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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.

To evaluate whether providing a constipation action plan (CAP) to families of children with constipation at outpatient gastroenterology (GI) visits affects health care utilization of the GI department.We created a CAP to be included in the after visit summary of children seen in our GI Clinic. We compared the number of patient telephone calls, electronic messages, and urgent care (UC) visits, emergency department (ED) visits, and hospitalizations within 3 months after the visit of patients who received the CAP compared to those who did not using inverse probability treatment weighting (IPTW) analysis. For families who received the CAP at a follow-up visit, we compared these variables in the 3 months before and after the CAP was provided using paired t test and McNemar's test as appropriate.We included 336 patients who received the CAP and 2812 who did not. After IPTW adjustment, there were fewer patient telephone calls for patients who received the CAP (P = 0.0006). The difference in patient electronic messages was not statistically significant (P = 0.09). For the 45 patients who received the CAP at a follow-up visit, there were on average 1.8 more patient telephone calls made prior to receiving the CAP than after (95% confidence interval (CI) = 0.8-2.8; P = 0.0007) and 2.3 more patient electronic messages received (95% CI = 0.1-4.5; P = 0.04). There were no differences in UC/emergency department visits or hospitalizations.We found that providing a CAP to families of children with constipation decreases health care utilization. Further studies are needed to determine whether this impacts patient outcomes.

We have had success treating children with severe rumination syndrome using a multidisciplinary intensive outpatient program (IOP) involving multiple treatment sessions daily. During the COVID-19 pandemic, we temporarily transitioned care to telemedicine. The objective of this study is to compare outcomes of patients with rumination syndrome who completed IOP treatment in person versus by telemedicine.We performed a retrospective review of patients diagnosed with rumination syndrome who participated in IOP treatment from 2018-2020. Similar treatment sessions were performed involving medical and behavioral techniques provided by a multidisciplinary team during telemedicine visits. Families/patients were asked to complete a survey outlining their child's current rumination symptom severity and review the IOP.We included 34 patients (79% F, median age 15 years, range 7-19 years) who completed IOP treatment. Twenty-six patients (76%) were treated in person and 8 patients (24%) by telemedicine. For patients treated in person, 76% (19/25) had improvement in symptoms while 16% (4/25) had complete resolution of symptoms. For patients treated by telemedicine, 88% (7/8) had improvement in their symptoms. There were no significant differences between groups in likelihood of improvement. Overall, 78% (18/23) preferred in person therapy while 17% (4/23) did not have a preference. All 18 of the in-person cohort who completed follow-up surveys preferred in-person management.Multidisciplinary intensive outpatient treatment for children and adolescents with severe rumination syndrome is effective. Although telemedicine may be an alternative to in person therapy, majority of families prefer in person visits.

Introduction Anal sphincter botulinum toxin injections (BTIs) are used in the treatment of children with severe defecation disorders, including Hirschsprung disease (HD) and functional constipation (FC). Our objective was to evaluate the outcomes of BTI in these children. Materials and Methods We performed a prospective cohort study of children undergoing BTI from July 2018 to December 2018. We recorded perceived effect of the BTI, including effectiveness ranging from 0 (not at all effective) to 4 (extremely effective). In addition, we recorded symptoms and the Cleveland Clinic Constipation Score (CCCS). Data were collected at baseline and at 2 weeks, 2 months, and 4 months post-injection. Results Forty-two children (HD = 25, FC = 17) were included in the study (median age 4.3 years, IQR 2.4–7.2, 52% male). Twenty-two (88%) children with HD and eight (47%) children with FC had previously undergone a BTI. BTIs were perceived effective in 16 (76%) and 12 (71%) children with HD and eight (47%) and seven (47%) children with FC at 2-week and 2-month follow-up, respectively. Effectiveness was not rated differently between groups except at the 2-month follow-up, when patients with HD rated the BTI more effective compared to those with FC (median 2 [HD] vs. median 1 [FC], p = 0.022). Over the course of the study, 17/39 (44%) children reported self-limiting adverse effects such as fecal incontinence and pain at the injection site. Conclusion Anal sphincter BTIs can be effective in the treatment of constipation in both HD and FC patients.

