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BackgroundEsophageal atresia (EA) is one of the most common congenital digestive anomalies. With improvements in surgical techniques and intensive care treatments, the focus of care of these patients has shifted from mortality to morbidity and quality-of-life issues. These children face gastrointestinal (GI) problems not only in early childhood but also through adolescence and adulthood. There is, however, currently a lack of a systematic approach to the care of these patients. The GI working group of International Network on Esophageal Atresia comprises members from ESPGHAN/NASPGHAN and was charged with the task of developing uniform evidence-based guidelines for the management of GI complications in children with EA.MethodsThirty-six clinical questions addressing the diagnosis, treatment, and prognosis of the common GI complications in patients with EA were formulated. Questions on the diagnosis, and treatment of gastroesophageal reflux, management of "cyanotic spells," etiology, investigation and management of dysphagia, feeding difficulties, anastomotic strictures, congenital esophageal stenosis in EA patients were addressed. The importance of excluding eosinophilic esophagitis and associated GI anomalies in symptomatic patients with EA is discussed as is the quality of life of these patients and the importance of a systematic transition of care to adulthood. A systematic literature search was performed from inception to March 2014 using Embase, MEDLINE, the Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Clinical Trials, and PsychInfo databases. The approach of the Grading of Recommendations Assessment, Development and Evaluation was applied to evaluate outcomes. During 2 consensus meetings, all recommendations were discussed and finalized. The group members voted on each recommendation, using the nominal voting technique. Expert opinion was used where no randomized controlled trials were available to support the recommendation.

Objectives: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients., Methods: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model., Conclusions: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD., Competing Interests: Dr Friedlander is the Chief Medical Officer and Co-Founder of EvoEndo, Inc. He is an employee, stockholder, and on the board of directors of EvoEndo, Inc. He is co-inventor on several University of Colorado and EvoEndo, Inc patents and patent pendings related to virtual reality, endoscope design, endoscopic methods, endoscopic training methods, and its associated technologies. Dr Prager is a cofounder of EvoEndo, Inc. The remaining 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.)

Objectives: After surgical repair, up to 70% of esophageal atresia (EA) patients suffer from gastroesophageal reflux disease (GERD). The ESPGHAN/NASPGHAN guidelines on management of gastrointestinal complications in EA patients were published in 2016. Yet, the implementation of recommendations on GERD management remains poor.We aimed to assess GERD management in EA patients in more detail, to identify management inconsistencies, gaps in current knowledge, and future directions for research., Methods: A digital questionnaire on GERD management in EA patients was sent to all members of the ESPGHAN EA working group and members of the International network of esophageal atresia (INoEA)., Results: Forty responses were received. Thirty-five (87.5%) clinicians routinely prescribed acid suppressive therapy for 1-24 (median 12) months. A fundoplication was considered by 90.0% of clinicians in case of refractory GERD with persistent symptoms despite maximal acid suppressive therapy and in 92.5% of clinicians in case of GERD with presence of esophagitis on EGD. Half of clinicians referred patients with recurrent strictures or dependence on transpyloric feeds. Up to 25.0% of clinicians also referred all long-gap EA patients for fundoplication, those with long-term need of acid suppressants, recurrent chest infections and feedings difficulties., Conclusions: Respondents' opinions on the optimal duration for routine acid suppressive therapy and indications for fundoplication in EA patients varied widely. To improve evidence-based care for EA patients, future prospective multicenter outcome studies should compare different diagnostic and treatment regimes for GERD in patients with EA. Complications of therapy should be one of the main outcome measures in such trials., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 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: Functional luminal imaging probe (FLIP) measures pressure-geometry relationships of digestive luminal space. When used in esophageal disorders, it provides several luminal parameters that help better understand the pathophysiology. Data about the potential utility of FLIP in pediatrics are scarce and there is no standardized use in children. We aim to describe the use of FLIP in our center, its safety, feasibility, and clinical impact in esophageal disorders in children., Methods: Consecutive FLIP recordings performed at the Centre Hospitalier Universitaire-Sainte-Justine, Montréal, Canada between February 2018 and January 2021 were extracted. A chart review was conducted for demographics and medical history. Symptomatology after the procedure was evaluated with validated dysphagia scores., Key Results: Nineteen patients were included (11 girls, median age 16 years, range 3.2-19.6) with achalasia (n = 5), post-Heller's myotomy dysphagia (n = 3), esophagogastric junction outflow obstruction (n = 3), congenital esophageal stenosis (n = 2); post-esophageal atresia repair stricture (n = 3), and post-fundoplication dysphagia (n = 3). There was no significant correlation between integrated relaxation pressure measured with high resolution manometry and distensibility index (DI). The use of FLIP made it possible to differentiate between dysphagia related to an esophageal obstruction (DI < 2.8 mm2/mmHg) and dysphagia without major motility disorder (DI > 2.8 mm2/mmHg) that guided the indication for dilation. FLIP led to a change in management in 47% of the patients. Forty-seven percent of the patients were symptom free at the time of the evaluation., Conclusions Inferences: FLIP provides key esophageal luminal values and therefore can play an important role in pediatric esophageal disorders management., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: The aim of this study was to assess whether clinicians approached the management of children with esophageal atresia (EA) in accordance with the 2016 European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN)/North American Society of Paediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on the management of gastrointestinal and nutritional complications in this cohort., Methods: We invited expert physicians and surgeons closely involved in the care of children with EA (members of the International network on esophageal atresia [INoEA], ESPGHAN EA working group, French national EA registry, European pediatric surgical association (EUPSA), and European rare disease reference network [ERNICA]) to participate in an anonymous online survey containing 15 multiple choice questions concerning the management of gastrointestinal and nutritional complications in children with EA. Questions were based on the management of gastroesophageal reflux disease (GERD) dysphagia, cyanotic spells, feeding and nutrition, anastamotic strictures, and transition to adult care as detailed in the 2016 guidelines., Results: Median concordance with ESPGHAN/NASPHAN EA Guidelines was 69% (16-100%, SD 16%) across all responders. Areas of greatest concordance were in the fields of surveillance endoscopy and medical management of GERD. Areas for potential educational opportunities include: the differential diagnosis and appropriate investigation of dysphagia and the diagnostic evaluation of extraesophageal symptoms., Conclusions: This survey highlights the importance of improving the understanding and adherence to the EA guidelines amongst clinicians involved in the care of these patients., 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.)

