Aerodigestive Sequelae and Triple Endoscopy after Congenital Tracheoesophageal Fistula Repair in Children.

Unlabelled: 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.

Failure to Thrive, Metabolic Acidosis, and Diarrhea in a 7-Week-Old Infant.

A 7-week-old infant presented to the emergency department with fussiness, decreased oral intake, loose stool, and respiratory distress for 2 days. The patient was born full-term with an uncomplicated birth history but had a history of slow weight gain. He was alert, but toxic-appearing at presentation, hypothermic with signs of dehydration, and with respiratory failure.

Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

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.

Consensus on intake questionnaire data elements in the development of an aerodigestive registry.

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.

Laryngeal clefts in Prader-Willi syndrome: Feeding difficulties and aspiration not always caused by hypotonia.

Feeding difficulties, aspiration, and failure to thrive in infancy are commonly seen in patients with Prader-Willi Syndrome (PWS) and attributed to hypotonia. Patients with PWS and laryngeal clefts were identified by review of medical records at three tertiary care children's hospitals between 2017 and 2022. We present three patients with PWS with feeding difficulties who were also found to have laryngeal clefts which likely contributed to their feeding difficulties.

A multicenter registry study on percutaneous electrical nerve field stimulation for pediatric disorders of gut-brain interaction.

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.

Triple endoscopy and recurrent croup in children: A single aerodigestive center experience.

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.

Diagnostic Utility of Mucosal Biopsies Taken During Colonoscopy-Guided Colonic Manometry Catheter Placement.

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.

Pediatric Aerodigestive Medicine: Advancing Collaborative Care for Children With Oropharyngeal Dysphagia.

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 Role of Pediatric Gastroenterologists in the Evaluation of Complex Aerodigestive Disorders.

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.

A multi-disciplinary approach to chronic cough in children.

Objectives: (1) To highlight the important causes of chronic and recurrent cough in children. (2) To discuss multidisciplinary approach to management of chronic/recurrent pediatric cough.

Methods: Review of scholarly articles, guidelines, expert panels via PubMed and Google Scholar.

Gastrointestinal Dysmotility Is a Significant Feature in 2 Siblings With a Novel Inositol 1,4,5-Triphosphate Receptor 1 () Missense Variant.

We present 2 siblings with a novel type 1 inositol 1,4,5-triphosphate receptor () missense variant who exhibit gastrointestinal dysmotility (chronic constipation and gastroparesis). is expressed in the cerebellum and interstitial cells of Cajal. Periodic release of calcium by initiates pacemaker currents, resulting in smooth muscle contraction.

Eosinophilic esophagitis with and without airway involvement in children - A comparative analysis.

Background: Eosinophilic esophagitis (EoE) is an allergic disease characterized by marked eosinophilic infiltration and inflammation of the esophagus eventually leading to esophageal dysfunction. This condition at times may involve the airway leading to breathing difficulties.

Objective: To compare the course of EoE in patients with or without airway involvement.

Gastrointestinal Hemorrhage: A Manifestation of the Telomere Biology Disorders.

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.

Pediatric Tracheal Lobular Capillary Hemangioma: A Case Report and Review of the Literature.

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.

Development of a multidisciplinary colorectal and pelvic health program: Program implementation and clinical impact.

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).

Cost-Effectiveness Analysis of the Surgical Management of Infants Less than One Year of Age with Feeding Difficulties.

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.

Size and Prevalence of Pediatric Aerodigestive Programs in 2017.

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.

High-resolution manometric guidance during laparoscopic Heller myotomy: Impact on quality of life and symptom severity for children with achalasia.

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.