Quality of life in pediatric patients with unremitting constipation pre and post Malone Antegrade Continence Enema (MACE) procedure

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www.elsevier.com/locate/jpedsurgJournal 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. CroffieRiley Hospital for Children at Indiana University Health, Indianapolis, IN 46202, USAReceived 13 August 2012; revised 22 December 2012; accepted 27 January 20130hKey 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. BackgroundUp to 25% of all patients seen by pediatric gastroenter-ologists are seen for complaints of constipation [1].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: jcroffie@iu.edu (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 toiletmailto:jcroffie@iu.eduhttp://dx.doi.org/10.1016/j.jpedsurg.2013.01.0451734 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[2]. 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 [2].Antegrade enemas are administered with fecal eliminationthus preventing constipation and fecal incontinence [4]. 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 [5], 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 [6] 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 [35], 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. MethodsAppropriate 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 [68]. 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. ResultsOver 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 TotalPre-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, catheterdislodgement, difficulty flushing, bleeding, and skin irritation.No major adverse events were reported.Only 1 patient required oral medication for constipationpost-operatively. Eight patients required tap water only forflushing the MACE, 4 used PEG 3350 mixed with tap water,and 3 required mineral oil mixed with the tap water. Of thosewho used tap water only, the mean volume was 20.4 mL/kg/flush (range 12.734.7). All patients passed stool at leastonce following the antegrade enema. Serum electrolyteswere not measured; however, no patient demonstrated anysymptoms suggestive of electrolyte abnormality.The mean overall pre-MACE QOL score was 64.1 (range41.372.8). At 6 months post-MACE the mean overall QOLscore was 90.2 (range 75.098.9), and it was 92.0 (range 72.8100.0) at 12 months. Mean overall QOL scores and sub-categories are listed in Table 1. Baseline QOL scores werecompared to the 6 month follow-up for all 15 patients(Table 2). The changes in total QOL scores and all subdivisionsof QOLwere statistically significant. Comparison of the overallQOL scores from the 6 month to 12 month post-operative timedid not reach statistical significance (p=0.42), suggesting thatimprovement in the quality of life in these patients maximizesduring the first 6 months. None of the subcategories had asignificant change in the score from 6 months to 12 months.All families stated that if they were to make the decision overagain they would proceed with the MACE.Table 2 QOL p-values.Comparison(n=15)Physicalp valuePsychosocialp valueTotalp valuePre-mace to 6 months 4.4104 1.1107 1.91076 months to 12 months 0.75 0.14 0.424. DiscussionThe MACE procedure has been used in both adult andpediatric patients for treatment of constipation and fecalincontinence. To our knowledge this is the first studyspecifically addressing QOL after a MACE in patients withfunctional constipation/encopresis and no underlying organ-ic or anatomic disorder prospectively. The pre-operativemean QOL score was 64.0 in the current study compared to ascore of 70.0 found by Youssef et al. who previously studiedchildren with constipation using the same QOL scale [9]. Intheir study, children with inflammatory bowel disease had anoverall QOL score of 84, children with gastroesophagealreflux disease scored 80, and normal healthy controls scored88 [9]. At 6 months post-MACE, our patients showed astatistically significant improvement in overall QOL with amean of 90.2 (p=1.9107), which is comparable to ahealthy control score of 88 as mentioned above. Improve-ment was noted in the physical and psychosocial categoriesand we did not see any subsequent change in QOL from6 months to 1 year post-procedure. Parents of all thechildren involved in the study stated that they