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Refractory functional constipation: clinical management or appendicostomy? Please cite this article as: Arruda VP, Bellomo-Brandão MA, Bustorff-Silva JM, Lomazi EA. Refractory functional constipation: clinical management or appendicostomy? J Pediatr (Rio J). 2020;96:210-6. , ☆☆ ☆☆ Study carried out at Faculdade de Ciências Médicas, Universidade Estadual de Campinas (Unicamp), Campinas, SP, Brazil.

Abstract

Objective:

To compare the clinical evolution in patients with refractory functional constipation undergoing different therapeutic regimens: oral laxatives and antegrade enemas via appendicostomy or clinical treatment with oral laxatives and rectal enemas.

Methods:

Analysis of a series of 28 patients with a mean age of 7.9 years (2.4-11), followed-up in a tertiary outpatient clinic. Refractory functional constipation was defined as continuous retentive fecal incontinence after at least a 12-month period of consensus therapy. After the diagnosis of refractory condition, appendicostomy was proposed and performed in 17 patients. Outcomes: (1) persistence of retentive fecal incontinence despite the use of enemas, (2) control of retentive fecal incontinence with enemas, and (3) control of retentive fecal incontinence, spontaneous evacuations, with no need for enemas.

Results:

Six and 12 months after the therapeutic option, control of retentive fecal incontinence was observed only in patients who underwent surgery, 11/17 and 14/17, p = 0.001 and p = 0.001, respectively. At 24 months, control of retentive fecal incontinence was also more frequent in operated patients: 13/17 versus 3/11 with clinical treatment, p = 0.005. In the final evaluation, the median follow-up times were 2.6 and 3 years (operated vs. clinical treatment, p = 0.40); one patient in each group was lost to follow-up and 9/16 operated patients had spontaneous bowel movements vs. 3/10 in the clinical treatment group, p = 0.043. Surgical complications, totaling 42 episodes, were observed 14/17 patients.

Conclusion:

Appendicostomy, although associated with a high frequency of complications, controlled retentive fecal incontinence earlier and more frequently than clinical treatment. The choice of one of the methods should be made by the family, after adequate information about the risks and benefits of each alternative.

KEYWORDS
Functional constipation; Enema; Child; Adolescent; Fecal incontinence

Resumo

Objetivo

Comparar a evolução clínica em crianças com constipação intestinal funcional refratária sob diferentes regimes terapêuticos: laxativos orais e enemas anterógrados via apendicostomia ou tratamento clínico com laxativos orais e enemas via retal.

Métodos

Análise de uma série de 28 pacientes, 7,9 anos (2,4-11), acompanhados em ambulatório terciário. Constipação intestinal funcional refratária foi definida como manutenção da incontinência fecal retentiva, em terapia consensual, por pelo menos 12 meses. Após diagnóstico de refratariedade, era proposta apendicostomia. Dezessete pacientes realizaram o procedimento cirúrgico. Desfechos: 1. Manutenção de incontinência fecal retentiva em uso de enemas; 2. Controle da incontinência fecal retentiva em uso de enemas; e 3. Controle da incontinência fecal retentiva, evacuações espontâneas, sem necessidade de enemas.

Resultados

Seis e 12 meses após opção terapêutica, controle da incontinência fecal retentiva foi observado apenas nos pacientes operados, 11/17 e 14/17, p = 0,001 e p = 0,001. Aos 24 meses, controle da incontinência fecal retentiva também mais frequente nos operados 13/17 versus 3/11 tratamento clínico, p = 0,005. Na avaliação final, medianas de tempo de seguimento: 2,6 e 3 anos (operados versus tratamento clínico, p = 0,40), um paciente em cada grupo abandonou o seguimento e 9/16 operados apresentavam evacuações espontâneas versus 3/10 no tratamento clínico, p = 0,043. Complicações cirúrgicas, 42 episódios, acometeram 14/17 pacientes.

Conclusão

A apendicostomia, embora associada a elevada frequência de complicações, controlou a incontinência fecal retentiva de maneira mais precoce e frequente do que o tratamento clínico. A escolha de um dos métodos deverá caber à família, após adequada informação sobre riscos e benefícios de cada alternativa.

