International Core Outcome Set for Acute Simple Appendicitis in Children: Results of a Systematic Review, Delphi Study, and Focus Croups With Young People.

OBJECTIVE: To develop an international core outcome set (COS), a minimal collection of outcomes that should be measured and reported in all future clinical trials evaluating treatments of acute simple appendicitis in children. SUMMARY OF BACKGROUND DATA: A previous systematic review identified 115 outcomes in 60 trials and systematic reviews evaluating treatments for children with appendicitis, suggesting the need for a COS. METHODS: The development process consisted of 4 phases: (1) an updated systematic review identifying all previously reported outcomes, (2) a 2-stage international Delphi study in which parents with their children and surgeons rated these outcomes for inclusion in the COS, (3) focus groups with young people to identify missing outcomes, and (4) international expert meetings to ratify the final COS. RESULTS: The systematic review identified 129 outcomes which were mapped to 43 unique outcome terms for the Delphi survey. The first-round included 137 parents (8 countries) and 245 surgeons (10 countries), the second-round response rates were 61% and 85% respectively, with 10 outcomes emerging with consensus. After 2 young peoples' focus groups, 2 additional outcomes were added to the final COS (12): mortality, bowel obstruction, intraabdominal abscess, recurrent appendicitis, complicated appendicitis, return to baseline health, readmission, reoperation, unplanned appendectomy, adverse events related to treatment, major and minor complications. CONCLUSION: An evidence-informed COS based on international consensus, including patients and parents has been developed. This COS is recommended for all future studies evaluating treatment ofsimple appendicitis in children, to reduce heterogeneity between studies and facilitate data synthesis and evidence-based decision-making.

Management of Gastroschisis: Results From the NETS2G Study, a Joint British, Irish, and Canadian Prospective Cohort Study of 1268 Infants.

OBJECTIVE: In infants with gastroschisis, outcomes were compared between those where operative reduction and fascial closure were attempted ≤24 hours of age (PC), and those who underwent planned closure of their defect >24 hours of age following reduction with a pre-formed silo (SR). SUMMARY OF BACKGROUND DATA: Inadequate evidence exists to determine how best to treat infants with gastroschisis. METHODS: A secondary analysis was conducted of data collected 2006-2008 using the British Association of Pediatric Surgeons Congenital Anomalies Surveillance System, and 2005-2016 using the Canadian Pediatric Surgery Network.28-day outcomes were compared between infants undergoing PC and SR. Primary outcome was number of gastrointestinal complications. Interactions were investigated between infant characteristics and treatment to determine whether intervention effect varied in sub-groups of infants. RESULTS: Data from 341 British and Irish infants (27%) and 927 Canadian infants (73%) were used. 671 infants (42%) underwent PC and 597 (37%) underwent SR. The effect of SR on outcome varied according to the presence/absence of intestinal perforation, intestinal matting and intestinal necrosis. In infants without these features, SR was associated with fewer gastrointestinal complications [aIRR 0.25 (95% CI 0.09-0.67, P = 0.006)], more operations [aIRR 1.40 (95% CI 1.22-1.60, P < 0.001)], more days PN [aIRR 1.08 (95% CI 1.03-1.13, P < 0.001)], and a higher infection risk [aOR 2.06 (95% CI 1.10-3.87, P = 0.025)]. In infants with these features, SR was associated with a greater number of operations [aIRR 1.30 (95% CI 1.17-1.45, P < 0.001)], and more days PN [aIRR 1.06 (95% CI 1.02-1.10, P = 0.003)]. CONCLUSIONS: In infants without intestinal perforation, matting, or necrosis, the benefits of SR outweigh its drawbacks. In infants with these features, the opposite is true. Treatment choice should be based upon these features.

Abdominal Tuberculosis in an Infant Presenting With a Small Bowel Obstruction.

