Longitudinal Quality of Life and Glycemic Outcomes of Total Pancreatectomy With Islet Autotransplantation in Children With Chronic Pancreatitis Followed in a Pediatric Multidisciplinary Pancreas Clinic.

BACKGROUND: Total pancreatectomy with islet autotransplantation (TPIAT) is a potentially curative treatment for patients with chronic pancreatitis (CP) refractory to medical and endoscopic therapies. Patients often receive the initial follow-up medical care at the surgery-performing center, but then may follow up closer to where they live. We sought to describe the characteristics and outcomes of pediatric patients who underwent TPIAT at a national surgical referral center and were subsequently followed at our regional subspecialty center, the Children's Hospital Colorado. METHODS: We performed a retrospective analysis of baseline and outcomes data for the 10 pediatric patients who underwent TPIAT from 2007 to 2020 and received follow-up care at our institution. RESULTS: All patients had a diagnosis of CP, and nine of 10 patients had an identified underlying genetic risk factor. Insulin usage was common immediately following TPIAT, but at 1 year of follow-up, five of nine patients (55.6%) were insulin-independent and nine of nine had an HbA1c below 6.5%. For the four patients on insulin 1 year after TPIAT, total daily insulin dose ranged from 0.06 to 0.71 units/kg/day. All patients who underwent mixed meal tolerance testing had a robust peak C-peptide response at 1 year. There were significant improvements in nausea, school/work absences, narcotic dependence, and pancreas-related hospital admissions 1 year after TPIAT. CONCLUSIONS: Patients followed at our center had long-term improvements with low-insulin usage, detectable C-peptide, and improved pancreatitis-related outcomes after TPIAT. Pediatric patients who undergo TPIAT can be successfully co-managed in conjunction with the original surgery-performing center.

Pancreatic Enzyme Use Reduces Pancreatitis Frequency in Children With Acute Recurrent or Chronic Pancreatitis: A Report From INSPPIRE.

INTRODUCTION: Among children who suffer from acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP), acute pancreatitis (AP) episodes are painful, often require hospitalization, and contribute to disease complications and progression. Despite this recognition, there are currently no interventions to prevent AP episodes. In this retrospective cohort study, we assessed the impact of pancreatic enzyme therapy (PERT) use on clinical outcomes among children with pancreatic-sufficient ARP or CP. METHODS: Children with pancreatic-sufficient ARP or CP in the INSPPIRE-2 cohort were included. Clinical outcomes were compared for those receiving vs not receiving PERT, as well as frequency of AP before and after PERT. Logistic regression was used to study the association between development of AP episodes after starting PERT and response predictors. RESULTS: Among 356 pancreatic-sufficient participants, 270 (76%) had ARP, and 60 (17%) received PERT. Among those on PERT, 42% did not have a subsequent AP episode, during a mean 2.1 years of follow-up. Children with a SPINK1 mutation ( P = 0.005) and those with ARP (compared with CP, P = 0.008) were less likely to have an AP episode after starting PERT. After initiation of PERT, the mean AP annual incidence rate decreased from 3.14 down to 0.71 ( P < 0.001). DISCUSSION: In a retrospective analysis, use of PERT was associated with a reduction in the incidence rate of AP among children with pancreatic-sufficient ARP or CP. These results support the need for a clinical trial to evaluate the efficacy of PERT to improve clinical outcomes among children with ARP or CP.

A National Survey of the Training and Practice Patterns of Practicing Pediatric Advanced Endoscopists in the United States.

BACKGROUND AND AIMS: Pediatric advanced endoscopy consists primarily of endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS) and is becoming more common in pediatrics. This study aims to characterize the current landscape of pediatric advanced endoscopy training and practice by directly surveying independently practicing pediatric advanced endoscopists (PAEs). We also aim to ascertain expert opinion on competency in pediatric ERCP and EUS. METHODS: A 66-question REDCap survey and a 73-question Qualtrics survey were distributed to members of the ERCP Special Interest Group of North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition. Respondents currently performing ERCP or EUS independently in children were included. Statistical analysis was performed using Mann-Whitney U test. RESULTS: Of 41 PAEs surveyed, 38 (92.7%) responded and 27 independent practitioners were included. Thirteen respondents performed EUS. PAEs who completed an advanced endoscopy fellowship (AEF) were more comfortable performing American Society for Gastrointestinal Endoscopy grade 3 or grade 4 ERCPs ( P < 0.0008) and felt more prepared to practice EUS independently than other trainees. Expert opinion of PAEs felt a threshold of 200 procedures was needed to attain competency in either ERCP or EUS. Pediatric duodenoscope exposure improved comfort in performing ERCP in children <10 kg ( P = 0.009). CONCLUSIONS: Training of pediatric gastroenterologists in ERCP and EUS are highly variable, though the skills attained are similar. AEF-trained specialists reported greater training volumes and felt more prepared to practice independently than those who did not. Competency thresholds determined by expert PAEs for ERCP and EUS agree with American Society for Gastrointestinal Endoscopy guidelines for adult advanced endoscopy trainees.

