Search

Background and Aims: PTEN hamartoma tumor syndrome (PHTS) confers a high risk of specific cancers and is the most common genetic cause of autism spectrum disorder (ASD). Gastrointestinal (GI) phenotypes in PHTS are poorly characterized in children. Thus, we aimed to characterize the GI and hepatic manifestations in children with PHTS and to investigate genotype-phenotype associations., Methods: We performed a retrospective chart review of prospectively accrued children with PHTS at 2 tertiary-care centers. Wilcoxon rank-sum, Chi-squared, and Fisher's exact tests and Firth's logistic regression were utilized to explore associations between variables., Results: This series included 80 children with disease-causing PTEN variants. Common GI manifestations included constipation in 41 (51%), feeding issues in 31 (39%), and polyps in 22 (28%) children. The polyps were of mixed histologic types. Eosinophilic gastrointestinal disorders were observed in 5 (6%) children. Crohn's disease, celiac disease, and protein-losing enteropathy were observed once each. Eosinophilic gastrointestinal disorders were observed exclusively in patients without ASD ( P  = .052). Nonsense PTEN variants were enriched in those with polyps ( P  = .029). Missense PTEN variants (OR 2.9, P  = .034) and upper GI polyps (OR 4.4, P  = .018) were associated with increased odds of constipation., Conclusion: Constipation and feeding issues are common in children with PHTS. Polyps are more prevalent in children with PHTS than previously described and associated with nonsense PTEN variants. Children without ASD represent a distinct patient subset with a predisposition to eosinophilic gastrointestinal disorders and possibly upper GI polyps. Endoscopic evaluation should continue to be performed in symptomatic children with PHTS, with consideration of closer follow-up in those without ASD., (© 2024 The Authors.)