ABSTRACT: Abdominal tuberculosis (TB) is rare in children and usually spread in the peritoneum or gastrointestinal tract. Symptoms tend to be vague and nonspecific, with no extra-abdominal involvement, presenting a challenge for clinicians and delayed diagnosis. Postnatally acquired abdominal TB is most commonly transmitted through inhalation or ingestion of respiratory droplets with Mycobacterium tuberculosis from the mother.Abdominal TB in infants is rare. We present a case of a 2-month-old infant presenting with an acute bowel obstruction secondary to abdominal TB acquired through contact with maternal TB mastitis. This unique case emphasizes the importance of considering abdominal TB in the differential for at-risk infants presenting with small bowel obstruction.

Consensus Guidelines for Perioperative Care in Neonatal Intestinal Surgery: Enhanced Recovery After Surgery (ERAS®) Society Recommendations.

BACKGROUND: Enhanced Recovery After Surgery (ERAS®) Society guidelines integrate evidence-based practices into multimodal care pathways that have improved outcomes in multiple adult surgical specialties. There are currently no pediatric ERAS® Society guidelines. We created an ERAS® guideline designed to enhance quality of care in neonatal intestinal resection surgery. METHODS: A multidisciplinary guideline generation group defined the scope, population, and guideline topics. Systematic reviews were supplemented by targeted searching and expert identification to identify 3514 publications that were screened to develop and support recommendations. Final recommendations were determined through consensus and were assessed for evidence quality and recommendation strength. Parental input was attained throughout the process. RESULTS: Final recommendations ranged from communication strategies to antibiotic use. Topics with poor-quality and conflicting evidence were eliminated. Several recommendations were combined. The quality of supporting evidence was variable. Seventeen final recommendations are included in the proposed guideline. DISCUSSION: We have developed a comprehensive, evidence-based ERAS guideline for neonates undergoing intestinal resection surgery. This guideline, and its creation process, provides a foundation for future ERAS guideline development and can ultimately lead to improved perioperative care across a variety of pediatric surgical specialties.

Improving value and access to specialty medical care for families: a pediatric surgery telehealth program

Background: In Canada, access to subspecialty surgical services for children imposes inconvenience and financial hardship on geographically remote families. The purpose of this study was to evaluate a recently implemented pediatric surgical telehealth pilot program from the family and provider perspectives. Methods: Enabled by an existing telehealth infrastructure for pediatric subspecialty medicine and mental health, a pilot telehealth program for surgical consultation was established by a single surgeon in British Columbia. Following establishment of eligibility criteria, patients from remote communities requiring new consultation or clinical follow-up were offered a telehealth alternative. At the end of the encounter, both the parent and patient (if appropriate) provided feedback via a questionnaire. Provider satisfaction was also assessed via a questionnaire. We estimated costs avoided and analyzed data on pediatric surgery consultation wait time. Results: Between September 2014 and November 2017, 80 patients were seen in 19 remote telehealth centres, 23 as new referrals and 57 in follow-up consultation. Among new referrals, the commonest diagnosis was chest wall deformity. The average travel distance avoided was 705 km, with an estimated direct cost avoidance of $585. Sixty-four families (80%) completed the questionnaire. Almost all (63 [98%]) indicated high overall satisfaction with the telehealth experience. Provider satisfaction was similarly high, in terms of both the technology user interface and clinical effectiveness. Overall pediatric surgical consultation wait times were unaffected. Conclusion: Implementation of telehealth technology in a pediatric surgical practice offered high value to patients/families and, from the provider's perspective, yielded an acceptable alternative to in-person assessment.