Objectives: Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptable levels of morbidity and mortality. Major contributors to the disappointing lack of progress in paediatric CIPO include a dearth of clarity and uniformity across all aspects of clinical care from definition and diagnosis to management. In order to assist medical care providers in identifying, evaluating, and managing children with CIPO, experts in this condition within the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as well as selected external experts, were charged with the task of developing a uniform document of evidence- and consensus-based recommendations., Methods: Ten clinically relevant questions addressing terminology, diagnostic, therapeutic, and prognostic topics were formulated. A systematic literature search was performed from inception to June 2017 using a number of established electronic databases as well as repositories. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to evaluate outcome measures for the research questions. Levels of evidence and quality of evidence were assessed using the classification system of the Oxford Centre for Evidence-Based Medicine (diagnosis) and the GRADE system (treatment). Each of the recommendations were discussed, finalized, and voted upon using the nominal voting technique to obtain consensus., Results: This evidence- and consensus-based position paper provides recommendations specifically for chronic intestinal pseudo-obstruction in infants and children. It proposes these be termed paediatric intestinal pseudo-obstructive (PIPO) disorders to distinguish them from adult onset CIPO. The manuscript provides guidance on the diagnosis, evaluation, and treatment of children with PIPO in an effort to standardise the quality of clinical care and improve short- and long-term outcomes. Key recommendations include the development of specific diagnostic criteria for PIPO, red flags to alert clinicians to the diagnosis and guidance on the use of available investigative modalities. The group advocates early collaboration with expert centres where structured diagnosis and management is guided by a multi-disciplinary team, and include targeted nutritional, medical, and surgical interventions as well as transition to adult services., Conclusions: This document is intended to be used in daily practice from the time of first presentation and definitive diagnosis PIPO through to the complex management and treatment interventions such as intestinal transplantation. Significant challenges remain to be addressed through collaborative clinical and research interactions.

Objectives: Patients with esophageal atresia/tracheoesophageal fistula (EA-TEF) can develop Barrett esophagus as a long-term consequence of their condition. Intestinal metaplasia (IM), a risk factor for developing adenocarcinoma of the esophagus, has not been well characterized in the pediatric population., Methods: Retrospective review of patients with EA-TEF followed at 3 academic pediatric centers between the years 1997 and 2014., Results: Among 542 children and adolescents, 1.3% (7 patients, 5 girls) were diagnosed with IM based on endoscopy and pathology. Six of the patients had EA-TEF type C, whereas the last patient had a "long gap" type A atresia. Patients were diagnosed with gastric metaplasia either before the IM diagnosis in 4 patients or concomitantly in 3. The median (range) age of diagnosis for gastric metaplasia was 7.9 (range 2-17.2) and for IM 10.9 (2-17.2) years. Gastroesophageal reflux (GER) symptoms were nonspecific. Five patients were on proton pump inhibitor therapy for symptomatic GER at the time of diagnosis of IM. 2 of the 7 patients had previously undergone Nissen fundoplication. One patient, who had undergone a Nissen fundoplication, was restarted on proton pump inhibitor once the diagnosis of IM was made. All patients had repeated endoscopy and dysplasia was not observed with a median follow-up of 1.7 (range 1-4.9) years., Conclusions: IM occurs in patients with EA-TEF, some as young as 2 years. Therefore, early endoscopic surveillance should be considered in this GER-prone population.

