The promise and pitfalls of care standardization in congenital diaphragmatic hernia.

The aim of standardizing care is to enhance patient outcomes and optimize healthcare delivery by minimizing variations in care and ensuring the efficient allocation of healthcare resources. Despite these potential benefits to patients, healthcare providers and the healthcare system, standardization may also disadvantage these groups. With a specific focus on congenital diaphragmatic hernia, this article will review the promise and pitfalls of standardization, as well as a potential path forward that uses standardization to improve outcomes in this rare and complex disease process.

How can pediatric surgeons address racism and become actively anti-racist?

BACKGROUND/PURPOSE: This is an article submitted on behalf of the Canadian Association of Pediatric Surgeons. We assert that Pediatric Surgeons must work to dismantle systemic racism. Pediatric Surgeons have expertise in both common and rare surgical diseases affecting patients ranging from premature neonates to adolescents. Thus, our professional obligation is to transform our health and social systems to prevent the harms of racism to our patients. METHODS: Specific to the Canadian context, we describe a brief history, the ongoing impact on individuals and communities, and the harmful effect on the surgical community and trainees. Finally, we developed a series of practical recommendations to help surgeons become actively anti-racist. RESULTS: Four primary recommendations are made: (1) Increasing and supporting anti-racism education; (2) Changing individual behaviours to combat racism; (3) Developing strategies for organizational change; and (4) Committing to diversity in leadership. CONCLUSION: As surgeons, we are actors of change, and we can take meaningful steps to combat racism in our health systems. LEVEL OF EVIDENCE: V.

The First Two Years of the Association of Pediatric Surgery Training Program Directors (APSTPD) Transition to Fellowship Course: Lessons Learned and Future Directions.

OBJECTIVE: The first transition to fellowship course for incoming pediatric surgery fellows was held in the US in 2018 and the second in 2019. The course aimed to facilitate a successful transition in to fellowship by introduction of the professional, patient care, and technical aspects unique to pediatric surgery training. The purpose of this study was to evaluate the feasibility and effectiveness of the first two years of this course in the US and discuss subsequent evolution of this endeavor. DESIGN: This is a descriptive and qualitative analysis of two years' experience with the Association of Pediatric Surgery Training Program Directors' (APSTPD) Transition to Fellowship course. Course development and curriculum, including clinical knowledge, soft skills, and hands-on skills labs, are presented. Participating incoming fellows completed multiple choice, boards-style pre- and post-tests. Scores were compared to determine if knowledge was effectively transferred. Participants also completed post-course evaluations and subsequent 3- or 12-month surveys inquiring on the lasting impact of the course on their transition into fellowship. Standard univariate statistics were used to present results. SETTING: The first APSTPD Transition to Fellowship course was held at the Johns Hopkins Hospital in Baltimore, Maryland in 2018, and the second course was held at the Oregon Health and Science University in Portland, Oregon in 2019. PARTICIPANTS: All fellows entering ACGME-certified Pediatric Surgery fellowships in the United States were invited to participate. Twenty fellows accepted and attended in 2018, and fourteen fellows participated in 2019. RESULTS: There were 34 incoming pediatric surgery fellow participants over 2 years. Faculty represented more than 10 institutions each year. Pre- and post-test scores were similar between years, with a significant improvement of scores after completion of the course (67±10% vs 79±8%, p < 0.001). Feedback from participants was overwhelmingly positive, with skills labs being attendees' favorite component. When asked about usefulness of individual course sessions, more attendees found clinical sessions more useful than soft skills (93% vs 73%, p = 0.011). Almost all (90%) of participants reported the course met its stated purpose and would recommend the course to future fellows. This was further reflected on 3 and 12 month follow up surveys wherein 85% stated they found the course helpful during the first few months of fellowship and 90% would still recommend it. CONCLUSIONS: A transition to fellowship course in the US for incoming pediatric surgery fellows is logistically feasible, effective in transfer of knowledge, and highly regarded among attendees. Feedback from each course has been used to improve the subsequent courses, ensuring that it remains a valuable addition to pediatric surgical training in the US.

Congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a rare birth defect characterized by incomplete closure of the diaphragm and herniation of fetal abdominal organs into the chest that results in pulmonary hypoplasia, postnatal pulmonary hypertension owing to vascular remodelling and cardiac dysfunction. The high mortality and morbidity rates associated with CDH are directly related to the severity of cardiopulmonary pathophysiology. Although the aetiology remains unknown, CDH has a polygenic origin in approximately one-third of cases. CDH is typically diagnosed with antenatal ultrasonography, which also aids in risk stratification, alongside fetal MRI and echocardiography. At specialized centres, prenatal management includes fetal endoscopic tracheal occlusion, which is a surgical intervention aimed at promoting lung growth in utero. Postnatal management focuses on cardiopulmonary stabilization and, in severe cases, can involve extracorporeal life support. Clinical practice guidelines continue to evolve owing to the rapidly changing landscape of therapeutic options, which include pulmonary hypertension management, ventilation strategies and surgical approaches. Survivors often have long-term, multisystem morbidities, including pulmonary dysfunction, gastroesophageal reflux, musculoskeletal deformities and neurodevelopmental impairment. Emerging research focuses on small RNA species as biomarkers of severity and regenerative medicine approaches to improve fetal lung development.

Risk stratification helps identify congenital diaphragmatic hernia (CDH) infants in need of formal neurodevelopmental assessment: Observations from a structured, interdisciplinary long-term follow-up clinic.

BACKGROUND/PURPOSE: Neurodevelopmental delay (NDD) affects congenital diaphragmatic hernia (CDH) infants. Initial assessment by experienced developmental pediatricians, supported by Bayley-3 tests, is a viable pathway for NDD identification and surveillance. We risk stratified CDH infants to observe differences in incidence and type of NDD based on disease severity. METHODS: Patients from a CDH long-term follow-up database started in 2012 were reviewed (REB#2019-4583). Risk stratification into low, moderate, and high-risk cohorts was performed using the CDH Study Group Mortality Prediction Score. Patients requiring ECLS, supplemental oxygen at 30 days and patch repair were also considered high-risk (i.e. usual clinical criteria). Post-discharge NDD assessments by developmental pediatricians and occupational therapists (Bayley-3) were analyzed for all patients >18months. NDD incidence and type per risk group was determined using descriptive statistics. RESULTS: Of 102 CDH patients included for study, 26% (27/102) had NDD. Risk stratification identified 2(2%), 7(7%), and 18(18%) patients with NDD in the low, moderate and high-risk groups, respectively. Language delay (2 low; 6 moderate; 10 high) was the most prevalent. Three patients had both expressive and receptive language delay. Motor deficits were observed almost exclusively in the high-risk group. CONCLUSION: Based on our experience, NDD affects one-quarter of CDH infants. Risk stratification helped identify infants at increased risk of NDD. While language delays predominated across all risk groups, multiple deficits occurred in higher risk cohorts. These patients should receive structured NDD assessment as part of an optimal interdisciplinary CDH care pathway.

The Canadian Congenital Diaphragmatic Hernia Collaborative Mobile App: A National Guideline Implementation Strategy.

OBJECTIVE: Coinciding with the publication of the Canadian congenital diaphragmatic hernia (CDH) Collaborative's clinical practice guidelines (CPG), we developed a mobile smartphone app to increase guideline utilization and promote knowledge translation. STUDY DESIGN: This mobile app was organized into sections corresponding to the phases of CDH care (prenatal, perinatal/postnatal, and child/adolescent), and contained 22 recommendations supported by evidence summaries, PubMed links, levels of evidence, and strength of expert consensus. Download statistics were collected from September 2018 to June 2020 after release of two iOS versions and an Android platform. Data regarding user numbers/location, most visited sections, and individual session details were analyzed. RESULTS: During the study period, the CDH app had 1,586 users predominantly from Canada (40%), United States (30%), and Brazil (12%). The Android release increased app visibility, particularly in Brazil, which had the largest number of new users. Of 3,723 sessions, roughly one-third were returning users. The average session duration and screens viewed/session was 4 minutes and seven screens, respectively. Postnatal ventilation was the most frequently visited subsection after prenatal diagnosis/risk stratification. Measurement of observed-to-expected lung head ratio was the most visited individual recommendation. The guideline compliance checklist was the most frequently accessed resource highlighting its utility. CONCLUSION: The CDH app is an innovative platform to disseminate guidelines. The increasing global reach of the app suggests worldwide CPG relevance. With additional features planned, the CDH app will continue to support clinical decision-making and empower patients and families as they navigate the short and long-term challenges associated with CDH. KEY POINTS: · Mobile smartphone technology provides an optimal platform for guideline dissemination.. · International uptake supports worldwide CPG relevance.. · Future initiatives include the development of patient and family resources..

