Catheter salvage from central line-related bloodstream infections in pediatric intestinal failure.

OBJECTIVES: Patients with intestinal failure require central venous access which puts them at risk for central line-associated bloodstream infections (CLABSI). Maintaining vascular patency is critical for this population to receive nutrition support. When CLABSIs occur line salvage can help maintain vascular access. The aim of this study is to assess factors associated with safe and successful central venous catheter salvage. METHODS: Retrospective cohort study of patients with intestinal failure at two tertiary care institutions between 2012 and 2020. The study examined the rates of attempted salvage, factors associated with successful salvage, and complications associated with salvage attempts. RESULTS: Over the study period, 76 patients with intestinal failure were include while central venous access was in place. There were a total of 94 CLABSIs. Salvage was more likely to be attempted when patients were under the direct care of an intestinal rehabilitation service (95% vs. 68%, p = 0.04). The overall successful salvage rate was 91.6% (n = 77). Gram-positive, Gram-negative, and polymicrobial infections had successful salvage rates of 97%, 92%, and 94% respectively. The successful salvage rate for fungal infections was 40%. There was no difference in 30-day complication rates for hospital readmission, intensive care unit admission, and death between patients who underwent salvage attempt and those who did not. CONCLUSIONS: Central line salvage can be safely attempted for many infections in patients with intestinal failure, leading to vascular access preservation.

Transient End-Tidal Carbon Dioxide Elevation During Pediatric Upper Endoscopy With Carbon Dioxide Insufflation: Is It True Hypercapnia?

BACKGROUND: Endoscopic insufflation, long performed using air, is being replaced by carbon dioxide (CO2) at many pediatric centers, despite limited published data on its use in children. We have previously demonstrated that CO2 use during esophagogastroduodenoscopy (EGD) in non-intubated children is associated with transient elevations of end-tidal CO2 (EtCO2). This observation raised concerns about possible CO2 inhalation and systemic absorption. Here, we investigate this concern by concurrently measuring both EtCO2 and transcutaneous CO2 (tCO2) during upper endoscopic procedures in children. AIM: To determine if elevations in EtCO2 levels seen in non-intubated children undergoing CO2 insufflation during EGD are associated with elevated systemic CO2 levels. METHODS: Double-blinded, prospective, randomized clinical trial. Children were randomized 1:1 to receive either CO2 or air for endoscopic insufflation. EtCO2 was sampled with a CO2-sampling nasal cannula and tCO2 was monitored using the Radiometer transcutaneous monitoring device. RESULTS: Fifty nine patients were enrolled; 30 patients in the CO2 insufflation group and 29 in the air group. All patients underwent a procedure involving an EGD. Transient elevations in EtCO2 (defined as >60 mmHg) were observed only in the CO2 insufflation group. This contrasted with the similar elevations of tCO2 between the CO2 and air insufflation groups. None of these events were of clinically significant magnitude or duration. CONCLUSION: This study demonstrates that the observed transient elevations in EtCO2 seen during EGD in non-intubated children receiving CO2 insufflation are most likely measurements of eructated CO2 without evidence of excessive systemic absorption of CO2.

Impact of a Medical Scribe Program on Outpatient Pediatric Gastroenterology Clinic.

OBJECTIVES: Medical scribes may offer a route to improve physician productivity and workflow efficiency with reduced physician time for documentation. To our knowledge, there is no prior literature on medical scribe impact on outpatient pediatric gastroenterology clinic operations. The primary aim of our study was to address this knowledge gap. METHODS: Data were collected on encounters conducted by pediatric gastroenterology physicians at a tertiary center, during a summer scribe program. Scribes were trained and attended clinics in a nonrandomized fashion. Clinic efficiency was assessed by patient flow, tracked via the electronic medical record system. Medical note complexity codes, associated work relative value units (wRVUs), and note delinquency were compared between encounters with and without scribes. Patient satisfaction survey scores were compared between groups. RESULTS: One thousand nine hundred seventy encounters were included. Documented medical note complexity (and wRVUs), note delinquency, patient satisfaction, and perceived overall quality of service were similar between groups. Clinic time for established encounters was statistically shorter with scribes (median 18 vs 21 minutes, P = 0.01), a 14% reduction. No significant difference was noted in new encounter clinic time. The time to note completion was shorter for new encounters with scribes (2 vs 3 days, P = 0.048). More notes were finalized by the third day postencounter when a scribe was present (63% vs 57%, P = 0.02). CONCLUSIONS: The presence of medical scribes was associated with significantly more efficient clinic flow for established encounters and modest improvements in note completion rate. There were no measurable negative effects on documented medical note complexity or patient satisfaction scores.

