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ABSTRACT Introduction Laryngotracheoesophageal clefts (LTEC) are aspiration‐related lesions often assessed using swallow studies and symptoms like respiratory or feeding difficulties. However, these indicators are unreliable predictors of disease severity. Lung inflammation and injury, though significant concerns, are rarely and inconsistently evaluated or reported. As a result, the correlation between swallow study findings and actual pulmonary disease remains unclear. Methods A retrospective cohort of children with LTEC seen in our Aerodigestive Program between October 1, 2012, and September 30, 2023, who underwent videofluoroscopic swallow study (VFSS) or flexible endoscopic evaluation of swallowing (FEES) were included. Data collected included clinical history, risk factors, BAL, chest CT, impedance probe, VFSS/FEES findings, and FOIS‐P/I scores at baseline and follow‐up. Results Eight‐two subjects identified. Lung inflammation and bronchiectasis were common and correlated. Clinical factors, impedance probe, and the presence/severity of swallowing abnormalities on VFSS/FEES were not predictive of lung disease. Fifty‐seven patients underwent LTEC repair for a range of indications, only 49% based on CT and/or BAL findings. Of 57 children who had LTEC repair, only 49% were based on CT or BAL findings. LTEC repair was associated with modest improvement feeding and swallowing. Discussion Children with LTEC have a high prevalence of inflammatory lung disease and injury and undergo repair for a variety of reasons. Swallow studies detect functional swallowing issues but do not reliably identify lung disease. Despite this limitation, LTEC repair is linked to improved feeding and swallowing in some patients, though not all benefit.

Introduction: Laryngotracheoesophageal clefts (LTEC) are well-known aspiration-associated lesions. Results of swallow studies, and respiratory, feeding, and swallowing symptoms are commonly used indications for repair, despite being unclear predictors of disease. Although lung inflammation and injury are primary concerns, such findings are uncommonly and inconsistently evaluated and reported and therefore it remains unclear how well swallow studies predict lung disease. Methods: Children seen in our Aerodigestive program 10/1/2012 and 9/30/2023, with a LTEC and a videofluoroscopic swallow study (VFSS) or flexible endoscopic evaluation of swallowing (FEES) were included. Data regarding clinical history and risk factors, BAL, chest CT, and impedance probe results were abstracted, along with VFSS/FEES results and functional oral intake scale (FOIS-P/I) at baseline and follow-up. Results: 82 subjects identified. Lung inflammation and bronchiectasis were common and correlated. Clinical factors, impedance probe, and the presence/severity of swallowing abnormalities on VFSS/FEES were not predictive of lung disease. 57 patients underwent LTEC repair for a range of indications, only 49% based on CT and/or BAL findings. LTEC repair was associated with a trend towards improvement feeding and swallowing, yet none of these met statistical significance. Discussion: Children with LTEC have a high prevalence of inflammatory lung disease and injury and undergo repair for a variety of reasons. The insufficient ability of swallow study to identify patients with lung disease suggests that such studies identify functional abnormalities but not a disease state, per se. Nevertheless, LTEC repair is associated with improvements in feeding and swallowing in a subset of patients.

AbstractObjectivesEosinophilic esophagitis (EoE) is often diagnosed in school‐age children between 6‐ and 9‐year‐old. There is less known about those who are diagnosed with EoE that are younger than 6 years old. The objective of this study is to compare clinical presentation, comorbidities, and outcomes based on age at diagnosis of EoE.MethodsSingle‐center retrospective chart review of children (<18 years) diagnosed with EoE between 2005 and 2020. We recorded demographics, clinical presentation, family history, past medical history, treatment, and endoscopic findings. Children in this cohort were classified based on age into three age groups: <2 years, 2−<6 years, and 6−<18 years.ResultsWe identified 256 children with EoE, the mean age (SD) at the time of diagnosis was 9 (5.2) years and 184 (72%) were male. We had 164 (64%) patients with available follow‐up esophagogastroduodenoscopies (EGDs) data (495 EGDs in total) of those 99/164 (60%) reached mucosal remission. In the very young children (<2 years) vomiting was the most common presentation, while poor weight gain was seen more in the 2−<6‐year group in comparison to the >6‐years. Food impaction and abdominal pain were most likely to present in older children 6−18 years. Combination therapy, as opposed to a single therapy, induced remission at a higher frequency in the <6‐year group in comparison to the 6−<18‐year group (85% vs. 66%).ConclusionEoE should be considered in younger children presenting with feeding difficulty and poor weight gain. Combination therapy seems to be more effective in younger children with EoE, but further studies with bigger sample size are needed to study the efficacy of the different combination therapies.