The future is here! Pediatric surgery and the move to the royal college of physicians and surgeons of Canada's competence by design.

This interactive session was held at the 51st Annual Meeting of the Canadian Association of Pediatric Surgeons (CAPS) in preparation for the transition of Pediatric Surgery training in Canada to Competency by Design (a CBME-based model of residency training developed by the Royal College of Physicians and Surgeons of Canada).

Respiratory disorders in patients with omphalocele.

The respiratory difficulties experienced by infants with omphalocele are being appreciated with greater frequency. These problems represent self-limited difficulties related to omphalocele closure or are the result of severe pulmonary disease including pulmonary hypoplasia and pulmonary hypertension. Infants with giant omphalocele represent a unique group that may experience increased respiratory morbidity which may lead to chronic respiratory problems extending into childhood and adolescence. Importantly, respiratory insufficiency at birth is an independent predictor of mortality for patients with omphalocele. In this review, we will provide a summary of the respiratory difficulties experienced by patients with omphalocele as well as insight into management and surveillance.

Preoperative cardiopulmonary evaluation in specific neonatal surgery.

Preoperative assessment of surgical neonates often relates to issues of prematurity, low birth weight, or associated malformations. This review explores the preoperative cardiopulmonary evaluation in specific newborn surgical populations, the role of echocardiography in congenital diaphragmatic hernia perioperative management, the impact of bronchopulmonary dysplasia in the ex-preterm surgical neonate and a brief discussion on the risk of general anesthesia and specific anesthetic considerations for any surgical neonate. Newborns with congenital anomalies requiring early general surgical intervention should have an assessment for congenital heart disease. In the asymptomatic neonate, a thorough physical exam may be sufficient preoperatively. Neonates born with esophageal atresia or anorectal malformations should have a full evaluation for VACTERL associations. Initial echocardiography in congenital diaphragmatic hernia is used to evaluate anatomy, but there is emerging evidence to suggest the use of echocardiography in the ongoing surveillance of CDH to influence the timing of surgical intervention. Bronchopulmonary dysplasia is present in up to 40% of ex-premature neonates and increases the risk of postoperative apneas and need for ventilatory support. However, all surgical neonates have an increased risk of post-operative apneas, and the need for surgical intervention should be balanced with the risk of general anesthesia.

A transition to discipline curriculum for pediatric surgery trainees: Evaluation of a pediatric surgery boot camp from 2017 to 2018.

INTRODUCTION: Boot camps seek to impart knowledge and skills for individuals entering new roles. We sought to evaluate knowledge, skills, and confidence of in-coming pediatric surgery trainees with a 2.5-day pediatric surgery boot camp. METHODS: A curriculum included key aspects of pediatric surgery delivered during interactive lectures, small group discussions, and simulation. With REB approval, participant demographics were collected. Pre- and posttests assessed knowledge and trainee confidence. Comparative statistics and multivariate analysis of variance (MANOVA) were performed. RESULTS: Between 2017 and 2018, 16 individuals from North American pediatric surgery training programs participated in two boot camps. Ten had North American general surgery training, and eleven had no pediatric surgery exposure ≥1 year prior. All participants expressed increased confidence with course material after boot camp [F(18,11) = 3.137;p < 0.05]. Performance improved significantly (pre- vs. posttests, 47.0% vs. 62.4%; p < 0.05). MANOVA between faculty and trainees demonstrated agreement on the value of individual sessions [F(15,3) = 0.642;p = 0.76]. Neonatal bowel obstruction, gastrostomy tube complications, esophageal atresia, pain management, and informed consent were rated most useful. CONCLUSION: Trainees and teaching faculty considered the boot camp valuable. Trainees demonstrated significant improvements in core knowledge and confidence. The initial pediatric surgery boot camp experience shows promise in facilitating the transition to discipline for new trainees. STUDY TYPE: Prospective treatment study. LEVEL OF EVIDENCE: IV.

Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes.

PURPOSE: Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers. METHODS: Prenatal lung-head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. RESULTS: Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values >45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. CONCLUSIONS: This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review. LEVEL OF EVIDENCE: IV.

Risk stratification and outcome determinants in gastroschisis.

Selection of outcome determinants and risk stratification are necessary to identify patients at higher risk for morbidity and mortality. This facilitates human and material resource allocation and allows for improved family counseling. While several different factors, including prenatal ultrasonographic bowel features, the timing and mode of delivery, and the features of bowel injury have been investigated in gastroschisis, there is still significant debate as to which of these best predicts outcome. This article reviews the different outcome predictors and risk prognostication schemata currently available in the literature to help guide clinicians caring for infants with gastroschisis.

