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Objectives: The understanding of the impact of tethered cord syndrome (TCS) on the physiology of the colorectal area is limited. Our aim was to describe anorectal and colonic motility in children with TCS and compare the findings to those of children with functional constipation (FC)., Methods: We conducted a retrospective review of children with TCS who had an anorectal manometry (ARM) performed at our institution from January 2011 to September 2023. We recorded demographics, medical and surgical history, clinical symptoms, and treatment at time of ARM, ARM findings (resting pressure, push maneuver, rectal sensation, rectoanal inhibitory reflex [RAIR], and RAIR duration), and the final interpretation of colonic manometry (CM) if performed. We identified age and sex-matched control groups of children with FC., Results: We included 24 children with TCS (50% female) who had ARM testing (median age at ARM 6.0 years, interquartile range 4.0-11.8 years). All children had constipation at time of ARM. Nineteen children had detethering surgery before ARM was performed. No significant differences in ARM parameters were found between children who had detethering surgery before ARM and children with FC. Among the 24 children, 14 also had a CM performed (13/14 after detethering surgery). No significant differences in colonic motility were found between children with a history of TCS and children with FC., Conclusions: Anorectal physiology and colonic motility are similar between children with a history of TCS and children with FC, suggesting that the underlying pathophysiology of defecatory disorders in children with and without history of TCS is similar., (© 2024 The Author(s). Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Rumination syndrome (RS) is challenging to diagnose, which can lead to diagnostic delays. Our objective was to evaluate the length of time from RS symptom onset to diagnosis in patients referred to our institution and to examine whether this duration predicts treatment outcomes., Methods: We conducted a review of patients with RS evaluated at our institution. Data were collected from chart review and patient/family reported questionnaires. We evaluated the time from symptom onset to diagnosis over time and whether it was associated with symptom resolution., Results: We included 247 patients with RS (60% female, median age of 14 years, interquartile range [IQR]: 9-16 years). The median age at symptom onset was 11 years (IQR: 5-14 years) and median age at diagnosis was 13 years (IQR: 9-15 years) for a median duration of 1 year (IQR: 0-3 years) between symptom onset and diagnosis. Length of time between symptom onset and diagnosis did not change significantly at our institution from 2016 to 2022. Among the 164 children with outcome data, 47 (29%) met criteria for symptom resolution after treatment. A longer time to diagnosis was associated with a lower likelihood of symptom resolution after treatment (p = 0.01)., Conclusion: In our experience, the time to RS diagnosis after symptom onset is shorter than previously described. A longer delay in diagnosis is associated with lower likelihood of symptom resolution after treatment, emphasizing the importance of a prompt recognition of rumination symptoms and a timely diagnosis., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective:  The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility., Methods:  A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. p -Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States)., Results:  A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males n  = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% ( p  = 0.023) with pelvic dyssynergia underwent subsequent colonic resection., Conclusion:  In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: For children with constipation and fecal incontinence treated with antegrade continence enemas (ACE), a fluoroscopic study with contrast administered via appendicostomy/cecostomy can define the anatomy of the colon and simulate the flush to investigate associated symptoms or inadequate response. These studies can at times show retrograde flow into the small intestine. Our objective was to investigate the significance of this finding., Methods: We reviewed studies at our institution with contrast administered via appendicostomy/cecostomy in children treated with ACE, identifying those demonstrating retrograde flow of contrast. We recorded demographics, medical history, interventions, and outcomes., Results: We identified 162 studies (52% male, median age 10.7 years) with contrast via appendicostomy (76%) or cecostomy (24%). Diagnoses included anorectal malformation (38%), spinal cord anomaly (26%), functional constipation (24%), colonic dysmotility (18%), and Hirschsprung disease (12%). Fifty-nine (36%) studies showed retrograde flow: 28/59 children (48%) were not responding adequately and 21/59 (36%) had symptoms with ACE. Children with retrograde flow were more likely to have symptoms with ACE than those without (36% vs. 15%, p < 0.01). Fourteen children underwent interventions for this finding, including administering flushes more distally (4/8 responded), changing positioning of the child during flush administration, (1/2 responded), and slowing administration (1/1 responded). Retrograde flow was associated with younger age (p < 0.01), not sex or underlying diagnosis., Conclusion: Identifying retrograde flow during studies with contrast administered via appendicostomy/cecostomy can be useful for children with a poor response or symptoms associated with ACE, as adjustments to the mechanics of the flush can alleviate those symptoms., Level of Evidence: Prognostic study, Level III., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Our objective was to evaluate long-term outcomes of sacral nerve stimulation (SNS) for children with functional and organic defecation disorders., Methods: We performed a prospective study of children <21 years of age who started SNS treatment between 2012 and 2018. We recorded demographics, medical history, and diagnostic testing. We obtained measures of symptom severity and quality of life at baseline and follow up at 1, 6, 12, 24, 36, 48, and ≥60 months. Successful response was defined as bowel movements >2 times/week and fecal incontinence (FI) <1 time/week. Families were contacted to administer the Glasgow Children's Benefit Inventory and to evaluate patient satisfaction., Key Results: We included 65 patients (59% female, median age at SNS 14 years, range 9-21) with median follow-up of 32 months. Thirty patients had functional constipation (FC), 15 had non-retentive FI (NRFI), and 16 had an anorectal malformation (ARM). The percentage with FI <1 time/week improved from 30% at baseline to 64% at 1 year (p < 0.001) and 77% at most recent follow-up (p < 0.001). Patients with FC, NRFI, and ARM had sustained improvement in FI (p = 0.02, p < 0.001, p = 0.02). Patients also reported fewer hard stools (p = 0.001). Bowel movement frequency did not improve after SNS. At most recent follow-up, 77% of patients with a functional disorder and 50% with an organic disorder had responded (p = 0.03). Nearly all families reported benefit., Conclusions and Inferences: SNS led to sustained improvement in FI regardless of underlying etiology, but children with functional disorders were more likely to respond than those with organic disorders., (© 2024 John Wiley & Sons Ltd.)

