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Children post-tracheoesophageal fistula (TEF) repair may present with chronic respiratory and gastrointestinal symptoms that can affect quality of life., Objective: To identify factors associated with positive findings on triple endoscopy following neonatal TEF repair., Study Design: Case series with retrospective review of patients., Setting: Tertiary care center aerodigestive program., Methods: Children with neonatally repaired congenital TEF who had a triple endoscopy between 2011 and 2022 were reviewed. The presence of chronic cough, recurrent pulmonary infections, lipid-laden macrophages (LLM), and airway and esophageal anomalies were among the variables analyzed. Chi-square and Kruskal-Wallis univariate analysis was performed., Results: The mean age was 4.28 ± 4.65 years old, and the most common type of TEF repaired was type C (78%). Within our cohort, 87% of patients had GERD, 60% of patients had prior esophageal dilations, and 84% of patients had tracheomalacia. Thirty-one (46.3%) patients had laryngeal cleft, of which 77.4% had a history of prior esophageal dilations ( P  = .01). Twenty-one (33.9%) patients had tracheal diverticulum on bronchoscopy, which was associated with chronic cough, stridor, and coughing with feeds. Patients with positive LLM on BAL were associated with presentation of chronic cough and stridor ( P  = .03). Recurrent TEF was associated with chronic cough. Subglottic stenosis was associated with a history of prolonged intubation ( P  < .05)., Conclusion: Chronic cough was frequently reported and associated with tracheal diverticulum, recurrent TEF, and positive LLM findings on triple endoscopy in patients after congenital TEF repair. Patients presenting with chronic cough and stridor following congenital TEF repair may benefit from a multidisciplinary evaluation., Competing Interests: None., (© 2025 The Author(s). OTO Open published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Objective: To define the essential elements of the intake questionnaire that will be a part of a larger multicenter registry for aerodigestive patients., Methods: A modified Delphi method was utilized to obtain consensus on the data elements that should warrant inclusion in the final research database. Patient questionnaires from the eight participating institutions were reviewed and individual elements were aggregated into 14 categories., Results: A total of 198 initial elements were voted on for inclusion. The categories included demographics, respiratory symptoms, gastrointestinal symptoms, ear nose and throat symptoms, feeding, birth history, medical history, surgical history, family history, social history, medications prior to evaluation, devices used prior to evaluation, prior diagnostic evaluations, and prior evaluation by aerodigestive team members. 83 of the 198 elements met consensus for inclusion in the final registry for an inclusion rate of 41.9 %. Three separate rounds of ranking were required to obtain consensus., Conclusion: The aerodigestive registry is an important initiative that will help foster research and help guide future management. The intake questionnaire of the registry is a critical component of this project, and the consensus obtained during this study should help create a streamlined and efficient registry that will help all aerodigestive patients on a national level., Competing Interests: Declaration of competing interest There are no financial conflicts of interest from the authors., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Objectives: Percutaneous electrical nerve field stimulation (PENFS) has demonstrated promise in single-center trials for pediatric abdominal pain-related disorders of gut-brain interaction (DGBI). Our aim was to explore efficacy of PENFS as standard therapy for DGBI in a registry involving multiple pediatric gastroenterology referral centers., Methods: This was a multicenter, prospective open-label registry of children (8-18 years) undergoing PENFS for DGBI at seven tertiary care gastroenterology clinics. DGBI subtypes were classified by Rome IV criteria. Parents and patients completed Abdominal Pain Index (API), Nausea Severity Scale (NSS), and Functional Disability Inventory (FDI) questionnaires before, during therapy and at follow-up visits up to 1 year later., Results: A total of 292 subjects were included. Majority (74%) were female with median (interquartile range [IQR]) age 16.3 (14.0, 17.7) years. Most (68%) met criteria for functional dyspepsia and 61% had failed ≥4 pharmacologic therapies. API, NSS, and FDI scores showed significant declines within 3 weeks of therapy, persisting long-term in a subset. Baseline (n = 288) median (IQR) child-reported API scores decreased from 2.68 (1.84, 3.58) to 1.99 (1.13, 3.27) at 3 weeks (p < 0.001) and 1.81 (0.85, 3.20) at 3 months (n = 75; p < 0.001). NSS scores similarly improved from baseline, persisting at three (n = 74; p < 0.001) and 6 months later (n = 55; p < 0.001). FDI scores displayed similar reductions at 3 months (n = 76; p = 0.01) but not beyond. Parent-reported scores were consistent with child reports., Conclusions: This large, comprehensive, multicenter registry highlights efficacy of PENFS for gastrointestinal symptoms and functionality for pediatric DGBI., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective: To determine the utility of triple endoscopy (combined direct laryngoscopy, bronchoscopy (DLB), flexible bronchoscopy with bronchoalveolar lavage (FB + BAL), and esophagogastroduodenoscopy (EGD)) in the diagnosis and management of patients with recurrent croup (RC), and to identify predictors of endoscopic findings METHODS: A retrospective chart review was performed of pediatric patients (age <18 years) with RC evaluated by triple endoscopy at a tertiary care pediatric hospital from 2010 to 2021. Data including presenting symptoms, airway findings, BAL and EGD with biopsy findings were collected., Results: 42 patients with RC underwent triple endoscopy were included. The mean age was 4.55±2.84 years old. The most common symptom was chronic cough among 19 (45%) patients, while 23 (55%) patients had gastrointestinal (GI) symptoms. Airway findings included tracheomalacia in 19, laryngeal cleft in 17, and subglottic stenosis in 11 patients. On EGD with biopsy, abnormal gross findings were present in 6 and abnormal microscopic findings in 18 patients, including 6 with histologic findings suggestive of gastroesophageal reflux and 5 with eosinophilic esophagitis. Seventeen (40%) patients had positive culture on BAL. No findings in patient histories significantly predicted presence of lower airway malacia, subglottic stenosis, or abnormal EGD findings., Conclusions: Children with recurrent croup presenting to aerodigestive centers may not have any pertinent presenting symptoms that correlate with significant findings on triple endoscopy. Further work is needed to determine which children with recurrent croup may benefit from aerodigestive evaluation., Level of Evidence: Level 3., Competing Interests: Declaration of Competing Interest The authors have no relevant conflicts of interest, financial or otherwise, to disclose., (Copyright © 2023. Published by Elsevier B.V.)

Objectives: The diagnostic utility of mucosal biopsies taken during colonoscopy-guided colonic manometry catheter placement is unknown. The aims of our study were to determine the frequency and histopathology results of mucosal biopsies during these procedures and to assess whether there were any associations between the histology or gross findings with manometry results., Methods: We performed a retrospective chart review of children who had a colonic manometry study completed between 2008 and 2020 at a quaternary children's hospital. We captured patient demographics, biopsy locations, histopathology results, gross endoscopy findings, and manometry results. The chi-squared test and when appropriate Fisher exact test was used to evaluate categorical associations., Results: One hundred forty-eight patients were included. One hundred eighteen (80%) had colonic biopsy and 63 (43%) had ileal biopsy. Colonic histology findings, which patients could have multiple, included lymphonodular hyperplasia (34%), normal (27%), chronic inflammation (24%), melanosis coli (21%), colonic eosinophilia (10%), and acute inflammation (8%). Ileal histology findings included increased Peyer patches (44%), normal (44%), acute inflammation (11%), chronic inflammation (3%), eosinophilia (5%), and eosinophilic ileitis (3%). The majority of acute and chronic inflammation was graded as mild. There were no statistically significant associations of histology to gross endoscopy or manometry findings., Conclusions: Colonic biopsies are obtained in the majority of patients presenting for colonic manometry evaluation with ileal biopsies obtained less frequently. Histopathology findings are noted frequently, but the majority are the result of or did not impact clinical care. There were no associations between abnormal histopathology or abnormal gross endoscopy findings with colonic manometry results., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Purpose of Review: Children with aerodigestive disorders frequently have concerns regarding difficulty breathing, swallowing, and growing. In this review, we explored the role of pediatric gastroenterologists in the evaluation of complex aerodigestive disorders and the overall approach to these often-challenging patients., Recent Findings: Pediatric gastroenterologists evaluate children with aerodigestive concerns ranging from dysphagia and gastroesophageal reflux to complex congenital abnormalities such as esophageal atresia. Diagnostic tools, such as multichannel intraluminal impedance-pH monitoring, are used for diagnosing gastroesophageal reflux and assessing the correlation with symptoms. Endoscopic evaluation, and in some complex cases, with therapeutic dilations may also be performed. Gastrointestinal dysmotility evaluation with manometry studies are also being increasingly utilized. Multidisciplinary aerodigestive programs can provide a coordinated approach to children with complex airway, pulmonary and gastrointestinal tract disorders. A pediatric gastroenterologist's expertise and specialized skills not only offer many diagnostic tools for these complicated medical cases but are also important in long term medical management., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

We present 2 siblings with a novel type 1 inositol 1,4,5-triphosphate receptor ( ITPR1 ) missense variant who exhibit gastrointestinal dysmotility (chronic constipation and gastroparesis). ITPR1 is expressed in the cerebellum and interstitial cells of Cajal. Periodic release of calcium by ITPR1 initiates pacemaker currents, resulting in smooth muscle contraction. ITPR1 mutations are known to be associated with neurologic syndromes, and these variants have not previously been associated with significant gastrointestinal manifestations in humans. Using whole-genome sequencing, in silico prediction software, biopsy samples, and manometry, the identified novel ITPR1 variant is likely pathogenic and may have neurogastroenterology implications., (© 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)