Loop versus divided colostomy for the management of anorectal malformations: a systematic review and meta-analysis.

BACKGROUND: The ideal colostomy type for patients with anorectal malformations (ARM) is undetermined. We performed a systematic review and meta-analysis of short-term complications comparing loop and divided colostomies. METHODS: After review registration (PROSPERO: CRD42016036481), multiple databases were searched for comparative studies without language or date restrictions. Gray literature was sought. Complications investigated included stomal prolapse/hernia/retraction, wound infections, and urinary tract infections (UTIs). Two reviewers independently assessed study eligibility and the quality of included studies. Meta-analysis of selected complications was performed using Revman 5.3, with p<0.05 considered significant. RESULTS: Twenty-six studies were included, and four were multi-institutional. Reporting standards were highly variable. Studies scored between 6 and 9 of possible nine stars on the NOS. Overall, 3866 neonates with ARM were incorporated, in which 2241 loop colostomies and 1994 divided colostomies were reported. Of 10 studies reporting short-term complications, the overall rate was 27%. Meta-analysis demonstrated no significant difference in the incidence of UTIs, (OR: 2.55 [0.76, 8.58], p=0.12), while loop colostomies had a significantly higher prolapse rate (See figure). No publication bias was noted. CONCLUSIONS: A colostomy for patients with an ARM is a source of considerable morbidity. Divided colostomies reduce the risk of subsequent prolapse and may represent the preferred approach. LEVEL OF EVIDENCE: 3A.

Gastrojejunostomy tube complications - A single center experience and systematic review.

PURPOSE: Gastrojejunostomy tubes (GJTs) enable enteral nutrition in infants/children with feeding intolerance. However, complications may be increased in small infants. We evaluated our single-institution GJT complication rate and systematically reviewed existing literature. METHODS: With REB approval, a retrospective single-institution analysis of GJT placements between 2009 and 2015 was performed. For the systematic review, MOOSE guidelines were followed. RESULTS: At our institution, 48 children underwent 154/159 successful insertions primarily for gastroesophageal reflux (n=27; 55%) and aspiration (n=11; 23%). Median age at first GJT insertion was 2.2years (0.2-18). Thirty-five (73%) had an index insertion when ≤10kg. GJTs caused 2 perforations and 1 death. The systematic review assessed 48 articles representing 2726 procedures. Overall perforation rate was estimated as 2.1% (n=36 studies, 23/1092, 95% CI: 1.0-3.2). Perforation rates in children <10kg versus ≥10kg were estimated as 3.1%/procedure (95% CI: 1.1%-5.0%) and 0.1%/procedure (95% CI: 0%-0.3%), respectively. The relative risk of perforation was 9.4 (95% CI: 2.8-31.3). Overall mortality was estimated as 0.9%/patient (n=39 studies; 95% CI: 0.2-1.6%). Most perforations (19/23; 83%) occurred ≤30days of attempted tube placement. CONCLUSION: Gastrojejunostomy tubes are associated with significant complications and frequently require revision/replacement. Insertion in patients <10kg is associated with increased perforation risk. Caution is warranted in this subgroup. LEVEL OF EVIDENCE: Level II.

Outcome prediction in gastroschisis - The gastroschisis prognostic score (GPS) revisited.

PURPOSE: The GPS enables risk stratification for gastroschisis and helps discriminate low from high morbidity groups. The purpose of this study was to revalidate GPS's characterization of a high morbidity group and to quantify relationships between the GPS and outcomes. METHODS: With REB approval, complete survivor data from a national gastroschisis registry was collected. GPS bowel injury scoring was revalidated excluding the initial inception/validation cohorts (>2011). Length of stay (LOS), 1st enteral feed days (dFPO), TPN days (dTPN), and aggregate complications (COMP) were compared between low and high morbidity risk groups. Mathematical relationships between outcomes and integer increases in GPS were explored using the entire cohort (2005-present). RESULTS: Median (range) LOS, dPO, and dTPN for the entire cohort (n=849) was 36 (26,62), 13 (9,18), and 27 (20,46) days, respectively. High-risk patients (GPS≥2; n=80) experienced significantly worse outcomes than low risk patients (n=263). Each integer increase in GPS was associated with increases in LOS and dTPN by 16.9 and 12.7days, respectively (p<0.01). COMP rate was also increased in the high-risk cohort (46.3% vs. 22.8%; p<0.01). CONCLUSION: The GPS effectively discriminates low from high morbidity risk groups. Within the high risk group, integer increases in GPS produce quantitatively differentiated outcomes which may guide initial counseling and resource allocation. LEVEL OF EVIDENCE: IIb.