PALAVRAS-CHAVE
Constipação intestinal; Enema; Criança; Adolescente; Incontinência fecal

Introduction

The prevalence of functional constipation (FC) in children is high in Western societies.11 Cook IJ, Talley NJ, Benninga MA, Rao SS, Scott SM. Chronic constipation: overview and challenges. Neurogastroenterol Motil. 2009;21:S1-8. In Brazil, the importance of this condition in the demographic profile of pediatric diseases is demonstrated by its prevalence, between 17% and 36%, observed in schoolchildren and in specialized outpatient clinics, respectively.11 Cook IJ, Talley NJ, Benninga MA, Rao SS, Scott SM. Chronic constipation: overview and challenges. Neurogastroenterol Motil. 2009;21:S1-8.,22 de Morais MB, Maffei HV. Constipação intestinal. J Pediatr (Rio J). 2000;76:147-56.

Half of children followed-up for 6-12 months recover from FC, achieving regular bowel movements without the need for laxatives33 Pijpers M, Bongers M, Benninga M, Berger M. Functional constipation in children: a systematic review on prognosis and predictive factors. J Pediatr Gastroenterol Nutr. 2010;50:256-68.; however, in approximately 25% of patients treated in tertiary outpatient clinics, complaints of infrequent bowel movements and retentive fecal incontinence (RFI) persist for long periods, and even up to adulthood.33 Pijpers M, Bongers M, Benninga M, Berger M. Functional constipation in children: a systematic review on prognosis and predictive factors. J Pediatr Gastroenterol Nutr. 2010;50:256-68.

4 Tabbers MM, DiLorenzo C, Berger MY, Faure C, Langendam MW, Nurko S, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58:258-74.
-55 Bongers MEJ, van Wijk MP, Reitsma JB, Benninga MA. Long-term prognosis for childhood constipation: clinical outcomes in adulthood. Pediatrics. 2010;126:e156. When the therapeutic response is unsatisfactory at maximum doses of laxatives associated with the use of enemas and there are no alarm signals to investigate an organic cause justifying the symptoms, the condition is termed refractory or intractable intestinal constipation.44 Tabbers MM, DiLorenzo C, Berger MY, Faure C, Langendam MW, Nurko S, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58:258-74.

This condition includes a complex and heterogeneous group of patients, in which the symptom-triggering variables are not fully established. RFI negatively interferes in social interaction, and the quality of life of these patients was considered to be lower than that of individuals with inflammatory bowel disease and gastroesophageal reflux disease.66 Youssef NN, Langseder AL, Verga BJ, Mones RL, Rosh JR. Chronic childhood constipation is associated with impaired quality of life: a case-controlled study. J Pediatr Gastroenterol Nutr. 2005;41:56-60. The need to overcome the symptoms culminates, sometimes, in the consideration of a surgical procedure to eliminate RFI and keep the left colon and rectum empty.77 King SK, Sutcliffe JR, Southwell BR, Chait PG, Hutson JM. The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg. 2005;40:1935-40. The use of antegrade enemas was introduced by Malone in 1990 with the aim of emptying the left colon and achieving control of fecal incontinence.88 Malone PS, Ransley PG, Kiely EM. Preliminary report: the antegrade continence enema. Lancet. 1990;336:1217-8. The greatest benefit of this procedure in the long term is the possibility of colonic motility recovery.99 Rodriguez L, Nurko S, Flores A. Factors associated with successful decrease and discontinuation of antegrade continence enemas (ACE) in children with defecation disorders: a study evaluating the effect of ACE on colon motility. Neurogastroenterol Motil. 2013;25:140-81.

The literature regarding the rates of patients with refractory functional constipation (RFC) treated with appendicostomy who became independent of the ostomy is scarce.1010 Siminas S, Losty PD. Current surgical management of pediatric idiopathic constipation: a systematic review of published studies. Ann Surg. 2015;262:925-33. The aim of this study was to compare the long-term clinical evolution of the use of appendicostomy for antegrade enemas associated with laxatives with the clinical treatment, comprising oral laxatives and rectal enemas, in patients with RFC.

Methods

This series of 28 cases included patients with FC followed-up at a tertiary service outpatient pediatric gastroenterology clinic and in whom the symptoms met the diagnostic criteria of Roma III1111 Hyman PE, Milla PJ, Benninga MA, Davidson GP, Fleisher DF, Taminiau J. Childhood functional gastrointestinal disorders: neonate/toddler. Gastroenterology. 2006;130:1519-26.,1212 Rasquin A, Di Lorenzo C, Forbes D, Guiraldes E, Hyams JS, Staiano A, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology. 2006;130:1527-37. and showed no alarm signals justifying the investigation of organic causes. All patients underwent anorectal manometry with recording of the rectal sphincter reflex presence, to ensure the functional integrity of the myenteric plexus.

