Long-Term Outcomes of Antegrade Continence Enemas to Treat Constipation and Fecal Incontinence in Children.

OBJECTIVES: The objective of this study is to investigate long-term outcomes of antegrade continence enema (ACE) treatment in children with constipation or fecal incontinence. METHODS: Prospective cohort study including pediatric patients with organic or functional defecation disorders who started ACE treatment. Data were collected at baseline and at follow-up (FU) from 6 weeks until 60 months. We assessed parent and patient-reported gastrointestinal health-related quality of life (HRQoL) using the Pediatric Quality of Life Inventory Gastrointestinal Symptoms Module (PedsQL-GI), gastrointestinal symptoms, adverse events, and patient satisfaction. RESULTS: Thirty-eight children were included (61% male, median age 7.7 years, interquartile range 5.5-12.2). Twenty-two children (58%) were diagnosed with functional constipation (FC), 10 (26%) with an anorectal malformation, and 6 (16%) with Hirschsprung disease. FU questionnaires were completed by 22 children (58%) at 6 months, 16 children (42%) at 12 months, 20 children (53%) at 24 months, and 10 children (26%) at 36 months. PedsQL-GI scores improved overall with a significant increase at 12- and 24-month FU for children with FC and a significant increase in parent reported PedsQL-GI score at 36-month FU for children with organic causes. Minor adverse events, such as granulation tissue, were reported in one-third of children, and 10% of children needed a surgical revision of their ACE. The majority of all parents and children reported that they would "probably" or "definitely" choose ACE again. CONCLUSION: ACE treatment is perceived positively by patients and parents and can lead to long-term improvement in gastrointestinal HRQoL in children with organic or functional defecation disorders.

Prevalence of suspected child abuse in children with constipation: a case-control study.

BACKGROUND: A possible association between child abuse and neglect (CAN) and functional constipation (FC) has been described in adults, however, limited data are available in children. Our objective was to determine the prevalence of suspected CAN in children with FC as compared with their healthy peers. METHODS: A case-control study was carried out in children aged 3-10 years. Children with FC were recruited at a tertiary outpatient clinic, and healthy controls were recruited at schools. Parents were asked to fill out questionnaires about the history and behaviour of their child, children were inquired using a semistructured interview about experienced traumatic events and sexual knowledge. The interview was scored by two independent observers. The prevalence of suspected CAN was determined according to the questionnaires and interview. RESULTS: In total, 228 children with FC and 153 healthy controls were included. Both groups were age and gender comparable (50% females, median age 6 years (not significant)). No significant difference in the prevalence of suspected CAN was found between children with FC and healthy controls (23.3% vs 30.1%, 95% CI 0.44 to 1.12, p=0.14), including a suspicion of sexual, emotional and physical abuse. CONCLUSION: Suspected CAN was detected in both children with FC as in healthy controls. The possible association between CAN and FC in children could not be confirmed.

Hypnosis to Reduce Distress in Children Undergoing Anorectal Manometry: A Randomized Controlled Pilot Trial.

Background/Aims: To assess the effectiveness and feasibility of a brief session of hypnosis to reduce distress in children with functional constipation undergoing anorectal manometry (ARM). Methods: A partially-blinded randomized controlled pilot trial was conducted in children 4-18 years old scheduled for ARM. Children were randomized to receive a brief session of hypnosis prior to ARM or standard care. Non-blinded and blinded observers rated the child's level of distress using the Observation Scale of Behavioral Distress and a 4-point-Likert scale, respectively. Differences between groups were analyzed using Fisher's exact test or Mann-Whitney U test as appropriate. Results: Data from 32 children (15 hypnosis and 17 standard care) were analyzed. Prior to insertion of the catheter, the observed mean levels of distress were lower in the hypnosis group according to both the non-blinded observer (median 0.0 [interquartile range {IQR} 0.0-0.3] vs 1.4 [IQR 0.3-2.4]; P = 0.009) and the blinded observer (median 0.0 [IQR 0.0-0.0] vs 0.5 [IQR 0.0-1.0]; P = 0.044). During ARM, observed and reported levels of distress did not differ significantly. In the hypnosis group, 92.9% of parents and children reported that hypnosis helped the child to relax. There were no significant differences in resting pressure, squeeze pressure, or duration of the procedure between both groups. Conclusion: A brief session of hypnosis for children before ARM is an easily incorporable intervention that lowers distress levels prior to the procedure and is positively perceived by children and parents.

