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 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.

Identifying Posterior Fossa Abnormality in Pediatric Aerodigestive Patients, a Case Series.

OBJECTIVES: Multidisciplinary clinics like Aerodigestive programs focus on issues associated with airway, pulmonary, and gastrointestinal issues. Rarely, significant neurological issues like posterior fossa abnormality are identified as the primary etiology. We describe 3 such patients and compare their clinical presentation to the other patients seen in Aerodigestive clinic. METHODS: A retrospective chart review was conducted to review the 3 posterior fossa patients and the remainder of children that were referred to the Aerodigestive Clinic at Children's Hospital Los Angeles from June 2016 to August 2018. Clinical characteristics including triple endoscopies and sleep studies were recorded. RESULTS: Of the 110 patients included for review, 3 patients (3%) had an underlying posterior fossa abnormality; all of whom had symptoms of sleep disordered breathing along with dysphagia compared with 30% incidence of this symptom profile in the remaining Aerodigestive population. CONCLUSION: Presence of sleep disordered breathing and dysphagia, with underlying vomiting history, warrants considering evaluation for posterior fossa abnormalities in addition to traditional workup for aerodigestive disorders. Due to the rarity of this presentation and small sample size, future studies with multicenter collaboration may help better describe identifiers to delineate this population with similar aerodigestive symptoms and clarify diagnostic algorithms.

A guide on transnasal endoscopy: setting up a pediatric unsedated endoscopy program.

Background: Unsedated transnasal endoscopy is becoming an increasingly popular option for the evaluation of upper gastrointestinal tract disorders in adults and children worldwide. This innovative technology has transformative potential as it provides for a more efficient, safe, and cost-effective method for endoscopy and reduces the risks associated with anesthesia, which is particularly relevant in pediatrics as endoscopy is commonly done under general anesthesia or conscious sedation. The aim is to address knowledge gaps amongst pediatric gastroenterologists who may be considering the development of a TNE program, detailing how to implement sedation-free TNE into practice for pediatric patients and current and forthcoming technologies. Methods: We conducted a comprehensive review of current literature and collection of data from experts and clinicians in the field on how sedation-free programs were started and being conducted. We aimed to collate the data to provide a guide to address knowledge gaps with a focus on setting up and starting a sedation-free endoscopy program. Results: Here in, we provide a detailed guide for implementing a sedation-free endoscopy program in pediatrics including design and layout of a TNE unit, special staffing needs, equipment, current and forthcoming technologies, financial considerations and training considerations. We highlight special considerations that are relevant in pediatrics incorporating distraction or dissociation techniques such as Virtual Reality Systems, developmentally appropriate preparation for children, and topical analgesia. Conclusion: Sedation-free endoscopy is a rapidly growing option for pediatric patients. Development of an unsedated pediatric endoscopy program will improve patient care, decrease the need for anesthesia, provide a lower cost and safe alternative to traditional sedated endoscopy, and is a viable component to a pediatric gastroenterology practice.

Perioperative respiratory adverse events in children undergoing triple endoscopy.

OBJECTIVES: Children with aerodigestive disorders often have many of the reported risk factors for development of perioperative respiratory adverse events. This study sought to evaluate the incidence of such events in this group of patients undergoing general anesthesia for "triple endoscopy" (flexible bronchoscopy with bronchoalveolar lavage, rigid laryngoscopy and bronchoscopy, and esophagogastroduodenoscopy) and to identify any patient-specific or procedure-specific risk factors associated with higher incidence of perioperative respiratory adverse events. METHODS: We performed a retrospective chart review of children 18 years or younger who underwent triple endoscopy as part of an aerodigestive evaluation. Data collected from medical records included: preoperative polysomnography, symptoms of acute respiratory illness, medical comorbidities, demographics, postoperative hospital or intensive care unit admission, and all respiratory events and interventions in the perioperative period. Patient-specific and procedure-specific factors were investigated via univariate analysis for any correlations with perioperative respiratory adverse events. RESULTS: Of the 122 patients undergoing triple endoscopy, 69 (57%) experienced a perioperative respiratory adverse event. We found no difference in the incidence of perioperative respiratory adverse events among children with documented lung disease compared with those with no lung disease (OR: 0.89, p = .8 95% CI: 0.43, 1.8), and no significant difference between those children who had a respiratory illness at the time of surgery, 1-2 weeks prior, 3-4 weeks prior, and those with no preceding respiratory illness. A higher percentage of males had a perioperative respiratory adverse event, compared with females (OR: 2.7, p = .01 95% CI: 1.3, 5.09). CONCLUSION: Patients undergoing triple endoscopy for evaluation of aerodigestive disorders at our institution experienced perioperative respiratory adverse events at a rate of 57%.

