Impact of Trainee Involvement on Pediatric ERCP Procedures: Results From the Pediatric ERCP Initiative.

This study examines the role of trainee involvement with pediatric endoscopic retrograde cholangiopancreatography (ERCP) and whether it affects the procedure's success, post-procedural adverse outcomes, and duration. A secondary analysis of the Pediatric ERCP Database Initiative, an international database, was performed. Consecutive ERCPs on children <19 years of age from 18 centers were entered prospectively into the database. In total 1124 ERCPs were entered into the database, of which 320 (28%) were performed by trainees. The results showed that the presence of trainees did not impact technical success ( P = 0.65) or adverse events rates ( P = 0.43). Rates of post-ERCP pancreatitis, pain, and bleeding were similar between groups ( P > 0.05). Fewer cases involving trainees were in the top quartile (>58 minutes) of procedural time (19% vs 26%; P = 0.02). Overall, our findings indicate trainee involvement in pediatric ERCP is safe.

Pediatric ERCP in the Setting of Acute Pancreatitis: A Secondary Analysis of an International Multicenter Cohort Study.

Previous studies have demonstrated the safety of performing endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population; however, few have addressed the outcomes of children undergoing ERCP during acute pancreatitis (AP). We hypothesize that ERCP performed in the setting of AP can be executed with similar technical success and adverse event profiles to those in pediatric patients without pancreatitis. Using the Pediatric ERCP Database Initiative, a multi-national and multi-institutional prospectively collected dataset, we analyzed 1124 ERCPs. One hundred and ninety-four (17%) of these procedures were performed in the setting of AP. There were no difference in the procedure success rate, procedure time, cannulation time, fluoroscopy time, or American Society of Anesthesiology class despite patients with AP having higher American Society of Gastrointestinal Endoscopy grading difficulty scores. This study suggests that ERCP can be safely and efficiently performed in pediatric patients with AP when appropriately indicated.

Intestinal failure-associated liver disease in the neonatal ICU: what we know and where we're going.

PURPOSE OF REVIEW: Parenteral nutrition is an integral part of the care of infants in the neonatal ICU. However, prolonged use of parenteral nutrition can be associated with adverse outcomes, most notably parenteral nutrition-associated liver disease, now known as intestinal failure-associated liver disease (IFALD). This review highlights pertinent developments in the epidemiology of IFALD as it pertains to neonates and showcases recent advances in the pathophysiology, treatment, and outcomes of neonates with IFALD. RECENT FINDINGS: The role of intravenous lipid emulsions in the pathogenesis, prevention, and treatment of IFALD remains a target for investigative studies. Recent data continues to support the use of fish-oil based intravenous lipids, but its use is limited due to concerns for essential fatty acid deficiency. Use of soy-based lipids and mixed lipids is not wrought with such concerns as these are often used at greater doses but their use is limited due to higher proinflammatory fatty acid content, increased phytosterols and decreased antioxidants, risk factors for the development of IFALD. SUMMARY: Hepatic complications may limit the use of parenteral nutrition in the neonatal ICU. However, the pathophysiology of IFALD is continuing to be further elucidated and novel targets are being developed for the treatment of IFALD. As noninvasive disease monitoring strategies continue to be developed, early enteral nutrition ameliorates the risk of IFALD and should be considered when possible.

Predictors of Prolonged Fluoroscopy Exposure in Pediatric Endoscopic Retrograde Cholangiopancreatography: Results From the Large Pediatric Endoscopic Retrograde Cholangiopancreatography Database Initiative Multicenter Cohort.