Contexte: Au Canada, l'accès aux services chirurgicaux surspécialisés pour enfants est source d'inconvénients et de difficultés financières pour les familles vivant en région éloignée. L'objectif de cette étude était d'évaluer le point de vue des familles et des fournisseurs de services sur un programme pilote de télésanté pédiatrique récemment mis en oeuvre. Méthodes: Ce programme pilote de consultation chirurgicale à distance a été mis sur pied par un chirurgien de Colombie-Britannique, qui s'est servi d'une infrastructure de télésanté préexistante permettant la prestation de services surspécialisés de médecine et de santé mentale pour enfants. Une fois leur admissibilité établie, les patients vivant en région éloignée et ayant besoin d'une consultation ou d'un suivi clinique se sont vu offrir des services de télésanté. Après la consultation, les parents et le patient (lorsque c'était approprié) ont rempli un formulaire de rétroaction. La satisfaction des fournisseurs de soins a été également évaluée au moyen d'un questionnaire. Nous avons estimé les coûts évités et analysé les données sur le temps d'attente pour une consultation en chirurgie pédiatrique. Résultats: Entre septembre 2014 et novembre 2017, 80 patients ont consulté 19 centres de télésanté : 23 pour une nouvelle consultation et 57 pour un suivi. Parmi les nouveaux cas, le diagnostic le plus fréquent était une malformation de la paroi thoracique. En moyenne, 705 km de déplacements ont été évités, ce qui représente un coût estimé de 585 $. Soixante-quatre familles (80 %) ont rempli le questionnaire, et presque toutes (63 [98 %]) se sont dites globalement satisfaites de leur expérience de télésanté. La satisfaction des fournisseurs de soins était également élevée, tant en ce qui a trait à l'interface utilisateur des outils technologiques qu'à l'efficacité clinique. Le programme n'a eu aucun effet sur le temps d'attente général pour une consultation en chirurgie pédiatrique. Conclusion: L'utilisation de services de télésanté en chirurgie pédiatrique a été d'une grande valeur pour les patients et leur famille. Du point de vue des fournisseurs de soins, elle a constitué une solution de rechange acceptable aux consultations en personne.

Aggressive Surgical Management of Congenital Diaphragmatic Hernia: Worth the Effort?: A Multicenter, Prospective, Cohort Study.

OBJECTIVE: The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. BACKGROUND: Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. METHODS: Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA. RESULTS: A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P < 0.05) and increased incidence of anomalies (P < 0.0001). Low-volume centers ("Lo", n=44 total, < 10 CDH pts/yr) and high-volume centers ("Hi", n = 21) had median nonrepair rates of 19.8% (range 0%-66.7%) and 16.7% (5.1%-38.5%), respectively. High-volume centers were further dichotomized by rate of nonrepair (HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P < 0.0001). For every 100 CDH patients, HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation. CONCLUSIONS: There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.

Conservative therapy for appendicitis in children.

Question A 10-year-old girl who was seen in my office last week with acute-onset abdominal pain and fever was referred to an emergency department, was diagnosed with appendicitis, and was treated conservatively with antibiotics, without surgery. Has the paradigm for treating appendicitis changed, and which is the preferred treatment of appendicitis in children: antibiotics or appendectomy?Answer For more than 100 years, surgical management was the principal treatment of acute appendicitis. Potential adverse events associated with appendectomy include bleeding, surgical site infection, and ileus, as well as stress for children and their parents. The option of treating appendicitis with antibiotics has been known for decades, which has led to consideration of antibiotics alone as a therapeutic alternative to surgery for uncomplicated appendicitis. While there is a reasonable body of evidence in support of this practice in adults, the accumulation of evidence of the safety and effectiveness of non-operative management in children is ongoing. Large studies are still needed, and those are being conducted at this time, with results expected in the next few years.

Management of gastroschisis.

PURPOSE OF REVIEW: The diagnosis and treatment of gastroschisis spans the perinatal disciplines of maternal fetal medicine, neonatology, and pediatric surgery. Since gastroschisis is one of the commonest and costliest structural birth defects treated in neonatal ICUs, a comprehensive review of its epidemiology, prenatal diagnosis, postnatal treatment, and short and long-term outcomes is both timely and relevant. RECENT FINDINGS: The incidence of gastroschisis has increased dramatically over the past 20 years, leading to a renewed interest in causation. The widespread availability of maternal screening and ultrasound results in very high rates of prenatal diagnosis, which enables evaluation of the optimal timing and mode of delivery. The preferred method of surgical closure continues to be an issue of debate among pediatric surgeons, whereas postsurgical treatment seeks to expedite the initiation and progression of enteral feeding and minimize complications. A small subset of babies with complex gastroschisis leading to intestinal failure benefit from the knowledge and expertise of dedicated interdisciplinary teams, which seek to bring novel therapies and improved clinical outcomes. SUMMARY: The opportunities to increase the knowledge of causation, and identify best practices leading to improved outcomes, drive the ongoing need for collaborative clinical research in gastroschisis.