Objectives: Iatrogenic viscus perforation in pediatric gastrointestinal endoscopy (GIE) is a very rare, yet potentially life-threatening event. There are no evidence-based recommendations relating to immediate post-procedure follow-up to identify perforations and allow for timely management. This study aims to characterize the presentation of children with post-GIE perforation to better rationalize post-procedure recommendations., Methods: Retrospective study based on unrestricted pooled data from centers throughout Europe, North America, and the Middle East affiliated with the Endoscopy Special Interest Groups of European Society for Paediatric Gastroenterology Hepatology and Nutrition and North American Society for Pediatric Gastroenterology Hepatology and Nutrition. Procedural and patient data relating to clinical presentation of the perforation were recorded on standardized REDCap case-report forms., Results: Fifty-nine cases of viscus perforation were recorded [median age 6 years (interquartile range 3-13)]; 29 of 59 (49%) occurred following esophagogastroduodenoscopy, 26 of 59 (44%) following ileocolonoscopy, with 2 of 59 (3%) cases each following balloon enteroscopy and endoscopic retrograde cholangiopancreatography; 28 of 59 (48%) of perforations were identified during the procedure [26/28 (93%) endoscopically, 2/28 (7%) by fluoroscopy], and a further 5 of 59 (9%) identified within 4 hours. Overall 80% of perforations were identified within 12 hours. Among perforations identified subsequent to the procedure 19 of 31 (61%) presented with pain, 16 of 31 (52%) presented with fever, and 10 of 31 (32%) presented with abdominal rigidity or dyspnea; 30 of 59 (51%) were managed surgically, 17 of 59 (29%) managed conservatively, and 9 of 59 (15%) endoscopically; 4 of 59 (7%) patients died, all following esophageal perforation., Conclusions: Iatrogenic perforation was identified immediately in over half of cases and in 80% of cases within 12 hours. This novel data can be utilized to generate guiding principles of post-procedural follow-up and monitoring., Plain Language Summary: Bowel perforation following pediatric gastrointestinal endoscopy is very rare with no evidence to base post-procedure follow-up for high-risk procedures. We found that half were identified immediately with the large majority identified within 12 hours, mostly due to pain and fever., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: To review the clinical management and outcomes of magnet ingestions at a large tertiary children's hospital. To determine the association of frequency of high-powered magnet ingestion with the regulation of these magnets., Methods: Children <18 years who presented to the emergency room and were admitted to the Children's Hospital of Philadelphia for ingestion of single or multiple magnets from January 2008 to December 2020 were included. Demographics, symptoms, management, and outcomes were analyzed. The frequency of magnet ingestion was compared over 3 eras: (1) pre-ban (2008-2012), (2) intra-ban (2013-2016), and (3) post-ban (2017-2020)., Results: There were 167 magnet ingestions, including 99 with multiple magnets. Most patients (59%) were male and median age was 6 (interquartile range, 3-9) years. Most single magnet ingestions (86%) were discharged with outpatient monitoring, and none experienced severe outcomes. Multiple magnet ingestions led to significant morbidity including hospitalizations (68%), endoscopic procedures (48%), surgical procedures (14%), and severe outcomes (12%). Most patients (75%) were asymptomatic, however, there was a higher risk of surgery and severe complications based on the presence of symptoms ( P = 0.003). The rate of surgical intervention was higher with ≥3 magnets (31.7%) compared to 2 magnets (2.4%) ( P < 0.003). Additionally, we found an 160% increase in children with magnet ingestions in the post-ban period ( P = 0.021)., Conclusions: Multiple magnet ingestion is associated with high morbidity and rate of severe outcomes. There is a relationship between public policy of magnet sale and frequency of magnet ingestion., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Competing Interests: Douglas Fishman – royalties, up-to-date; Michael Wilsey – consultant, Mead Johnson; Clifton Huang – Paid Speaker, Abbvie; Kulwinder Dua – consultant (speaker), Boston Scientific. The other authors report no conflicts of interest., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Introduction: High-quality pediatric gastrointestinal procedures are performed when clinically indicated and defined by their successful performance by skilled providers in a safe, comfortable, child-oriented, and expeditious manner. The process of pediatric endoscopy begins when a plan to perform the procedure is first made and ends when all appropriate patient follow-up has occurred. Procedure-related standards and indicators developed to date for endoscopy in adults emphasize cancer screening and are thus unsuitable for pediatric medicine., Methods: With support from the North American and European Societies of Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used the methodological strategy of the Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument to develop standards and indicators relevant for assessing the quality of endoscopic procedures. Consensus was sought via an iterative online Delphi process and finalized at an in-person conference. The quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development, and Evaluation) approach., Results: The PEnQuIN working group achieved consensus on 14 standards for pediatric endoscopic procedures, as well as 30 indicators that can be used to identify high-quality procedures. These were subcategorized into three subdomains: Preprocedural (3 standards, 7 indicators), Intraprocedural (8 standards, 18 indicators), and Postprocedural (3 standards, 5 indicators). A minimum target for the key indicator, "rate of adequate bowel preparation," was set at ≥80%., Discussion: It is recommended that all facilities and individual providers performing pediatric endoscopy worldwide initiate and engage with the procedure-related standards and indicators developed by PEnQuIN to identify gaps in quality and drive improvement., Competing Interests: Conflicts of interest: P.B. has served on the advisory boards of Biocodex, Nutricia, and Avanos. P.B. has received honoraria for speaking engagements from AbbVie, Nutricia, and Avanos. N.M.C.'s institution received speaker fees, advisory board fees, and research funding on his behalf from AbbVie, Eli Lilly, Takeda, Shire, Pfizer, and 4D Pharma. D.S.F. has received royalties from UpToDate (“Pediatric Caustic Ingestions”). I.H. has received honoraria for speaking engagements from BioGaia, Oktal Pharma, Nutricia, Abela Pharm, and Nestle. H.Q.H. has received research support from Janssen, AbbVie, Takeda, and Allergan. H.Q.H. has served on the advisory boards of AbbVie and Jansen. K.J. has received research support from Janssen, AbbVie, and the Center for Drug Research and Development (CDRD). K.J. has served on the advisory boards of Janssen, AbbVie, and Merck and participates in the speaker's bureau for AbbVie and Janssen. D.G.L. has received consultant fees from EvoEndo. J.R.L. has received research support from AbbVie and an honorarium from Mead Johnson. A.R.O. has received research support from Janssen, AbbVie, Pfizer, and Eli Lilly. A.R.O. has served on the advisory boards of Janssen, AbbVie, and Eli Lilly and participates in the speaker's bureau for AbbVie and Janssen. J.R.R. has received research support from AbbVie and Janssen. J.R.R. has served on the advisory boards of Janssen, BMS, Eli Lilly, and Pfizer. C.M.W. has received research support from AbbVie., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Introduction: There is increasing international recognition of the impact of variability in endoscopy facilities on procedural quality and outcomes. There is also growing precedent for assessing the quality of endoscopy facilities at regional and national levels by using standardized rating scales to identify opportunities for improvement., Methods: With support from the North American and European Societies of Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used the methodological strategy of the Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument to develop standards and indicators relevant for assessing the quality of facilities where endoscopic care is provided to children. Consensus was reached via an iterative online Delphi process and subsequent in-person meeting. The quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development and Evaluation) approach., Results: The PEnQuIN working group achieved consensus on 27 standards for facilities supporting pediatric endoscopy, as well 10 indicators that can be used to identify high-quality endoscopic care in children. These standards were subcategorized into three subdomains: Quality of Clinical Operations (15 standards, 5 indicators); Patient and Caregiver Experience (9 standards, 5 indicators); and Workforce (3 standards)., Discussion: The rigorous PEnQuIN process successfully yielded standards and indicators that can be used to universally guide and measure high-quality facilities for procedures around the world where endoscopy is performed in children. It also underscores the current paucity of evidence for pediatric endoscopic care processes, and the need for research into this clinical area., Competing Interests: Conflicts of interest: P.B. has served on the advisory boards of Biocodex, Nutricia, and Avanos. P.B. has received honoraria for speaking engagements from AbbVie, Nutricia, and Avanos. N.M.C.'s institution received speaker fees, advisory board fees, and research funding on his behalf from AbbVie, Eli Lilly, Takeda, Shire, Pfizer, and 4D Pharma. D.S.F. has received royalties from UpToDate (“Pediatric Caustic Ingestions”). I.H. has received honoraria for speaking engagements from BioGaia, Oktal Pharma, Nutricia, Abela Pharm, and Nestle. H.Q.H. has received research support from Janssen, AbbVie, Takeda, and Allergan. H.Q.H. has served on the advisory boards of AbbVie and Jansen. K.J. has received research support from Janssen, AbbVie, and the Center for Drug Research and Development (CDRD). K.J. has served on the advisory boards of Janssen, AbbVie, and Merck and participates in the speaker's bureau for AbbVie and Janssen. D.G.L. has received consultant fees from EvoEndo. J.R.L. has received research support from AbbVie and an honorarium from Mead Johnson. A.R.O. has received research support from Janssen, AbbVie, Pfizer, and Eli Lilly. A.R.O. has served on the advisory boards of Janssen, AbbVie, and Eli Lilly and participates in the speaker's bureau for AbbVie and Janssen. J.R.R. has received research support from AbbVie and Janssen. J.R.R. has served on the advisory boards of Janssen, BMS, Eli Lilly, and Pfizer. C.M.W. has received research support from AbbVie., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Introduction: High-quality procedure reports are a cornerstone of high-quality pediatric endoscopy as they ensure the clear communication of procedural events and outcomes, guide patient care and facilitate continuous quality improvement. The aim of this document is to outline standardized reporting elements that achieved international consensus as requirements for high-quality pediatric endoscopy procedure reports., Methods: With support from the North American and European Societies of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used Delphi methodology to identify key elements that should be found in all pediatric endoscopy reports. Item reduction was attained through iterative rounds of anonymized online voting using a 6-point scale. Responses were analyzed after each round and items were excluded from subsequent rounds if ≤50% of panelists rated them as 5 ("agree moderately") or 6 ("agree strongly"). Reporting elements that ≥70% of panelists rated as "agree moderately" or "agree strongly" were considered to have achieved consensus., Results: Twenty-six PEnQuIN group members from 25 centers internationally rated 63 potential reporting elements that were generated from a systematic literature review and the Delphi panelists. The response rates were 100% for all three survey rounds. Thirty reporting elements reached consensus as essential for inclusion within a pediatric endoscopy report., Discussion: It is recommended that the PEnQuIN Reporting Elements for pediatric endoscopy be universally employed across all endoscopists, procedures and facilities as a foundational means of ensuring high-quality endoscopy services, while facilitating quality improvement activities in pediatric endoscopy., Competing Interests: Conflicts of interest: P.B. has served on the advisory boards of Biocodex, Nutricia, and Avanos. P.B. has received honoraria for speaking engagements from AbbVie, Nutricia, and Avanos. N.M.C.'s institution received speaker fees, advisory board fees, and research funding on his behalf from AbbVie, Eli Lilly, Takeda, Shire, Pfizer, and 4D Pharma. D.S.F. has received royalties from UpToDate (“Pediatric Caustic Ingestions”). I.H. has received honoraria for speaking engagements from BioGaia, Oktal Pharma, Nutricia, Abela Pharm, and Nestle. H.Q.H. has received research support from Janssen, AbbVie, Takeda, and Allergan. H.Q.H. has served on the advisory boards of AbbVie and Jansen. K.J. has received research support from Janssen, AbbVie, and the Center for Drug Research and Development (CDRD). K.J. has served on the advisory boards of Janssen, AbbVie, and Merck and participates in the speaker's bureau for AbbVie and Janssen. D.G.L. has received consultant fees from EvoEndo. J.R.L. has received research support from AbbVie and an honorarium from Mead Johnson. A.R.O. has received research support from Janssen, AbbVie, Pfizer, and Eli Lilly. A.R.O. has served on the advisory boards of Janssen, AbbVie, and Eli Lilly and participates in the speaker's bureau for AbbVie and Janssen. J.R.R. has received research support from AbbVie and Janssen. J.R.R. has served on the advisory boards of Janssen, BMS, Eli Lilly, and Pfizer. C.M.W. has received research support from AbbVie., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Introduction: High-quality pediatric endoscopy requires reliable performance of procedures by competent individual providers who consistently uphold all standards determined to assure optimal patient outcomes. Establishing consensus expectations for ongoing monitoring and assessment of individual pediatric endoscopists is a method for confirming the highest possible quality of care for such procedures worldwide. We aim to provide guidance to define and measure quality of endoscopic care for children., Methods: With support from the North American and European Societies of Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used the methodological strategy of the Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument to develop standards and indicators relevant for assessing the quality of endoscopists. Consensus was sought via an iterative online Delphi process and finalized at an in-person conference. The quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development, and Evaluation) approach., Results: The PEnQuIN working group achieved consensus on 6 standards that all providers who perform pediatric endoscopy should uphold and 2 standards for pediatric endoscopists in training, with 7 corresponding indicators that can be used to identify high-quality endoscopists. Additionally, these can inform continuous quality improvement at the provider level. Minimum targets for defining high-quality pediatric ileocolonoscopy were set for 2 key indicators: cecal intubation rate (≥90%) and terminal ileal intubation rate (≥85%)., Discussion: It is recommended that all individual providers performing or training to perform pediatric endoscopy initiate and engage with these international endoscopist-related standards and indicators developed by PEnQuIN., Competing Interests: Conflicts of interest: P.B. has served on the advisory boards of Biocodex, Nutricia, and Avanos. P.B. has received honoraria for speaking engagements from AbbVie, Nutricia, and Avanos. N.M.C.'s institution received speaker fees, advisory board fees, and research funding on his behalf from AbbVie, Eli Lilly, Takeda, Shire, Pfizer, and 4D Pharma. D.S.F. has received royalties from UpToDate (“Pediatric Caustic Ingestions”). I.H. has received honoraria for speaking engagements from BioGaia, Oktal Pharma, Nutricia, Abela Pharm, and Nestle. H.Q.H. has received research support from Janssen, AbbVie, Takeda, and Allergan. H.Q.H. has served on the advisory boards of AbbVie and Jansen. K.J. has received research support from Janssen, AbbVie, and the Center for Drug Research and Development (CDRD). K.J. has served on the advisory boards of Janssen, AbbVie, and Merck and participates in the speaker's bureau for AbbVie and Janssen. D.G.L. has received consultant fees from EvoEndo. J.R.L. has received research support from AbbVie and an honorarium from Mead Johnson. A.R.O. has received research support from Janssen, AbbVie, Pfizer, and Eli Lilly. A.R.O. has served on the advisory boards of Janssen, AbbVie, and Eli Lilly and participates in the speaker's bureau for AbbVie and Janssen. J.R.R. has received research support from AbbVie and Janssen. J.R.R. has served on the advisory boards of Janssen, BMS, Eli Lilly, and Pfizer. C.M.W. has received research support from AbbVie., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Introduction: Pediatric-specific quality standards for endoscopy are needed to define best practices, while measurement of associated indicators is critical to guide quality improvement. The international Pediatric Endoscopy Quality Improvement Network (PEnQuIN) working group was assembled to develop and define quality standards and indicators for pediatric gastrointestinal endoscopic procedures through a rigorous guideline consensus process., Methods: The Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument guided PEnQuIN members, recruited from 31 centers of various practice types representing 11 countries, in generating and refining proposed quality standards and indicators. Consensus was sought via an iterative online Delphi process, and finalized at an in-person conference. Quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development, and Evaluation) approach., Results: Forty-nine quality standards and 47 indicators reached consensus, encompassing pediatric endoscopy facilities, procedures, endoscopists, and the patient experience. The evidence base for PEnQuIN standards and indicators was largely adult-based and observational, and downgraded for indirectness, imprecision, and study limitations to "very low" quality, resulting in "conditional" recommendations for most standards (45/49)., Conclusions: The PEnQuIN guideline development process establishes international agreement on clinically meaningful metrics that can be used to promote safety and quality in endoscopic care for children. Through PEnQuIN, pediatric endoscopists and endoscopy services now have a framework for auditing, providing feedback, and ultimately, benchmarking performance. Expansion of evidence and prospective validation of PEnQuIN standards and indicators as predictors of clinically relevant outcomes and high-quality pediatric endoscopic care is now a research priority., Competing Interests: Conflicts of interest: P.B. has served on the advisory boards of Biocodex, Nutricia, and Avanos. P.B. has received honoraria for speaking engagements from AbbVie, Nutricia, and Avanos. N.M.C.'s institution received speaker fees, advisory board fees, and research funding on his behalf from AbbVie, Eli Lilly, Takeda, Shire, Pfizer, and 4D Pharma. D.S.F. has received royalties from UpToDate (“Pediatric Caustic Ingestions”). I.H. has received honoraria for speaking engagements from BioGaia, Oktal Pharma, Nutricia, Abela Pharm, and Nestle. H.Q.H. has received research support from Janssen, AbbVie, Takeda, and Allergan. H.Q.H. has served on the advisory boards of AbbVie and Jansen. K.J. has received research support from Janssen, AbbVie, and the Center for Drug Research and Development (CDRD). K.J. has served on the advisory boards of Janssen, AbbVie, and Merck and participates in the speaker's bureau for AbbVie and Janssen. D.G.L. has received consultant fees from EvoEndo. J.R.L. has received research support from AbbVie and an honorarium from Mead Johnson. A.R.O. has received research support from Janssen, AbbVie, Pfizer and Eli Lilly. A.R.O. has served on the advisory boards of Janssen, AbbVie, and Eli Lilly and participates in the speaker's bureau for AbbVie and Janssen. J.R.R. has received research support from AbbVie and Janssen. J.R.R. has served on the advisory boards of Janssen, BMS, Eli Lilly, and Pfizer. C.M.W. has received research support from AbbVie., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Protein-losing enteropathy (PLE) is a disorder of intestinal lymphatic flow resulting in leakage of protein-rich lymph into the gut lumen. Our primary aim was to report the imaging findings of dynamic contrast magnetic resonance lymphangiography (DCMRL) in patients with PLE. Our secondary objective was to use these imaging findings to characterize lymphatic phenotypes., Methods: Single-center retrospective cohort study of patients with PLE unrelated to single-ventricle circulation who underwent DCMRL. We report imaging findings of intranodal (IN), intrahepatic (IH), and intramesenteric (IM) access points for DCMRL., Results: Nineteen patients 0.3-58 years of age (median 1.2 years) underwent 29 DCMRL studies. Primary intestinal lymphangiectasia (PIL) was the most common referring diagnosis (42%). Other etiologies included constrictive pericarditis, thoracic insufficiency syndrome, and genetic disorders. IN-DCMRL demonstrated a normal central lymphatic system in all patients with an intact thoracic duct and localized duodenal leak in one patient (1/19, 5%). IH-DCMRL detected a duodenal leak in 12 of 17 (71%), and IM-DCMRL detected duodenal leak in 5 of 6 (83%). Independent of etiology, lymphatic leak was only visualized in the duodenum., Conclusions: In patients with PLE, imaging via DCMRL reveals that leak is localized to the duodenum regardless of etiology. Comprehensive imaging evaluation with three access points can provide detailed information about the site of duodenal leak., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention., Methods: In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed., Results: During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics., Conclusion: We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal., Key Points: • We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity., (© 2021. The Author(s).)