BACKGROUND AND AIMS: Ionizing radiation exposure during endoscopic retrograde cholangiopancreatography (ERCP) is an important quality issue especially in children. We aim to identify factors associated with extended fluoroscopy time (FT) in children undergoing ERCP. METHODS: ERCP on children <18 years from 15 centers were entered prospectively into a REDCap database from May 2014 until May 2018. Data were retrospectively evaluated for outcome and quality measures. A univariate and step-wise linear regression analysis was performed to identify factors associated with increased FT. RESULTS: 1073 ERCPs performed in 816 unique patients met inclusion criteria. Median age was 12.2 years (interquartile range [IQR] 9.3-15.8). 767 (71%) patients had native papillae. The median FT was 120 seconds (IQR 60-240). Factors associated with increased FT included procedures performed on patients with chronic pancreatitis, ERCPs with American Society of Gastrointestinal Endoscopy (ASGE) difficulty grade >3, ERCPs performed by pediatric gastroenterologist (GI) with adult GI supervision, and ERCPs performed at non-free standing children's hospitals. Hispanic ethnicity was the only factor associated with lower FT. CONCLUSION: Several factors were associated with prolonged FTs in pediatric ERCP that differed from adult studies. This underscores that adult quality indicators cannot always be translated to pediatric patients. This data can better identify children with higher risk for radiation exposure and improve quality outcomes during pediatric ERCP.

Technical Outcomes in Pediatric Endoscopic Retrograde Cholangiopancreatography: Data from an International Collaborative.

OBJECTIVES: Endoscopic retrograde cholangiopancreatography (ERCP) in adults has been extensively studied through multicenter prospective studies. Similar pediatric studies are lacking. The Pediatric ERCP Database Initiative (PEDI) is a multicenter collaborative aiming to evaluate the indications and technical outcomes in pediatric ERCPs. METHODS: In this prospective cohort study, data were recorded for pediatric ERCPs performed across 15 centers. A pre-procedure, procedure, 2-week post-procedure follow-up, and adverse event form were completed for each ERCP performed. Univariate and stepwise linear regression was performed to identify factors associated with technically successful procedures and adverse events. RESULTS: A total of 1124 ERCPs were performed on 857 patients from May 1, 2014 to May 1, 2018. The median age was 13.5 years [interquartile range (IQR) 9.0-15.7]. Procedures were technically successful in the majority of cases (90.5%) with success more commonly encountered for procedures with biliary indications [odds ratio (OR) 4.2] and less commonly encountered for native papilla anatomy (OR 0.4) and in children <3 years (OR 0.3). Cannulation was more often successful with biliary cannulation (95.9%) compared to pancreatic cannulation via the major papilla (89.6%, P < 0.0001) or minor papilla (71.2%, P < 0.0005). The most commonly identified adverse events included post-ERCP pancreatitis (5%), pain not related to post-ERCP pancreatitis (1.8%), and bleeding (1.2%). Risk factors for the development of each were identified. CONCLUSIONS: This large prospective study demonstrates that ERCP is reliable and safe in the pediatric population. It highlights the utility of PEDI in evaluating the technical outcomes of pediatric ERCPs and demonstrates the potential of PEDI for future studies in pediatric ERCPs.

Menstruation-Associated Acute Pancreatitis in Patients with Hereditary Pancreatitis.

Two adolescent girls presented with recurrent episodes of menstrual cycle-associated acute pancreatitis and are diagnosed with hereditary pancreatitis, a cause of chronic pancreatitis. Hereditary pancreatitis should remain in the differential diagnosis for teenage girls with catamenial acute pancreatitis and family history of pancreatic disease.

New Insights Into Intestinal Failure-Associated Liver Disease in Children.

Development of intestinal failure-associated liver disease (IFALD) is a common complication of long-term parenteral nutrition (PN) in children and adults. The molecular and cellular mechanisms and the phases of IFALD are now being delineated. Components of PN lipid emulsions, including plant sterols, interact with hepatic innate immune activation promoted by products of gut bacterial overgrowth/dysbiosis and altered intestinal barrier function (gut-liver axis) and by episodes of sepsis to cause cholestasis and IFALD. New therapeutic strategies, including modifications of intravenous lipid emulsions to reduce pro-inflammatory fatty acids and plant sterol content, can lower the risk of IFALD, reverse cholestasis, and reduce complications, although the significance of persisting hepatic fibrosis is unknown. This review will provide an update on advances in the pathogenesis of IFALD, newer therapeutic and preventative strategies, and challenges that confront managing patients with IFALD.

