A Child with an Esophageal Web Treated with Functional Lumen Imaging Probe.

A 14-month-old male presented to the emergency department with a 4-day history of vomiting after the intake of liquids or solids. During the admission, imaging studies revealed an esophageal web, a form of congenital esophageal stenosis. He was treated with a combination of Endoluminal Functional Lumen Imaging Probe (EndoFLIP) and controlled radial expansion (CRE) balloon dilation, followed by EndoFLIP and EsoFLIP dilation 1 month later. The patient's vomiting resolved after treatment, and he was able to gain weight. This report describes one of the first cases of applying EndoFLIP and EsoFLIP to treat an esophageal web in a pediatric patient.

Esophageal dilation with EsoFLIP is faster than CRE balloon dilation combined with EndoFLIP in children.

BACKGROUND: Controlled radial expansion (CRE) balloon dilators are traditionally used to dilate esophageal strictures during an esophagogastroduodenoscopy (EGD). EndoFLIP is a diagnostic tool used during an EGD to measure important parameters of the gastrointestinal lumen, capable of assessing treatment before and after dilation. EsoFLIP is a related device that combines a balloon dilator with high-resolution impedance planimetry to provide some of the luminal parameters in real time during dilation. We sought to compare procedure time, fluoroscopy time, and safety profile of esophageal dilation using either CRE balloon dilation combined with EndoFLIP (E + CRE) versus EsoFLIP alone. METHODS: A single-center retrospective review was performed to identify patients ≤ 21 years of age who underwent an EGD with biopsy and esophageal stricture dilation using E + CRE or EsoFLIP between October 2017 and May 2022. RESULTS: Twenty-nine EGDs with esophageal stricture dilation were performed in 23 patients (19 E + CRE and 10 EsoFLIP). The two groups did not differ in age, gender, race, chief complaint, type of esophageal stricture, or history of prior gastrointestinal procedures (all p > 0.05). The most common medical history in the E + CRE and EsoFLIP groups were eosinophilic esophagitis and epidermolysis bullosa, respectively. Median procedures times were shorter in the EsoFLIP cohort compared to E + CRE balloon dilation (40.5 min [IQR 23-57 min] for the EsoFLIP group; 64 min [IQR 51-77 min] for the E + CRE group; p < 0.01). Median fluoroscopy times were also shorter for patients who underwent EsoFLIP (0.16 min [IQR 0-0.30 min] for EsoFLIP dilation; 0.30 min [IQR 0.23-0.55] for the E + CRE group; p = 0.003). There were no complications or unplanned hospitalizations in either group. CONCLUSION: EsoFLIP dilation of esophageal strictures was faster and required less fluoroscopy than CRE balloon dilation combined with EndoFLIP in children, while being equally as safe. Prospective studies are needed to further compare the two modalities.

Endoluminal Functional Lumen Imaging Probe Is Safe in Children Under Five Years Old.

BACKGROUND: Endoluminal functional lumen imaging probe (EndoFLIP) is a minimally invasive, novel device that uses high-resolution impedance planimetry to measure important parameters of the gastrointestinal lumen that aid in the diagnosis of esophageal disorders. EndoFLiP is approved by the US Food and Drug Administration (FDA) for children 5 years and older. We sought to compare its safety and luminal characteristics between children under 5 years of age with children 5 years and older. METHODS: A single-center retrospective review was performed to identify all patients < 21 years of age who underwent esophagogastroduodenoscopy (EGD) with EndoFLIP between October 2017 and November 2020. Results: Sixty-seven EGDs with EndoFLIP were performed in 56 patients, including 14 that were done in children < 5 years and 53 in children ≥5years. The median age in the < 5-year group was 1.7years (interquartile range [IQR], 0.9-4.4) and the youngest patient was 1 month old. The median age in ≥5-year group was 14.3 years (IQR, 8.2-16.2). Median procedure times were similar (32 minutes [IQR, 25-48] for the < 5-year group; 28 minutes [IQR, 20-33] for ≥5-year group; P  = 0.08). There were no adverse events or unplanned hospitalizations in either group. At 30 mL inflation, the pressure of the lower esophageal sphincter (LES) was statistically different between the 2 groups (P = 0.02). CONCLUSION: EndoFLIP appears to be safe for use in our small cohort of children < 5 years of age and may be an important tool in the management of esophageal disorders in this age group. There was no difference in procedure time between our 2 age groups and there were no procedure-related complications.

Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis.

Based on systematic review and meta-analysis, the risk for developing cancers in patients with cystic fibrosis (CF) is known to be significantly greater than in the general population, including site-specific cancers of the esophagus, small bowel, colon, liver, biliary tract, and pancreas. An even higher risk has been found in patients who have severe CF transmembrane conductance regulator (CFTR) genotypes or who have undergone organ transplantation and are immunosuppressed. The risk continues to rise as life expectancies steadily climb due to advancements in medical care and treatment for CF. The colorectal cancer risk is at such a high level that CF has now been declared a hereditary colon cancer syndrome by the Cystic Fibrosis Foundation. The CFTR gene has been strongly-associated with the development of gastrointestinal (GI) cancers and mortality in the CF population. Even CF carriers have shown an increased rate of GI cancers compared to the general population. Several limitations exist with the reported guidelines for screening of GI and hepatopancreatobiliary cancers in the CF population, which are largely universal and are still emerging. There is a need for more precise screening based on specific risk factors, including CFTR mutation, medical co-morbidities (such as gastroesophageal reflux disease, distal intestinal obstruction syndrome, and diabetes mellitus), familial risks for each cancer, gender, age, and other factors. In this review, we propose changes to the guidelines for GI screening of patients with CF. With the development of CFTR modulators, additional studies are necessary to elucidate if there is an effect on cancer risk.

A Child with COVID-19 and Immunoglobulin A Vasculitis.

We describe the case of a child with progressive abdominal pain and rash admitted to a large tertiary children's hospital in the Chicago metropolitan area and subsequently found to have immunoglobulin A (IgA) vasculitis and coronavirus disease 2019 (COVID-19). This patient presented with abdominal pain, purpuric lesions, hematochezia, increasingly elevated D-dimer, and abnormal inflammatory markers on laboratory evaluation. To the best of our knowledge, this article describes the only reported pediatric case of COVID-19 associated with IgA vasculitis. [Pediatr Ann. 2021;50(1):e44-e48.].