AbstractObjectivesEosinophil‐derived neurotoxin (EDN) is a viable marker of eosinophilic esophagitis (EoE) disease activity. We studied the utility of measuring EDN from esophageal epithelial brushings for diagnosing EoE, focusing on two scenarios: (1) cases of exclusive distal eosinophilia and (2) cases of discrepancy between endoscopy and histology.MethodsRecords of patients who underwent esophagogastroduodenoscopy (EGD) with EDN measured via esophageal brushings at Arnold Palmer Hospital for Children in Orlando, Florida from January 2014 to October 2018 were retrospectively reviewed. Demographics, clinical, endoscopic, and histologic data were collected.ResultsWe reviewed 231 patient records (66.7% male, mean age 10.3 years, range 1–22 years). EDN values correlated with endoscopic reference score (EREFS) and peak eosinophil count (PEC) (Spearman's rho = 0.756 (p < 0.001) and 0.824 (p < 0.001) respectively). Average PEC, EREFS, and EDN concentrations were higher in patients with active EoE than in controls or patients with EoE in remission (inactive). When grouping patients based on esophageal eosinophilia distribution, EDN mirrored PEC, and EREFS. Patients with exclusive distal eosinophilia had lower EDN concentrations than those with eosinophilia in >1 level of the esophagus (23.8 ± 46.1 mcg/mL vs. 171.3 ± 205.8 mcg/mL respectively, p < 0.001). EDN values were more consistent with EREFS in cases of discrepancies between endoscopic findings and pathology (p < 0.001).ConclusionEDN measured in esophageal brushing samples reflects disease activity objectively and accurately. It also offers significant value in cases of exclusive distal esophageal eosinophilia and when discrepancies exist between endoscopy and histology.

Objectives:Patients with inflammatory bowel disease (IBD) frequently have extraintestinal manifestations. The goal of this pilot study was to assess exocrine pancreatic function in cases with suspicion for or an established diagnosis of IBD.Methods:Direct stimulated endoscopic pancreatic function test (ePFT) was performed in 74 children with IBD, in both newly diagnosed and established cases. Demographic, clinical, and laboratory parameters were entered into a database and analyzed.Results:Among the 74 children, 49 were newly diagnosed and 25 had an established diagnosis of IBD. A majority had the diagnosis of Crohn disease (CD) (n = 48; 32 new and 16 established cases) with male predominance (64.6%). Altogether, 42 (56.7%) children had either generalized or partial exocrine pancreatic insufficiency (EPI). Twenty‐four of the 48 CD children (50%) had abnormal ePFT. In those with ulcerative colitis (UC), 18 of the 26 (62.9%) had abnormal ePFT. The highest abnormality rate was in lipase enzyme activity. Weight z scores were significantly lower in those with abnormal ePFT (Crohn cases: P = 0.008; UC cases: P = 0.046). Peak protein concentration in collected pancreatic fluid was significantly lower in children with CD who had abnormal ePFT (P = 0.013).Conclusions:This pilot study revealed a relatively high prevalence of EPI in children with IBD through use of ePFT. EPI can result in maldigestion, with decreased capacity to digest fat. Further prospective studies are needed to assess need and efficacy of pancreatic enzyme replacement therapy in children with IBD and abnormal ePFT.