Do we need an extra dimension? A pilot study on the use of three-dimensional anorectal manometry in children with functional constipation.

BACKGROUND: Recently, a new high-definition (or three-dimensional "3D") high-resolution anorectal manometry (3D-ARM) catheter has been introduced. This catheter allows for a more detailed visualization of the anal canal. However, its clinical utility and tolerability in children with constipation are unknown. Our primary objective was to evaluate the agreement between findings from solid-state high-resolution anorectal manometry (HR-ARM) and 3D-ARM. Secondary objectives were to investigate if 3D-ARM has additional value over HR-ARM and to evaluate patient and provider experience. METHODS: Prospective pilot study including children (8-18 years of age) with functional constipation scheduled for anorectal manometry. Children underwent HR-ARM and 3D-ARM consecutively. We compared manometry results of both procedures and collected data on patient and provider experience via self-developed questionnaires. KEY RESULTS: Data of ten patients were analyzed (60% female, median age 14.9 years). In the majority of patients, ARMs were performed awake (n = 8, 80%). In two patients, the recto-anal inhibitory reflex (RAIR) was visualized during HR-ARM but not during 3D-ARM. Anal canal resting pressures were significantly higher during 3D-ARM compared to HR-ARM (median 77 mmHg [IQR 59-94] vs. 69 mmHg [IQR 51-91], respectively, p = 0.037). No significant anatomical or muscular abnormalities were visualized during the 3D-ARM. The majority of children identified the 3D-ARM as the more unpleasant (5/7 [71%]) and more painful procedure (6/7 [86%]) and therefore preferred the HR-ARM (4/7 [57%]). CONCLUSIONS & INFERENCES: In our patient sample, 3D-ARM was associated with more discomfort without providing more useful information and even resulted in an inconsistent visualization of the RAIR.

Outcomes after enterostomies in children with and without motility disorders: A description and comparison of postoperative complications.

PURPOSE: To provide an overview of complications after ileostomy or colostomy procedures in children, and to compare outcomes between patients with gastrointestinal motility disorders (i.e. functional constipation, Hirschsprung's disease, pediatric intestinal pseudo-obstruction (PIPO)) and children without motility disorders (including necrotizing enterocolitis, anorectal malformation and inflammatory bowel disease). METHODS: We performed a retrospective study of children who underwent an enterostomy procedure at our institution. The number and type of complications and subsequent reoperations after ostomy formation were determined. Complications were scored using the Clavien-Dindo classification. A complication of ≥ grade III-b was considered a high-grade complication. RESULTS: 129 children with an ileostomy and 61 children with a colostomy were included. Of these, 62 children (32.6%) had motility disorders; functional constipation (n=40), Hirschsprung's disease (n=18) and PIPO (n=4). The total prevalence of complications was 73.2%. Comparing the perioperative data, children with motility disorders significantly more often underwent a laparoscopic procedure (59.7% vs. 10.9%, p=0.000) and had an end stoma-configuration (37.1% vs. 14.1%, p=0.000) as compared to children without motility disorders. Children with motility problems had a higher complication rate (88.7% vs. 65.5%, OR 4.1, 95% CI 1.7-9.8, p=0.001) compared to children without motility problems, and a larger proportion of complications was classified as high-grade complications (61.8% vs. 31.0% p =0.002). CONCLUSION: A high complication rate after enterostomy formation was detected. Children with gastrointestinal motility disorders had more and more severe complications as compared to children without motility disorders. LEVEL OF EVIDENCE: Level III TYPE OF STUDY: Retrospective comparative study.