Objectives: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States., Methods: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM., Results: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017)., Conclusions: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: For children with intractable functional constipation (FC), there are no evidence-based guidelines for subsequent evaluation and treatment. Our objective was to assess the practice patterns of a large, international cohort of pediatric gastroenterologists., Methods: We administered a survey to physicians who attended the 2nd World Congress of Pediatric Neurogastroenterology and Motility held in Columbus, Ohio (USA) in September 2023. The survey included 29 questions on diagnostic testing, nonpharmacological and pharmacological treatment, and surgical options for children with intractable FC., Results: Ninety physicians from 18 countries completed the survey. For children with intractable FC, anorectal manometry was the most commonly used diagnostic test. North American responders were more likely than Europeans to use stimulant laxatives (97% vs. 77%, p = 0.032), prosecretory medications (69% vs. 8%, p < 0.001), and antegrade continence enemas (ACE; 83% vs. 46%, p = 0.009) for management. Europeans were more likely than North Americans to require colonic transit testing before surgery (85% vs. 30%, p < 0.001). We found major differences in management practices between Americans and the rest of the world, including use of prosecretory drugs (73% vs. 7%, p < 0.001), anal botulinum toxin injections (81% vs. 58%, p = 0.018), ACE (81% vs. 58% p = 0.018), diverting ileostomies (56% vs. 26%, p = 0.006), and colonic resections (42% vs. 16%, p = 0.012). No differences were found when respondents were compared by years of experience., Conclusions: Practice patterns in the evaluation and treatment of children with intractable FC differ widely among pediatric gastroenterologists from around the world. A clinical guideline regarding diagnostic testing and surgical decision-making is needed., (© 2024 The Author(s). Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Rumination syndrome (RS) beginning in early childhood or infancy is understudied and challenging to treat. Our objective is to compare the characteristics and outcomes of early-onset (EO) and adolescent-onset (AO) patients with RS., Methods: We conducted an ambidirectional cohort study of children diagnosed with RS at our institution. Patients were included in two groups: EO (RS symptom onset ≤5 years and diagnosis ≤12 years) and AO (onset >12 years). Patient characteristics, severity, and outcomes were compared between the groups., Results: We included 49 EO and 52 AO RS patients. The median ages of symptom onset and diagnosis in EO were 3.5 and 6 years, respectively; AO, 14.5 and 15 years. EO RS had a slight male predominance while AO was predominantly female (p = 0.016). EO patients were more likely to have developmental delay (24% vs. 8%, p = 0.029) and less likely to have depression (0% vs. 23%, p < 0.001) or anxiety (14% vs. 40%, p = 0.004). At baseline, EO RS was less severe than AO RS: EO RS had greater regurgitation frequency (p < 0.001) but lower vomiting frequency (p = 0.001), resulting in less meal skipping (p < 0.001), reliance on tube feeding or parenteral nutrition (p < 0.001), and weight loss (p = 0.035). EO RS symptoms improved over time: at follow-up, patients had lower regurgitation (p < 0.001) and vomiting frequency (p < 0.001) compared to baseline., Conclusion: EO RS is clinically distinct from AO RS, with differences in sex distribution, comorbid conditions, and severity of initial presentation. The pathogenesis and natural history of EO RS may be distinct from that of AO RS., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: None declared., (Thieme. All rights reserved.)