Chronic cough is a common complaint in the pediatric population and can have many different etiologies. We present a rare case of a tracheal lobular capillary hemangioma (LCH), also known as pyogenic granuloma, causing chronic cough in a child. In this case, the tracheal LCH was managed successfully with laser ablation. A review of the literature reveals only 2 other reported pediatric cases of tracheal LCH. Laryngoscope, 131:1729-1731, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Objective: To describe the clinical features, therapeutic interventions, and patient outcomes of gastrointestinal (GI) hemorrhage in individuals with a telomere biology disorder, including dyskeratosis congenita, Hoyeraal-Hreidarsson syndrome, Revesz syndrome, and Coats plus., Study Design: Clinical Care Consortium for Telomere Associated Ailments members were invited to contribute data on individuals with telomere biology disorders at their institutions who experienced GI bleeding. Patient demographic, laboratory, imaging, procedural, and treatment information and outcomes were extracted from the medical record., Results: Sixteen patients who experienced GI hemorrhage were identified at 11 centers. Among 14 patients who underwent genetic testing, 8 had mutations in TINF2, 4 had mutations in CTC1 or STN1, and 1 patient each had a mutation in TERC and RTEL1. Ten patients had a history of hematopoietic cell transplantation. The patients with Coats plus and those without Coats plus had similar clinical features and courses. Angiodysplasia of the stomach and/or small bowel was described in 8 of the 12 patients who underwent endoscopy; only 4 had esophageal varices. Various medical interventions were trialed. No single intervention was uniformly associated with cessation of bleeding, although 1 patient had a sustained response to treatment with bevacizumab. Recurrence was common, and the overall long-term outcome for affected patients was poor., Conclusions: GI bleeding in patients with telomere biology disorders is associated with significant morbidity and with vascular ectasias rather than varices., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Pediatric patients with complex colorectal and genitourinary conditions often require coordinated multidisciplinary care; however, this coordinated care can be hard to structure and deliver. The purpose of this paper is to review the development and implementation of a multidisciplinary colorectal and pelvic health program, one year after the program's initiation., Methods: This is an observational retrospective 1-year study (10/1/2017 to 9/30/2018). In fiscal year (FY) 2018, a multidisciplinary colorectal and pelvic health program was initiated. The program development incorporated bimonthly team meetings, educational conferences, and initiation of three clinics: a complex colorectal and genitourinary reconstruction clinic, a bowel management clinic, and a colonic motility clinic. Conditions treated included complex anorectal and cloacal malformations, Hirschsprung disease, and idiopathic constipation. The fiscal year was selected to provide comparative administrative data after program implementation., Results: During the study period, 121 patients underwent comprehensive collaborative evaluation of which 58 (47%) were new to the institution compared to 12 (19%) new patients in the previous year (p < 0.001). In FY 2018, there were 130 procedures performed and 512 collaborative visits with an average of 47 visits per month. This was a 3.4-fold increase in visits compared to FY2017 (171 visits). Of the new patients, 60% (35/58), traveled a median of 181 miles, representing 33 statewide counties, and 4 states compared to a median of 93 miles in the previous fiscal year (p = 0.004)., Conclusion: The development of a colorectal and pelvic health program is feasible and requires a collaborative approach, necessitating multiple service lines within an institution. Program creation and implementation can result in rapid institutional clinical growth by filling a local and regional need through coordinated multidisciplinary care., Level of Evidence: IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: We compared the cost-effectiveness of the common surgical strategies for the management of infants with feeding difficulty., Methods: Infants with feeding difficulty undergoing gastrostomy alone (GT), GT and fundoplication, or gastrojejunostomy (GJ) tube were enrolled between 2/2017 and 2/2018. A validated GERD symptom severity questionnaire (GSQ) and visual analog scale (VAS) to assess quality of life (QOL) were administered at baseline, 1 month, and every 6 months. Data collected included demographics, resource utilization, diagnostic studies, and costs. VAS scores were converted to quality adjusted life months (QALMs), and costs per QALM were compared using a decision tree model., Results: Fifty patients initially had a GT alone (71% laparoscopically), and one had a primary GJ. Median age was 4 months (IQR 3-8 months). Median follow-up was 11 months (IQR 5-13 months). Forty-three did well with GT alone. Six (12%) required conversion from GT to GJ tube, and one required a fundoplication. Of those with GT alone, six (14%) improved significantly so that their GT was removed after a mean of 7 ± 3 