Objectives: The aim of the present study was to evaluate the efficacy and short-term safety of topical mitomycin-C, an antifibrotic agent, in preventing the recurrence of anastomotic strictures after surgical repair of esophageal atresia (EA)., Methods: We retrospectively reviewed the medical records of patients with recurrent anastomotic strictures after EA surgery who underwent at least 3 esophageal dilations. We compared the outcome (ie, resolution of the stricture) of the group that received topical mitomycin-C treatment with endoscopic esophageal dilation with a historical cohort treated by dilations alone., Results: A total of 11 children received mitomycin-C concurrently with endoscopic dilations. After a median follow-up of 33 months (range 18-72), and a mean number of 5.4 dilations per patient (range 3-11), 8 of 11 patients achieved a resolution of their strictures, 2 patients remained with stenosis, and 1 patient needed a surgical correction. In the control group, 10 patients required an average of 3.7 (range 3-7) total dilations. After a follow-up of 125 months (range 35-266) after the last dilation, strictures in 9 of 10 children disappeared and the remaining patient was symptom free. No dysplasia related to mitomycin-C was demonstrated., Conclusions: There is no benefit in the resolution of the stricture when adding mitomycin-C treatment compared with repeated esophageal dilations alone in historical controls. Further randomized controlled studies and a short- and long-term evaluation of safety are needed.

Dual pH-multichannel intraluminal impedance (pH-MII) is a sensitive tool for evaluating overall gastroesophageal reflux disease, and particularly for permitting detection of nonacid reflux events. pH-MII technology is especially useful in the postprandial period or at other times when gastric contents are nonacidic.pH-MII was recently recognized by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as being superior to pH monitoring alone for evaluation of the temporal relation between symptoms and gastroesophageal reflux. In children, pH-MII is useful to correlate symptoms with reflux (particularly nonacid reflux), to quantify reflux during tube feedings and the postprandial period, and to assess efficacy of antireflux therapy. This clinical review is simply an evidence-based overview addressing the indications, limitations, and recommended protocol for the clinical use of pH-MII in children.

Objectives: Esophageal atresia with or without tracheo-esophageal fistula is a frequent malformation that occurs in about 1 of 3000 live births. It can be associated with other congenital malformations. The aim of this study was to measure the frequency of heterotopic pancreas (HP) in children with esophageal atresia (EA) and to evaluate possible linkage with other malformations., Materials and Methods: All patients with EA were prospectively followed since 2005 at Hôpital Sainte-Justine and since 2006 at the Montreal Children's Hospital. We compared 91 patients who underwent gastroscopy during that period with 182 control patients who submitted to gastroscopy for other indications. The presence or the absence of HP and its localization were noted in both groups. The following data were also collected on patients with EA: sex, gestational age, EA type, and other malformations., Results: Seventeen (18.7%) of the 91 patients with EA had gastric HP compared with 1 (0.5%) in the control group (OR 42, 95% confidence interval 7-249, P < 0.001). There were no differences between patients with or without HP regarding sex, prematurity, EA type, and the presence or absence of other congenital abnormalities., Conclusions: This study demonstrates, for the first time, that gastric HP is associated with EA irrespective of other malformations.

Background: Abdominal pain related to irritable bowel syndrome (IBS) and functional abdominal pain (FAP) is frequent in children and can be of variable severity. Both IBS and FAP are associated with rectal hypersensitivity. We hypothesized that in children with IBS and FAP, the rectal sensory threshold for pain (RSTP) is associated with symptom severity., Patients and Methods: A total of 47 patients (34 girls; median age, 14.2 years) with IBS (n = 29) and FAP (n = 18), according to the Rome II criteria, underwent a rectal barostat examination to determine their RSTP. Gastrointestinal symptom severity was assessed by validated questionnaires. During the rectal barostat exam, symptoms were documented using a visual analog scale and by measuring the area coloured on a human body diagram corresponding to painful sensations., Results: The median RSTP was 16 mmHg and was similar in IBS and FAP patients. Eighty-three percent of the patients had rectal hypersensitivity (RSTP < or = 30.8 mmHg, the 5th percentile of control children studied in our laboratory). Fifty-one percent and 36%, respectively, reported missing school and social activities at least once per week. Increased frequency of pain, missed days of school, missed social activities, and pain during the barostat examination were not associated with lower RSTP values in either the whole group or in the subset of children with rectal hypersensitivity., Conclusions: Rectal hypersensitivity is not proportional to the severity of symptoms in children with IBS and FAP, indicating that symptom severity is influenced by other factors in addition to visceral hypersensitivity.