Objectives: Our objective was to investigate if there is a difference in the detection of the rectoanal inhibitory reflex (RAIR) when an anorectal manometry (ARM) is performed awake or under general anesthesia., Methods: A retrospective review of ARM studies was performed to identify children who had undergone ARMs both while awake and under general anesthesia. We compared ARM outcomes including the detection of the RAIR and anal canal resting pressure., Results: Thirty-four children had received ARMs both while awake and under general anesthesia (53% female, median age at first ARM 7.5 years [range 3-18 years]). In 9 of 34 (26%) children the RAIR was solely identified during ARM under general anesthesia and not during ARM while awake. In 6 of 9 (66%) this was unrelated to the balloon volumes used during balloon inflations. In 4 of 34 (12%) children, assessment of the RAIR was inconclusive during ARM under general anesthesia due to too low, or loss of anal canal pressure. In 2 of those children, ARMs while awake showed presence of a RAIR. Anal canal resting pressures were higher during ARM while awake versus ARM under general anesthesia (median 70 [interquartile range, IQR 59-85] vs 46 mmHg [IQR 36-65] respectively, P < 0.001)., Conclusions: General anesthesia may affect the detection of a RAIR in 2 ways. On the one hand, it may facilitate better visualization in children in whom a RAIR could not be visualized while awake. On the other hand, it may cause a loss of anal canal pressure resulting in an inconclusive test result., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Baclofen can decrease rumination frequency in adults with rumination syndrome. Outcomes of baclofen treatment in children with rumination syndrome have not been described. The aim of this study was to examine the safety and efficacy of baclofen in children with rumination syndrome in combination with behavioral therapy at a single center., Methods: We performed a retrospective review of children aged 0-18 years with rumination syndrome based on Rome criteria and prescribed baclofen by a pediatric gastroenterologist at the Nationwide Children's Hospital, Columbus, Ohio, USA, between 2012 and 2019. Children without follow-up data or who were prescribed baclofen for other symptoms were excluded., Results: We identified 44 children with rumination syndrome who were prescribed baclofen by a pediatric gastroenterologist. Seventeen either did not have follow-up data or never started the medication. We included 27 patients in the study: 22 (81.5%) female, median age 14.5 years (range 10-18 years) and 100% Caucasian. Twenty patients (74%) received baclofen 5 mg and seven patients (26%) received baclofen 10 mg three times daily. Most patients received behavioral therapy and baclofen simultaneously. Thirteen patients (48%) reported improvement in symptoms, primarily a decrease in rumination frequency, at their first follow-up visit. Regurgitation frequency per week decreased after starting baclofen (p < 0.05). One patient experienced dizziness. No other side effects were reported., Conclusion: Nearly half of our patients with rumination syndrome improved after baclofen. It was well tolerated with minimal side effects. This suggests that baclofen in addition to behavioral therapy can improve symptoms of rumination syndrome. Prospective, controlled studies in a larger cohort of children with rumination syndrome are needed to confirm these findings., (© 2023. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Objectives: Rumination syndrome (RS) can be challenging to treat and data on treatment outcomes in children are limited. The objective of this study was to evaluate outcomes of children with RS treated with tailored outpatient and inpatient strategies., Methods: We performed a retrospective cohort study of children <18 years old with RS evaluated at our institution from 2018 to 2020. At our institution, we use a multidisciplinary, tiered approach to treatment based on presentation severity. Children with RS either undergo outpatient treatment program (OP) or participate in an intensive outpatient program (IOP) or an intensive inpatient program (IP). We reviewed baseline characteristics and assessed severity (including frequency of regurgitation/vomiting, route of nutrition, and weight loss) at baseline, at completion of treatment, and at a follow-up time point., Results: We included 171 children with RS (64% female, median age 13 years, interquartile range (IQR) 10-15), 123 of whom had post-treatment data after completing OP, IOP, or IP. At baseline, 66% of patients were vomiting daily and 40% were losing weight. After treatment, 72% of OP, 95% of IOP, and 96% of IP patients reported that symptoms were better or fully resolved compared to baseline. In all 3 treatment groups, patients were vomiting, losing weight, and skipping meals significantly less after treatment compared to baseline. At follow-up (median 5.3 months), 86% of IOP and 66% of IP patients had symptoms that remained better or resolved., Conclusions: RS can cause severe symptoms, impacting nutritional status and school participation. However, multidisciplinary care in a tiered approach leads to significant symptomatic improvement., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: We have had success treating children with severe rumination syndrome using a multidisciplinary intensive outpatient program (IOP) involving multiple treatment sessions daily. During the coronavirus disease 2019 (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., Methods: We performed a retrospective review of patients diagnosed with rumination syndrome who participated in IOP treatment from 2018 to 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., Results: 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., Conclusions: 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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: 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., Methods: 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., Results: 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., Conclusions: 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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., (© 2022 The Authors. Neurogastroenterology & Motility published by John Wiley & Sons Ltd.)

