conclusions: A MACE procedure is of benefit to otherwise normal pediatric patients who haveunremitting functional constipation with failure of medical treatment. Our patients had a significantimprovement in all QOL categories and overall QOL. 2013 Elsevier Inc. All rights reserved.1. Background
Up to 25% of all patients seen by pediatric gastroenter-ologists are seen for complaints of constipation .Constipation can be functional or due to an organic condition Corresponding author. Tel.: +1 317 944 3774; fax: +1 317 944 8521.E-mail address: firstname.lastname@example.org (J.M. Croffie).
022-3468/$ see front matter 2013 Elsevier Inc. All rights reserved.ttp://dx.doi.org/10.1016/j.jpedsurg.2013.01.045including colonic dysmotility secondary to a multitude ofdisorders. These disorders include cerebral palsy, spina bifidaand congenital anomalies, such as anorectal malformations.Chronic constipation can lead to overflow fecal incontinencewhen there is a fecal impaction in the rectum, and this canhave negative effects on the patient's self-esteem, socialinteractions, and the development of independence.
The management of chronic constipation varies depend-ing on the primary disorder, but may involve the use of toilet
1734 A.F. Har et al.training, bulking agents, laxatives, or biofeedback. For somepatients, medical management fails and surgical options areexplored. The Malone Antegrade Continence Enema(MACE) is a procedure which has been used to treatconstipation or fecal incontinence in both adults and children. The appendicostomy is usually hidden within theumbilicus or placed in the right lower quadrant. If theappendix is absent, a neoappendix can be formed using acecal flap or a tubularized segment of terminal ileum .Antegrade enemas are administered with fecal eliminationthus preventing constipation and fecal incontinence . Todate, there have been no prospective studies of quality oflife (QOL) with the MACE procedure in the pediatricpopulation with functional constipation/encopresis. In oneretrospective study , of the 65 patients who had had theMACE procedure in the preceding 4 years, 89% werehighly satisfied with the outcome and there was improve-ment in social confidence and hygiene complaints. Anotherretrospective study  surveyed pediatric patients under-going a MACE procedure for fecal incontinence post repairof imperforate anus and found that the QOL, as rated by thepatient, parents and teachers, improved significantly. Threeother retrospective studies have looked at complicationsand outcomes post-MACE procedure in pediatric popula-tions , but none focused on change in QOL. Moststudies interviewed patients and families years after theprocedure therefore, the results may have been affected byrecall bias.
The primary aim of this study was to determine if there isa change in the quality of life in pediatric patients withunremitting functional constipation and/or encopresis afterundergoing a MACE procedure.2. Methods
Appropriate institutional review board approval wasobtained prior to initiating the study. Patients, ages 5 to18 years, with unremitting functional constipation and anormal evaluation, including both anorectal manometry andcolonic manometry, who decided to undergo a MACEprocedure were contacted to participate in the study.Consecutive patients were enrolled for 20 months (May2009Jan 2011). Subjects were excluded from the study ifthey were less than 5 years or greater than 18 years of age,had congenital anorectal malformations, or congenital/acquired spinal cord disorders.
Cases were all performed at a single-center; tertiary carereferral hospital. All but 1 patient underwent their MACEprocedure with the same pediatric surgeon. The surgeonsutilized a right lower quadrant incision and created a non-refluxing appendicostomy in a standard fashion with thestoma in the base of the umbilicus. A superiorly based skinflap was utilized to prevent a cicatrix. Flushes were advancedpostoperatively until an adequate volume to lead to completeevacuation was determined. The patient was then released onpost-operative day 25.
A QOL survey was completed by a parent prior to theoperation, at 6 months, and at 12 months postoperatively.The PedsQLTM Generic Core Scales were utilized todetermine the patient's QOL . The survey is comprisedof 23 questions encompassing physical, emotional, socialand school functioning and the questions are scored using a 5point Likert-scale (0=never a problem, 4=almost always aproblem). The questions were then reverse scored andlinearly transformed as per questionnaire protocol (0=100,1=75, 2=50, 3=25, 4=0) with the highest possible scorebeing 100, indicating perfect quality of life, and the lowestscore being 0 indicating severe QOL dysfunction.
A two-tailed Student's paired t-test was used to comparethe QOL scores obtained at the different time points. A pvalue of0.05 was considered to be statistically significant.We also recorded patient demographics and any complica-tions related to the MACE.3. Results
Over the course of 20 months, 15 consecutive patients wereenrolled and their QOLwas followed for 12 months. Themeanage of the patients at the time of surgical creation of theappendicostomy was 9.8 years (range 7.011.1). Five of thepatients were female. The most common laxative used prior tosurgical intervention was polyethylene glycol without electro-lytes (PEG 3350) (15 patients, 100%), followed by Senna andmineral oil (13 patients), and magnesium salts (7 patients). Anumber of patients also used bisacodyl, lactulose, docusate,and sorbitol. Themean number of different laxatives trialed perpatient was 4 (range 28). 13 patients (87%) required someform of disimpaction involving phosphate enemas (67%), highvolume nasogastric PEG 3350 solution with electrolytes(20%), or manual disimpaction (60%). Eight patients (53%)had treatment by a psychologist and 8 (53%) underwentbiofeedback prior to their MACE procedure.
All patients in our study had their native appendix utilizedfor the appendicostomy. Following surgery, a MicKey typeballoon tip catheter was initially left for 34 weeks in orderto allow the anastomosis to heal without the trauma of havinga catheter introduced every day. At the 34 week clinic visitthe initial catheter was removed and a button type catheterwas placed. This was placed as usually the child did not wantthe stoma intubated daily. Families were offered the optionof daily intubation at the time of flush without placement of abutton. We did not identify factors to predict stomal stenosis,other than failure to intubate the stoma daily. One patientwho did not have a button placed had stomal stenosis11 months after his MACE procedure requiring revision.One patient required a Chait Tube placement by interven-tional radiology after the catheter had been out for severaldays and the stoma had closed.
Table 1 Mean QOL scores.
Time Physical Psychosocial Total
Pre-MACE (range) 69.4 (25100) 61.3 (5081.7) 64.1 (41.372.8)6 months (range) 94.2 (87.5100) 87.0 (76.798.3) 90.2 (7598.9)12 months (range) 94.7 (83.4100) 90.2 (60100) 92.0 (72.8100)
1735Quality of life pre and post MACE procedureThemost commonMACE complications encounteredwereoccasional soiling (5 patients, 33%), leakage from the ostomy(5 patients, 33%), and granulation tissue (5 patients, 33%).These complications were minor and did not interfere with thepatient's functioning, in fact while there were 5 children whocontinued to have intermittent soiling, a comparison of theirmean total QOL scores pre and 12 months post MACErevealed means of 67.4 (range 62.071.7) and 88.9 (range72.8100.0) respectively (p=0.03). For the 5 patients who hadissues with stoma leakage their pre and 12 month postMACE,total QOL scores were 63.0 (range 47.872.8) and 86.1 (range72.896.7) respectively (p=0.05). Therefore, despite inter-mittent soiling or stoma leakage there was a significantincrease in these patients QOL compared to baseline. Otherreported complications were pain at the catheter site,occasional constipation, post-operative cellulitis, catheterdislo