Background: Achalasia is an esophageal motility disorder characterized by esophagogastric junction (EGJ) dysfunction and impaired esophageal peristalsis with significant impact on quality of life. While the functional luminal imaging probe (FLIP) has been used to assess EGJ distensibility in achalasia, its clinical utility in pediatrics is limited due to absence of normative values and correlations with clinical outcomes in children. Thus, we sought to evaluate FLIP's use in a pediatric achalasia cohort undergoing dilations and non-achalasia controls., Methods: We conducted a retrospective study of pediatric patients with achalasia who underwent FLIP before and immediately after balloon dilations and compared to a non-achalasia cohort., Key Results: Thirty patients with achalasia (mean age, 15.2 years; 40% female), including fourteen treatment-naïve and thirteen controls (mean age, 7.9 years; 61% female) were identified. Median EGJ distensibility index (EGJ-DI) 2.07 mm 2  mmHg -1 and diameter (9.23 mm) in treatment-naïve patients were significantly lower compared to controls (EGJ-DI 6.8 mm 2  mmHg -1 ; diameter 18.61 mm; (p < 0.001). Balloon dilations resulted in a significant increase in EGJ-DI immediately after the dilation, particularly in treatment-naïve patients (p < 0.001), and a significant improvement in Eckardt scores (p < 0.001)., Conclusions & Inferences: Functional luminal imaging probe measurements of EGJ-DI in pediatric patients with achalasia are mostly consistent with adult findings. However, normal EGJ-DI is seen in symptomatic patients, including treatment-naive, highlighting the need for pediatric reference data. Balloon dilations achieve a significant increase in EGJ-DI with improvement in Eckardt scores, confirming the therapeutic value of dilations in achalasia management., (© 2021 John Wiley & Sons Ltd.)