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.

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., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose of Review: Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD., Recent Findings: Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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 <21 years treated with GES at our institution between 2009 and 2019. Encounters were selected at baseline prior to GES and at follow-up at 1 month, 12 months, and the most recent visit. We compared symptoms, route of nutrition, and medication usage at baseline to follow-up timepoints. Factors associated with improvement were evaluated. We recorded complications and need for battery replacement. We contacted families to administer the Glasgow Children's Benefit Inventory (GCBI) and a parent satisfaction questionnaire., Key Results: Eighty-five patients (68.2% female, median age 15.8 years) completed a trial of temporary GES due to severe nausea and vomiting. Seventy-seven (90.6%) had a positive response and underwent permanent stimulator placement. Use of tube feeding or parenteral nutrition (PN) decreased from 72.7% at baseline to 29.9% at the most recent follow-up (p < 0.001). Higher baseline vomiting severity was associated with the ability to stop supplemental nutrition by 1 month (p < 0.05). Fourteen patients (18.2%) had complications, primarily due to stimulator-associated discomfort, and 29 (37.7%) required battery replacement. Median GCBI was +52.1, indicating health-related benefit., Conclusions and Inferences: Children with severe nausea and vomiting treated with GES experienced significant and durable improvement in symptom severity and their ability to tolerate oral nutrition., (© 2021 John Wiley & Sons Ltd.)

Objectives: To identify predictors of hospitalization in pediatric patients presenting to an emergency department (ED) for a cyclic vomiting syndrome (CVS) attack., Study Design: We retrospectively reviewed patients with CVS seen at our institution between 2015 and 2018 and included those who met the Rome IV criteria for CVS. We identified all CVS-related ED visits and subsequently performed a case-control analysis, utilizing multivariate logistic regression, to identify clinical and demographic factors that may predict hospitalization., Results: In total, 219 patients with CVS (using International Statistical Classification of Diseases and Related Health Problems, 10th Revision) were identified, of which 65% met the inclusion criteria (median age 11 years). We identified 152 CVS-related ED visits, of which 62% resulted in hospitalization. Factors found to predict hospitalization using multivariate analyses included male sex (P = .04), younger age (P = .027), delayed presentation (>24 hours) to the ED (P < .001), and longer wait time prior treatment with antiemetics (P = .029)., Conclusion: One-quarter of all patients with CVS had presented to the ED and nearly two-thirds of these ED visits resulted in hospitalization. A delayed presentation to the ED following the onset of symptoms was the strongest independent predictor of hospital admission, alongside male sex, younger age, and longer ED wait times before treatment with antiemetics. These findings suggest that early intervention may be key to successfully mitigating the risk of hospitalization for a CVS attack., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Linaclotide is a well-tolerated and effective agent for adults with functional constipation (FC) or irritable bowel syndrome with constipation (IBS-C). However, data in children are lacking. The aim of this study is to examine the efficacy and safety of linaclotide in children., Methods: We performed a retrospective review of children < 18 years old who started linaclotide at our institution (Nationwide Children's Hospital, Columbus, Ohio). We excluded children already using linaclotide or whom had an organic cause of constipation or abdominal pain. We recorded information on patient characteristics, medical and surgical history, symptoms, clinical response, course of treatment, and adverse events at baseline, first follow-up, and after 1 year of linaclotide use. A positive clinical response was based on the physician's global assessment of symptoms at the time of the visit as documented., Results: We included 93 children treated with linaclotide for FC (n = 60) or IBS-C (n = 33); 60% were female; median age was 14.7 years (IQR 13.2-16.6). Forty-five percent of patients with FC and 42% with IBS-C had a positive clinical response at first follow-up a median of 2.5 and 2.4 months after starting linaclotide, respectively. Approximately a third of patients experienced adverse events and eventually 27% stopped using linaclotide due to adverse events. The most common adverse events were diarrhea, abdominal pain, nausea, and bloating., Conclusion: Nearly half of children with FC or IBS-C benefited from linaclotide, but adverse events were relatively common. Further prospective, controlled studies are needed to confirm these findings and to identify which patients are most likely to benefit from linaclotide.