Patients were included in a study for scintigraphic assessment of colonic transit and continued to be systematically followed-up on the same basis.1313 Carmo RL, Oliveira RP, Ribeiro AE, Lima MC, Amorim BJ, Ribeiro AF, et al. Colonic transit in children and adolescents with chronic constipation. J Pediatr (Rio J). 2015;91:386-9. The evaluation was systematized, the attendance to follow-up consultations was monitored, and families were asked about their access to the medication, either with their own resources or through donations. They were also asked about possible side effects of the medication and advised against modifying medication dose without medical consultation. The presence of fecal impaction was confirmed by the report of retentive fecal incontinence in the anamnesis, presence of fecal mass at the abdominal palpation, and digital rectal examination. If it was not possible to perform a rectal examination, due to the patient's non-consent, a plain abdominal X-ray in the anteroposterior view was requested. Once the fecal impaction was confirmed, the patient was admitted to the university hospital to undergo rectal enemas with glycerin solution at 12%, 20 mL/kg, three times a day for three to five days and, depending on the clinical evaluation of the enema output, a radiological assessment was performed prior to hospital discharge.

The diagnosis of RFC was established when RFI persisted after at least 12 months of follow-up with the medical specialty, during treatment with maximum tolerable doses of osmotic laxatives (lactulose 2 mL/kg/day or polyethylene glycol 3350, 1.5-2 g/kg/day), associated with rectal enemas (12% glycerin solution or saline solution, 20 mL/kg/day, up to 500 mL); the enemas were recommended if spontaneous evacuation did not occur after two days, in accordance with international guidelines.1010 Siminas S, Losty PD. Current surgical management of pediatric idiopathic constipation: a systematic review of published studies. Ann Surg. 2015;262:925-33.

11 Hyman PE, Milla PJ, Benninga MA, Davidson GP, Fleisher DF, Taminiau J. Childhood functional gastrointestinal disorders: neonate/toddler. Gastroenterology. 2006;130:1519-26.
-1212 Rasquin A, Di Lorenzo C, Forbes D, Guiraldes E, Hyams JS, Staiano A, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology. 2006;130:1527-37.

When the diagnosis of RFC was made, a study of the colonic transit time was carried out by scintigraphy, and the appendicostomy procedure was offered to all patients, being accepted by 17 of them. After the colonic transit study, and after the decision for any of the therapeutic measures, the evolution of these patients continued to be systematically recorded in their medical records, on the same basis described above.

To analyze the evolution of intestinal constipation symptoms during follow-up, three outcomes were considered: (1) maintenance of the RFI with enemas, (2) control of the RFI with enemas and (3) control of the RFI and spontaneous evacuations, without enemas.

Colon transit study - the nuclear study of colonic transit was carried out according to the service protocol.1313 Carmo RL, Oliveira RP, Ribeiro AE, Lima MC, Amorim BJ, Ribeiro AF, et al. Colonic transit in children and adolescents with chronic constipation. J Pediatr (Rio J). 2015;91:386-9. Two types of motor patterns were observed: slow colonic transit (SCT), when 48-h images showed a large part of the marker in the proximal and transverse colon, and distal retention (DR), if the radioisotope had passed the transverse colon within 30 h after the test meal but remained retained in the rectosigmoid region for at least 48 h of observation.1313 Carmo RL, Oliveira RP, Ribeiro AE, Lima MC, Amorim BJ, Ribeiro AF, et al. Colonic transit in children and adolescents with chronic constipation. J Pediatr (Rio J). 2015;91:386-9.,1414 Yik YI, Southwell BR, Hutson JM. Targeting the causes of intractable chronic constipation in children: the Nuclear Transit Study (NTS). Neurogastroenterol Motil. 2010;22:71.

Operative technique - the appendicostomy was performed by laparotomy or laparoscopy, and the patient was hospitalized for approximately two days. After two weeks, intermittent catheterization was started.1515 Herndon CDA, Rink RC, Cain MP, Lerner M, Kaefer M, Yerkes E, et al. In situ Malone antegrade continence enema in 127 patients: a 6-year experience. J Urol. 2004;172:1689-91.