Factors impacting participation in flexible endoscopic evaluation of swallowing in children.

OBJECTIVES: Flexible endoscopic evaluation of swallowing (FEES) is a common and useful tool for assessment of dysphagia and swallowing safety in children, however an important problem is limited participation in some children. We examine the factors associated with adequate participation in children undergoing FEES, including the completion rate, whether a clear diagnosis or feeding plan was made, and the incidence of adverse events (AEs). METHODS: We conducted a retrospective review of children undergoing FEES at an urban children's hospital. Data collected included age, gender, race, comorbidities, AEs and outcomes of FEES. RESULTS: Of the 130 patients, 46 (35%) were female with a median age of 2.6 years. 113 (87%) patients cooperated and obtained a result, while 15 (12%) patients did not cooperate and no result was obtained; there was no significant difference in gender, comorbidities, or race between those groups. The age distribution of FEES subjects in this study was skewed with nearly 75% below age 5. Patients who cooperated had a higher median age (3 years) than those who did not (1.2 years) and 47% of non-cooperators were less than 1 year of age. There were no significant AEs, the most common mild AE was excessive crying (34 subjects, 26%). In a multivariate model controlling for age and gender, excessive crying was associated with a decreased odds of cooperation (OR: 0.16, p = 0.004, 95% CI: 0.04, 0.54). CONCLUSION: Children who undergo FEES have an overall favorable completion rate and no serious adverse events, however its utility is limited in cases where children refuse to participate. Understanding the factors associated with failure to cooperate with FEES is important in developing strategies to improve participation. Excessive crying is identified as such a factor in this study.

Strategy for Food Reintroduction Following Empiric Elimination and Elemental Dietary Therapy in the Treatment of Eosinophilic Gastrointestinal Disorders.

PURPOSE OF REVIEW: This review presents the available data regarding efficacy of nutritional therapy, highlighting clinical decision points and a strategy for reintroduction of foods following an elemental diet for treatment of eosinophilic gastrointestinal disorders. RECENT FINDINGS: Elemental and empiric elimination diets are highly effective treatments for eosinophilic gastrointestinal diseases. Standardization in the reintroduction phase, after utilizing the diet for disease remission, is lacking. Clinicians are confronted with multiple challenges regarding the best practice for food reintroduction and identification of potential dietary triggers including order of foods being challenged and duration between endoscopic procedures. Individualization is required for preference and adherence to optimize quality of life and treatment success for this burdensome and life altering immune driven gastrointestinal disorder. Age specific concerns for children, teenagers, and adults should be assessed using a patient centric approach.

Mural Eosinophilic Gastrointestinal Disease in 2 Pediatric Patients Presenting as Focal Mass.

Eosinophilic gastrointestinal diseases (EGIDs) are a diverse group of intestinal diseases involving the infiltration of eosinophils into the bowel wall. EGID can present with a variety of clinical conditions, which are largely dependent on the location of eosinophils in the intestinal wall. We describe the first reported pediatric cases of EGID presenting with symptomatic partial bowel obstruction from intestinal masses due to isolated focal mural involvement. Both patients subsequently responded favorably to therapy with exclusive elemental nutrition in the first case and exclusive elemental nutrition with steroids in the second case. These cases reveal the wide-ranging clinical manifestations of EGID, expand on the differential diagnosis of focal intestinal masses, and provide guidance on the evaluation of ambiguous cases.

Autoimmune Gastritis in Pediatrics: A Review of 3 Cases.