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., (© 2020 The Authors. Neurogastroenterology & Motility published by John Wiley & Sons Ltd.)

Abstract: 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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (© 2020 The Authors. Neurogastroenterology & Motility published by John Wiley & Sons Ltd.)

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., (© 2019 John Wiley & Sons Ltd.)

Background and Aim: Botulinum toxin (botox) is a commonly used treatment for functional anorectal and colonic disorders. Although generally regarded as safe, complications associated with botox injection into the anal sphincters in children with severe defecation disorders are not well described. We aimed to review our institutional experience and the existing literature to better understand the safety of this practice., Methods: We performed a retrospective review of pediatric patients undergoing botox administration into the anal sphincter for treatment of a variety of defecation disorders between 2014 and 2018. Additionally, we performed a review of all published literature reporting complications from botox injection in this patient population., Results: 881 patients ranging from 5 weeks to 19.7 years underwent a total of 1332 botox injections including our institution (332 patients/526 injections) and the reviewed series (549 patients/806 injections). Overall, complications were seen after 9 (0.7%) injections and included urinary incontinence (n = 5), pelvic muscle paresis (n = 2), perianal abscess (n = 1), pruritis ani (n = 1), and rectal prolapse (n = 1). Patient age, weight, and diagnosis were not associated with an increased rate of complication in our institutional experience. All complications were self-limited and did not require intervention. There were no episodes of systemic botulinum toxicity., Conclusion: Botox injection into the anal sphincters is accepted practice in children with Hirschsprung disease, severe functional constipation, and internal anal sphincter achalasia and appears to be safe from this review. The precise dosing and age at which complications are more likely to arise could not be ascertained and require further study., Level of Evidence: IV TYPE OF STUDY: Retrospective cohort study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Using water-perfused (WP) high-resolution manometry, we recently demonstrated that children with functional constipation (FC) lacked the postprandial increase in distal colonic cyclic motor patterns that was observed in healthy adults. Our aim was to determine if similar results could be detected using a solid-state (SS) manometry catheter., Methods: We performed a retrospective analysis of 19 children with FC (median age 11.1 years, 58% male) who underwent colonic manometry with a SS catheter (36 sensors, 3 cm apart). Data were compared with previously published data using a WP catheter (36 sensors, 1.5 cm apart) recorded from 18 children with FC (median age 15 years; 28% male)., Key Results: The cyclic motor patterns recorded by the SS catheter did not differ from those previously recorded by the WP catheter. There was no detected increase in this activity in response to the meal in either group. Long-single motor patterns were recorded in most patients (n = 16, 84%) with the SS catheter. The number of these events did not differ from the WP recordings. In the SS data, HAPCs were observed in 4 children prior to the meal, in 5 after the meal. This did not differ significantly from the WP data., Conclusions & Inferences: These data recorded by SS manometry did not differ from WP manometry data. Regardless of the catheter used, both studies revealed an abnormal colonic response to a meal, indicating a pathology which is not related to the catheter used to record these data., (© 2018 The Authors. Neurogastroenterology & Motility Published by John Wiley & Sons, Ltd.)