Colonoscopy is an important diagnostic and therapeutic tool in evaluating and treating gastrointestinal tract pathologies. Adequate visualization of the intestinal lumen is necessary for detection of lesions, and thus bowel preparation is a key component of the process. It is estimated that over 25% percent of pediatric patients have sub-optimal bowel preparations, which can lead to longer procedure times, missed pathology, unsuccessful ileal intubation, and possibly repeat procedure/anesthesia. There is no universal protocol for bowel preparation in pediatrics and there is a wide variability of practices around the world. The purpose of this paper is to review the recent published literature regarding bowel preparations for pediatric colonoscopy with focus on published work in the last decade exploring a number of factors involved in bowel preparation including the role of patient education, types of bowel preparation, and their efficacy and safety., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Mamula and Nema.)

Gastrointestinal (GI) bleeding from pediatric vascular malformation is uncommon and difficult to diagnose and manage. The preferred treatment is surgical resection; however, it can be challenging to precisely localize the lesion, particularly if it is not serosal., Objectives: To describe a technique of intentional preoperative coil localization of symptomatic pediatric GI vascular malformations by pediatric interventional radiology to facilitate fluoroscopically assisted laparoscopic resection., Methods: We searched the electronic privacy information center and picture archive and communication system in our center and found 3 cases. The electronic privacy information center and picture archive and communication system databases were the sources for retrieval of demographic, medical, radiological, and procedural information in all 3 cases., Results: After many nondiagnostic investigations in all 3 patients, a GI vascular malformation as a cause of GI bleeding was diagnosed with computed tomography angiography/magnetic resonance angiography and catheter angiography. A preoperative 0.018-inch Hilal coil was placed as close as possible to the vascular malformation during super selective angiography. Laparoscopic surgery was performed within 24 hours of coil placement. In all cases, histology confirmed the resected bowel lesions to be vascular malformations., Conclusions: Intentional endovascular coil localization has the potential to increase the precision of lesion localization and may reduce laparoscopic operative time, when guided by the coil position., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Juvenile polyposis syndrome (JPS) is a clinically diagnosed hamartomatous polyposis syndrome that increases the risk of gastrointestinal cancer. Approximately 40%-50% of JPS is caused by a germline disease-causing variant (DCV) in the SMAD4 or BMPR1A genes. The aim of this study was to characterize the phenotype of DCV-negative JPS and compare it with DCV-positive JPS. Herein, we analyzed a cohort of 145 individuals with JPS from nine institutions, including both pediatric and adult centers. Data analyzed included age at diagnosis, family history, cancer history, need for colectomy/gastrectomy, and polyp number and location. Compared with DCV-positive JPS, DCV-negative JPS was associated with younger age at diagnosis ( P < 0.001), lower likelihood of having a family history of JPS ( P < 0.001), and a lower risk of colectomy ( P = 0.032). None of the DCV-negative individuals had gastric or duodenal polyps, and polyp burden decreased after the first decade compared with DCV-positive JPS. Subgroup analysis between SMAD4 and BMPR1A carriers showed that SMAD4 carriers were more likely to have a family history of JPS and required gastrectomy. Taken together, these data provide the largest phenotypic characterization of individuals with DCV-negative JPS to date, showing that this group has distinct differences compared with JPS due to a SMAD4 or BMPR1A variant. Better understanding of phenotype and cancer risk associated with JPS both with and without a DCV may ultimately allow for individualized management of polyposis and cancer risk. Prevention Relevance: Juvenile Polyposis Syndrome (JPS) is a gastrointestinal cancer predisposition syndrome requiring lifelong surveillance, however there is limited data comparing individuals with and without a germline disease-causing variant in SMAD4 or BMPR1A Herein we show that individuals with JPS without an underlying disease-causing variant have distinct phenotypic differences including lack of upper gastrointestinal polyps and lower rates of a family history of JPS, suggesting that a different approach to management may be appropriate in this population., (©2020 American Association for Cancer Research.)

Competing Interests: The authors report no conflicts of interest.

Despite ongoing efforts of the national Button Battery Task Force (BBTF), cases of major morbidity and mortality continue to be reported at a higher rate after ingestion of higher voltage and larger button batteries. Initiation of sucralfate or honey shortly after some button battery ingestions is crucial to prevent further injury while awaiting emergent removal. Endoscopic acetic acid irrigation post removal of button battery may halt deeper injury. Gastroenterologists need to be aware of the updates to the National Capital Poison Center algorithm for button battery ingestion.

Background: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare, severe, sporadically occurring disorder characterized by multiple venous malformations., Aims: To present and analyze a case series of pediatric patients with BRBNS and to describe diagnostic approaches and management options applied., Patients and Methods: Multicenter, retrospective study, evaluating the diagnosis and management of children with BRBNS., Results: Eighteen patients diagnosed with BRBNS were included. Cutaneous venous malformations were observed in 78% and gastrointestinal venous malformations in 89%. Lesions were also found in other organs including muscles, joints, central nervous system, eyes, parotid gland, spine, kidneys and lungs. Gastrointestinal lesions were more common in the small intestine than in stomach or colon. The management varied significantly among centers. Endoscopic therapy and surgical therapy alone failed to prevent recurrence of lesions. In younger children and in patients with musculoskeletal or other organ involvement, sirolimus was used with 100% success rate in our series (5 patients treated) although poor compliance with subtherapeutic sirolimus trough levels led to recurrence in a minority., Conclusions: Considering the multi-organ involvement in BRBNS, diagnosis and management requires a multidisciplinary approach. The treatment includes conservative, medical, endoscopic and surgical options. Prospective multicenter studies are needed to identify the optimal management of this rare condition., (Copyright © 2019 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Objectives: To describe the technique and report on our initial experience with the use of intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) for evaluation of the lymphatics in patients with hepatic lymphatic flow disorders., Methods: This is a retrospective review of the imaging and clinical findings in six consecutive patients undergoing IH-DCMRL. The technique involves injection of a gadolinium contrast agent into the intrahepatic lymphatic ducts followed by imaging of the abdomen and chest with both heavily T2-weighted imaging and dynamic time-resolved imaging., Results: In six consecutive patients, IH-DCMRL was technically successful. There were four patients with protein-losing enteropathy (PLE) and two with ascites in this study. In the four patients with PLE, IH-DCMRL demonstrated hepatoduodenal connections with leak of contrast into the duodenal lumen not seen by conventional lymphangiography. In one patient with ascites, IH-DCMRL demonstrated lymphatic leakage into the peritoneal cavity not seen by intranodal lymphangiography. In the second patient with ascites, retrograde lymphatic perfusion of mesenteric lymphatic networks and nodes was seen. Venous contamination was seen in two patients. No biliary contamination was identified. There were no short-term complications., Conclusions: IH-DCMRL is a cross-sectional technique which successfully evaluated hepatic lymphatic flow disorders and warrants further investigation., Key Points: • Intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) is a new imaging technique to evaluate hepatic lymphatic flow disorders such as protein-losing enteropathy. • In comparison to conventional liver lymphangiography, IH-DCMRL offers a 3D imaging technique and better distal lymphatic contrast distribution and does not use ionizing radiation.