A prospective multicenter analysis from the Pediatric ERCP Database Initiative: predictors of choledocholithiasis at ERCP in pediatric patients.

BACKGROUND AND AIMS: The management of suspected choledocholithiasis remains a challenge in pediatric endoscopy. Several recommendations are available for adult patients; however, it is unknown which pediatric patients are most likely to benefit from ERCP for evaluation of choledocholithiasis. The primary aim of this study was to evaluate adult-based criteria in the evaluation of pediatric patients with choledocholithiasis. A secondary aim was to evaluate the role of conjugated (or direct) bilirubin to improve the sensitivity of detecting choledocholithiasis. METHODS: This was a prospective multicenter study in pediatric patients as part of the Pediatric ERCP Database Initiative (PEDI) with additional post-hoc analysis of updated guidelines. Patients <19 years of age undergoing ERCP for suspected choledocholithiasis or gallstone pancreatitis were enrolled at participating sites. RESULTS: Ninety-five patients were enrolled (69 with choledocholithiasis confirmed at ERCP and 26 with no stones at ERCP). Adverse event rates were similar in both groups. Specificity ranged from 27% to 91% using adult guidelines, but a sensitivity of only 20% to 69%. The were no significant differences between the 2 groups using preprocedure transabdominal US (P = 1.0). Significant differences between groups were identified using either the total or conjugated bilirubin (P = .02). There was also a significant difference between the stone and no-stone groups when conjugated bilirubin was dichotomized to >2 mg/dL (P = .03). CONCLUSIONS: Abdominal imaging and laboratory indices may be used to predict pediatric choledocholithiasis with varying sensitivity and specificity. Pediatric-specific guidelines may allow for improved stone prediction compared with existing adult recommendations.

Influence of Acid Blockade on the Aerodigestive Tract Microbiome in Children With Cystic Fibrosis.

BACKGROUND: Acid blockade is commonly prescribed in patients with cystic fibrosis (CF). Growing concerns, however, exist about its possible role in the pathophysiology of pulmonary infections. We aimed to investigate if acid blockade alters esophageal and respiratory microbiota leading to dysbiosis and inflammation. METHODS: We performed a cross sectional study of children with CF who were either prescribed acid blockade or not. Samples from the gastrointestinal and respiratory tracts were obtained and microbiome analyzed. Mixed effect models were used to compare outcomes between cohorts and across sampling sites. A random subject intercept was included to account for the multiple sampling sites per individual. RESULTS: A cohort of 25 individuals, 44% girls with median age of 13.8 years [IQR 11.2--14.8] were enrolled. Alpha diversity, total bacterial load, and beta diversity were similar across anatomic compartments, across the upper gastrointestinal tract, and in respiratory samples. Similar alpha diversity, total bacterial load, and beta diversity results were also observed when comparing individuals on versus those off acid blockade. IL-8 was elevated in the distal versus proximal esophagus in the whole cohort (P < 0.01). IL-8 concentrations were similar in the distal esophagus in patients on and off acid blockade, but significantly greater in the proximal esophagus of subjects on treatment (P < 0.01). CONCLUSIONS: On the basis of these data, acid blockade use does not appear to influence the microbiome of the aerodigestive tract in children with cystic fibrosis suggesting a complex interplay between these medications and the bacterial composition of the esophagus and lung.

Medical Management of Chronic Pancreatitis in Children: A Position Paper by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Pancreas Committee.

ABSTRACT: This position paper summarizes the current understanding of the medical management of chronic pancreatitis (CP) in children in light of the existing medical literature, incorporating recent advances in understanding of nutrition, pain, lifestyle considerations, and sequelae of CP. This article complements and is intended to integrate with parallel position papers on endoscopic and surgical aspects of CP in children. Concepts and controversies related to pancreatic enzyme replacement therapy (PERT), the use of antioxidants and other CP medical therapies are also reviewed. Highlights include inclusion of tools for medical decision-making for PERT, CP-related diabetes, and multimodal pain management (including an analgesia ladder). Gaps in our understanding of CP in children and avenues for further investigations are also reviewed.