Solutions for enemas through appendicostomy/rectal administration - enemas were performed when spontaneous evacuations did not occur after 48 h with 0.9% saline solution or 12% glycerin solution, 10-20 mL/kg. In the operated patients, this solution was infused with the patient sitting in the toilet. When they had spontaneous bowel movements and did not use the stoma, patients were instructed to catheterize the orifice at least once a day, in order to avoid stenosis.

Statistical analysis

The data were analyzed using IBM Statistic SPSS (IBM SPSS Statistics for Windows, version 20.0. NY, EUA). The Mann-Whitney and Fisher exact tests were applied to continuous and categorical variables, respectively. p-values < 0.05 were considered significant.

The study was approved and authorized by the institution's Research Ethics Committee on July 28, 2015, #: 163,392. CAAE 47138715.9.0000.5404.

Results

During a five-year period, 28 patients were diagnosed with RFC. Clinical data regarding duration and treatment of constipation are shown in Table 1. The median age at constipation onset was 1 year, ranging from 3 months to 7 years of age in the group that chose appendicostomy, and 1 year, ranging from 3 months to 3 years of age in the group that chose clinical treatment (p = 0.68, Mann-Whitney's test). The median age of the patients at the beginning of the follow-up in the tertiary service was 8.3 years (2.5-10.8) and 6.7 years (2.4-11), respectively, for the appendicostomy and clinical treatment groups (p = 0.70, Mann-Whitney's test).

Table 1
Duration and type of treatment for constipation in 28 patients with refractory functional constipation, classified according to the type of treatment: appendicostomy or clinical treatment.

The comparative analysis of the outcomes between the appendicostomy and clinical treatment groups is shown in Fig. 1 and Table 2. In the final evaluation, one patient in each group was lost to follow-up. Among the patients who underwent the appendicostomy, two out of 16 discontinued stomal irrigation for stenosis after four months and 2.4 years, respectively, and restarted using rectal enemas, persisting with RFI; for the purpose of outcome analysis, these patients were maintained in the surgical group.

Figure 1
Percentage of patients with retentive fecal incontinence at 6, 12, 24 months and final evaluation after choice of treatment: appendicostomy or clinical treatment.

Table 2
Clinical symptoms assessed at follow-up of 28 patients with refractory functional constipation, distributed according to the type of treatment: appendicostomy or clinical treatment.

A total of 42 complications were observed in 17 patients, with some patients having more than one complication; these complications resulted in the need for 22 surgical reinterventions and stoma use discontinuation by three patients. The use of appendicostomy was discontinued in three situations: one patient with morbid obesity, who had contraindication for surgical reintervention, one who decided against the reintervention after stoma stenosis, and another who abandoned treatment after seven complications. Two patients required reconstruction of the left stoma. In the presence of stoma leakage, an anti-reflux valve was created, and the stoma was re-constructed. Therefore, 14/17 patients (88.2%) maintained adherence to antegrade irrigations even after complications were observed.

The colonic transit patterns and association with the outcome at the end of the study are shown in Table 3.

Table 3
Outcomes according to colonic transit pattern in patients with refractory functional constipation and treated with appendicostomy or clinical treatment.

Discussion

This study assessed RFI control at 6 and 12 months after appendicostomy or clinical treatment; at 24 months, the frequency of RFI control remained higher in those submitted to the operation, however, these patients had many surgical complications.

The conditions associated with the therapeutic refractoriness of FC are not clarified, and it has not been defined whether this condition would result from long-term fecal impaction or if it is a primary alteration in colonic motility.1414 Yik YI, Southwell BR, Hutson JM. Targeting the causes of intractable chronic constipation in children: the Nuclear Transit Study (NTS). Neurogastroenterol Motil. 2010;22:71. Treatment delay, defined as the time between the age of onset of intestinal constipation and admission to a specialized service, is inversely related to the time of recovery of the clinical condition.55 Bongers MEJ, van Wijk MP, Reitsma JB, Benninga MA. Long-term prognosis for childhood constipation: clinical outcomes in adulthood. Pediatrics. 2010;126:e156.