Ketorolac Is Safe and Associated With Lower Rate of Post-Endoscopic Retrograde Cholangiopancreatography Pancreatitis in Children With Pancreatic Duct Manipulation.

OBJECTIVES: Use of non-steroidal anti-inflammatory drugs (NSAIDs) for post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) prevention in pediatrics is not well studied. Because of difficulty in accurately dosing indomethacin suppositories in pediatric patients, our center has used intravenous ketorolac for PEP prevention and present data on its safety and associated PEP rates. METHODS: Prospective monitoring of PEP for all patients who underwent ERCP at a pediatric tertiary care center from July 2010 to June 2018. Retrospective review of patient and procedural factors and severity of PEP. Routine use of ketorolac for PEP prevention began in 2014. RESULTS: Two hundred and ninety-eight ERCPs were analyzed. One hundred and sixty-six patients received intraprocedural ketorolac and 132 did not. One patient had post-ERCP bleeding and bleeding rates were not significantly different between ketorolac and non-ketorolac groups (0.6% vs 0%, P = 1). Overall rates of PEP were not significantly different between the ketorolac and no ketorolac group (9% vs 13%, P = 0.29); however, for high-risk pediatric patients with injection of contrast into and/or cannulation of the pancreatic duct, the rates of PEP were significantly lower for patients who received ketorolac (11% vs 25%, P = 0.035). CONCLUSIONS: Pediatric patients undergoing ERCP with manipulation of the pancreatic duct are high risk for PEP, and ketorolac was associated with a lower rate of PEP in these patients. Ketorolac was well tolerated without a higher rate of bleeding after ERCP. These results are the first to provide evidence showing an association with intraprocedural NSAID use and lower rates of PEP in select pediatric patients.

Results of duodenoscope culture and quarantine after manufacturer-recommended cleaning process.

BACKGROUND AND AIMS: Outbreaks of infections transmitted by duodenoscopes have prompted changes to duodenoscope cleaning protocols. However, little is known about effectiveness of clinical cleaning practices. We present culture data after duodenoscope manufacturer-recommended high-level disinfection (HLD) and quarantine. METHODS: Our institution adopted a combination of manufacturer-recommended cleaning with the Centers for Disease Control and Prevention-recommended culture and quarantine in 2015. Duodenoscopes (models TJF-Q180V, TJF-160, and PJF-160; Olympus, Center Valley, Penn, USA) underwent HLD according to the manufacturer's reprocessing manual protocols after use. Two culture specimens were then obtained using a sterile brush from the distal tip, including elevator mechanism, and by flushing sterile water through the working channel. Duodenoscopes were quarantined until cultures resulted. Positive cultures were defined as >10 colony-forming units (CFUs) of low-concern organisms or any CFUs of high-concern organisms according to Centers for Disease Control and Prevention recommendations. If either culture specimen was positive, the process was repeated until cultures were negative. Data were collected from December 2015 to July 2018. RESULTS: There were 140 instances of duodenoscope cleaning with 280 specimens. Twenty-eight of 234 (12%) initial culture specimens (18% of cultured duodenoscopes) were positive. Five of 36 (14%) second cultures were positive. Two of 8 (25%) third cultures were positive. Ninety-eight percent of organisms cultured were gram positive. In 8 instances both culture methods (brushing and flush) were positive; otherwise, only 1 method was positive. In 11 instances (8%) duodenoscopes were removed from quarantine before final culture results. No patient had infections related to ERCP. CONCLUSIONS: Eighteen percent of duodenoscopes had a positive culture after initial HLD. Only 3% were gram-negative bacteria. Repeated HLD was 86% and 75% effective at eliminating initial and repeat positive cultures, respectively. Initial HLD per manufacturer recommendations is not always effective at eliminating bacterial contamination. Additional steps are necessary to decrease risks of duodenoscope-transmitted infections.

Algorithm to Predict Which Children With Chronic Abdominal Pain Are Low Suspicion for Significant Endoscopic Findings.

Chronic abdominal pain (CAP) is a common and challenging problem in pediatric primary and specialty care. We developed a diagnostic algorithm to organize workup for gastrointestinal causes of CAP and improve identification of patients who are low suspicion (LS) or high suspicion (HS) to have significant intestinal pathology identified with endoscopy. We retrospectively used this algorithm to categorize 150 outpatients with CAP as LS (n = 99) or HS (n = 51) and examined subsequent endoscopic findings for all patients. There were 6% significant diagnoses in the LS group compared with 34% in the HS group ( P < .0001). The LS group had no patients with celiac or inflammatory bowel disease. These results can be used to help a clinician approach CAP, and discuss with families the likelihood of endoscopy finding a cause for CAP based on LS or HS designation.