Is Carbon Dioxide Insufflation During Endoscopy in Children as Safe and as Effective as We Think?

OBJECTIVES: Distension of the gastrointestinal lumen is crucial for visualization and advancement during endoscopic procedures. An increasing number of pediatric centers now use carbon dioxide (CO2) preferentially over air as many adult studies and a few pediatric studies have concluded that CO2 is better tolerated than air, especially for colonoscopy. AIMS: The aim of the study was to determine if CO2 is as safe and as effective as air and if it reduces abdominal discomfort and distension in children undergoing upper endoscopy and colonoscopy. METHODS: Double blinded, prospective, randomized clinical study. Patient- and nursing-reported outcomes of pain and distension were recorded. End tidal CO2 (EtCO2) was monitored continuously with a CO2-sampling nasal cannula for patients undergoing procedural sedation and via the endotracheal tube for those who were intubated. RESULTS: One hundred seventy-eight patients with 180 procedures were enrolled, 91 procedures were randomized to receive CO2, and 89 to air. Groups did not differ significantly with respect to nursing-assessed abdominal discomfort, change in girth from baseline, or endoscopist-perceived ease of inflation. Use of CO2 was associated with transient spikes in the EtCO2 (≥60 mmHg) in a significant number of patients during sedated upper endoscopy without endotracheal intubation. There was a reduction of bloating and flatulence for all procedures in the CO2 group. CONCLUSIONS: The benefits of using CO2 for insufflation were minimal in our patients. The observed transient elevations of EtCO2 during sedated upper endoscopy raise concerns of possible systemic hypercarbia. The wisdom of its routine use for all pediatric endoscopic procedures is questioned.

Successful Colonoscopy-assisted Cecostomy Tube Replacement to Salvage Lost Cecostomy Tract Access in Children.

OBJECTIVES: Cecostomy tubes are commonly used for antegrade enema delivery in children with spinal defects and anorectal malformations to help address chronic constipation and fecal incontinence. Once surgically or radiologically placed, cecostomy tubes require changes by a percutaneous approach, which may be unsuccessful requiring repeat laparoscopy or open surgery to re-establish the cecostomy tract. The role of colonoscopy assistance to salvage lost cecostomy access in children who fail percutaneous replacement is not well described. The primary aim was to describe the safety and effectiveness of a colonoscopy-assisted approach to re-establish lost cecostomy access in children. METHODS: This was a retrospective cohort study of the methods, success and complication rates associated with colonoscopy assisted cecostomy tube replacement in children between 2000 and 2017 at a pediatric tertiary care center. RESULTS: Ninety-five patients with 841 attempted procedures were included with only 1% of procedures requiring endoscopic assistance. These included 7 colonoscopy-assisted cecostomy tube replacement procedures in 6 patients (median age 9.2 years, median weight 26.3 kg, 33% girls). The most common reason for using colonoscopy assistance was a failed percutaneous approach. The colonoscopy-assisted approach was successful in all cases without documented complications. CONCLUSIONS: Colonoscopy-assisted cecostomy tube replacement is safe and highly successful in re-establishing lost cecostomy access in children after failed attempts with percutaneous or fluoroscopic-guided approaches.

Safety and Efficacy of the Push Endoscopic Technique in the Management of Esophageal Food Bolus Impactions in Children.