would gothrough the operation again if given the choice.The patients enrolled in this study had symptoms ofconstipation and encopresis for more than 5 years. All wereexperiencing soiling several times a day and many werewearing pull-up diapers. The patients and their families wereexperiencing a significant amount of social and emotionaldisruption and expressed frustration at the lack of progress.All but two patients had previously required fecal disimpac-tion. The patients, who had been treated with fiber andlaxatives by their physicians prior to referral, were treated byus with an appropriate dose of an osmotic or lubricantlaxative plus intense behavioral modification with mainte-nance of star charts and use of incentives. Patients whopresented with recurrent impactions were given additionalstimulant laxatives in an attempt to improve rectal tone.Those with significant psychiatric problems who wererefusing to even sit on the toilet were referred to a childpsychiatrist for additional treatment as needed. Patientsfailing initial treatment underwent anorectal manometry torule out short segment Hirschsprung's disease or analachalasia. Those with dyssynergic defecation were referredfor biofeedback therapy and completed at least 3 sessions at1736 A.F. Har et al.2 week intervals. Ongoing constipation or encopresis led tocolonic manometry studies to determine need for segmentalor subtotal colonic resection for focal or diffuse myopathy orneuropathy. Finally, patients who had normal colonicmotility who had very few, if any, options left for treatmentwere then given the option of continuing laxative andbehavioral treatment or the MACE procedure as a temporarytreatment option to provide aggressive antegrade enematherapy. The indication for the MACE procedure, therefore,was chronicity of functional constipation and unrelentingsymptoms and failure of conventional treatment withsignificant psychosocial disruption.All previous studies on QOL post-MACE have been onmixed populations, and predominantly patients with spinalcord or congenital anatomic disorders. Two studies retro-spectively evaluated QOL in adults and children withconstipation/encopresis secondary to organic disorders[10,11] observing that in 89%100% QOL improved. Twopediatric studies with organic disorders reported a 97%100% improvement in QOL [12,13]. In 29 pediatric patientswith documented slow transit constipation who underwent aMACE procedure, 93% reported improved QOL, 7% nochange, and 1%worsened [14]. A single prospective study of23 children with spina bifida utilizing a QOL survey beforeand after MACE noted significant improvement in QOL inboth the patients and their parents [15].The most commonly encountered complications in ourpatients were occasional soiling (33%), although significant-ly less often and of significantly less volume than prior to theMACE, leakage from the MACE (33%), and granulationtissue (33%). Others observed continued soiling in 6.4%77% [10,13,14,1619] and leakage from the MACE site in10%35% [13,14,20]. No studies have specifically com-mented on granulation tissue rates, a fairly commonlyobserved finding at stoma sites. For many patients withinitial soiling increasing the volume of flush resolved theproblem. Rates of appendicostomy stenosis have rangedfrom 14% to 55% [1114,16,1822]. While leaving in agastrostomy-like button may potentially increase leakagefrom the MACE, this is outweighed by the benefit ofavoiding revisional surgery or daily dilations of the stoma.Use of a MIC-KEY button in cases of stomal stenosis hasbeen previously reported [23] in a retrospective cohort studyof 56 consecutive patients post-MACE procedure.A limitation of this study is obviously the small number ofpatients. Most children with functional constipation andencopresis can be managed with laxatives and behaviormodification if aggressive therapy is started early. This studywas aimed at the small number of patients who have chronicrelentless symptoms that disrupt their psychosocial func-tioning and quality of life and for whom options for treatmentare limited. Future multi center studies with larger number ofpatients may help determine if the conclusions reached herestill hold. Another limitation is that we conducted oursurveys with the parents only and not with the children. Thiswas due to the majority of the surveys being conducted overthe phone during school hours and the patients not beingreached for comment. Future studies should consider boththe parent and patient assessment of QOL to determine howstrongly these correlate.5. ConclusionsA MACE procedure is of benefit to otherwise normalpediatric patients who have unremitting functional cons-tipation with failure of medical treatment. Our patients hada significant improvement in all QOL categories andoverall QOL.References[1] Loening-Baucke V. Chronic constipation in children. Gastroenterol-ogy 1993;105(5):1557-64.