Esophageal Distensibility Defines Fibrostenotic Severity in Pediatric Eosinophilic Esophagitis.

BACKGROUND & AIMS: Identification of fibrosis in pediatric eosinophilic esophagitis (EoE) relies on symptom assessment and endoscopy. Symptoms are highly variable, and early fibrotic remodeling may go undetected on endoscopy yet contribute to esophageal dysfunction. We aimed to assess whether esophageal distensibility has utility in defining fibrostenotic severity in a cohort of pediatric patients with EoE with symptoms of esophageal dysfunction. METHODS: We analyzed a prospectively recruited a cohort of children ages 9 to 21 years undergoing upper endoscopy and Endoscopic Functional Lumen Imaging Probe (EndoFLIP) for suspected or previously diagnosed EoE. Esophageal distensibility was evaluated by the distensibility index (DI) and esophageal diameter at the distensibility plateau. The association of esophageal distensibility to clinical, endoscopic, and histologic parameters of disease severity was assessed. Receiver operating characteristic analysis was performed to determine the utility of distensibility in defining esophageal rigidity in pediatric EoE. RESULTS: We identified 59 pediatric patients with EoE undergoing endoscopy and EndoFLIP at a single pediatric tertiary referral center. DI (mm2/mmHg) was significantly lower in patients with fibrotic as compared with inflammatory features on endoscopy (median, 3.3; interquartile range, 2.3-4.4) vs median, 5.5; interquartile range, 4.1-6.0; P = .02) and showed no correlation with eosinophil count. DI <4.5 mm2/mmHg predicted grade 2 rings on endoscopy with area under the curve of 0.81 (P = .0004). DI predicted food impaction in both unadjusted and adjusted models (fully adjusted odds ratio, 1.44; 95% confidence interval, 1.02-2.14; P = .0486). CONCLUSION: Esophageal distensibility determined by EndoFLIP is a measure of fibrostenotic severity that can be used to clinically phenotype pediatric EoE. We propose parameters of DI <4.5 mm2/mmHg for defining esophageal rigidity in pediatric patients with EoE ages 9 years and older.

A Single-Food Milk Elimination Diet Is Effective for Treatment of Eosinophilic Esophagitis in Children.

BACKGROUND & AIMS: Cow's milk protein (CMP) is the most common trigger of inflammation in children and adults with eosinophilic esophagitis (EoE). We sought to assess the clinical, endoscopic, and histologic efficacy of dietary elimination of all CMP-containing foods in EoE. METHODS: We performed a prospective observational study in children with EoE treated with the 1-food elimination diet (1FED), excluding all CMP. Children and their caretakers were educated by a registered dietitian regarding dietary elimination of all CMP-containing foods, with substitutions to meet nutritional needs for optimal growth and development, and daily meal planning. Upper endoscopy with biopsies was performed after 8 to 12 weeks of treatment. The primary end point was histologic remission, defined as fewer than 15 eosinophils per high-power field. Secondary end points were symptomatic, endoscopic, and quality-of-life (QOL) improvements. RESULTS: Forty-one children (76% male; ages, 9 ± 4 years; 88% white) underwent 1FED education and post-treatment endoscopy with biopsies. Histologic remission occurred in 21 (51%) children, with a decrease in peak eosinophils per high-power field from a median of 50 (interquartile range, 35-70) to a median of 1 (interquartile range, 0-6; P < .0001). Endoscopic abnormalities improved in 24 (59%) patients, while symptoms improved in 25 (61%). Improved symptoms included chest pain, dysphagia, and pocketing/spitting out food. Parents perceived worse QOL, while children perceived improved QOL with the 1FED. CONCLUSIONS: One-food elimination of CMP-containing foods from the diet induced histologic remission in more than 50% of children with EoE and led to significant improvement in symptoms and endoscopic abnormalities. The ease of implementation and adherence supports the 1FED as first-line dietary treatment.

One-Hour Esophageal String Test: A Nonendoscopic Minimally Invasive Test That Accurately Detects Disease Activity in Eosinophilic Esophagitis.

OBJECTIVES: Eosinophilic esophagitis (EoE), a chronic food allergic disease, lacks sensitive and specific peripheral biomarkers. We hypothesized that levels of EoE-related biomarkers captured using a 1-hour minimally invasive Esophageal String Test (EST) would correlate with mucosal eosinophil counts and tissue concentrations of these same biomarkers. We aimed to determine whether a 1-hour EST accurately distinguishes active from inactive EoE or a normal esophagus. METHODS: In a prospective, multisite study, children and adults (ages 7-55 years) undergoing a clinically indicated esophagogastroduodenoscopy performed an EST with an esophageal dwell time of 1 hour. Subjects were divided into 3 groups: active EoE, inactive EoE, and normal esophageal mucosa. Eosinophil-associated protein levels were compared between EST effluents and esophageal biopsy extracts. Statistical modeling was performed to select biomarkers that best correlated with and predicted eosinophilic inflammation. RESULTS: One hundred thirty-four subjects (74 children, 60 adults) with active EoE (n = 62), inactive EoE (n = 37), and patient controls with a normal esophagus (n = 35) completed the study. EST-captured eosinophil-associated biomarkers correlated significantly with peak eosinophils/high-power field, endoscopic visual scoring, and the same proteins extracted from mucosal biopsies. Statistical modeling, using combined eotaxin-3 and major basic protein-1 concentrations, led to the development of EoE scores that distinguished subjects with active EoE from inactive EoE or normal esophagi. Eighty-seven percent of children, 95% of parents, and 92% of adults preferred the EST over endoscopy if it provided similar information. DISCUSSION: The 1-hour EST accurately distinguishes active from inactive EoE in children and adults and may facilitate monitoring of disease activity in a safe and minimally invasive fashion.