OBJECTIVES: Adult-based guidelines support the use of the pull (extraction) endoscopic technique in managing esophageal food bolus impactions (FBIs) but allow the consideration of the push (advancement) technique with caution based on available published literature. The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition guidelines mention the use of gentle endoscopic pushing for disimpaction but elaborate that this technique has not been studied in children. Our study aimed at assessing the safety and efficacy of the push technique in managing pediatric esophageal FBIs. METHODS: This was a retrospective cohort study of all pediatric patients presenting with esophageal FBIs to a pediatric tertiary care center from 2003 to 2016. RESULTS: Two hundred forty-two procedures for esophageal foreign body removal were reviewed. Thirty-nine procedures for managing esophageal FBIs were included in a total of 23 patients (1-4 procedures per patient). The most common underlying diagnoses were eosinophilic esophagitis and repaired tracheoesophageal fistula. The cohort had a median age of 8 years and median weight of 34.4 kg. Initial endoscopic disimpaction methods included 20 push and 19 pull technique attempts with success rates of 65% and 68%, respectively (P = 1.0). Unsuccessful attempts using 1 technique were successfully accomplished using the other technique. Patients initially managed by either of the 2 disimpaction techniques did not differ in age, weight, sex, or underlying diagnoses. No procedure-related complications were reported at our center. CONCLUSION: The present study shows that the push technique is as safe and effective as the pull technique in managing esophageal FBIs in pediatric patients.

Difficulty Achieving Vitamin D Sufficiency With High-Dose Oral Repletion Therapy in Infants With Cholestasis.

OBJECTIVES: Oral high-dose repletion vitamin D therapy, also known as stoss therapy, can be effective in the treatment of nutritional vitamin D deficiency rickets in infants and young children without liver disease and in patients with cystic fibrosis. There is no literature about this approach in infants with new-onset cholestasis. METHODS: This was a retrospective chart review of infants with cholestasis from March 2010 to March 2012 at a pediatric tertiary care center. Four cases satisfied the inclusion criteria, and were described in detail. RESULTS: All of the patients received oral high-dose repletion therapy with ergocalciferol (vitamin D2) 300,000 IU daily for 2 to 3 days. Follow-up vitamin D levels approximately 4 weeks later showed failure to achieve sufficiency levels (>20 ng/dL) in any patient. CONCLUSIONS: Unlike infants without liver disease, use of oral high-dose repletion therapy may not be adequate as treatment of vitamin D deficiency in the setting of cholestasis.

Is Total Pancreatectomy with Islet Autotransplantation A Reasonable Choice for Pediatric Pancreatitis?

Chronic pancreatitis is an emerging and poorly understood disease in childhood. Total pancreatectomy with islet cell autotransplantation is being proposed as a treatment for chronic pancreatitis and recent studies report a more favorable outcome in children compared to adults. Herein, we review the therapeutic alternatives for pediatric chronic pancreatitis, focusing primarily on TP/IAT.

Effects of 70% ethanol locks on rates of central line infection, thrombosis, breakage, and replacement in pediatric intestinal failure.

OBJECTIVES: Parenteral nutrition is essential for the growth and nutrition of patients with intestinal failure (IF). Catheter-related bloodstream infections (CRBSI) are a major complication of parenteral nutrition use. Few retrospective studies have shown that 70% ethanol lock (ETL) therapy for central lines can reduce the infection rate. Studies assessing line breakage, thrombosis, and replacement with the use of ETL are lacking in the pediatric literature. METHODS: The present study is a retrospective chart review, with a primary outcome of CRBSI rate per 1000 catheter days, and secondary outcomes of line thrombosis, line breakage requiring repair, and line replacement rates with use of ETL compared with heparin locks. Rates were compared using the Wilcoxon signed-rank test for paired nonparametric data. RESULTS: Seven patients satisfied the inclusion criteria that included having a single-lumen silicone central line exposed to both heparin and ETL therapy during the study period. There was a statistically significant decrease in overall and Gram-negative CRBSI rates per 1000 catheter days with a use of ETL therapy (10.3-1.4 per 1000 catheter days for overall CRBSIs, P = 0.02; 6.7-0 per 1000 catheter days for Gram-negative CRBSI, P = 0.03). There was an increasing trend in line thrombosis and repair rates with ETL therapy. Logistic regression analysis evaluating the impact of line luminal size on line repair rate showed a reduced risk of line repair when using larger-diameter central lines. CONCLUSIONS: ETL therapy is an effective method for decreasing CRBSI in patients with IF; however, it may have a negative impact on line integrity. Patients should be carefully selected when deciding on ETL therapy use for central line care. Studies are needed on the effect of different ethanol concentrations on infection rate and line integrity to optimize the outcome in this high-risk population.