Characterization of the colonic response to bisacodyl in children with treatment-refractory constipation.

BACKGROUND: Colonic manometry with intraluminal bisacodyl infusion can be used to assess colonic neuromuscular function in children with treatment-refractory constipation. If bisacodyl does not induce high-amplitude propagating contractions (HAPCs), this can be an indication for surgical intervention. A detailed characterization of the colonic response to intraluminal bisacodyl in children with constipation may help to inform clinical interpretation of colonic manometry studies. METHODS: Studies were performed in five pediatric hospitals. Analysis included identification of HAPCs, reporting HAPCs characteristics, and an area under the curve (AUC) analysis. Comparisons were performed between hospitals, catheter type, placement techniques, and site of bisacodyl infusion. RESULTS: One hundred and sixty-five children were included (median age 10, range 1-17 years; n = 96 girls). One thousand eight hundred and ninety-three HAPCs were identified in 154 children (12.3 ± 8.8 HAPCs per child, 0.32 ± 0.21 HAPCs per min; amplitude 113.6 ± 31.5 mm Hg; velocity 8.6 ± 3.8 mm/s, propagation length 368 ± 175 mm). The mean time to first HAPC following bisacodyl was 553 ± 669 s. Prior to the first HAPC, there was no change in AUC when comparing pre- vs post-bisacodyl (Z = -0.53, P = .60). The majority of HAPCs terminated in a synchronous pressurization in the rectosigmoid. Defecation was associated with HAPCs (χ2 (1)=7.04, P < .01). Site of bisacodyl administration, catheter type, and hospital location did not alter the response. CONCLUSIONS AND INFERENCES: Intraluminal bisacodyl induced HAPCs in 93% of children with treatment-refractory constipation. The bisacodyl response is characterized by ≥1 HAPC within 12 minutes of infusion. The majority of HAPCs terminate in a synchronous pressurization in the rectosigmoid. Optimal clinical management based upon colonic manometry findings is yet to be determined.

Comparison of antegrade continence enema treatment and sacral nerve stimulation for children with severe functional constipation and fecal incontinence.

BACKGROUND: To compare antegrade continence enema (ACE) treatment and sacral nerve stimulation (SNS) in children with intractable functional constipation (FC) and fecal incontinence (FI). METHODS: We performed a retrospective review of children 6-18 years old with FC and FI treated with either ACE or SNS at our institution. We recorded symptoms at baseline, 6 months, 12 months, 24 months, and their most recent visit after starting treatment. We compared improvement in FI, bowel movement (BM) frequency, abdominal pain, laxative use, and complications. Patients were contacted to evaluate perceived benefit using the Glasgow Children's Benefit Inventory. KEY RESULTS: We included 23 patients treated with ACE (52% female, median age 10 years) and 19 patients treated with SNS (74% female, median age 10 years). Improvement in FI was greater with SNS than ACE at 12 months (92.9% vs 57.1%, P = .03) and 24 months (100% vs 57.1%, P = .02). Improvement in BM frequency was greater with ACE, and children were more likely to discontinue laxatives at all follow-up time points (all P < .05). Improvement in abdominal pain was greater with ACE at the most recent visit (P < .05). Rate of complications requiring surgery was similar between groups (26.3% vs 21.7%). Benefit was reported in 83.3% and 100% of ACE and SNS groups, respectively (NS). CONCLUSIONS AND INFERENCES: Although both ACE and SNS can lead to durable improvement in children with FC and FI, SNS appears more effective for FI and ACE more effective in improving BM frequency and abdominal pain and in discontinuation of laxatives.

Management of functional constipation in children and adults.