OBJECTIVES: To bring heightened awareness to a condition, autoimmune gastritis (AIG), which is a well-established entity in adults; however, rarely described in pediatrics. Currently, the literature describes AIG in pediatric patients who also suffer from other autoimmune disorders, which precedes the diagnosis of AIG, and often presents with unexplained anemia. Additionally, there have been case reports describing patients with immunodeficiencies and AIG, which progress to gastric adenocarcinoma. AIG is a histopathologic diagnosis, demonstrating chronic inflammatory process with loss of parietal cells with or without intestinal metaplasia and enterochromaffin-like cell hyperplasia. Management of these patients includes nutritional replacement as well as routine surveillance endoscopy with biopsy in search of metaplastic and dysplastic changes. METHODS: We queried the pathology database at Children's Hospital Los Angeles (CHLA) for cases with a final diagnosis of AIG and for those with a differential diagnosis that includes AIG in the diagnostic comment. All cases that were identified were selected as long as they did not only meet the histopathologic criteria, but also the biochemical criteria for this condition. RESULTS: Of the 3 patients, 2 were referred to gastroenterology for the evaluation of iron-deficiency anemia in the context of diabetes mellitus and Addison's disease; and diabetes mellitus and Hashimoto's thyroiditis. AIG was confirmed on the biopsies, which showed a reduction in parietal cell mass, pseudopyloric metaplasia and enterochromafin-like cell hyperplasia. Both patients were treated with iron replacement therapy. The third patient presented with symptomatic anemia and diagnosed with pernicious anemia without other autoimmune disorders. She was successfully treated with oral vitamin supplementation. In this case, serial gastric biopsies demonstrated stable intestinal metaplasia without evidence of dysplasia. CONCLUSION: Although AIG is rare in children, pediatric gastroenterologists and pathologists should have a heightened suspicion for this entity in those patients with a history of autoimmune disorders and/or pernicious anemia.

Intravenous Fish Oil Lipid Emulsion Prevents Catheter-Related Thromboses in Pediatric Patients with Intestinal Failure.

A central venous catheter is a risk factor for deep vein thrombosis. We compared the incidence of deep vein thrombosis in children with intestinal failure patients receiving soy oil lipid emulsion (n = 35) vs fish oil lipid emulsion (n = 35). Ten deep vein thrombosis occurred in the soy oil lipid emulsion group, and none in the fish oil lipid emulsion group (P < .001).

Neuregulin-4 is a survival factor for colon epithelial cells both in culture and in vivo.

Expression of the ErbB4 tyrosine kinase is elevated in colonic epithelial cells during inflammatory bowel disease, whereas ErbB4 overexpression in cultured colonocytes blocks TNF-induced apoptosis in a ligand-dependent manner. Together, these observations suggest that ErbB4 induction may be a protective response. However, the effects of ErbB4 signaling in the colonic epithelium in vivo are not known. Furthermore, previous work on ErbB4 used ligands shared with other receptors, raising the question of whether the observed responses are explicitly due to ErbB4. In this study, we used the ErbB4-specific ligand neuregulin-4 (NRG4) to activate ErbB4 and define its role in colonocyte biology. NRG4 treatment, either in cultured cells or in mice, blocked colonic epithelial apoptosis induced by TNF and IFN-γ. It was also protective in a murine experimental colitis model. NRG4 stimulated phosphorylation of ErbB4 but not other ErbB receptors, indicating that this is a specific response. Furthermore, in contrast to related ligands, NRG4 enhanced cell survival but not proliferation or migration, and stimulated phosphorylation of the anti-apoptotic mediator Akt but not ERK MAPK. Pharmacological inhibition of PI3K/Akt signaling reversed the anti-apoptotic effects of NRG4, confirming the role of this cascade in NRG4-induced cell survival. With regard to the potential clinical importance of this pathway, NRG4 expression was decreased in human inflammatory bowel disease samples and mouse models of colitis, suggesting that activation of ErbB4 is altered in disease. Thus, exogenous NRG4 may be beneficial for disorders in which epithelial apoptosis is part of the pathology.