Gastrointestinal polyps are mucosal overgrowths that, if unchecked, can undergo malignant transformation. Although relatively uncommon in the pediatric age group, they can be the harbingers of multiorgan cancer risk and require close management and follow-up. Additionally, as many polyposis syndromes are inherited, appropriate genetic testing and management of relatives is vital for the health of the entire family. In this review, we discuss both common and uncommon childhood gastrointestinal polyposis syndromes in terms of clinical presentation, management, and surveillance. We also detail any additional malignancy risk and surveillance required in the pediatric age group (<21 years old). Through this review, we provide a framework for gastroenterologists to manage the multifaceted nature of pediatric polyposis syndromes.

Background: Recent studies have shown that oral combination antibiotics may improve disease course in refractory inflammatory bowel disease (IBD). Here, we describe the use of combination oral antibiotics as salvage therapy in refractory ulcerative colitis (UC), Crohn's colitis, and IBD-unclassified (IBD-U) at a large pediatric IBD center., Methods: Clinical response, disease activity indices, adverse events, and clinical outcomes were measured up to 1 year after antibiotic treatment in this retrospective cohort study of children with medically refractory IBD colitis., Results: Sixty-three patients with refractory UC, Crohn's colitis, and IBD-U (median age [interquartile range {IQR}], 15.3 [11.2-16.5] years; median disease duration [IQR], 1.2 [0.41-4.6] years) received a combination of 3 or 4 oral antibiotics (most commonly amoxicillin, metronidazole, and either doxycycline or ciprofloxacin) for a median (IQR) of 29 (21-58) days. Thirty-four patients (54%) were deemed corticosteroid-refractory or -dependent, with the majority (62/63) having a previous or present loss of response or primary nonresponse to anti-tumor necrosis factor alpha (anti-TNFα) therapy. Use of combination antibiotics led to a significant decrease in median Pediatric Ulcerative Colitis Activity Index (PUCAI) score (IQR) from 55 (40-65) to 10 (0-40; P < 0.0001) over 3 ± 1 weeks, with 25/63 (39.7%) patients achieving clinical remission (PUCAI <10 points). The clinical benefits of oral antibiotics were independent of anti-TNFα therapy optimization. Among children entering clinical remission (n = 25), only 1 patient required surgery at 1-year follow-up, vs 10 patients in the nonresponder group. Negative predictors of response to combination antibiotics were exposure to doxycycline (odds ratio [OR], 0.25; 95% CI, 0.08-0.76) and PUCAI ≥65 at baseline (OR, 0.2; 95% CI, 0.05-0.74)., Conclusions: Oral combination antibiotics appears to be an effective rescue and steroid-sparing therapy to induce remission in the short term in patients failing a biologic., (© 2019 Crohn’s & Colitis Foundation. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Assessment is critical to support pediatric endoscopy training. Although trainee engagement in assessment is encouraged, the use of self-assessment and its accuracy among pediatric endoscopists is not well described. We aimed to determine the self-assessment accuracy of novice, intermediate, and experienced pediatric endoscopists., Methods: Novice (performed <50 previous colonoscopies), intermediate (50-500), and experienced (>1000) pediatric endoscopists from 3 North American academic teaching hospitals each performed a clinical colonoscopy. Endoscopists were assessed in real-time by 2 experienced endoscopists using the Gastrointestinal Endoscopy Competency Assessment Tool for Pediatric Colonoscopy (GiECATKIDS). In addition, participants self-assessed their performance using the same instrument. Self-assessment accuracy between the externally assessed and self-assessed scores was evaluated using absolute difference scores, intraclass correlation coefficients, and Bland-Altman analyses., Results: Forty-seven endoscopists participated (21 novices, 16 intermediates, and 10 experienced). Overall, there was moderate agreement of externally assessed and self-assessed GiECATKIDS total scores with an intraclass correlation coefficient of 0.72 (95% confidence interval, 0.55-0.83). The absolute difference scores among the 3 groups were significantly different (P = 0.005), with experienced endoscopists demonstrating a more accurate self-assessment compared to novices (P = 0.003). Bland-Altman plots revealed that novice endoscopists' self-assessed scores tended to be higher than their externally assessed scores, indicating they overestimated their performance., Conclusions: We found that endoscopic experience was positively associated with self-assessment accuracy among pediatric endoscopists. Novices were inaccurate in assessing their endoscopic competence and were prone to overestimation of their performances. Our findings suggest novices may benefit from targeted interventions aimed at improving their insight and self-awareness.

Objectives: Identify MRE features of colonic strictures in children/young adults with Crohn's disease., Methods: Included are patients with colonic strictures on colonoscopy imaged with MRE. Bowel wall thickening (BWT) with luminal narrowing, pre-stenotic dilatation (PSD), bowel wall enhancement, and diffusion restriction were evaluated., Results: All potential strictures (n=12) had BWT with luminal narrowing on MRE. 8/12 had PSD, meeting our MRE stricture criteria. MRE diagnosed 8/12 colonic strictures compared to colonoscopy but was concordant with findings in all surgical patients., Conclusion: Strictures on MRE did not always agree with colonoscopy, but when correlating with surgery, MRE findings were all concordant., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: Iron deficiency anemia (IDA) is a common complication of pediatric inflammatory bowel disease (IBD), yet the effectiveness of oral iron supplementation is limited. Intravenous iron sucrose is an effective and safe alternative treatment for IDA in adults with IBD, but its role in the treatment of IDA in pediatric IBD is unclear. The primary aim of this study was to evaluate the use of iron sucrose in pediatric IBD subjects with IDA and determine the clinical response as measured by improvement in hemoglobin concentration. The secondary aim was to describe adverse events associated with iron sucrose use in this cohort., Methods: A retrospective chart review was performed of all pediatric patients with IBD receiving iron sucrose infusions for IDA at a single tertiary care center between 2011 and 2015., Results: Seventy-two subjects (53 with Crohn disease, 11 with ulcerative colitis, and 8 with IBD-unclassified) received a total of 273 iron sucrose infusions. Forty-three subjects qualified for the efficacy analysis. There was a significant increase in hemoglobin over the treatment course, with mean (±SD) hemoglobin increasing from 9.6 ± 1.2 g/dL at baseline to 12.1 ± 1.3 g/dL after iron sucrose treatment (P < 0.001). Eighteen adverse events were reported in 13 subjects (18.1% of subjects and 6.6% of infusions). No anaphylaxis reactions occurred and none of the adverse events were, however, life-threatening or required hospitalization., Conclusions: Intravenous iron sucrose is a safe and potentially efficacious treatment choice for IDA in pediatric IBD.