Functional constipation is common in children and adults worldwide. Functional constipation shows similarities in children and adults, but important differences also exist regarding epidemiology, symptomatology, pathophysiology, diagnostic workup and therapeutic management. In children, the approach focuses on the behavioural nature of the disorder and the initial therapeutic steps involve toilet training and laxatives. In adults, management focuses on excluding an underlying cause and differentiating between different subtypes of functional constipation - normal transit, slow transit or an evacuation disorder - which has important therapeutic consequences. Treatment of adult functional constipation involves lifestyle interventions, pelvic floor interventions (in the presence of a rectal evacuation disorder) and pharmacological therapy. When conventional treatments fail, children and adults are considered to have intractable functional constipation, a troublesome and distressing condition. Intractable constipation is managed with a stepwise approach and in rare cases requires surgical interventions such as antegrade continence enemas in children or colectomy procedures for adults. New drugs, including prokinetic and prosecretory agents, and surgical strategies, such as sacral nerve stimulation, have the potential to improve the management of children and adults with intractable functional constipation.

Quality of Life in Children with Functional Constipation: A Systematic Review and Meta-Analysis.

OBJECTIVE: To systematically review the literature on health-related quality of life (HRQoL) in children with functional constipation and to identify disease-related factors associated with HRQoL. STUDY DESIGN: The Pubmed, Embase, and PsycINFO database were searched. Studies were included if they prospectively assessed HRQoL in children with functional constipation according to the Rome criteria. Articles were excluded if patients had organic causes of constipation and if HRQoL was only assessed after successful therapeutic interventions. A meta-analysis was performed calculating sample size-weighted pooled mean and SD of HRQoL scores. The quality of the studies was also assessed. RESULTS: A total of 20 of 2658 studies were included, providing HRQoL data for 2344 children. Quality of evidence was considered to be poor in 9 of the 20 studies (45%); 13 of the 20 studies reported sufficient data to be included in the meta-analysis. Pooled total HRQoL scores of children with functional constipation were found to be lower compared with healthy reference samples (65.6 vs 86.1; P < .01). Similar HRQoL scores were found according to self-report and parent proxy report. Hospital-based studies reported lower HRQoL scores as compared with community-based studies. Two studies reported on HRQoL scores of children with and without fecal incontinence, but no significant difference was found. CONCLUSIONS: HRQoL is compromised in children with functional constipation.

A comparison of Malone appendicostomy and cecostomy for antegrade access as adjuncts to a bowel management program for patients with functional constipation or fecal incontinence.

BACKGROUND: Appendicostomy and cecostomy are two approaches for antegrade enema access for children with severe constipation or fecal incontinence as adjuncts to a mechanical bowel management program. Each technique is associated with a unique set of complications. The purpose of our study was to report the rates of various complications associated with antegrade enema access techniques to help guide which option a clinician offers to their patients. METHODS: We reviewed all patients in our Center who received an appendicostomy or cecostomy from 2014 to 2017 who were participants in our bowel management program. RESULTS: 204 patients underwent an antegrade access procedure (150 appendicostomies and 54 cecostomies). Skin-level leakage (3% vs. 22%) and wound infections (7% vs. 28%) occurred less frequently in patients with appendicostomy compared to cecostomy. Nineteen (13%) appendicostomies required revision for stenosis, 4 (3%) for mucosal prolapse, and 1 (1%) for leakage. The rates of stenosis (33 vs. 12%) and wound infection (13 vs. 6%) were higher in patients who received a neoappendicostomy compared to an in situ appendicostomy. Intervention was needed in 19 (35%) cecostomy patients, 15 (28%) for an inability to flush or a dislodged tube, and 5 for major complications including intraperitoneal spillage in 4 (7%) and 1 (2%) for a tube misplaced in the ileum, all occurring in patients with a percutaneously placed cecostomy. One appendicostomy (1%) patient required laparoscopic revision after the appendicostomy detached from the skin. CONCLUSION: Patients had a lower rate of minor and major complications after appendicostomy compared to cecostomy. The unique complication profile of each technique should be considered for patients needing these procedures as an adjunct to their care for constipation or fecal incontinence. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Prevalence of Functional Defecation Disorders in Children: A Systematic Review and Meta-Analysis.