Introduction: We previously reported our surgical technique for functional constipation for patients who have failed medical management using a novel collaborative approach with gastroenterology input, pre-operative contrast enema, colonic manometry, and laxative protocol combined with a laparoscopic colonic resection with Malone appendicostomy. Now we report our intermediate outcomes., Methods: Patients who failed bowel management program for functional constipation were reviewed from 3/2014-2/2017. Patients with Hirschsprung disease, anorectal malformation, tethered cord, spina bifida, Trisomy 21, cerebral palsy, mitochondrial disease, or prior colon resection were excluded., Results: Of 31 patients (14 females; median age 12years, follow-up 10.3months) with functional constipation and failed medical management, 26 (84%) had preoperative colonic manometry which, in addition to the contrast enema, guided laparoscopic colon resection. Ten patients (32.3%) are clean with no flushes (1 takes no laxatives, 8 are on low dose laxatives only, and 1 patient was clean on laxatives but chose to switch back to flushes). Of the 21 patients that remain on antegrade flushes, 20 (95.2%) are clean, and one patient (4.8%) continues to soil. We define clean as no soiling and no abnormal stool burden on x-ray. Laxative trials are planned for all patients on an antegrade flush regimen., Conclusion: Our intermediate results show that laparoscopic colon resection with Malone appendicostomy allows the majority of patients to be clean on antegrade flushes, and some to be on no or minimal laxatives., Type of Study: Retrospective review., Level of Evidence: 3., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To evaluate the long-term efficacy of sacral nerve stimulation (SNS) in children with constipation and describe patient benefit and parent satisfaction., Methods: Using a prospective patient registry, we identified patients <21 years old with constipation treated with SNS for >2 years. We compared symptoms, medical treatment, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), and Fecal Incontinence Severity Index (FISI) before SNS and at follow-up. We contacted parents to administer the Glasgow Children's Benefit Inventory (GCBI) and a parent satisfaction questionnaire., Key Results: We included 25 children (52% male, median age 10 years): 16 had functional constipation, six anorectal malformation, two tethered spinal cord, and one Hirschsprung's disease. Defecation frequency did not change after SNS but patients reporting fecal incontinence decreased from 72% to 20% (P<.01) and urinary incontinence decreased from 56% to 28% (P=.04). Patients using laxatives decreased from 64% to 44% (ns) and patients using antegrade enemas decreased from 48% to 20% (P=.03). GSS, most FIQL domains, and FISI were improved at follow-up. Six (24%) patients had complications requiring further surgery. Of the 16 parents contacted, 15 (94%) parents indicated positive health-related benefit and all would recommend SNS to other families., Conclusions & Inferences: Sacral nerve stimulation is a promising and durable treatment for children with refractory constipation, and appears particularly effective in decreasing fecal incontinence. Although a quarter of patients experienced complications requiring additional surgery, nearly all parents reported health-related benefit. Future studies to identify predictors of treatment response and complications are needed., (© 2017 John Wiley & Sons Ltd.)

Background: Colonic dilation is common in children with intractable functional constipation (FC). Our aim was to describe the association between segmental colonic dilation and colonic dysmotility in children with FC., Methods: We performed a retrospective study on 30 children with intractable FC (according to the Rome III criteria) who had undergone colonic manometry and contrast enema within a 12-month time period. Colonic diameter was measured at 5 cm intervals from the anal verge up to the splenic flexure. Moreover, the distance between the lateral margins of the pedicles of vertebra L2 was measured to provide a ratio (colonic diameter or length/distance between the lateral margins; "standardized colon size" [SCS]). All manometry recordings were visually inspected for the presence of high-amplitude propagating contractions (HAPCs); a parameter for colonic motility integrity. The intracolonic location of the manometry catheter sensors was assessed using an abdominal X-ray., Key Results: Colonic segments with HAPCs had a significantly smaller median diameter than colonic segments without HAPCs (4.08 cm vs 5.48 cm, P<.001; SCS 1.14 vs 1.66, P=.001). Children with prematurely terminating HAPCs had significantly larger SCS ratios for colonic diameter than children with fully propagating HAPCs (P=.008). SCS ratios for the length of the rectosigmoid and the descending colon and the SCS ratio for sigmoid colon diameter were significantly larger in children with FC compared to a previously described normative population (P<.0001, P<.0001 and P=.0007 respectively)., Conclusions & Inferences: Segmental colonic dilation was associated with prematurely terminating HAPCs and may be a useful indicator of colonic dysmotility., (© 2017 John Wiley & Sons Ltd.)