Recommendations for surgical safety checklist use in Canadian children's hospitals.

BACKGROUND: There is ample evidence that avoidable harm occurs in patients, including children, who undergo surgical procedures. Among a number of harm mitigation strategies, the use of surgical safety checklists (SSC) is now a required organizational practice for accreditation in all North American hospitals. Although much has been written about the effects of SSC on outcomes of adult surgical patients, there is a paucity of literature on the use and role of the SSC as an enabler of safe surgery for children. METHODS: The Pediatric Surgical Chiefs of Canada (PSCC) advocates on behalf of all Canadian children undergoing surgical procedures. We undertook a survey of the use of SSC in Canadian children's hospitals to understand the variability of implementation of the SSC and understand its role as both a measure and driver of patient safety and to make specific recommendations (based on survey results and evidence) for standardized use of the SSC in Canadian children's hospitals. RESULTS: Survey responses were received from all 15 children's hospitals and demonstrated significant variability in how the checklist is executed, how compliance is measured and reported, and whether or not use of the checklist resulted in specific instances of error prevention over a 12-month observation period. There was near unanimous agreement that use of the SSC contributed positively to the safety culture of the operating room. CONCLUSION: Based on the survey results, the PSCC have made 5 recommendations regarding the use of the SSC in Canadian children's hospitals.

CONTEXTE: Il a été prouvé maintes fois que les patients, y compris les enfants, sur qui des interventions chirurgicales sont pratiquées peuvent subir des méfaits évitables. Les normes d'agrément exigent dorénavant de tous les hôpitaux d'Amérique du Nord qu'ils utilisent une liste de contrôle de la sécurité chirurgicale, l'une des stratégies susceptibles de réduire ces méfaits. Si de nombreux articles portent sur l'efficacité d'une telle liste lors d'interventions chirurgicales sur des patients adultes, peu d'études ont été publiées sur l'utilisation et le rôle de ces listes dans le contexte de la sécurité des interventions chirurgicales effectuées sur des enfants. MÉTHODES: L'organisme Pediatric Surgical Chiefs of Canada (PSCC) représente tous les enfants canadiens qui subissent des interventions chirurgicales. Nous avons mené un sondage auprès des hôpitaux canadiens pour enfants pour déterminer le degré de mise en oeuvre de la liste de contrôle d'un endroit à l'autre, pour comprendre son rôle dans l'évaluation et l'amélioration de la sécurité des patients et pour formuler des recommandations précises (d'après les données probantes et les résultats obtenus) afin d'en normaliser l'utilisation dans les hôpitaux pour enfants du Canada. RÉSULTANTS: Les 15 hôpitaux pour enfants ont tous répondu à notre sondage. Leurs réponses étaient très variées en ce qui concerne la mise en oeuvre de la liste de contrôle, la façon dont ils évaluent et documentent le respect de la liste et l'efficacité de celle-ci à prévenir des erreurs concrètes pendant la période de 12 mois à l'étude. Les répondants ont indiqué presque à l'unanimité que la liste de contrôle favorisait grandement une culture de sécurité en salle d'opération. CONCLUSION: À partir de ces résultats, le PSCC a formulé 5 recommandations concernant l'utilisation de la liste de contrôle dans les hôpitaux pour enfants du Canada.

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.

Delivery planning for pregnancies with gastroschisis: findings from a prospective national registry.