Challenges in meeting fellowship procedural guidelines in pediatric therapeutic endoscopy and liver biopsy.

OBJECTIVE: The aims of this study were to assess the opportunities for therapeutic endoscopy, liver biopsies, and percutaneous endoscopic gastrostomy (PEG) placements available to fellows during a 3-year pediatric gastroenterology fellowship, and to evaluate access to ancillary procedural-training opportunities. METHODS: Data were collected from 12 pediatric gastroenterology fellowship programs in the United States. Procedures completed in the years 2009-2011 were queried using CPT codes and endoscopy databases. The maximal opportunity for procedures was based on the total procedures performed by the institution in 3 years divided by the total number of fellows in the program. The centers completed a questionnaire regarding ancillary opportunities for endoscopic training. RESULTS: There is significant variability in pediatric endoscopic training opportunities in specialized gastrointestinal (GI) procedures. Under the 1999 guidelines, no centers were able to meet the thresholds for polypectomy and control of nonvariceal bleeding. The 2013 guidelines allowed the number of programs reaching polypectomy thresholds to increase by 67% but made no difference for control of bleeding despite a decrease in the threshold. Training in PEG placement was not available in 42% of the surveyed centers. Elective ancillary procedural training is offered by 92% of the surveyed centers. CONCLUSIONS: Most training programs do not have the volume of therapeutic endoscopy procedures for all of the fellows to meet the training guidelines. Training in therapeutic endoscopy, PEG placement, and liver biopsy in pediatric GI fellowships should be supplemented using all of the possible options including rotations with adult GI providers and hands-on endoscopy courses. A shift toward evaluating competency via quality measures may be more appropriate.

Role of thiopurine metabolite testing and thiopurine methyltransferase determination in pediatric IBD.

Thiopurines have been used in inflammatory bowel disease (IBD) for >30 years, and measurements of both thiopurine methyltransferase (TPMT) and thiopurine (TP) metabolites, 6-thioguanine nucleotides (6-TGN) and 6-methylmercaptopurine (6-MMP), have been readily available. The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) Committee on Inflammatory Bowel Disease thought it appropriate to review the present indications for use of TPMT and TP metabolite testing. Substantial evidence demonstrates that TP therapy is useful for both Crohn disease and ulcerative colitis. Review of the existing data yielded the following recommendations. TPMT testing is recommended before initiation of TPs to identify individuals who are homozygote recessive or have extremely low TPMT activity, with the latter having more reliability than the former. Individuals who are homozygous recessive or have extremely low TPMT activity should avoid the use of TPs because of concerns for significant leukopenia. TMPT testing does not predict all cases of leukopenia and has no value to predict hypersensitivity adverse effects such as pancreatitis. Any potential value to reduce the risk of malignancy has not been studied. All individuals taking TPs should have routine monitoring with complete blood cell count and white blood cell count differential to evaluate for leukopenia regardless of TPMT testing results. Metabolite testing can be used to determine adherence with TP therapy. Metabolite testing can be used to guide dose increases or modifications in patients with active disease. Consideration would include either increasing the dose, changing therapy or for those with elevated transaminases or an elevated 6-MMP, using adjunctive allopurinol to help raise 6-thioguanine metabolites and suppress formation of 6-MMP. Routine and repetitive metabolite testing has little or no role in patients who are doing well and taking an acceptable dose of a TP.

Gastrojejunal feeding tube use by gastroenterologists in a pediatric academic center.