Background/aim: Pediatric ileocolonic Crohn disease (CD) may be difficult to distinguish from ulcerative colitis (UC) with backwash ileitis (BWI). The primary aim of the study was to determine the probability of CD in children with a confluent colitis and ileitis when newly diagnosed with inflammatory bowel disease (IBD)., Methods: A retrospective observational study of 100 newly diagnosed patients with IBD was performed. Two pathologists reviewed ileal biopsy specimens for 8 histological features. Biopsy and clinical features were evaluated for predictive ability of a final diagnosis of CD., Results: The presence of crypt distortion, lamina propria (LP) expansion, and acute LP inflammation combined with 4 clinical variables in multivariate regression analysis had adequate discriminative validity when comparing the mean probability of a final CD diagnosis between CD and not-CD groups (0.90 vs. 0.59, p value <0.001). When crypt distortion, LP expansion, and acute LP inflammation are present in any combination, the sensitivity and specificity for presence of CD ranges 38.4-57% and 92.9-100%, respectively., Conclusions: Combining histological features of ileitis and clinical variables can adequately discriminate between the presence and absence of Crohn disease in children who present with confluent colitis and ileitis. Combined presence of certain histological features has high specificity for CD., (Copyright © 2017 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Objectives: Diverting ileostomy is used as a temporizing therapy in patients with perianal Crohn disease; however, little data exist regarding its use for colonic disease. The primary aim of the present study was to determine the role of diversion in severe refractory colonic inflammatory bowel disease (IBD) in a pediatric population., Methods: Retrospective study of patients who underwent diverting ileostomy at The Children's Hospital of Philadelphia from 2000 to 2014 for the management of severe, refractory colonic IBD. Clinical variables were compared in the 1 year before ileostomy and 1 year after diversion. Surgical and disease outcomes including changes in diagnosis were reviewed through 2015., Results: Twenty-four patients underwent diverting ileostomy for refractory colonic disease. Initial diagnoses were Crohn disease in 10 (42%), ulcerative colitis in 1 (4%), and IBD-unclassified in 13 patients (54%). Comparing data before and after surgery, there were statistically significant improvements in height and weight velocities, height velocity z score, blood transfusion requirement, hemoglobin, and hospitalization rates. Chronic steroid use decreased from 71% to 22%. At the conclusion of the study, 10 patients had undergone subsequent colectomy, 7 had successful bowel reanastomosis, and 7 remain diverted. Seven patients (29%) had a change in diagnosis. There were 13 surgical complications in 7 subjects, including prolapse reduction, stoma revision, and resection of ischemic bowel., Conclusions: In pediatric patients with refractory colonic IBD, diverting ileostomy can be a successful intervention to induce clinical stability. Importantly, diversion is a steroid-sparing therapy and allows additional time to clarify the diagnosis.

Background and Objectives: The safety and utility of endoscopic ultrasound (EUS) for the evaluation and management of gastrointestinal (GI) tract disorders among adults has been established. The literature on safety and efficacy in a pediatric referral population (under 21 years of age) is limited. We hypothesized that EUS is safe and useful in the pediatric population. We reviewed the pediatric EUS experience at a single tertiary-care system. We describe the indications, findings, safety, technical success rate, and impact on clinical outcomes., Patients and Methods: All patients 21 years of age or younger referred for EUS between 5, 2007 and 11, 2012 were identified from our electronic medical record databases. Retrospective chart review was then conducted to document demographics, procedure indications, procedure type (diagnostic or therapeutic), type of anesthesia used, EUS findings, and the clinical impact of EUS on the subsequent management of the patients., Results: Seventy EUS procedures were attempted in 58 patients during the study. Of these, two EUS procedures were aborted due to inadequate moderate sedation and 68 were successfully completed. The median age at initial endoscopy was 18 years (range 6-21 years), 50% were male and 65% were Caucasian. Four patients underwent EUS-guided pseudocyst drainage. Among the remaining 54 patients, the indications for EUS were the evaluation of GI mucosal/submucosal lesions (n = 14), acute or recurrent pancreatitis (n = 10), localization of suspected insulinoma (n = 8), evaluation of pancreatic abnormalities seen on prior imaging (n = 6), surveillance of tumors or evaluation of luminal lesions in hereditary syndromes (n = 6), abdominal pain of suspected pancreatobiliary origin (n = 5), and other rare indications (n = 5). Fine-needle aspiration was performed in 13 (9 diagnostic, 4 therapeutic) and trans-gastric fine-needle contrast injection of the pancreatic duct was performed in one patient without any complications. Sedation (data available for 66 procedures) included general endotracheal anesthesia in 38 (57%), monitored anesthesia care (MAC) in 19 (29%), and moderate sedation in 9 (14%). There were 4 minor intra-procedural anesthesia-related complications (laryngospasm in 2 and hypoxemia from airway obstruction and secretions in 2) in MAC and general endotracheal anesthesia (GA) cases, and 1 postprocedural complication (fever after pseudocyst drainage). EUS can achieve the diagnostic or therapeutic goal and ruled out suspected pathology in 88% of cases precluding need for additional testing., Conclusions: (1) EUS in the pediatric population is technically successful and efficacious. (2) Therapeutic and diagnostic EUS impacted clinical care decisions. (3) There is a low risk of immediate significant complications. (4) The overall efficacy and safety support the performance of EUS in a pediatric population by experienced endoscopists.

Objectives: Gastrointestinal bleeding is one of the most common indications for urgent endoscopy in the pediatric setting. The majority of these procedures are performed for control of variceal bleeding, with few performed for nonvariceal upper gastrointestinal (NVUGI) bleeding. The data on therapeutic endoscopy for NVUGI are sparse. The aims of our study were to review our experience with NVUGI bleeding, describe technical aspects and outcomes of therapeutic endoscopy, and determine gastroenterology fellows' training opportunities according to the national training guidelines., Methods: We performed a retrospective review of endoscopy database (Endoworks, Olympus Inc, Center Valley, PA) from January 2009 to December 2014. The search used the following keywords: bleeding, hematemesis, melena, injection, epinephrine, cautery, clip, and argon plasma coagulation. The collected data included demographics, description of bleeding lesion and medical/endoscopic therapy, rate of rebleeding, relevant laboratories, physical examination, and need for transfusion and surgery. The study was approved by the institutional review board., Results: During the study period 12,737 upper endoscopies (esophagogastroduodenoscopies) were performed. A total of 15 patients underwent 17 esophagogastroduodenoscopies that required therapeutic intervention to control bleeding (1:750 procedures). The mean ± standard deviation (median) age of patients who required endoscopic intervention was 11.6 ± 6.0 years (14.0 years). Seven out of 17 patients received dual therapy to control the bleeding lesions. All but 3 patients received medical therapy with intravenous proton pump inhibitor, and 3 received octreotide infusions. Six of the patients experienced rebleeding (40%), with 4 out of 6 initially only receiving single modality therapy. Two of these patients eventually required surgical intervention to control bleeding and both patients presented with bleeding duodenal ulcers. There were no cases of aspiration, perforation, or deaths. There were a total of 24 fellows trained in our program during the study period. Less than 1 therapeutic endoscopy per fellow for NVUGI bleeding was performed., Conclusions: NVUGI bleeding requiring therapeutic endoscopic intervention is rare in pediatrics. A high rate (40%) of rebleeding was noted with a large proportion (66%) of patients receiving single modality therapy. Two patients required surgical intervention to control bleeding and both presented with bleeding duodenal ulcers. An insufficient number of therapeutic procedures is available for adequate fellow training requiring supplemental simulator and hands-on animal model, or adult endoscopy unit training.