Magnetic resonance imaging findings following button battery ingestion.

BACKGROUND: Lithium button battery ingestions have been increasing in frequency since the early 2000s and can develop severe and sometimes fatal complications from caustic injury even after rapid battery removal. To aid in clinical decision-making, we began obtaining magnetic resonance imaging (MRI)/MR angiography in these patients. OBJECTIVE: Our goal was to review MRI/MR angiography imaging in button battery ingestion cases and compare with other imaging, clinical data and outcomes in these patients. MATERIALS AND METHODS: In this retrospective institutional-review-board-approved study, we reviewed all button battery ingestion cases with MRI from April 2012 to September 2018. Clinical data, endoscopic findings and all imaging studies were rereviewed. MRIs were evaluated for inflammation, blooming artifact and complications including vascular injury, tracheoesophageal fistula, esophageal perforation and spondylodiscitis, and compared to endoscopy, esophagram and bronchoscopy. RESULTS: Twenty-three patients with button battery ingestions had a total of 51 MRI/MR angiograms. Seventy percent of the cohort was male with a median age of 2 years (range: 0.94-17 years). Severe complications were found in 48% of patients (11/23), including esophageal perforation (n=11), tracheoesophageal fistula (n=3) and spondylodiscitis (n=1). No patients had vascular injury. Cervical location of the battery was significantly associated with severe complications (10/11 cases). The length of the blooming artifact was greater than 2 cm in those with severe complications and, in most cases, <2 cm in those without severe complications. All complications were seen on initial screening MR exam with serial exams showing decreased inflammation. CONCLUSION: MRI/MR angiography can provide valuable information about complications, including esophageal perforation, tracheoesophageal fistula and spondylodiscitis. Decreasing inflammation surrounding the esophagus and vasculature is believed to be clinically reassuring and aids in managing button battery ingestion patients.

Gastric injury secondary to button battery ingestions: a retrospective multicenter review.

BACKGROUND AND AIMS: Removal of gastric button batteries (BBs) remains controversial. Our aim was to better define the spectrum of injury and to characterize clinical factors associated with injury from retained gastric BBs. METHODS: In this multicenter retrospective cohort study from January 2014 through May 2018, pediatric gastroenterologists from 4 pediatric tertiary care centers identified patients, aged 0 to 18 years, who had a retained gastric BB on radiography and subsequently underwent endoscopic assessment. Demographic and clinical information were abstracted from electronic health records using a standard data collection form. RESULTS: Sixty-eight patients with a median age of 2.5 years underwent endoscopic retrieval of a gastric BB. At presentation, 17 (25%) were symptomatic. Duration from ingestion to endoscopic removal was known for 65 patients (median, 9 hours [interquartile range, 5-19]). Median time from ingestion to first radiographic evaluation was 2 hours. At endoscopic removal, 60% of cases had visual evidence of mucosal damage, which correlated with duration of BB retention (P = .0018). Time to retrieval of the BB was not statistically significant between symptomatic and asymptomatic subjects (P = .12). After adjusting for age and symptoms, the likelihood of visualizing gastric damage among patients who had BBs removed 12 hours post ingestion was 4.5 times that compared with those with BB removal within 12 hours of ingestion. CONCLUSIONS: In this study, swallowed BBs posed a risk of damage to the stomach, including a single case of impaction and perforation of the gastric wall. Clinicians may want to consider retrieval within 12 hours of ingestion of gastric BBs. Larger prospective studies to assess risk of injury are needed.

Button Battery Powered Fidget Spinners: A Potentially Deadly New Ingestion Hazard for Children.

Toys entering the marketplace may have unrecognized hazard risks until data on injury become known. The fidget spinner is a new popular toy mass marketed to children and is primarily sold without warning labels. The US Consumer Product Safety Commission has recently issued a formal statement on potential safety concerns related to ingestion of the toy parts and other hazards. Button batteries within this toy pose the greatest danger to children as ingestion can lead to lethal injury. We report 2 cases of children who swallowed a button battery from a fidget spinner, causing severe esophageal injury. Various aspects of this type of ingestion important for clinicians to be aware of are reviewed.