Objective: To document the use of diagnostic testing in adolescents who ultimately were diagnosed with rumination syndrome, a functional gastrointestinal disorder. We examined the diagnostic yield of each test as well as the associated costs, and we determined if any demographic or illness-related variables impacted the magnitude of the work-up., Study Design: A retrospective chart review was conducted for 68 patients with rumination syndrome admitted to our inpatient treatment program. The cost and findings of patients' diagnostic investigations were gathered, as well as demographic and illness-related variables to determine factors that may be related to evaluation size., Results: The most commonly used tests in the evaluation of rumination syndrome included esophagogastroduodenoscopy, gastric emptying, antroduodenal manometry, upper gastrointestinal series, and abdominal ultrasound scan. Each patient underwent an average of 8.8 tests, with the average cost for each patient's diagnostic work-up being US $19 795. Few tests were found to be beneficial in the diagnosis of rumination syndrome, and few demographic or illness variables were found to be related to the overall extent of the investigation., Conclusions: Extensive testing for rumination syndrome in adolescents is common in clinical practice, and comes at a high financial cost with low yield, likely delaying diagnosis and treatment. Symptom-based criteria should be used to make the diagnosis of rumination syndrome., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Sacral 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., Methods: Using a prospective patient registry, we identified patients <21years old who were receiving ACE prior to SNS placement. We compared ACE/laxative usage, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), Fecal Incontinence Severity Index (FISI), and Vancouver Dysfunctional Elimination Syndrome Score (DES) at baseline and progressive follow-up time intervals., Results: Twenty-two patients (55% male, median 12years) were included. Median ACE frequency decreased from 7 per week at baseline to 1 per week at 12months (p<0.0001). Ten children (45%) had their cecostomy/appendicostomy closed. Laxative use, GSS, FIQL, and DES did not change. FISI improved over the first 12months with statistical significance reached only at 6months (p=0.02). Six (27%) children experienced complications after SNS that required further surgery., Conclusions: In children with severe constipation dependent on ACE, SNS led to a steady decrease in ACE usage with nearly half of patients receiving cecostomy/appendicostomy closure within 2years., Level of Evidence: IV., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.

Background: The cost of hospital care for adults with gastroparesis (GP) is increasing. Our objective was to evaluate the cost of hospital care for children with GP., Methods: Using the Pediatric Health Information System, we selected hospitalizations with a diagnosis of GP (ICD-9 536.3), dyspepsia and other specified disorders of function of stomach (DYS, 536.8) and unspecified functional disorder of stomach (UFD, 536.9) from 2004 to 2013. We recorded dates of hospitalization, demographics, costs, and length of stay (LOS)., Key Results: From 2004 to 2013, 4015 patients were admitted for GP (54.2% female, median age 8 years). Total cost of hospitalization for GP increased 5.8 fold from $6 185 390 to $35 654 075 (p = 0.0001). Cost per hospitalization did not change. Cost of initial hospitalization was highest in patients 0-5 years and lowest in patients 16-21 years (p < 0.0001). Number of hospitalizations each year for GP increased from 252 to 1310 (p < 0.0001) and unique patients hospitalized increased from 174 to 723 (p < 0.0001). Number of hospitalizations and unique patients for DYS/UFD also increased (p < 0.0001). LOS for GP did not change with time. Females and younger GP patients had more repeat hospitalizations (p < 0.0001, p < 0.0001)., Conclusions & Inferences: The financial burden of hospitalization for pediatric GP has increased dramatically from 2004 to 2013, driven by a rise in number of hospitalizations and unique patients hospitalized each year. Cost and LOS per hospitalization remain stable. Unlike in adults, hospitalizations for DYS/UFD have also increased, suggesting that the increase in hospitalizations for GP is not secondary to changing diagnostic practices., (© 2016 John Wiley & Sons Ltd.)