BACKGROUND AND OBJECTIVES: Enteral feeding through gastrojejunal (GJ) tubes is an established method of nutrition for patients with feeding difficulty who do not tolerate intragastric feedings. The pediatric literature about the long-term outcome, safety, and complications of different GJ tubes and placement methods is lacking. Our study aims to provide information about indications, techniques, and long-term outcome of GJ tube use in children. METHODS: Retrospective chart review for GJ tube placement procedures was used at our center for 10 years (1999-2009). Data collected included demographics, placement indications, underlying diagnosis, tube type, placement methods, complications, tube survival, and patient outcome. RESULTS: Thirty-three patients using GJ tubes were identified, with a total of 160 successful procedures documented (overall success rate of 97.6%). At initial placement, the mean age was 6 years (range 0.6-21.6) and the mean weight was 19.4 kg (range 6.6-72.2). Patients had a mean of 4.9 tubes placed per patient (range 1-20) during a follow-up of 26.8 months (range 0.4-115.3). The most common indications for replacement included accidental dislodgement, tube obstruction, coiling back into the stomach, and broken tube component. At the end of the study, 39% continued using GJ tubes, 30% were transitioned back to gastrostomy or oral feeds, and 15% underwent a surgical intervention. CONCLUSIONS: Long-term GJ tube use is possible and safe in children. Various feeding tubes and placement methods can be used by pediatric gastroenterologists to provide long-term jejunal feeds in children.

Polygenic Infantile Juvenile Polyposis Syndrome Managed With Sirolimus and Endoscopic Polypectomy.

In the following clinical case of infantile juvenile polyposis syndrome (JPS), administration of a pharmacologic agent sirolimus was associated with reduced disease burden without need for bowel resection. The positive impact included improvement in protein-losing enteropathy, decreased intestinal blood loss, and improved weight gain. In addition, the number of polyps resected per unit time and frequency of upper and lower endoscopic evaluation needed dropped after initiation of sirolimus. This case report describes a positive clinical outcome and discusses the use of sirolimus with aggressive polypectomy as a potential treatment for the rare disease entity of polygenic infantile JPS. Through this case, we aim to emphasize that while administration of this drug may mitigate many sequelae of infantile JPS, it does not appear to eliminate the need for aggressive polypectomy.

Impact of central venous catheter repair in pediatric intestinal failure.

OBJECTIVES: Patients with intestinal failure (IF) require long-term parenteral nutrition through central venous catheters (CVCs). When damaged, catheter replacement or repair is considered. Limited literature exists on repair outcomes in this population. We aimed to assess the impact of repair on durability of exiting CVCs and infection rates. METHODS: This was a retrospective cohort study of pediatric IF patients with tunneled silicone CVCs over 10 years. Outcomes were evaluated by assessing CVC longevity, repair success, replacement, and postrepair infection rates. RESULTS: One hundred thirty-eight repairs and 45 replacements were conducted in 37 patients with repair and replacement rates of 4.7 and 1.5 per 1000 catheter days, respectively. Twenty patients (54%) required ≥1 repair. For CVCs requiring repair, median CVC durability without and with repairs were at 123 and 391 days, respectively (P < .0001). Overall repair success rate was 96% with significantly lower success in the emergency department at 81% (P = .007). The 7-day postrepair infection rate was 2.2% without specific risk factors identified. Most repairs (76%) were performed by the Pediatric Gastroenterology division. Variability in practice was noted among services, including frequency of periprocedural antibiotic use and performance of temporary repairs before permanent repairs. A gradual increase in CVC repair rate was noted over time. CONCLUSIONS: Our study showed that CVC repair is effective in prolonging CVC durability in pediatric IF patients without increasing infection rates. Incorporating a temporary repair as a step before permanent repair may offer a route to address potential intraluminal thrombosis before permanent repair.

Anticipated impact of recent ethanol lock shortage and price increase on practice among pediatric intestinal rehabilitation programs across the United States.