After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is unfortunately not the case for a significant number of patients who struggle following their pull-through procedure. Many clinicians are puzzled by these outcomes as they can occur in patients who they believe have had a technically satisfactory described operation. This review presents an organized approach to the evaluation and treatment of the post HD pull-through patient who is not doing well. Patients with HD who have problems after their initial operation can have: (1) fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis (a more severe subset of obstructive symptoms). After employing a systematic diagnostic approach, successful treatments can be implemented in almost every case. Patients may need medical management (behavioral interventions, dietary changes, laxatives, or mechanical emptying of the colon), a reoperation when a specific anatomic or pathologic cause is identified, or botulinum toxin when non-relaxing sphincters are the cause of the obstructive symptoms or recurrent enterocolitis.

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.

Neurogastroenterology and motility (NGM) disorders are common in childhood and are often very debilitating. Although pediatric gastroenterology fellows are expected to obtain training in the diagnosis and management of patients with these disorders, there is an ongoing concern for unmet needs and lack of exposure and standardized curriculum. In the context of tailoring training components, outcome and expressed needs of pediatric gastroenterology fellows and programs, members of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and American Neurogastroenterology and Motility Society (ANMS) developed guidelines for NGM training in North America in line with specific expectations and goals of training as delineated through already established entrustable professional activities (EPAs). Members of the joint task force applied their expertise to identify the components of knowledge, skills, and management, which are expected of NGM consultants. The clinical knowledge, skills and management elements of the NGM curriculum are divided into domains based on anatomic regions including esophagus, stomach, small bowel, colon and anorectum. In addition, dedicated sections on pediatric functional gastrointestinal (GI) disorders, research and collaborative approach, role of behavioral health and surgical approaches to NGM disorders and transition from pediatric to adult neurogastroenterology are included in this document. Members of the NASPGHAN-ANMS task force anticipate that this document will serve as a resource to break existing barriers to pursuing a career in NGM and provide a framework towards uniform training expectations at 3 hierarchical tiers corresponding to EPA levels.

Constipation Is a common pediatric symptom that can affect children of all ages. It impacts children both physically and psychosocially while also causing significant emotional and financial stress to their families. Constipation presenting early in life can be secondary to conditions such as anorectal malformations, anal stenosis, Hirschsprung disease, spine abnormalities, cystic fibrosis, or metabolic dysfunction. Yet, the majority of children and adolescents who struggle with constipation do so as a result of a functional disorder. Diagnosis Is primarily based on fulfilling defined clinical criteria. Despite being so prevalent, functional constipation is often misdiagnosed and inappropriately treated. Laxative use Is often avoided due to misconceptions related to their efficacy and safety. This review addresses how to effectively diagnose and treat functional constipation in children of different ages and with variable severity at the time of presentation. Guidelines from several medical societies for the evaluation and treatment of a child presenting with constipation have been published. It has become increasingly clear that early treatment of functional constipation is associated with improved long-term outcomes. The diagnostic role of anorectal and colonic manometry has been clarified and these tests have been standardized in the pediatric population. New medical and surgical treatments have been developed and Are leading to improved outcomes in children who fail conventional medical and behavioral interventions. Childhood constipation Requires a comprehensive therapeutic plan, comprised of education, behavioral intervention, and medication that jointly produce effective defecation. Children with chronic constipation who Are unresponsive to maximal medical treatment benefit from further evaluation with colonic transit studies, anorectal and colonic manometry, imaging studies of the spine, and defecography—especially if more invasive interventions such as anal sphincter botulinum toxin injection, appendicostomy, cecostomy, colonic resection, ileostomy, and sacral nerve stimulation are being considered. These children Are best evaluated in specialized centers that offer a multi-disciplinary approach to both the physical and psychosocial components of chronic constipation treatment.

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.