months. Overall, the median GSQ score improved from 173 at baseline to 18 after 1 year (p < 0.001). VAS scores also improved from 70/100 at baseline to 85/100 at 1 year (p < 0.001). ED visits (59%), readmissions (47%), and clinic visits (88%) cost $58,091, $1,442,139, and $216,739, respectively. GJ tube had significantly higher costs for diagnostic testing compared to GT (median $8768 vs. $1007, p < 0.001). Conversion to GJ tube resulted in costs of $68,241 per QALM gained compared to GT only., Conclusions: Most patients improved with GT alone without needing GJ tube or fundoplication. GT and GJ tube were associated with improvement in symptoms and QOL. GJ tube patients reported greater gains in QALMS but incurred higher costs. Further analysis of willingness to pay for each additional QALM will help determine the value of care., Study and Level of Evidence: Cost-effectiveness study, Level II., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: Pediatric aerodigestive programs appear to be rapidly proliferating and provide multidisciplinary, coordinated care to complex, medically fragile children. Pediatric subspecialists are considered essential to these programs. This study evaluated the state of these programs in 2017 by surveying their size, composition, prevalence, and the number of patients that they serve., Methods: The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Aerodigestive Special Interest Group leadership distributed an 11-question survey to the Pediatric Gastroenterology International Listserv. The mean time of the programs' existence, number of half-day clinics, number of procedure days, number of patients evaluated, and the lead primary specialty were evaluated., Results: Thirty-four programs responded. Twenty-five were based in academic centers. Thirty-one programs were located across the United States. The average time of program existence was 5.3 years (standard deviation [SD] = 4.3; range 1-17 years). Approximately 64.7% were started in the past 5 years. Twelve programs were based in the division of gastroenterology. The average number of gastroenterologists serving aerodigestive programs was 2 (SD = 1.1). The mean number of half-day clinic sessions and procedure days were 2.8 (SD = 2.9) and 2.6 (SD = 2), respectively. New and follow-up visits per year in each program averaged 184 (SD = 168; range 10-750)., Conclusions: Pediatric aerodigestive programs are prevalent, proliferating, and serve a large number of complex patients across North America and the world. This survey demonstrated that programs are predominantly based in academic settings. The number of patients cared for by aerodigestive centers varies widely depending on size and age of program.

Background: High-resolution esophageal manometry (HREM) during laparoscopic Heller myotomy (LHM) with fundoplication for achalasia allows tailoring of myotomy length and wrap tightness. The purpose of this study is to quantify long-term postoperative symptom severity and quality of life using validated questionnaires., Methods: Children ≤18 years with achalasia who previously underwent LHM with intraoperative HREM from 2010 to 2017 were prospectively surveyed. Eckardt Symptom Score (ESS), Achalasia Severity Questionnaire (ASQ), Pediatric Quality of Life Inventory (PedsQL), and Pediatric GERD Symptom and Quality of Life (PGSQ) questionnaires were administered. Scores for historical controls were obtained from prior survey instrument validation studies as comparison., Results: Of 30 eligible patients, 12 (40%) completed the surveys. Mean age at time of surgery was 13 ± 3 years. Assessment was performed at least 10 months after surgery with mean time elapsed of 3.6 ± 2 years. Average premyotomy lower esophageal sphincter (LES) pressure, postmyotomy LES pressure, and postfundoplication LES pressure were 30 ± 10 mmHg, 14 ± 6 mmHg, and 18 ± 9, respectively. ESS (2.3/12), ASQ (39/100 ± 16), PGSQ (symptom: 0.6/4 ± 0.4, school: 0.4/4 ± 0.4), and overall PedsQL (82/100 ± 15) were similar to those of healthy historical controls., Conclusion: Children with achalasia undergoing LHM with intraoperative HREM had sustained long-term symptom improvement and quality of life scores comparable to healthy patients., Study and Level of Evidence: Retrospective, II., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: This project compares the degree of tracheal collapse determined by rigid and flexible bronchoscopy in paediatric patients with tracheomalacia., Methods: A total of nine patients with tracheomalacia underwent both rigid and flexible video bronchoscopy. All patients were breathing spontaneously. Cross-sectional images of the airway were processed using the ImageJ program and analysed via colour histogram mode technique in order to delineate the luminal area. Paired t-tests (conducted using Stata software version 13.0) quantified differences between rigid and flexible bronchoscopes regarding the ratios of luminal pixels at maximum airway collapse to expansion. Correlation between both techniques in terms of airway collapse to expansion ratios was determined by calculating the Pearson correlation coefficient (R)., Results: The difference in ratios of maximum collapse to expansion between rigid and flexible bronchoscopy was not statistically significant (p = 0.4656) and was positively correlated (R = 0.523)., Conclusion: The ratios suggest that rigid and flexible bronchoscopy are equally efficacious in assessing tracheomalacia severity, and may be used interchangeably in a clinical setting.