In this study, a minimum period of 12 months with monthly return visits, under protocol therapeutics, systematized anamnesis, and schedule control, was used to demonstrate adherence to therapy, as best as possible, and to define refractoriness. For the definition of therapeutic non-response, studies have considered periods varying from six months to two or three years of clinical follow-up.1616 Hutson JM, Chase JW, Clarke MC, King SK, Sutcliffe J, Gibb S, et al. Slow-transit constipation in children: our experience. Pediatr Surg Int. 2009;25:403-6.,1717 Southwell BR, King SK, Hutson JM. Chronic constipation in children: organic disorders are a major cause. J Paediatr Child Health. 2005;41:1-15. In 2014, the duration of symptoms to define refractoriness was established as a period of at least three months, under appropriate treatment, in a consensus of the European and American societies of Pediatric Gastroenterology.44 Tabbers MM, DiLorenzo C, Berger MY, Faure C, Langendam MW, Nurko S, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58:258-74. In Brazil, nonadherence to therapy is a factor that definitely impairs the evolution of children with functional intestinal constipation.1818 Steiner AS, Torres RF, Penna JF, Gazzinelli FB, Corradi GA, Costa SA, et al. Chronic functional constipation in children: adherence and factors associated with drug treatment. J Pediatr Gastroenterol Nutr. 2014;58:598-602. The systematic outpatient follow-up allowed us to more concretely assess the magnitude of the risks and benefits observed when comparing therapeutic options.

Appendicostomy is an alternative for a minority of patients with functional intestinal constipation, followed-up in tertiary services and who do not respond to consensus therapy. In literature, the procedure success is reported to range between 57% and 100% of the cases, and such rates were obtained based on different outcomes: complete RFI remission, improvement of abdominal pain or quality of life, presence of evacuation after irrigation via appendicostomy, and recovery of regular bowel movements.77 King SK, Sutcliffe JR, Southwell BR, Chait PG, Hutson JM. The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg. 2005;40:1935-40.,1919 Basson S, Zani A, McDowell S, Athanasakos E, Cleeve S, Phelps S, et al. Antegrade continence enema (ACE): predictors of outcome in 111 patients. Pediatr Surg Int. 2014;30:1135-44.,2020 Siddiqui AA, Fishman SJ, Bauer SB, Nurko S. Long-term follow-up of patients after antegrade continence enema procedure. J Pediatr Gastroenterol Nutr. 2011;52:574-80. Dolejs et al.2121 Dolejs SC, Smith JK, Sheplock J, Croffie JM, Rescorla FJ. Contemporary short- and long-term outcomes in patients with unremitting constipation and fecal incontinence treated with an antegrade continence enema. J Pediatr Surg. 2017;52:79-83. observed that, three months after surgery, 89/93 (96%) of the patients had RFI control. In the present series, six months after the appendicostomy, 82% of patients no longer had RFI, and it was also observed that the early occurrence of complications close to the procedure may have contributed to a delay in RFI remission. Dolejs et al.2121 Dolejs SC, Smith JK, Sheplock J, Croffie JM, Rescorla FJ. Contemporary short- and long-term outcomes in patients with unremitting constipation and fecal incontinence treated with an antegrade continence enema. J Pediatr Surg. 2017;52:79-83. also observed that 24% of patients recovered their capacity to evacuate spontaneously and regularly, with appendicostomy reversion after a median time of 40 months (22/93 children). In the group of 25 articles systematically reviewed by Siminas et al.,1010 Siminas S, Losty PD. Current surgical management of pediatric idiopathic constipation: a systematic review of published studies. Ann Surg. 2015;262:925-33. this outcome was verified in 9.5% of the patients. The recovery of spontaneous evacuation movements is an outcome achieved, on average, in less than half of the patients with refractory constipation submitted to appendicostomy, even after long-term follow-up.99 Rodriguez L, Nurko S, Flores A. Factors associated with successful decrease and discontinuation of antegrade continence enemas (ACE) in children with defecation disorders: a study evaluating the effect of ACE on colon motility. Neurogastroenterol Motil. 2013;25:140-81.,2222 Mugie SM, Machado RS, Mousa HM, Punati JB, Hogan M, Benninga MA, et al. Ten-year experience using antegrade enemas in children. J Pediatr. 2012;161:700-4.,2323 van den Berg MM, Hogan M, Caniano DA, Di Lorenzo C, Benninga MA, Mousa HM. Colonic manometry as predictor of cecostomy success in children with defecation disorders. J Pediatr Surg. 2006;41:730-6. Colonic manometry studies have shown that the presence of high-amplitude peristaltic contractions (HAPCs) and peristaltic response to bisacodyl can predict success with the use of antegrade irrigation.2323 van den Berg MM, Hogan M, Caniano DA, Di Lorenzo C, Benninga MA, Mousa HM. Colonic manometry as predictor of cecostomy success in children with defecation disorders. J Pediatr Surg. 2006;41:730-6. Rodriguez et al. observed that the recovery of manometric indicators of colonic peristalsis function is associated with the possibility of discontinuing the use of the appendicostomy, an outcome observed in 11/40 of the studied patients.99 Rodriguez L, Nurko S, Flores A. Factors associated with successful decrease and discontinuation of antegrade continence enemas (ACE) in children with defecation disorders: a study evaluating the effect of ACE on colon motility. Neurogastroenterol Motil. 2013;25:140-81.