OBJECTIVE: To systematically review the literature regarding the epidemiology of functional constipation and functional nonretentive fecal incontinence (FNRFI) in children. Secondary objectives were to assess the geographical, age, and sex distribution of functional constipation and FNRFI and to evaluate associated factors. STUDY DESIGN: The Cochrane Library, PubMed, and Embase databases were searched from 2006 until September 2017. The following inclusion criteria were applied: (1) prospective studies of population-based samples; (2) reporting on the prevalence of functional constipation or FNRFI according to the Rome III/IV criteria; (3) in children aged 0-18 years; and (4) published in full manuscript form. A quality assessment of included studies was conducted. Random effect meta-analyses with meta-regression analyses of study characteristics were performed. RESULTS: Thirty-seven studies were included, of which 35 reported on the prevalence of functional constipation and 15 of FNRFI. The reported prevalence of functional constipation ranged from 0.5% to 32.2%, with a pooled prevalence of 9.5% (95% CI 7.5-12.1). The prevalence of FRNFI ranged from 0.0% to 1.8%, with a pooled prevalence of 0.4% (95% CI 0.2-0.7). The prevalence of functional constipation was 8.6% in boys compared with 8.9% in girls (OR 0.99, 95% CI 0.9-1.4). Geographical location, dietary habits, and exposure to stressful life events were reported to be associated with the prevalence of functional constipation. Data on FNRFI were scarce and no associated factors were identified. CONCLUSION: Functional constipation is common in childhood and is associated with geographical location, lifestyle factors, and stressful life events. FNRFI is rare, and no associated factors were identified.

Awareness and Implementation of the 2014 ESPGHAN/NASPGHAN Guideline for Childhood Functional Constipation.

OBJECTIVE: The aim of the study was to assess whether physicians approach children with functional constipation according to the 2014 European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN)/North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guideline. METHODS: We invited pediatricians and pediatric gastroenterologists in the Netherlands and the United States to participate in this anonymous survey using a self-developed questionnaire containing 19 multiple choice questions concerning evaluation and treatment of children with constipation. RESULTS: We included 328 physicians (67% from the United States, 34% from the Netherlands). The majority of US responders (53%) worked in primary care, whereas all Dutch responders worked in a hospital. In total, 31% of responders were not familiar with the guideline (38% US responders vs 16% Dutch responders, P < 0.001). Perianal inspection was frequently ("often" or "always" on a 5-point Likert scale) conducted by 78% of responders. Digital rectal examination was frequently done by 42%. Inquiry about sexual abuse was made by 18%. Commonly reported reasons for omitting these items were perceived patient or parental discomfort. Most frequently implemented initial nonpharmacological interventions included a toilet training program (89%), optimizing fluid and fiber intake (86% and 81%), a defecation diary (62%), and a reward system (60%). Polyethylene glycol was the most prescribed medication for disimpaction (68%) and maintenance treatment (57% for infants, 97% for children ≥1 year). CONCLUSIONS: Many responders were not familiar with the ESPGHAN/NASPGHAN guideline for functional constipation. Nonetheless, therapeutic decisions correlated fairly well with recommendations from the guideline, especially for children 1 year of age or older. Guideline awareness and adherence remain to be improved. Future studies should focus on exploring strategies to improve guideline implementation through the development of digital learning tools.

Attention Deficit Hyperactivity Disorder and Functional Defecation Disorders in Children.