Comparative Analysis of Adverse Events After Esophageal Balloon and Bougie Dilations in Children.

BACKGROUND: Pneumatic balloon and bougie dilation are widely used methods for pediatric esophageal dilation. There are no studies directly comparing the safety of these techniques in pediatric patients. This study compared adverse events (AEs) of balloon and bougie dilation in children at a single institution. METHODS: AEs were identified by means of a prospective clinical registry of all procedure related AEs from 2012 to 2015 at a single institution. Identified AEs underwent retrospective review of procedural and clinical details. The category of each AE was recorded and severity was assigned using a 5-point scoring system. AEs were compared between balloon and bougie dilation for different severities. RESULTS: There were 105 patients who underwent 246 dilation sessions. Balloon dilation was performed more commonly (n = 190, 77%) as compared to Maloney dilators (n = 56, 23%). Patients with balloon dilation were younger (3.0 vs 14.5 years, P = 0.0001) and more likely to have strictures from caustic ingestion (42% vs 2%, P < 0.0001) or surgical anastomoses (34% vs 5% P < 0.0001). Bougie dilation was used more commonly in patients with eosinophilic esophagitis (77% vs 7%, P < 0.0001)). In multivariate analysis, each year of increasing age was associated with a 12% increase in any AEs (P = 0.015), but no difference in clinically significant AEs (grade 2 or higher) was identified between dilation methods. CONCLUSIONS: Bougie and balloon dilation did not have significant differences in AE rates, but the patient populations differed between the 2 methods. The dilation method should depend on stricture characteristics and endoscopist expertise with each method.

Impact of fellow training level on adverse events and operative time for common pediatric GI endoscopic procedures.

BACKGROUND AND AIMS: Previous studies on pediatric endoscopic training have not examined in detail if adverse events (AEs) are affected by the fellow's training level. We aimed to determine whether trainee presence and educational level increase AEs or operative time (OT) for pediatric intestinal endoscopy. METHODS: This was a prospective observational study of AEs for all endoscopic procedures and retrospective analysis of OT (time of endoscope insertion until removal) for a sample of specified procedures at a tertiary children's hospital. AEs were categorized by severity grades: 1, home management; 2, outpatient evaluation; 3, hospitalization and/or repeat endoscopy; 4, surgery and/or intensive care unit admission; and 5, death. RESULTS: A total of 15,886 procedures (6257 with trainee) including 1627 therapeutic procedures (733 with trainee) were analyzed for AEs. Four hundred thirteen total AEs (2.60%) and 213 AEs grade 2 to 4 (1.34%) were identified. Fellow presence at any training level did not increase AE rates for any procedures. Median OT for 3762 EGDs decreased from 17 to 11 minutes from the first quarter to the fourth quarter of first-year fellowship and then remained stable. EGDs without fellows were shorter (9 minutes, P < .0001) compared with any training level. Median times of 1291 colonoscopies with EGD decreased from 55 to 51 to 47 minutes for fellows in the first half, second half of first-year fellowship, and second and third year, respectively. Attendings alone were faster (37 minutes, P < .0001). CONCLUSIONS: Current pediatric endoscopic training for is safe regardless of fellow training level. Trainee efficiency improves during and after fellowship.

Use of Fully Covered Self-expanding Metal Biliary Stents in Pediatrics: A Case Series.

OBJECTIVES: Endoscopic retrograde cholangiopancreatography is used to manage biliary pathology in pediatric patients. Plastic biliary stents have been used in this population for obstructive lesions and bile leaks; however, they are sometimes not effective due to migration, occlusion, or ineffective sealing. Fully covered self-expanding metal stents (FCSEMS) have larger diameters making them more suitable for some situations. Their use in pediatrics has, however, not been defined. The aim of the present study is to describe our experience with FCSEMS at our institution. METHODS: We present a series of all patients who underwent FCSEMS placement at Children's Hospital Colorado including 3 adolescents and 1 young adult with complex medical needs. RESULTS: Patient age range was 12 to 24 years and the weight ranged between 36 and 75 kg. All patients underwent previous endoscopic retrograde cholangiopancreatography and 1 or more rounds of plastic stenting without adequate clinical response before consideration of FCSEMS placement. Indications included recalcitrant biliary anastomotic stricture after liver transplant, persistent bile leak after needle perforation, recurrent obstructive choledocholithiasis after cholecystectomy, and malignant biliary stricture. Sizes of FCSEMS depended on patient bile duct size and biliary pathology. Dwell time was 6 to 8 weeks. Three patients had resolution of biliary pathology after FCSEMS therapy. One patient had distal migration of FCSEMS necessitating repeat stenting. There were no adverse events from FCSEMS placement or removal. CONCLUSIONS: FCSEMS therapy should be considered in appropriate pediatric patients when plastic biliary stents are not effective. Further studies are needed to evaluate the safety and efficacy of FCSEMS in the pediatric age group.