Competing Interests: Conflict of Interest: None

Objectives: To determine the efficacy of physical therapy (PT) for fecal incontinence in children with pelvic floor dyssynergia (PFD)., Study Design: Retrospective chart review of children with PFD completing >1 PT session for fecal incontinence at a quaternary children's hospital. The frequency of fecal incontinence (primary outcome), constipation-related medication use, number of bowel movements (in those with <3 per week at baseline) and pelvic floor muscle (PFM) function were captured at baseline and at the final PT visit. Outcomes were categorized as excellent (complete continence), good (>50% decrease in fecal incontinence frequency), fair (not worsening but <50% fecal incontinence frequency decrease), and poor (more frequent fecal incontinence). Compliance with PT was determined by the percentage of attended PT appointments., Results: Children included met the following primary outcomes: 27 (42.2%) excellent, 24 (37.5%) good, 11 (17.1%) fair, and 2 (3.1%) poor. Factors associated with an excellent or good outcome included improved PFM functioning and good (≥70% PT attendance) compliance. Children with a history of surgically corrected tethered spinal cord were more likely to have a fair outcome (P = .015). Use of constipation-related medications decreased (1.9 ± 0.7 vs 1.5 ± 0.9, P = .005). Weekly bowel movement frequency increased (1.6 ± 0.6 vs 6.4 ± 4.8, P < .001) in those with infrequent bowel movements (n = 26) at baseline., Conclusions: Pelvic floor PT is effective in the majority of children with fecal incontinence related to PFD. Factors associated with PT efficacy include improved PFM functioning, good compliance with PT, and history of tethered cord., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Bronchogenic cysts are congenital malformations of the tracheobronchial tree. We describe a 20-month-old male who presented with persistent non-bilious emesis; manometry and imaging were consistent with esophageal achalasia. During a planned laparoscopic Heller myotomy, an intramural bronchogenic cyst was discovered in the anterior esophagus at the level of the gastroesophageal junction and successfully resected with resolution of his symptoms.

Background: Esophagogastroduodenoscopy (EGD) and gastric emptying scintigraphy (GES) are commonly performed in the evaluation of children with upper gastrointestinal symptoms. It has been presumed, but not clarified, that gastroparesis increases the likelihood of identifying abnormalities on EGD. We sought to determine whether the presence of gastroparesis influenced the diagnostic yield of EGD in children., Methods: We conducted a retrospective chart review of children who underwent both an EGD and GES within 3 months of each other for evaluation of upper gastrointestinal symptoms (eg, abdominal pain). Clinical history (symptoms, comorbidities, medications, and surgical procedures), GES results, and EGD histology reports were captured., Results: A total of 125 children (46% female) were included, of whom, 70 (56%) had gastroparesis. Thirty-three (26%) children had liquid meal GES (1.2 ± 1.1 y of age, mean ± SD) and 92 (64%) had solid meal GES (12.4 ± 3.6 y of age). There was an overall trend toward a decreased frequency of biopsy abnormalities in those with gastroparesis (P=0.09). Those with gastroparesis identified through liquid meal GES were less likely to have reflux esophagitis on biopsy (P=0.002). Those with gastroparesis identified on solid meal GES were less likely to have gastritis (P=0.04). Symptoms, comorbidities, or medications were not predictive of GES or EGD results., Conclusions: Children with gastroparesis may be less likely to have biopsy abnormalities identified on EGD in comparison to those without gastroparesis. Further prospective, larger, and multicenter studies are needed to validate our findings.