Other authors have indicated that those patients who do not reach this response may be those with primary colonic motility disorders.2020 Siddiqui AA, Fishman SJ, Bauer SB, Nurko S. Long-term follow-up of patients after antegrade continence enema procedure. J Pediatr Gastroenterol Nutr. 2011;52:574-80. Appendicostomy evaluation for longer follow-up periods, on average 10 years, showed a loss of efficacy over time, and about 60% of patients maintained the use of antegrade irrigations after this period. These patients, nevertheless, report high rates of satisfaction and several reported that the transition to adult outpatient clinics was associated with a loss of professional support they had as children and abandonment of the technique.2424 Yardley IE, Pauniaho SL, Baillie CT, Turnock RR, Coldicutt P, Lamont GL, et al. After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure. J Pediatr Surg. 2009;44:1274-6.

Unsatisfactory appendicostomy results were associated with three factors: patient's age at the time of the procedure,2525 Curry JI, Osborne A, Malone PSJ. How to achieve a successful Malone antegrade continence enema. J Pediatr Surg. 1998;33:138-41. as children under 6 years of age would have more difficulty accepting the procedure due to the incorrect use of enemas, which appear to be more effective if performed in the postprandial period and as infusions with a duration of 30 and 60 min, and the undesirable effects of irrigation, such as pain, during catheterization or infusion.77 King SK, Sutcliffe JR, Southwell BR, Chait PG, Hutson JM. The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg. 2005;40:1935-40.,2626 Cascio S, Flett ME, De la Hunt M, Barrett AM, Jaffray B. MACE or caecostomy button for idiopathic constipation in children: a comparison of complications and outcomes. Pediatr Surg Int. 2004;20:484-7. There are no controlled studies to evaluate the best irrigation solution, and different services have been using various guidelines regarding composition and volumes of irrigation solutions, as well as the addition or not of osmotically active agents. Siddiqui et al.2020 Siddiqui AA, Fishman SJ, Bauer SB, Nurko S. Long-term follow-up of patients after antegrade continence enema procedure. J Pediatr Gastroenterol Nutr. 2011;52:574-80. found that saline solution has a lower risk of electrolytic imbalance and that the addition of osmotic agents (polyethylene glycol) increases the success rates of irrigation.

High rates of surgical complications have been recorded in all evaluated cases.77 King SK, Sutcliffe JR, Southwell BR, Chait PG, Hutson JM. The antegrade continence enema successfully treats idiopathic slow-transit constipation. J Pediatr Surg. 2005;40:1935-40.,2020 Siddiqui AA, Fishman SJ, Bauer SB, Nurko S. Long-term follow-up of patients after antegrade continence enema procedure. J Pediatr Gastroenterol Nutr. 2011;52:574-80.,2323 van den Berg MM, Hogan M, Caniano DA, Di Lorenzo C, Benninga MA, Mousa HM. Colonic manometry as predictor of cecostomy success in children with defecation disorders. J Pediatr Surg. 2006;41:730-6.,2727 Shaul DB, Harrison EA, Muenchow SK. Avoidance of leakage and strictures when creating an invisible conduit for antegrade colonic enemas. J Pediatr Surg. 2002;37:1768-71. The most prevalent complications, which usually require surgical reinterventions, are stoma stenosis and leakage. In an attempt to minimize these complications, several modifications have been proposed to the initial surgical technique: skin flap creation with other types of skin suture, implantation of devices in cecostomies, button, broviacs, stoppers, and left Malone.2828 Bani-Hani AH, Cain MP, Kaefer M, Meldrum KK, King S, Johnson CS, et al. The Malone antegrade continence enema: single institutional review. J Urol. 2008;180:1106-10. The devices reduce stenoses or leakage, but are associated with other complications such as displacements, infections, and formation of granulation tissue with the need for surgical reintervention in 29% of the cases, besides being visually identifiable.2929 Fonkalsrud EW, Dunn JC, Kawaguchi AI. Simplified technique for antegrade continence enemas for fecal retention and incontinence. J Am Coll Surg. 1998;187:457-60.,3030 Chait PG, Shandling B, Richards HM, Connolly BL. Fecal incontinence in children: treatment with percutaneous cecostomy tube placement - a prospective study. Radiology. 1997;203:621-4. In this service, the autors chose to make an appendicostomy with an appendix in situ and without use of devices, a technique of choice in the literature.1010 Siminas S, Losty PD. Current surgical management of pediatric idiopathic constipation: a systematic review of published studies. Ann Surg. 2015;262:925-33.