Literature Review and a Relevant Case of Pediatric Collagenous Gastritis: A Rare but Important Etiology of Iron-Deficiency Anemia.

An adolescent male with fatigue, weight loss, and iron-deficiency anemia failed to improve with iron supplementation and a gluten-free diet. Endoscopy revealed collagenous gastritis. Pediatric patients with refractory iron deficiency and family history of autoimmune disorders should be referred to pediatric gastroenterology for evaluation of collagenous gastritis and celiac disease.

Optimal Donor Allograft Function: The Search for the Lowest Acceptable Donor Left Ventricular Ejection Fraction in Pediatric Heart Transplantation.

BACKGROUND: The availability of heart donors is limited by organ shortage. Due to concerns of reduced survival, donors with depressed left ventricular ejection fraction (LVEF <50%) have been cautiously used in pediatric heart transplantation. One strategy to expand the donor pool is to re-evaluate whether lower donor LVEF may be acceptable for transplantation. METHODS: We performed a multicenter retrospective cohort study of patients <18 y receiving heart transplants from April 2007 to September 2021 using the United Network of Organ Sharing dataset. We excluded retransplants and multiorgan transplants. Cut-point analyses of LVEF was performed and Kaplan-Meier method was used to compare 1-y survival for new cut-points and the standard (LVEF >50%). RESULTS: The analytic sample consisted of 5255 patients. Recipients receiving hearts with lower LVEFs were more likely to be on ventilator and extracorporeal membrane oxygenation support. Recipients did not differ in waitlist times or transplant status. Cut-point analysis identified LVEF 45% as a potentially new cutoff. One-year survival of recipients of donors with LVEF ≥45% (92.1%; 95% confidence interval [CI], 91.3%-92.8%) was similar to that of LVEF >50% (92.1%; CI, 91.4%-92.9%). Survival for the LVEF 45%-49% (88.8%; CI, 72.9%-95.7%) cohort was slightly lower than the ≥50% cohort, albeit nonsignificant. CONCLUSIONS: One-year survival among pediatric heart transplants using a donor heart LVEF threshold of 45% or 40% was similar to a threshold of 50%. However, the finding is based on a small number of patients with LVEF <50%, and future larger prospective studies are warranted to confirm the findings of this study before a lower LVEF threshold is considered.

Quality Improvement Initiative to Improve Endoscopic Reassessment of Eosinophilic Esophagitis.

The eosinophilic esophagitis (EoE) endoscopic reference score (EREFS) is a validated system for description, recognition, and reporting of EoE findings during esophagogastroduodenoscopy (EGD). This scoring system correlates with esophageal eosinophilia and therapeutic responses and has validated diagnostic accuracy with good inter- and intraobserver reliability in pediatric and adult patients. In this study, we aimed to improve physician education on and documentation of EREFS and correlate EREFS scoring with eosinophil density on histology. Methods: Applying the "Plan, Do, Study, Act" methodology for quality improvement between October 2018 and November 2019, we established a baseline rate of EREFS completion by review of the electronic medical record (EMR). Key drivers were identified, and 3 interventions were implemented. Results: Over 12 months, 542 distinct endoscopies were performed on 410 patients for EoE surveillance. Patients were 68% male with a mean age of 10.9 years (SD 5.7 years), mean EREFS score of 2.14 (SD 1.88), and mean peak eosinophil count 30.9 eos/hpf (SD 37.1 eos/hpf). Baseline EREFS completion rate of 72.7% (90% CI, 67.4-77.4). Following all 3 PDSA cycles, EREFS completion rate significantly improved to greater than desired target of 90% (94.9%; 90% CI, 90.6-97.6; P < 0.001). Conclusion: Interventions including provider education and the inclusion of EREFS in documentation templates can increase adoption rates of EREFS among providers caring for patients with known EoE.