BACKGROUND: Ethanol lock use has been associated with significantly lower rates of central line-associated bloodstream infection (CLABSI) in children with intestinal failure. Concerns have been raised among intestinal rehabilitation program providers regarding the impact of recent changes in cost and availability of ethanol locks in the US. METHODS: We conducted a survey among the members of the North American Society For Pediatric Gastroenterology, Hepatology & Nutrition Intestinal Rehabilitation Special Interest Group (NASPGHAN IR-SIG) regarding practice changes among providers to tackle this issue and the anticipated effect on CLABSI rates. RESULTS: The results show that the vast majority of US participants use ethanol locks in their population with intestinal failure, with most anticipating or already experiencing reduction in access to ethanol locks. Most worrisome is that more than half of participating programs expect an increase in CLABSI rates in this vulnerable patient population as a consequence of limited access to ethanol locks. CONCLUSION: Further multicenter prospective studies to assess the efficacy of alternative locking agents, besides ethanol, are needed in order to have readily available and affordable options for CLABSI prevention in the future.

Making the case for multidisciplinary pediatric aerodigestive programs.

Multidisciplinary pediatric aerodigestive centers have been proposed to address the needs of children with complex multi-system problems affecting the respiratory and upper gastrointestinal tracts. The setup of a multidisciplinary service allows for the complex coordination needed between different subspecialties. This allows for rapid communication and family-centered decision making and agreement on further diagnostic and/or therapeutic next steps such as offering triple endoscopy when indicated. Triple endoscopy entails performing rigid upper airway assessment, flexible bronchoscopy and upper gastrointestinal endoscopy and has been linked to reduced time to diagnosis/treatment, reduced costs and anesthesia exposure. This review summarizes the available literature on the structure and benefits of multidisciplinary pediatric aerodigestive services.

Refractory Pediatric Fibrostenotic Eosinophilic Esophagitis Treated With Dupilumab.

Eosinophilic esophagitis (EoE) is a progressive inflammatory disease of the esophagus. Untreated or uncontrolled disease over time can lead to the development of fibrosis and formation of strictures. Once the patient develops strictures, it is difficult to treat with the available medical therapies and will often require esophageal dilations. The Food and Drug Administration recently approved dupilumab for the treatment of EoE in patients older than 12 years. The clinical trials excluded patients with esophageal strictures. We describe a case of EoE with fibrostenotic stricture who had stricture resolution while on dupilumab therapy.

30% Ethanol Locks Are Effective in Preventing Central Line-Associated Bloodstream Infections in Pediatric Intestinal Failure: A Pilot Study.

BACKGROUND: Complications related to central venous lines (CVLs) are common in intestinal failure (IF), including central line-associated bloodstream infections (CLABSIs). Replacing heparin with 70% ethanol locks can reduce infection rates; however, concerns exist about potential negative effects on catheter integrity. We hypothesized that a lower ethanol concentration combined with citrate would prevent catheter-related complications, including CLABSIs. METHODS: This was a retrospective cohort study in pediatric IF patients assessing rates of CLABSIs, catheter repair, catheter replacement, and thrombolytic (alteplase) use for suspected catheter-related thromboses comparing heparin, 70% ethanol, and 30% ethanol-2.8% citrate locks. RESULTS: Six patients were included with a total of 8777 catheter days. All patients utilized silicone single-lumen CVLs for venous access. The rate of CLABSIs was highest with heparin at 13.9 events per 1000 catheter days, which significantly dropped to 1.6 per 1000 catheter days on 70% ethanol (P = .02) and remained significantly low at 0.4 per 1000 catheter days when transitioning to 30% ethanol-2.8% citrate locks as compared with heparin (P = .01). No statistically significant differences were noted among the groups in rates of catheter repair and replacement and alteplase use. CONCLUSION: This pilot study provides supportive evidence that a lower ethanol lock concentration may be effective in maintaining a low rate of CLABSIs in pediatric IF patients using silicone CVLs. Although this study did not show significant positive impact on catheter integrity and durability, there are other potential benefits to using lower ethanol concentrations including reduced systemic ethanol exposure in children.