Background: Children with intractable functional constipation (FC) may eventually require surgery, often guided by motility testing. However, there are no evidence-based guidelines for the surgical management of intractable FC in children., Aim: To assess the diagnostic and surgical approach of pediatric surgeons and pediatric gastroenterologists towards children with intractable FC., Methods: A survey was administered to physicians attending an international conference held simultaneously in Columbus (Ohio, USA) and Nijmegen (the Netherlands). The survey included 4 questions based on cases with anorectal and colonic manometry results., Results: 74 physicians completed the questionnaire. Anorectal manometry was used by 70%; 52% of them would consider anal sphincter botulinum toxin injections for anal achalasia and 21% would use this to treat dyssynergia. Colonic manometry was used by 38%; 57% of them reported to use this to guide surgical decision-making. The surgical approach varied considerably among responders answering the case questions based on motility test results; the most commonly chosen treatments were antegrade continence enemas and anal botulinum injections., Conclusion: Surgical decision-making for children with intractable FC differs among physicians. There is a need for clinical guidelines regarding the role of anorectal and colonic manometry in surgical decision-making in children with intractable FC., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose of Review: Constipation is a very common problem in pediatrics with both the severity of presentations and treatments varying across a broad spectrum. The majority of children with functional constipation are managed successfully without the need for specialized testing and surgical intervention. Those who present with intractable constipation, with or without fecal soiling, require referrals for motility testing that helps determine both medical and surgical management, and interventions. The literature was reviewed for publications on surgical approaches to children with severe constipation, including assessing the quality and levels of evidence and the use of objective measures to determine outcomes., Recent Findings: There is very little in the way of recent studies evaluating surgical indications or treatment approaches for functional constipation, apart from one systematic review and one recent expert review. Although the systematic review was published in the last year, most of the studies it analyzes are older. The vast majority of studies comprise level 4 and 5 evidence., Summary: The indication for most surgical procedures is 'failed' medical management, yet no standardized definition for this exists. Many surgical procedures are proposed with little evidence to show outcomes. We recommend that the surgical evaluation and treatment of children with constipation needs to be protocolized and studied in a prospective manner using validated outcomes measures. Our center's current protocol is described.

Background: The decision to perform autologous intestinal lengthening in patients with short bowel syndrome (SBS) depends on total bowel length and the diameter and length of dilated segments. This study evaluated the accuracy of radiologic measurements of intestinal length and diameter., Methods: Patients who underwent an intestinal lengthening procedure with preoperative upper gastrointestinal study (UGI) were identified from 10/2012 through 1/2015. Measurements of total length and diameters and lengths of dilated segments on UGI were compared to intraoperative measurements using Spearman's rank correlation coefficients and Bland-Altman plots., Results: Fourteen patients underwent 15 lengthening procedures. Median age was 3.6years. Most common causes of SBS were complicated gastroschisis (43%) and small bowel atresia (36%). Intra-operative bowel lengths prior to performing lengthening procedures ranged from 21 to 170cm. The median measurements of radiographic and operative measurements (respectively) were total bowel lengths 77cm and 69cm (r=0.93, p<0.0001), maximum diameters 7.7cm and 7cm (r=0.86, p=0.001), and lengths of dilated segments 13cm and 14cm (r=0.41, p=0.36)., Conclusions: The correlation between UGI and operative measurements suggests that prediction of total bowel length and maximum diameter of dilation is accurate and can assist with operative planning., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.

Objective: To determine whether children with symptoms of internalizing psychiatric disorders have a greater prevalence of pain-predominant functional gastrointestinal disorders (FGIDs) and migraine-like headaches., Study Design: Children and adolescents aged 6-18 years were recruited from a behavioral health center (n = 31) and a primary care center (n = 36). Subjects completed Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition-based symptom inventory questionnaires to screen for internalizing psychiatric disorders, the Questionnaire on Pediatric Gastrointestinal Symptoms, and a somatic distress assessment interview., Results: Thirty-three subjects (19 of 31 from the behavioral health center and 14 of 36 from the primary care center) screened positive for symptoms of anxiety or depressive disorders. The remainder screened negative and served as controls. Pain-predominant FGIDs were more common in the group with symptoms of anxiety or depression compared with controls (prevalence, 51.5% vs 8.8%; P = .0002). Migraine headaches occurred in 57.6% of the subjects with internalizing psychiatric disorders vs 23.5% of the control group (P = .006). The prevalence of functional constipation did not differ significantly between the 2 groups. The data remained essentially unchanged when analyzed within each center of recruitment., Conclusion: Youths with anxiety or depressive symptoms are more likely to suffer from pain-predominant FGIDs and migraine-like headaches, but not from functional constipation. The lack of an association between functional constipation and internalizing psychiatric symptoms suggests that FGIDs associated with pain may bear a specific relationship to emotional disorders., (Copyright © 2013 Mosby, Inc. All rights reserved.)