BACKGROUND Gastric electrical stimulation (GES) is a promising therapy for children with severe nausea and vomiting, but information on long-term outcomes is limited. We sought to evaluate the long-term efficacy and safety of GES and to describe patient benefit and satisfaction. METHODS Using a prospective registry, we identified patients aged

Background Our objective is to describe the prevalence of patients with internal anal sphincter achalasia (IASA) without Hirschsprung disease (HD) among children undergoing anorectal manometry (ARM) and their clinical characteristics. Methods We performed a retrospective review of high‐resolution ARM studies performed at our institution and identified patients with an absent rectoanal inhibitory reflex (RAIR). Clinical presentation, medical history, treatment outcomes, and results of ARM and other diagnostic tests were collected. We compared data between IASA patients, HD patients, and a matched control group of patients with functional constipation (FC). Key results We reviewed 1,072 ARMs and identified 109 patients with an absent RAIR, of whom 28 were diagnosed with IASA. Compared to patients with FC, patients with IASA had an earlier onset of symptoms and were more likely to have abnormal contrast enema studies. Compared to patients with HD, patients with IASA were more likely to have had a normal timing of meconium passage, a later onset of symptoms, and were diagnosed at an older age. At the latest follow‐up, the majority of patients diagnosed with IASA (54%) were only using oral laxatives. Over half of patients with IASA had been treated with anal sphincter botulinum toxin injection, and 55% reported a positive response. Conclusions and Inferences Patients diagnosed with IASA may represent a more severe patient population compared to patients with FC, but have a later onset of symptoms compared to patients with HD. They may require different treatments for their constipation and deserve further study., Internal anal sphincter achalasia (IASA) is a finding in children with intractable constipation that has not been well‐recognized. Children diagnosed with IASA had an earlier onset of symptoms and were more likely to have an abnormal contrast enema compared to children with intractable functional constipation. They may represent a more severe patient population and deserve further study.

OBJECTIVES Motility and functional disorders are common in children and often debilitating, yet these disorders remain challenging to treat effectively. At the 2018 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the Neurogastroenterology and Motility Committee held a full day symposium entitled, 2018 Advances In Motility and In NeuroGastroenterology - AIMING for the future. The symposium aimed to explore clinical paradigms in pediatric gastrointestinal motility disorders and provided a foundation for advancing new scientific and therapeutic research strategies. METHODS The symposium brought together leading experts throughout North America to review the state of the art in the diagnosis and management of motility and functional disorders in children. Presentations were divided into esophageal, antral duodenal, and colorectal modules. Each module included oral presentations by experts in the respective fields, leading to thought-provoking discussions. There were 2 breakout sessions with small group discussions on select topics, focusing on defining scientific insights into the diagnosis and management of pediatric functional gastrointestinal and motility disorders in a systematic, segment-based approach. CONCLUSIONS The field of neurogastroenterology has made remarkable progress in the last decade. The current report summarizes the major learning points from the symposium highlighting the diagnosis and promising therapies on the horizon for pediatric neurogastrointestinal and motility disorders.

Functional gastrointestinal disorders (FGIDs), including functional abdominal pain (FAP), account for a large portion of conditions seen by paediatric gastroenterologists. Despite the commonality of FGIDs, there remains significant stigma around these diagnoses among medical providers, patients and families. This is due to the absence of easily identifiable biological markers in FGIDs and the overlay with psychological and social factors contributing to symptom onset and maintenance. As such, the biopsychosocial model is essential in conceptualising, evaluating and treating FGIDs. The way in which medical providers explain FGIDs and the manner in which they collaborate with other specialists (eg, psychologists, dieticians, physical therapists, school nurses) is paramount to the patient and family acceptance of an FGID diagnosis and the success of subsequent treatment. The following review outlines paediatric FGIDs with a focus on FAP in adolescents, in particular within the context of the biopsychosocial approach to pathophysiology, diagnosis and treatment.