Objectives:Coffee and caffeinated products have been widely consumed for many centuries. Previous adult studies have suggested that both coffee and decaffeinated beverages induce colonic motility. However, no study has been conducted in pediatrics, and the role of caffeine alone in pediatric colonic motility needs to be explored.Methods:A prospective study of pediatric patients undergoing standard colonic motility testing that were able to consume caffeinated coffee, decaffeinated coffee, and caffeine tablet during colonic manometry. Patients who had a gastrocolonic reflex and high amplitude propagated contractions (HAPCs) in response to intraluminal administration of bisacodyl in the colon were included in the final analyses.Results:Thirty‐eight patients were recruited, 22 of which were excluded, 11 due to abnormal studies (no HAPC seen in response to intraluminal response to bisacodyl), and 11 due to inability to consume all study agents or complete the study. Sixteen patients met criteria for final analyses. Intracolonic bisacodyl produced a larger area under the curve (AUC) compared to all other agents. Caffeinated coffee resulted in a higher AUC, motility index (MI), and time to HAPC compared with decaffeinated coffee (P < 0.05). There was no significant difference between caffeinated coffee and caffeine tablet, or caffeine tablet and decaffeinated coffee.Conclusions:Caffeine is indeed a colonic stimulant; however, other components of caffeinated and non‐caffeinated beverages likely induce colonic response and require further evaluation for possible use as a colonic stimulant.

ABSTRACTObjectives:To evaluate the efficacy of transanal irrigation (TAI) in pediatric patients with neurogenic bowel dysfunction (NBD) who were treatment naïve to catheter‐based TAI using Peristeen device (Coloplast).Methods:Prospective recruitment of patients with NBD who were unsatisfied with their bowel regimen or had no bowel regimen in place, were assessed using the neurogenic bowel dysfunction score (NBDS) before initiating treatment (Time 0) with Peristeen. NBDS scores were reassessed twice: within the first 6 months (Time 1) of initiation of Peristeen and again after greater than 6 months of usage with Peristeen (Time 2).Results:Over a 26‐month period, 104 patients with NBD were enrolled. Mean age was 10.6 years ± 4.7 (range 3–18 years). The NBDS at Time 1 had an average reduction of 14 points from the original score. A similar trajectory was seen at Time 2, with an average reduction of 13 points from original score. There was a statistically significant decrease of 14 points, P < 0.001 at Time 1 and this response was sustained at Time 2 with a statistically significant decrease in scores from initiation by 13 points, P < 0.001. Improved patient satisfaction and quality of life with Peristeen was seen at Time 1 and Time 2.Conclusion:Our results suggest that Peristeen can improve quality of life in pediatric patients with NBD. Significant improvement in NBDS occurred in our pediatric patients with NBD when initiated on Peristeen. Lower scores were seen at both Time 1 and Time 2, which indicated an improvement in their overall NBD.

ABSTRACTObjectives:There are no tests or patient factors to help predict the best treatment approach for a patient with eosinophilic esophagitis (EoE). The prevalence of proton‐pump inhibitor (PPI) responsive EoE in children ranges from 30% to 71% with multiple studies showing similar characteristics in responders and nonresponders. Eosinophil‐derived neurotoxin (EDN), an eosinophilic granule protein, measured in esophageal brushing has been shown to be a viable measure of disease activity in EoE. Our aim is to determine if EDN can help predict response to PPI in pediatric patients with EoE.Methods:We conducted a prospective cross‐sectional study to compare EDN between PPI‐responsive and PPI‐nonresponsive EoE subjects from 2018 through 2020. Enrolled patients with active EoE were treated with high‐dose PPI and underwent repeat endoscopy to determine PPI‐responsiveness. EDN was measured at baseline endoscopy, before any treatment, and at follow up endoscopy, after PPI therapy. Subjects were divided into PPI‐responsive and nonresponsive groups. EDN, endoscopic reference score (EREFS), and peak eosinophilic count (PEC) were compared.Results:Fifteen out of the 36 enrolled subjects with EoE (age range 2–18 years, 73.3% male) were PPI‐responsive and 21 (age range 2–19 years, 95.2% male) were PPI‐nonresponsive. EDN concentration was significantly higher in the PPI‐nonresponsive group than in the PPI‐responsive group (219.1 ± 229 mcg/mL vs 75.7 ± 60 mcg/mL, respectively, P = 0.036). There was no difference between the two groups in EREFS (P = 0.55) or PEC (P = 0.15).Conclusions:EDN measured in esophageal epithelial samples obtained by brushing during endoscopy may predict PPI‐responsiveness in children and young adults with EoE.