Objective: To assess preventive care measure prescribing in children exposed to glucocorticoids and identify prescribing variation according to subspecialty and patient characteristics., Study Design: Retrospective cohort study of children initiating chronic glucocorticoids in the gastroenterology, nephrology, and rheumatology divisions at a pediatric tertiary care center. Outcomes included 25-hydroxyvitamin D (25OHD) and lipid testing, pneumococcal polysaccharide (PPV) and influenza vaccination, and stress dose hydrocortisone prescriptions., Results: A total of 701 children were followed for a median of 589 days. 25OHD testing was performed in 73%, lipid screening in 29%, and PPV and influenza vaccination in 16% and 78%, respectively. Hydrocortisone was prescribed in 2%. Across specialties, 25OHD, lipid screening, and PPV prescribing varied significantly (all P < .001). Using logistic regression adjusting for specialty, 25OHD testing was associated with older age, female sex, non-Hispanic ethnicity, and lower baseline height and body mass index z-scores (all P < .03). Lipid screening was associated with older age, higher baseline body mass index z-score, and lower baseline height z-score (all P < .01). Vaccinations were associated with lower age (P < .02), and PPV completion was associated with non-White race (P = .04)., Conclusions: Among children chronically exposed to glucocorticoids, 25OHD testing and influenza vaccination were common, but lipid screening, pneumococcal vaccination, and stress dose hydrocortisone prescribing were infrequent. Except for influenza vaccination, preventive care measure use varied significantly across specialties. Quality improvement efforts are needed to optimize preventive care in this high-risk population., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Vedolizumab is effective for inducing and maintaining remission in adults with inflammatory bowel disease (IBD); however, there is limited pediatric data. This study aimed to describe the adverse events and clinical response to vedolizumab in refractory pediatric IBD., Methods: Disease activity indices, clinical response, concomitant medication use, and adverse events were measured over 22 weeks in an observational prospective cohort study of children with refractory IBD who had failed anti-tumor necrosis factor therapy and subsequently initiated vedolizumab therapy., Results: Twenty-one subjects, 16 with Crohn disease, received vedolizumab. Clinical response was observed in 6/19 (31.6%) of the evaluable subjects at week 6 and in 11/19 (57.9%) by week 22. Before induction, 15/21 (71.4%) participants were treated with systemic corticosteroids, as compared with 7/21 (33.3%) subjects at 22 weeks. Steroid-free remission was seen in 1/20 (5.0%) subjects at 6 weeks, 3/20 (15.0%) at 14 weeks, and 4/20 (20.0%) at 22 weeks. There was statistically significant improvement in serum albumin and hematocrit; however, C-reactive protein increased by week 22 (P < 0.05). There were no infusion reactions. Vedolizumab was discontinued in 2 patients because of severe colitis, requiring surgical intervention., Conclusions: There is limited experience with vedolizumab therapy in pediatric IBD. There seems to be a marked number of subjects with clinical response in the first 6 weeks that increases further by week 22 despite the severity of disease in this cohort. Adverse events may not be directly related to vedolizumab. This study is limited by small sample size, and larger prospective studies are warranted.

Objectives: Electrolyte-free polyethylene glycol powder (PEG-3350) has been widely used for colonoscopy preparation (prep); however, limited safety data on electrolyte changes exists with 1-day prep regimens. The primary aim of this study was to determine the proportion of patients with significant serum chemistry abnormalities before and at the time of colonoscopy. Secondary aims included evaluation of prep tolerance and bowel cleansing efficacy., Methods: We performed a prospective descriptive observational study of pediatric patients scheduled for outpatient colonoscopy who received our standard 1-day, weight-based 4 g/kg PEG-3350 prep with a single stimulant laxative dose and had serum chemistry testing within 60 days before and at the time of colonoscopy. A standardized bowel cleanliness tool (Aronchick scale) was completed by the endoscopist., Results: One hundred fifty-five patients had serum electrolytes data pre- and postprep. Comparison of each patient's chemistries demonstrated statistical equivalence with the 1 exception of blood urea nitrogen levels (P = 0.56). Hypokalemia was detected postprep in 37 subjects (24%), but none had a serum level <3.3 mmol/L, which was deemed to be of no clinical significance. Five patients were hypoglycemic post prep; 3 were 7 years or younger (P = 0.02). The colon cleanliness rating was excellent or good in 77% and suboptimal in 23% of patients., Conclusions: A 1-day, weight-based PEG-3350 bowel prep in children appears safe. Changes in electrolyte levels and renal function were not clinically significant. Children of 7 years or younger seem to be at a higher risk of hypoglycemia compared with older children.

Background and Aims: Studies in adults have shown that postprocedural abdominal pain is reduced with the use of carbon dioxide (CO(2)) instead of air for insufflation during colonoscopy. The aim of our study was to compare postprocedural abdominal pain and girth in children undergoing colonoscopy using CO(2) or air for insufflation., Methods: This was a prospective, randomized, double-blind study that included 76 consecutive pediatric patients undergoing colonoscopy for various indications. Patients were randomly assigned to either CO(2) or air insufflation. At 2, 4, and 24 hours after the examination, the patients' pain was assessed by using the 11-point numerical rating scale. The waist circumference was measured 10 minutes and 2 and 4 hours after colonoscopy., Results: A significantly higher proportion of patients had no pain after colonoscopy in the CO(2) group compared with the air group (82 vs 37% at 2 hours and 95% vs. 63% at 4 hours, P < .001). Mean abdominal pain scores 2 and 4 hours after the procedure were statistically significantly lower in the CO(2) group compared with the control air group (0.5 vs 2.6 at 2 hours and 0.1 vs 1.2 at 4 hours, P < .001). There was no difference in waist circumference between the 2 groups at all time intervals., Conclusion: The results of this randomized trial show clear benefits of CO(2) insufflation for colonoscopy in reducing postprocedural discomfort. (, Clinical Trial Registration Number: NCT02407639.)., (Copyright © 2016 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc. All rights reserved.)

Background: Children with very early-onset inflammatory bowel disease (VEO-IBD), those diagnosed at less than 5 years of age, are a unique population. A subset of these patients present with a distinct phenotype and more severe disease than older children and adults. Host genetics is thought to play a more prominent role in this young population, and monogenic defects in genes related to primary immunodeficiencies are responsible for the disease in a small subset of patients with VEO-IBD., Case Presentation: We report a child who presented at 3 weeks of life with very early-onset inflammatory bowel disease (VEO-IBD). He had a complicated disease course and remained unresponsive to medical and surgical therapy. The refractory nature of his disease, together with his young age of presentation, prompted utilization of whole exome sequencing (WES) to detect an underlying monogenic primary immunodeficiency and potentially target therapy to the identified defect. Copy number variation analysis (CNV) was performed using the eXome-Hidden Markov Model. Whole exome sequencing revealed 1,380 nonsense and missense variants in the patient. Plausible candidate variants were not detected following analysis of filtered variants, therefore, we performed CNV analysis of the WES data, which led us to identify a de novo whole gene deletion in XIAP., Conclusion: This is the first reported whole gene deletion in XIAP, the causal gene responsible for XLP2 (X-linked lymphoproliferative Disease 2). XLP2 is a syndrome resulting in VEO-IBD and can increase susceptibility to hemophagocytic lymphohistocytosis (HLH). This identification allowed the patient to be referred for bone marrow transplantation, potentially curative for his disease and critical to prevent the catastrophic sequela of HLH. This illustrates the unique etiology of VEO-IBD, and the subsequent effects on therapeutic options. This cohort requires careful and thorough evaluation for monogenic defects and primary immunodeficiencies.