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, Follow‐up data was available for 41 children at 6 months after treatment, 35 children at 12 months, 26 children at 24 months and all 42 children at most recent follow‐up. p‐value refers to difference in improvement from baseline. *p

BackgroundSacral nerve stimulation (SNS) can be beneficial for children with constipation, but no studies have focused on children with constipation severe enough to require antegrade continence enemas (ACEs). Our objective was to evaluate the efficacy of SNS in children with constipation treated with ACE.MethodsUsing a prospective patient registry, we identified patients

Background Due to scarcity of scientific literature on pediatric gastroparesis, there is a need to summarize current evidence and identify areas requiring further research. The aim of this study was to provide an evidence-based review of the available literature on the prevalence, pathogenesis, clinical presentation, diagnosis, treatment, and outcomes of pediatric gastroparesis. Methods A search of the literature was performed using the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines with the following databases: PubMed, EMBASE, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects, Cochrane Central Register of Controlled Trials, and Web of Science. Two independent reviewers screened abstracts for eligibility. Key results Our search yielded 1085 original publications, 135 of which met inclusion criteria. Most articles were of retrospective study design. Only 12 randomized controlled trials were identified, all of which were in infants. The prevalence of pediatric gastroparesis is unknown. Gastroparesis may be suspected based on clinical symptoms although these are often non-specific. The 4-hour nuclear scintigraphy scan remains gold standard for diagnosis despite lack of pediatric normative comparison data. Therapeutic approaches include dietary modifications, prokinetic drugs, and postpyloric enteral tube feeds. For refractory cases, intrapyloric botulinum toxin and surgical interventions such as gastric electrical stimulation may be warranted. Most interventions still lack rigorous supportive data. Conclusions Diagnosis and treatment of pediatric gastroparesis are challenging due to paucity of published evidence. Larger and more rigorous clinical trials are necessary to improve outcomes.

Recent developments in the evaluation and treatment of childhood constipation are likely to influence the way we deal with pediatric defecation disorders in the near future. Innovations in both colonic and anorectal manometry are leading to novel insights into functional defecation disorders in children. Promising results have been achieved with innovative therapies such as electrical stimulation and new drugs with targets that differ from conventional pharmacological treatments. Also, new surgical approaches, guided by manometric findings, have led to improvement in patient outcome. Finally, utilization of non-pharmacological interventions such as fiber and probiotics has been a field of particular interest in recent years. The aim of this article is to provide an update on these and other novel diagnostic and therapeutic tools related to childhood constipation.

Defecatory disorders are relatively common in children and can have a significant detrimental impact on a child’s quality of life. Treatment options for constipation and fecal incontinence refractory to conventional treatment are limited and often invasive. Neuromodulation is a promising treatment modality for this population. Evidence for the use of sacral neuromodulation to treat children with refractory constipation and fecal incontinence has been growing over the past decade, and there are reports of clinical response in children with a variety of underlying disorders, including functional constipation, anorectal malformation, and spinal cord abnormalities. However, sacral neuromodulation does require surgery and can be associated with certain known risks of complications, which may require additional procedures. Less invasive forms of neuromodulation, like abdominal transcutaneous electrical stimulation and posterior tibial nerve stimulation, have been used for children with defecatory disorders to a limited extent and with mixed results. In this chapter, we review current applications of neuromodulation for treatment of pediatric defecatory disorders and discuss potential future applications.

Background Contrast enemas with barium or water-soluble contrast agents are sometimes performed in children with severe intractable constipation to identify anatomical abnormalities. However there are no clear definitions for normal colonic size or abnormalities such as colonic dilation or sigmoid redundancy in children. Objective To describe characteristics of colonic anatomy on air contrast enemas in children without constipation to provide normal values for colonic size ratios in children. Materials and methods We performed a retrospective chart review of children aged 0–5 years who had undergone air contrast enemas for intussusception. The primary outcome measures were the ratios of the diameters and lengths of predetermined colonic segments (lengths of rectosigmoid and descending colon; diameters of rectum, sigmoid, descending colon, transverse colon and ascending colon) in relation to the L2 vertebral body width. Results We included 119 children (median age 2.0 years, range 0–5 years, 68% boys). Colonic segment length ratios did not change significantly with age, although the differences for the rectosigmoid/L2 ratio were borderline significant (P = 0.05). The ratios that involved the rectal and ascending colon diameters increased significantly with age, while diameter ratios involving the other colonic segments did not. Differences by gender and race were not significant. Conclusion These data can be used for reference purposes in young children undergoing contrast studies of the colon.