[2] Kiely EM, Ade-Ajayi N, Wheeler RA. Caecal flap conduit forantegrade continence enemas. Br J Surg 1994;81(8):1215.[3] Driver CP, Barrow C, Fishwick J, et al. The Malone antegrade colonicenema procedure: outcome and lessons of 6 years' experience. PediatrSurg Int 1998;13(5-6):370-2.[4] Kim J, Beasley SW,Maoate K. Appendicostomy stomas and antegradecolonic irrigation after laparoscopic antegrade continence enema.J Laparoendosc Adv Surg Tech A 2006;16(4):400-3.[5] Krogsgaard SM, Milling MD, Qvist N. Appendicostomy in thetreatment of severe defecation disorders in children. Ugeskr Laeger2006;168(7):692-4.[6] Varni JW, Seid M, Rode CA. The PedsQL: measurement model for thepediatric quality of life inventory. Med Care 1999;37(2):126-39.[7] Varni JW, Seid M, Knight TS, et al. The PedsQL 4.0 Generic CoreScales: sensitivity, responsiveness, and impact on clinical decision-making. J Behav Med 2002;25(2):175-93.[8] Varni JW, Burwinkle TM, Seid M, et al. The PedsQL 4.0 as a pediatricpopulation health measure: feasibility, reliability, and validity. AmbulPediatr 2003;3(6):329-41.[9] Youssef NN, Langseder AL, Verga BJ, et al. Chronic childhoodconstipation is associated with impaired quality of life: a case-controlled study. J Pediatr Gastroenterol Nutr 2005;41(1):56-60.[10] Yerkes EB, Cain MP, King S, et al. The Malone antegrade continenceenema procedure: quality of life and family perspective. J Urol 2003;169(1):320-3.[11] Ellsworth PI, Webb HW, Crump JM, et al. The Malone antegradecolonic enema enhances the quality of life in children undergoingurological incontinence procedures. J Urol 1996;155(4):1416-8.[12] Mattix KD, Novotny NM, Shelley AA, et al. Malone antegradecontinence enema (MACE) for fecal incontinence in imperforate anusimproves quality of life. Pediatr Surg Int 2007;23(12):1175-7.[13] Tiryaki S, Ergun O, Celik A, et al. Success of Malone's antegradecontinence enema (MACE) from the patients' perspective. Eur JPediatr Surg 2010;20(6):405-7.[14] Marshall J, Hutson JM, Anticich N, et al. Antegrade continenceenemas in the treatment of slow-transit constipation. J Pediatr Surg2001;36(8):1227-30.[15] Ok JH, Kurzrock EA. Objective measurement of quality of lifechanges after ACE Malone using the FICQOL survey. J Pediatr Urol2011;7(3):389-93.[16] Lawal TA, Rangel SJ, Bischoff A, et al. Laparoscopic-assisted maloneappendicostomy in the management of fecal incontinence in children. JLaparoendosc Adv Surg Tech A 2011;21(5):455-9.1737Quality of life pre and post MACE procedure[17] Bani-Hani AH, Cain MP, Kaefer M, et al. The Malone antegradecontinence enema: single institutional review. J Urol 2008;180(3):1106-10.[18] Zerhau P, Husar M, Tuma J. Malone antegrade continence enemastoma in children with dysfunctions of pelvic organs. Rozhl Chir 2008;87(11):593-5.[19] Soylet Y, Yesildag E, Besik C, et al. Antegrade continence enemaananalysis of 20 children with faecal incontinence. Eur J Pediatr Surg2006;16(4):251-4.[20] Siddiqui AA, Fishman SJ, Bauer SB, et al. Long-term follow-upof patients after antegrade continence enema procedure. J PediatrGastroenterol Nutr 2011;52(5):574-80.[21] Heshmat S, DeFoor W, Minevich E, et al. Use of customized MIC-KEY gastrostomy button for management of MACE stomalcomplications. Urology 2008;72(5):1026-9.[22] Yardley IE, Pauniaho SL, Baillie CT, et al. After the honeymooncomes divorce: long-term use of the antegrade continence enemaprocedure. J Pediatr Surg 2009;44(6):1274-6 [discussion 1276-7].Quality of life in pediatric patients with unremitting constipation pre and post Malone Antegrade Continence Enema (MACE) p.....1. Background2. Methods3. Results4. Discussion5. ConclusionsReferences

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