OBJECTIVE: The purpose of this study was to determine the influence of planned mode and planned timing of delivery on neonatal outcomes in infants with gastroschisis. STUDY DESIGN: Data from the Canadian Pediatric Surgery Network cohort were used to identify 519 fetuses with isolated gastroschisis who were delivered at all tertiary-level perinatal centers in Canada from 2005-2013 (n = 16). Neonatal outcomes (including length of stay, duration of total parenteral nutrition, and a composite of perinatal death or prolonged exclusive total parenteral nutrition) were compared according to the 32-week gestation planned mode and timing of delivery with the use of the multivariable quantile and logistic regression. RESULTS: Planned induction of labor was not associated with decreased length of stay (adjusted median difference, -2.6 days; 95% confidence interval [CI], -9.9 to 4.8), total parenteral nutrition duration (adjusted median difference, -0.2 days; 95% CI, -6.4 to 6.0), or risk of the composite adverse outcome (relative risk, 1.7; 95% CI, 0.1-3.2) compared with planned vaginal delivery after spontaneous onset of labor. Planned delivery at 36-37 weeks' gestation was not associated with decreased length of stay (adjusted median difference, 5.9 days; 95% CI, -5.7 to 17.5), total parenteral nutrition duration (adjusted median difference, 3.2 days; 95% CI, -7.9 to 14.3), or risk of composite outcome (relative risk, 2.3; 95% CI, 0.8-5.4) compared with planned delivery at ≥38 weeks' gestation. CONCLUSION: Infants with gastroschisis who were delivered after planned induction or planned delivery at 36-37 weeks' gestation did not have significantly better neonatal outcomes than planned vaginal delivery after spontaneous onset of labor and planned delivery at ≥38 weeks' gestation.

Maternal risk factors for gastroschisis in Canada.

BACKGROUND: Gastroschisis is a congenital abdominal wall defect that occurs in one per 2200 pregnancies. Birth defect surveillance in Canada has shown that the prevalence of gastroschisis has increased threefold over the past 10 years. The purpose of this study was to compare maternal exposures data from a national gastroschisis registry with pregnancy exposures from vital statistics to understand maternal risk factor associations with the occurrence of gastroschisis. METHODS: Using common definitions, pregnancy cohorts were developed from two databases. The Canadian Pediatric Surgery Network database, a population-based dataset was used to record maternal exposures for women who experienced a gastroschisis pregnancy, while a contemporaneous, geographically cross-sectional "control" cohort of pregnant women and their exposures was developed from Canadian Community Health Survey data. Groups comparison of maternal risk factors was performed using univariate and multivariate logistic generalized estimating equation techniques. RESULTS: A total of 692 gastroschisis pregnancies (from Canadian Pediatric Surgery Network) and 4708 pregnancies from Canadian Community Health Survey were compared. Younger maternal age (odds ratio, 0.85; 95% confidence interval, 0.83-0.87; p < 0.0001), smoking (odds ratio, 2.86; 95% confidence interval, 2.22-3.66; p < 0.0001), a history of pregestational or gestational diabetes (odds ratio, 2.81; 95% confidence interval, 1.42-5.5; p = 0.0031), and use of medication to treat depression (odds ratio, 4.4; 95% confidence interval, 1.38-11.8; p = 0.011) emerged as significant associations with gastroschisis pregnancies. CONCLUSION: Gastroschisis in Canada is associated with maternal risk factors, some of which are modifiable. Further studies into sociodemographic birth defect risk are necessary to allow targeted improvements in perinatal health service delivery and health policy.

Maternal and community predictors of gastroschisis and congenital diaphragmatic hernia in Canada.

PURPOSE: The incidence of gastroschisis (GS) has increased globally. Maternal age and smoking are risk factors and aboriginal communities may be more commonly affected. Factors leading to this increased incidence are otherwise unclear. We investigate maternal sociodemography, air pollution and personal risk factors comparing mothers of infants with GS with a control group of infants with diaphragmatic hernia (CDH) in a large population-based analysis. METHODS: Data were collected from a national, disease-specific pediatric surgical database (May 2006-June 2013). Maternal community sociodemographic information was derived from the Canadian 2006 Census. Univariate and multivariable analyses were performed examining maternal factors related to diagnosis of GS. RESULTS: GS infants come from poorer, less educated communities with more unemployment, less pollution, fewer immigrants, and more aboriginal peoples than infants with CDH. Teen maternal age, smoking, and illicit drug use, are associated with GS. CONCLUSION: Mothers of infants with GS are younger, more likely to smoke and come from socially disadvantaged communities with higher proportions of aboriginal peoples but lower levels of air pollution compared to mothers of CDH infants. Identification of maternal risks provides direction for prenatal screening and public health interventions.