In a recently published meta-analysis, Li et al. compared appendicostomy to cecostomy, concluding that the two techniques have similar percentages of incontinence control; the appendicostomy for antegrade irrigation, however, is more often associated with complications requiring surgical reintervention.3131 Li C, Shanahan S, Livingston MH, Walton JM. Malone appendicostomy versus cecostomy tube insertion for children with intractable constipation: a systematic review and meta-analysis. J Pediatr Surg. 2018;53:885-91.

The importance of the colonic transit study in the management and prognosis of refractory functional constipation has not been well defined. Associations of the transit pattern with the type of RFC clinical manifestation have been identified.1313 Carmo RL, Oliveira RP, Ribeiro AE, Lima MC, Amorim BJ, Ribeiro AF, et al. Colonic transit in children and adolescents with chronic constipation. J Pediatr (Rio J). 2015;91:386-9.,1616 Hutson JM, Chase JW, Clarke MC, King SK, Sutcliffe J, Gibb S, et al. Slow-transit constipation in children: our experience. Pediatr Surg Int. 2009;25:403-6. Pioneering researchers using this investigation tool have found that patients with a slow colonic transit pattern do not respond to retrograde enemas and may have neurotransmitter deficiencies.1616 Hutson JM, Chase JW, Clarke MC, King SK, Sutcliffe J, Gibb S, et al. Slow-transit constipation in children: our experience. Pediatr Surg Int. 2009;25:403-6. Data from the pediatric population are not yet sufficient to define surgical indications based on this type of investigation. In the present series, the transit pattern was not a sufficiently adequate tool for patient selection regarding the therapeutic method.

Despite the high frequency of complications and the absence of validated quality of life assessment, the procedure appears to have been accepted by the relatives and patients of the present series, since the majority persisted using the route after the successful management of complications. Benefits of the appendicostomy in the quality of life of children with RFC have been described as significant.3232 Har AF, Rescorla FJ, Croffie JM. Quality of life in pediatric patients with unremitting constipation pre and post Malone Antegrade Continence Enema (MACE) procedure. J Pediatr Surg. 2013;48:1733-7.

Appendicostomy, despite the high number of complications, appears to control the fecal leaking resulting from intestinal constipation more frequently and earlier than isolated clinical management. The recovery of the spontaneous evacuation condition was observed in a larger number of patients when compared with the isolated clinical management; however, the small number of participants and the conclusions based on risk estimation suggest that studies with larger samples and longer follow-up periods should be carried out to allow a more definitive assessment of this outcome. For now, it seems possible to suggest that the information of surgical possibility should be provided to the cases selected by systematic evaluation and that the final decision on the choice of any of the methods should be made by the family, after being appropriately informed about the risks and benefits of each alternative.

  • Please cite this article as: Arruda VP, Bellomo-Brandão MA, Bustorff-Silva JM, Lomazi EA. Refractory functional constipation: clinical management or appendicostomy? J Pediatr (Rio J). 2020;96:210-6.
  • ☆☆
    Study carried out at Faculdade de Ciências Médicas, Universidade Estadual de Campinas (Unicamp), Campinas, SP, Brazil.
  • Funding
    FAEPEX - Fundo de Apoio ao Ensino, à Pesquisa e à Extensão da Unicamp, Project No. 149/09.

References

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Publication Dates

  • Publication in this collection
    11 May 2020
  • Date of issue
    Mar-Apr 2020

History

  • Received
    20 May 2018
  • Accepted
    4 Sept 2018
  • Published
    22 Oct 2018
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