The Canadian Pediatric Surgery Network Congenital Diaphragmatic Hernia Evidence Review Project: Developing national guidelines for care.

The Canadian Pediatric Surgery Network (CAPSNet) has been collecting population-based data regarding congenital diaphragmatic hernia (CDH) across its 17 perinatal sites since 2005. With >500 infants registered to date, CAPSNet has addressed many critical knowledge gaps pertaining to CDH care. Most importantly, it has identified variability in both CDH practice and outcome across Canada. Using the successful Evidence-based Practice for Improving Quality (EPIQ) method, CAPSNet is undertaking a national, multidisciplinary effort to standardize best practices for CDH, from prenatal diagnosis to hospital discharge, based on the best available evidence. The present article outlines the value of clinical research networks and the process CAPSNet will undertake to produce national consensus guidelines for CDH care.

Le Réseau canadien de chirurgie pédiatrique recueille des données en population sur la hernie diaphragmatique congénitale (HDC) dans 17 sites périnatals depuis 2005. Puisque plus de 500 nourrissons y sont inscrits jusqu'à présent, le Réseau a corrigé de nombreuses lacunes liées aux soins de la HDC. Qui plus est, il a décelé la variabilité dans la pratique et les résultats de la HDC au Canada. Selon la méthode EPIC (un acronyme anglais qui signifie pratique fondée sur des données probantes pour améliorer la qualité), le Réseau déploie des efforts nationaux et multidisciplinaires pour normaliser les pratiques exemplaires en matière de soins de la HDC, du diagnostic prénatal au congé de l'hôpital, d'après les meilleures données probantes. Le présent article souligne la valeur des réseaux de recherche clinique et le processus que le Réseau entreprendra pour produire des lignes directrices consensuelles nationales sur les soins de la HDC.

Flap versus fascial closure for gastroschisis: a systematic review and meta-analysis.

BACKGROUND: Flap closure represents an alternative to fascial closure for gastroschisis. We performed a systematic review and meta-analysis of outcomes comparing these techniques. METHODS: A registered systematic review ( PROSPERO: CRD42015016745) of comparative studies was performed, querying multiple databases without language or date restrictions. Gray literature was sought. Outcomes analyzed included: mortality, ventilation days, feeding parameters, length of stay (LOS), wound infection, resource utilization, and umbilical hernia incidence. Multiple reviewers independently assessed study eligibility and literature quality. Meta-analysis of outcomes was performed where appropriate (Revman 5.2). RESULTS: Twelve studies met inclusion criteria, of which three were multi-institutional. Quality assessment revealed unbiased patient selection and exposure, but group comparability was suboptimal in four studies. Overall, 1124 patients were evaluated, of which 350 underwent flap closure (210 immediately; 140 post-silo). Meta-analysis revealed no significant differences in mortality, LOS, or feeding parameters between groups. Flap patients had less wound infections (OR 0.40 [95%CI 0.22-0.74], P=0.003). While flap patients had an increased risk of umbilical hernia, they were less likely to undergo repair (19% vs. 41%; P=0.01). CONCLUSIONS: Flap closure has equivalent or superior outcomes to fascial closure for patients with gastroschisis. Given potential advantages of bedside closure and reduced sedation requirements, flap closure may represent the preferred closure strategy.

The Canadian Pediatric Surgery Network (CAPSNet): Lessons Learned from a National Registry Devoted to the Study of Congenital Diaphragmatic Hernia and Gastroschisis.

The Canadian Pediatric Surgery Network (CAPSNet) was created in 2005 by a geographically representative, multidisciplinary group of clinicians and researchers with the intent of establishing a national research registry for gastroschisis (GS) and congenital diaphragmatic hernia (CDH). Since then, CAPSNet has used this registry and its 16-center network to make contributions to the knowledge base informing best practices for GS and CDH care. More recently, CAPSNet has expanded its focus to include quality assurance and improvement at each of its sites, by issuing a benchmarked outcomes "report card" with its annual report. Finally, a major objective of CAPSNet has been to establish and adopt standardized, evidence-based practice guidelines for GS and CDH across all Canadian perinatal centers.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee.

OBJECTIVE: Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS: Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS: Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS: Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.