Advances in the Surgical Treatment of Gastroschisis.

Gastroschisis (GS) is a structural defect of the anterior abdominal wall, usually diagnosed antenatally, that occurs with a frequency of approximately 4 per 10,000 pregnancies. Babies born with GS require neonatal intensive care and surgical management of the abdominal wall defect soon after birth. Although contemporary survival rates for GS are over 90%, these babies are at risk for significant morbidity, and require 4 to 6 weeks of costly, resource-intensive care in specialized neonatal units. Much consideration has been given to how best to treat the abdominal wall defect of GS. The traditional approach, necessitated by a need to establish enteral feeding as quickly as possible, consists of early postnatal visceral reduction and sutured abdominal closure. Advances in neonatal nutritional support have enabled the development of surgical approaches, which permit gradual visceral reduction and delayed abdominal closure. In cases where early visceral reduction cannot be achieved, delayed closure enabled by the initial placement of a prosthetic silo has been a live-saving alternative. The development of preformed silos has simplified their use and led to an interest in treating all cases with a delayed closure philosophy. Most recently, a sutureless technique of abdominal closure has been reported, which has the benefit of avoiding general anesthesia and offers other outcome improvements over sutured closure of the defect. The debate over primary closure versus silo placement and delayed closure continues to receive much attention. The goal of this article is to review historical aspects of gastroschisis closure, and then focus on current surgical techniques, including the innovative sutureless closure, with an analysis of the comparative clinical effectiveness of these approaches to treatment of the abdominal wall defect in GS.

The impact of a care bundle with an emphasis on hemodynamic assessment on the short-term outcomes in neonates with congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the short-term outcomes of implementing a care bundle emphasizing frequent hemodynamic assessments by echocardiography in neonates with congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective cohort study of infants with CDH admitted to a quaternary perinatal unit from January 2013 to March 2021. The primary composite outcome was defined as mortality or use of extracorporeal membrane oxygenation or need for respiratory support at discharge. RESULTS: We identified 37 and 20 CDH infants in Epoch I and II, respectively. More patch repairs (50% vs. 21.9%, p = 0.035) and echocardiograms (6[4-8] vs. 1[0-5], p = 0.003) were performed in Epoch II. While there were no differences in the primary outcome, there was a reduction in mortality in Epoch II (0% vs. 27%, p = 0.01). CONCLUSION: With the implementation of a CDH care bundle with an emphasis on hemodynamic assessment, we demonstrated a significant reduction in mortality.

Association of Exclusive Breast Milk Intake and Outcomes in Infants With Uncomplicated Gastroschisis: A National Cohort Study.

BACKGROUND: Enteral feeding is an essential part of the management of infants with gastroschisis. We hypothesized that exclusive breast milk is associated with improved neonatal outcomes. METHODS: We conducted a retrospective review of infants with uncomplicated gastroschisis through the Canadian Pediatric Surgery Network (CAPSNet) and Canadian Neonatal Network (CNN). The primary outcome was time to full enteral feeds. RESULTS: We identified 411 infants with gastroschisis treated at CAPSNet centres from 2014 to 2022. 144 patients were excluded due to gestational age <32 weeks, birth weight <1500 g, other congenital anomalies, or complicated gastroschisis. Of the remaining 267 participants, 78% (n = 209) received exclusive breast milk diet in the first 28 days of life, whereas 22% (n = 58) received supplemental or exclusive formula. Infants who received exclusive breast milk experienced higher time to reach full enteral feeding (median 24 vs 22 days, p = 0.047) but were more likely to have undergone delayed abdominal closure (32% vs 17%, p = 0.03). After adjustment, there were no significant differences between groups in time to reach full enteral feeds, duration of parenteral nutrition, or length of stay. Infants who received supplemental or exclusive formula had a similar risk of necrotizing enterocolitis (4% vs 3%) but were less likely to transition to exclusive breast milk at discharge (73% vs 11%, p < 0.001). CONCLUSION: Early use of exclusive breast milk in infants with uncomplicated gastroschisis is associated with similar outcomes compared to supplemental or exclusive formula. Patients who received supplemental or exclusive formula were unlikely to transition to exclusive breastfeeding by discharge. LEVEL OF EVIDENCE: Level IIb (Individual Cohort Study).

