Progressive Dysphagia and Chronic Abdominal Pain From Vascular Anomalies: A Case Report and Literature Review.

Median arcuate ligament syndrome and symptomatic aberrant right subclavian artery are uncommon in the pediatric population and are rarely found in the same individual. We present the case of a teenager with 2 rare vascular anomalies leading to chronic postprandial abdominal pain, dysphagia, and weight loss. The purpose of this case report is to raise awareness about these rare anomalies and their presentations in the pediatric population.

Clinical diagnostic predictive score for Meckel diverticulum.

BACKGROUND/PURPOSE: Meckel diverticulum (MD) is present in 2% of the population. Many practitioner feel the diagnosis relies upon technetium-99 m pertechnetate scintigraphy. When negative, patients undergo additional invasive procedures delaying definitive therapy. This study aims to identify features of bleeding MD and generate a risk score, which could preclude unnecessary testing and facilitate earlier operation. METHODS: All patients <18-years-old who presented with hematochezia from 2005 to 2015 were identified. MD diagnosis was based on histopathology of operative tissue. Controls were patients with hematochezia undergoing colonoscopy. A points system was used generate a risk score. RESULTS: A total of 215 patients presented with hematochezia out of which 42 patients with MD were identified. Predictive variables included infant (OR 7, 95%CI 2-29) and toddler (OR 20, 95%CI 8-50) age groups, duration <6 days (OR 18, 95%CI 8-43), presence of large blood volume (OR 16, 95% CI 7-36), hemoglobin <7 g/dL (OR 6, 95% CI 3-15) and transfusion requirement (OR 16, 95% CI 7-38). A score of 6 or higher is highly suggestive of MD. CONCLUSIONS: This scoring system identifies children with bleeding MD who may benefit from exploratory surgery without undergoing endoscopy. This novel scoring system can be applied to provide accurate clinical diagnosis, reduce unnecessary tests and allow prompt surgical management.

Adherence to Biopsy and Follow-Up Guidelines in a Population-Based Cohort of Children With Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated condition that is becoming more widely recognized in children. Recent EoE practice guidelines provide clear recommendations on adequate biopsy sampling at diagnostic endoscopy and necessity of close follow-up endoscopy with biopsy to ensure mucosal healing with therapy.1 Despite these recommendations, adherence to biopsy guidelines, time to first follow-up endoscopy, and overall surveillance endoscopy rates have not been robustly studied. Using a population-based cohort of children diagnosed with EoE in Utah, we assessed adherence to guidelines across multiple provider types, including academic pediatric gastroenterologists (PGIs), private practice PGIs, and adult-trained providers performing endoscopy in children.

Sensitivity and Specificity of Administrative Medical Coding for Pediatric Eosinophilic Esophagitis.

OBJECTIVES: Eosinophilic esophagitis (EoE) is a delayed-type hypersensitivity with increasing rates among pediatric populations. Although studies have used International Classification of Diseases (ICD) coding to define local cohorts and report disease epidemiology, the accuracy of the EoE ICD code for pediatric EoE is unknown. METHODS: We searched the Intermountain Healthcare Database for pediatric cases with the EoE ICD code over a 5-year period. We cross-referenced these results with a recently published pediatric EoE cohort from the same region and period, where incident cases were identified via retrospective review of pathology reports and medical records. Using the retrospective review cohort as the reference standard, we evaluated the accuracy of the EoE ICD code. RESULTS: Via retrospective review, we identified 1129 new pediatric EoE cases in the Intermountain Healthcare system over 5 years. Six hundred ten of these had the EoE ICD code associated with their chart. Out of 878,872 unique pediatric records in the Intermountain Healthcare system, 219 had the EoE ICD code incorrectly applied. The specificity of the EoE ICD code in children was 99%, but sensitivity and positive predictive value were 61% and 79%, respectively. CONCLUSIONS: The EoE ICD code has strengths and weaknesses in pediatrics. The EoE ICD code is specific, with few false positives across a large population, but not sensitive. The low sensitivity is likely multifactorial and requires further evaluation. Compared to retrospective chart review, which allows for application of clinicopathologic EoE diagnostic criteria, sole use of ICD codes results in underascertainment of EoE cases and key misclassifications.

Incidence and Prevalence of Pediatric Eosinophilic Esophagitis in Utah Based on a 5-Year Population-Based Study.

BACKGROUND & AIMS: Eosinophilic esophagitis (EoE) is often detected in children and is considered to be a rare disease, with prevalence values reported to be below 60 cases per 100,000 persons. To determine whether the incidence of EoE in children in Utah exceeds estimates from regional reports, we calculated incidence and prevalence values over a 5-year period. METHODS: Using consensus guidelines for the diagnosis of EoE, we reviewed pathology records from the Intermountain Healthcare pathology database, from July 1, 2011 through June 31, 2016. We collected data on 10,619 pediatric patients with available esophageal biopsy results, and identified cases of esophageal eosinophilia (>14 eosinophils in a high-power microscopy field in an endoscopic biopsy). An EoE case required the presence of esophageal eosinophilia, symptoms of esophageal dysfunction, and the absence of co-morbid conditions that may cause esophageal eosinophilia. Annual pediatric EoE incidence and prevalence values were calculated per 100,000 children, based on averaged pediatric population estimates from census figures of Utah in 2010 and 2016. RESULTS: We identified 1281 unique pediatric patients who met criteria for esophageal eosinophilia. Of those, 1060 patients met criteria for newly diagnosed EoE. Over the 5-year period studied, the average annual pediatric EoE incidence in Utah was 24 cases per 100,000 children. The prevalence in year 5 of the study was 118 cases per 100,000 children. CONCLUSION: In a population-based study of children in Utah, we found the incidence and prevalence of pediatric EoE to be higher than previously reported. This could be due to the prominence of EoE risk factors in this region, as well as Utah's searchable medical record system that allows for reliable case ascertainment. Further studies of this type could increase disease awareness, prompting early referral to pediatric gastroenterologists and trials to strengthen evidence-based, algorithmic approaches to EoE diagnosis and treatment in children.