OBJECTIVES: The aim of the study was to assess the prevalence of attention deficit hyperactivity disorder (ADHD) in children presenting with functional defecation disorders (FDDs) and to assess the prevalence of FDDs in children with ADHD. METHODS: A cross-sectional cohort study was carried out between September 2014 and May 2016. Group 1: Parents of children with FDDs according to the Rome III criteria completed the Child Behavior Checklist and the VvGK (Dutch questionnaire based on the American Disruptive Behavior Disorder rating scale). Patients with ADHD subarea scores ≥70 on the Child Behavior Checklist and/or ≥16 on the VvGK were referred for further psychiatric evaluation. Group 2: Parents of children treated for ADHD at a specialized ADHD outpatient clinic completed a standardized questionnaire regarding their child's defecation pattern. RESULTS: In group 1 (282 children with FDDs), 10.3% (7.1%-13.5% bias-corrected and accelerate confidence interval) were diagnosed with ADHD. Group 2 consisted of 198 children with ADHD, 22.7% (17.6-28.8 bias-corrected and accelerate confidence interval) fulfilled the Rome III criteria for an FDD. Children with both an FDD and ADHD reported urinary incontinence significantly more often compared to children with an FDD or ADHD alone: 57.1% in FDD + ADHD versus 22.8% in FDD alone (P < 0.001) and 31.1% in ADHD + FDD versus 7.8% in ADHD alone (P < 0.001). CONCLUSIONS: Approximately 10.3% of children with FDDs had ADHD and 22.7% of children with a known diagnosis of ADHD fulfilled the Rome III criteria for an FDD. This observation suggests that screening for behavioral disorders and FDDs should be incorporated into the diagnostic workup of these groups of children.

Assessing Children's Report of Stool Consistency: Agreement Between the Pediatric Rome III Questionnaire and the Bristol Stool Scale.

OBJECTIVES: To assess the agreement between the Questionnaire on Pediatric Gastrointestinal Symptoms-Rome III (QPGS-RIII) and the Bristol Stool Scale (BSS) in evaluating stool consistency and the diagnosis of functional constipation in children. STUDY DESIGN: Children aged 8-18 years were asked to describe their stool consistency in the previous month according to the QPGS-RIII and the BSS. Stool consistency according to both instruments was categorized into 3 categories: "hard," "normal," and "liquid." The children's reported stool consistency using the QPGS-RIII and the BSS were compared, and the intrarater agreement between the 2 instruments was measured using the Cohen kappa coefficient (κ). The diagnosis of functional constipation was based on the Rome III criteria, incorporating the assessment of stool consistency according to the QPGS-RIII and the BSS. RESULTS: A total of 1835 children were included. Only slight agreement existed between the QPGS-RIII and the BSS for assessing stool consistency (κ = .046; P = .022). Significantly more children reported hard stools on the BSS compared to the QPGS-RIII (18.0% vs 7.1%; P = .000). The prevalence of functional constipation was 8.6% using the QPGS-RIII and 9.3% using the BSS (P = .134). CONCLUSIONS: Only slight agreement exists between the QPGS-RIII and the BSS in the evaluation of stool consistency in children. Better instruments are needed to assess the consistency of stools with a high degree of reliability, both in research and in the clinical setting.

Parental characteristics and functional constipation in children: a cross-sectional cohort study.

OBJECTIVE: To evaluate personality, psychological health, physical health and childrearing practices in mothers and fathers of children with functional constipation (FC) compared with mothers and fathers of healthy controls. DESIGN: Cross-sectional cohort study. SETTING: Outpatient paediatric gastroenterology clinic at a tertiary hospital in the Netherlands. PATIENTS: Parents of children (4-16 years) presenting with FC were included between January 2010 and August 2012. Participating parents were asked to recruit parents of another child of the same age without FC as their own controls. Data of 116 mothers and 115 fathers of 127 children with FC, and 84 mothers and 73 fathers of 91 children without FC were collected. MAIN OUTCOME MEASURES: Parental characteristics were evaluated by using the NEO Five-Factor Inventory to assess personality, the Brief Symptom Inventory and Physical Symptom Checklist to assess psychological and physical health and the Ghent Parental Behavior Scale to assess childrearing practices. RESULTS: Mothers of constipated children had significant higher scores on the neuroticism personality factor and reported higher rates of overall psychological distress and depression. Both mothers and fathers of children with FC reported significant more physical symptoms than parents of children without FC. Mothers of children with FC showed more positive childrearing practices compared with controls. CONCLUSIONS: Personality, psychological and physical health, and childrearing practices differ significantly between parents of children with FC and parents of control subjects. Parental factors should be taken into account when evaluating children with FC.