Decreased ß-catenin Protein in Lungs From Human Congenital Diaphragmatic Hernia Archival Pathology Specimens: A Case-control Study.

BACKGROUND: Lung hypoplasia contributes to congenital diaphragmatic hernia (CDH) associated morbidity and mortality. Changes in lung wingless-type MMTV integration site family member (Wnt)-signalling and its downstream effector beta-catenin (CTNNB1), which acts as a transcription coactivator, exist in animal CDH models but are not well characterized in humans. We aim to identify changes to Wnt-signalling gene expression in human CDH lungs and hypothesize that pathway expression will be lower than controls. METHODS: We identified 51 CDH cases and 10 non-CDH controls with archival formalin-fixed paraffin-embedded (FFPE) autopsy lung tissue from 2012 to 2022. 11 liveborn CDH cases and an additional two anterior diaphragmatic hernias were excluded from the study, leaving 38 CDH cases. Messenger ribonucleic acid (mRNA) expression of Wnt-signalling effectors WNT2B and CTNNB1 was determined for 19 CDH cases and 9 controls. A subset of CDH cases and controls lung sections were immunostained for ß-catenin. Clinical variables were obtained from autopsy reports. RESULTS: Median gestational age was 21 weeks. 81% (n = 31) of hernias were left-sided. 47% (n = 18) were posterolateral. Liver position was up in 81% (n = 31) of cases. Defect size was Type C or D in 58% (n = 22) of cases based on autopsy photos, and indeterminable in 42% (n = 16) of cases. WNT2B and CTNNB1 mRNA expression did not differ between CDH and non-CDH lungs. CDH lungs had fewer interstitial cells expressing ß-catenin protein than non-CDH lungs (13.2% vs 42.4%; p = 0.006). CONCLUSION: There appear to be differences in the abundance and/or localization of ß-catenin proteins between CDH and non-CDH lungs. LEVEL OF EVIDENCE: Level III. TYPE OF STUDY: Case-Control Study.

Diagnosis and management of congenital diaphragmatic hernia: a 2023 update from the Canadian Congenital Diaphragmatic Hernia Collaborative.

OBJECTIVE: The Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative sought to make its existing clinical practice guideline, published in 2018, into a 'living document'. DESIGN AND MAIN OUTCOME MEASURES: Critical appraisal of CDH literature adhering to Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Evidence accumulated between 1 January 2017 and 30 August 2022 was analysed to inform changes to existing or the development of new CDH care recommendations. Strength of consensus was also determined using a modified Delphi process among national experts in the field. RESULTS: Of the 3868 articles retrieved in our search that covered the 15 areas of CDH care, 459 underwent full-text review. Ultimately, 103 articles were used to inform 20 changes to existing recommendations, which included aspects related to prenatal diagnosis, echocardiographic evaluation, pulmonary hypertension management, surgical readiness criteria, the type of surgical repair and long-term health surveillance. Fifteen new CDH care recommendations were also created using this evidence, with most related to the management of pain and the provision of analgesia and neuromuscular blockade for patients with CDH. CONCLUSIONS: The 2023 Canadian CDH Collaborative's clinical practice guideline update provides a management framework for infants and children with CDH based on the best available evidence and expert consensus.