Background & Aims: Very early onset inflammatory bowel disease (VEO-IBD), IBD diagnosed at 5 years of age or younger, frequently presents with a different and more severe phenotype than older-onset IBD. We investigated whether patients with VEO-IBD carry rare or novel variants in genes associated with immunodeficiencies that might contribute to disease development., Methods: Patients with VEO-IBD and parents (when available) were recruited from the Children's Hospital of Philadelphia from March 2013 through July 2014. We analyzed DNA from 125 patients with VEO-IBD (age, 3 wk to 4 y) and 19 parents, 4 of whom also had IBD. Exome capture was performed by Agilent SureSelect V4, and sequencing was performed using the Illumina HiSeq platform. Alignment to human genome GRCh37 was achieved followed by postprocessing and variant calling. After functional annotation, candidate variants were analyzed for change in protein function, minor allele frequency less than 0.1%, and scaled combined annotation-dependent depletion scores of 10 or less. We focused on genes associated with primary immunodeficiencies and related pathways. An additional 210 exome samples from patients with pediatric IBD (n = 45) or adult-onset Crohn's disease (n = 20) and healthy individuals (controls, n = 145) were obtained from the University of Kiel, Germany, and used as control groups., Results: Four hundred genes and regions associated with primary immunodeficiency, covering approximately 6500 coding exons totaling more than 1 Mbp of coding sequence, were selected from the whole-exome data. Our analysis showed novel and rare variants within these genes that could contribute to the development of VEO-IBD, including rare heterozygous missense variants in IL10RA and previously unidentified variants in MSH5 and CD19., Conclusions: In an exome sequence analysis of patients with VEO-IBD and their parents, we identified variants in genes that regulate B- and T-cell functions and could contribute to pathogenesis. Our analysis could lead to the identification of previously unidentified IBD-associated variants., (Copyright © 2015 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Objectives: Validated assessment tools are required to support competency-based education. We aimed to assess the reliability and validity of the Gastrointestinal Endoscopy Competency Assessment Tool for Pediatric Colonoscopy (GiECATKIDS), an instrument developed by 41 North American experts using Delphi methodology., Methods: GiECATKIDS consists of a 7-item global rating scale (GRS) and an 18-item checklist (CL). An attending physician assessed 104 colonoscopies performed at 3 North American hospitals by 56 endoscopists, including 25 novices (<50 previous procedures), 21 intermediates (50-250), and 10 advanced endoscopists (>500). Another observer rated procedures to assess interrater reliability using intraclass correlation coefficient (ICC). Test-retest reliability was measured with ICC comparing endoscopists' first and second procedure scores. Discriminative validity was examined by comparing experience level with scores. Concurrent validity was assessed by correlating scores with colonoscopy experience, cecal and terminal ileal intubation rates, and physician global assessment., Results: Interrater reliability of the GiECATKIDS was high (total: ICC = 0.88; GRS: ICC = 0.79; CL: ICC = 0.89). Test-retest reliability was excellent (total: ICC = 0.94; GRS: ICC = 0.94; CL: ICC = 0.84). GiECATKIDS total, GRS, and CL scores differed significantly among novice, intermediate, and advanced endoscopists (P < 0.001). There was a significant positive correlation (P < 0.001) between scores and number of previous colonoscopies (total: ρ = 0.91, GRS: ρ = 0.92, CL: ρ = 0.84), cecal intubation rate (total: ρ = 0.82, GRS: ρ = 0.85, CL: ρ = 0.77), ileal intubation rate (total: ρ = 0.82, GRS: ρ = 0.82, CL: ρ = 0.80), and physician global assessment (total: ρ = 0.95, GRS: ρ = 0.94, CL: ρ = 0.89)., Conclusions: The GiECATKIDS demonstrates strong reliability and validity as a measure of performance of pediatric colonoscopy that can be used to support training and assessment.

Foreign body ingestions in children are some of the most challenging clinical scenarios facing pediatric gastroenterologists. Determining the indications and timing for intervention requires assessment of patient size, type of object ingested, location, clinical symptoms, time since ingestion, and myriad other factors. Often the easiest and least anxiety-producing decision is the one to proceed to endoscopic removal, instead of observation alone. Because of variability in pediatric patient size, there are less firm guidelines available to determine which type of object will safely pass, as opposed to the clearer guidelines in the adult population. In addition, the imprecise nature of the histories often leaves the clinician to question the timing and nature of the ingestion. Furthermore, changes in the types of ingestions encountered, specifically button batteries and high-powered magnet ingestions, create an even greater potential for severe morbidity and mortality among children. As a result, clinical guidelines regarding management of these ingestions in children remain varied and sporadic, with little in the way of prospective data to guide their development. An expert panel of pediatric endoscopists was convened and produced the present article that outlines practical clinical approaches to the pediatric patient with a variety of foreign body ingestions. This guideline is intended as an educational tool that may help inform pediatric endoscopists in managing foreign body ingestions in children. Medical decision making, however, remains a complex process requiring integration of clinical data beyond the scope of these guidelines. These guidelines should therefore not be considered to be a rule or to be establishing a legal standard of care. Caregivers may well choose a course of action outside of those represented in these guidelines because of specific patient circumstances. Furthermore, additional clinical studies may be necessary to clarify aspects based on expert opinion instead of published data. Thus, these guidelines may be revised as needed to account for new data, changes in clinical practice, or availability of new technology.

Background: Duodenal hematoma (DH) is a rare complication of esophagogastroduodenoscopy (EGD) with duodenal biopsy and uncommon, but better described following blunt abdominal trauma (BAT). We aimed to describe DH incidence and investigate risk factors for DH development post-EGD and compare its features to those post-BAT., Methods: Multiple electronic databases were searched for the diagnosis of DH from 2000 to 2012. Inclusion criteria were patients 0 to 21 years of age who developed a DH following EGD with biopsy or BAT. Exclusion criteria were DH secondary to any other mechanism, EGD performed at another medical center, and insufficient information in the electronic medical record to determine treatments or outcomes., Results: A total of 14 post-EGD and 15 post-BAT patients with DH were included in the study. There were 26,905 EGDs with duodenal biopsies performed during the study period, for an incidence of 1:1922 procedures. Thirteen of 14 (93%) post-EGD DH events occurred between 2007 and 2012 (P < 0.001). The proportion of procedures performed under general anesthesia versus moderate sedation, and performed in the supine position versus left lateral decubitus were close to but did not reach statistical significance. DH-related complications and time to hematoma resolution was similar between groups., Conclusions: In a 13-year study period, 14 patients developed DH after EGD, for an incidence of 1:1922. Method of sedation and supine positioning of the patient during endoscopy warrant further investigation as potential risks. The clinical course and time to recovery with conservative management are similar between patients with EGD and BAT-induced DH.