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.

High frequency jet ventilation for congenital diaphragmatic hernia.

BACKGROUND: The optimal role of high frequency jet ventilation (HFJV) in lung protective stabilization of congenital diaphragmatic hernia (CDH) remains uncertain. We aimed to describe our center's experience with HFJV as both a rescue (following failed stabilization with CMV) and primary ventilation mode in the management of CDH. METHODS: Liveborn CDH patients treated from 2013 to 2021 in a single institution were reviewed. We compared 3 groups based on their primary and last ventilation mode prior to surgery: CMV (Group 1); HFJV (Group 2); and CMV/HFJV (Group 3). Outcomes included a composite primary outcome (≥1 of mortality, need for ECMO or need for supplemental O2 at discharge), total invasive ventilation days and development of pneumothorax. A descriptive analysis including univariate group comparisons was performed. Multivariate logistic regression models investigating the relationship between mode of ventilation and the primary outcome adjusted by potentially confounding covariates were constructed. RESULTS: 56 patients (32 Group 1, 18 Group 2, 6 Group 3) were analyzed. Group 2 and 3 patients had more severe disease based on liver position, SNAP-II score, pulmonary hypertension severity, need for inotropic support, CDHSG defect size and need for patch repair. There were no group differences in survival, need for ECMO, or pneumothorax occurrence, although infants receiving HFJV required longer invasive ventilation and had a greater need for O2 at discharge. Multivariate logistic regression revealed no associations between mode of ventilation and outcome. CONCLUSIONS: HFJV appears effective, both for CMV rescue and as a primary ventilation strategy in high risk CDH. LEVEL OF EVIDENCE: Level IV.

Disparities in surgical health service delivery and outcomes for indigenous children.

BACKGROUND: Evidence of health disparities for Indigenous children requiring surgical care is lacking. We present a systematic review of the literature examining possible disparities in surgical care and outcomes for pediatric patients of Indigenous ethnicity. DATA SOURCES: PubMed, Cochrane, MEDLINE, gray literature. METHODS: Literature review, using PubMed, Cochrane, MEDLINE, and gray literature was conducted to identify articles published more than 2010-2020 examining children's surgical health service delivery (epidemiology, access, operations provided) and outcomes for pediatric patients of Indigenous ethnicity compared with others. Extracted data included study design, setting, participant race/ethnicity, operations examined, and surgical outcomes. Article quality was assessed using the Newcastle-Ottawa Scales. RESULTS: From 411 abstracts, 125 articles were reviewed and 33 included for data abstraction. These were cohort and cross-sectional studies investigating a wide range of patient populations and procedures across the United States, Canada, Australia, and New Zealand. Articles were organized naturally by theme into birth malformations (15 articles), trauma (6 articles), pediatric general surgery/appendicitis (5 articles), pediatric otolaryngology (6 articles), and renal transplant (1 article) surgery. Four articles also described access and resource utilization related to inpatient care. Notable disparities observed included apparent increased prevalence of gastroschisis, rates of traumatic fatality, non accidental injury, and self harm among North American Indigenous children. CONCLUSIONS: Indigenous children appear to be vulnerable to a number of health and treatment outcome disparities related to conditions treated by surgeons. Surgeons are thus uniquely poised to act in identifying and eliminating Indigenous ethnicity-based pediatric health disparities.

Practice Summary of Antimicrobial Therapy for Commonly Encountered Conditions in the Neonatal Intensive Care Unit: A Canadian Perspective.

Neonates are highly susceptible to infections owing to their immature cellular and humoral immune functions, as well the need for invasive devices. There is a wide practice variation in the choice and duration of antimicrobial treatment, even for relatively common conditions in the NICU, attributed to the lack of evidence-based guidelines. Early decisive treatment with broad-spectrum antimicrobials is the preferred clinical choice for treating sick infants with possible bacterial infection. Prolonged antimicrobial exposure among infants without clear indications has been associated with adverse neonatal outcomes and increased drug resistance. Herein, we review and summarize the best practices from the existing literature regarding antimicrobial use in commonly encountered conditions in neonates.

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.

The medical and surgical management of gastroschisis.

Gastroschisis (GS) is a full-thickness abdominal wall defect in which fetal intestine herniates alongside the umbilical cord into the intrauterine cavity, resulting in an intestinal injury of variable severity. An increased prevalence of gastroschisis has been observed across several continents and is a focus of epidemiologic study. Prenatal diagnosis of GS is common and allows for delivery planning and treatment in neonatal intensive care units (NICUs) by collaborative interdisciplinary teams (neonatology, neonatal nursing and pediatric surgery). Postnatal treatment focuses on closure of the defect, optimized nutrition, complication avoidance and a timely transition to enteral feeding. Babies born with complex GS are more vulnerable to complications, have longer and more resource intensive hospital stays and benefit from standardized care pathways provided by teams with expertise in managing infants with intestinal failure. This article will review the current state of knowledge related to the medical and surgical management and outcomes of gastroschisis with a special focus on the role of the neonatologist in supporting integrated team-based care.

Case Reports: Management Challenges in Pediatric Germ Cell Tumors.

Germ cell tumors in infants are most frequently extragonadal, benign, and amenable to surgical resection. An unusual feature of germ cell tumors is the potential coexistence of malignant with benign disease which makes it possible for patients with incompletely resected tumors to experience either a benign or malignant recurrence. A challenge to postoperative surveillance is the interpretation of serum alpha fetoprotein, a marker of malignancy, that is physiologically elevated during the first year of life. A rare subset of germ cell tumors occur in the retroperitoneum. Although the vast majority are benign, these tumors are often large and distort normal anatomy, and may demonstrate local invasiveness that increases risk of resection. The intent of these reports is to caution readers about these unusual features of germ cell tumors of infancy.

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.

Initial experience of the use of ethylene-vinyl alcohol polymer (EVOH) as an alternative technique for lung nodule localization prior to VATS.

Identifying pulmonary nodules for resection that are small or are deep within the lung parenchyma is a frequently encountered challenge during video-assisted thoracoscopic surgery (VATS). Several image-guided localizing techniques have been described; however, there is limited literature on using these techniques in pediatric patients. We assessed the feasibility of using a commercially available ethylene-vinyl alcohol polymer (EVOH) as an alternative technique for lung nodule localization prior to VATS. We describe our experience of successful EVOH lung nodule localization in three pediatric patients with an oncologic history presenting with new lung nodules.

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 the paediatric surgery patient experience: an 8-year analysis of narrative quality data.

BACKGROUND: Narrative data about the patient experience of surgery can help healthcare professionals and administrators better understand the needs of patients and their families as well as provide a foundation for improvement of procedures, processes and services. However, units often lack a methodological framework to analyse these data empirically and derive key areas for improvement. The American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) is aimed at improving the quality of surgical care by collecting patient data and reporting risk-adjusted surgical outcomes for each participant hospital in the programme. Though qualitative data about patient experience are captured as part of the NSQIP database, to date no framework or methodology has been proposed, or reported on, to analyse these data for the purposes of quality improvement. The goal of this study was to demonstrate the feasibility of using content analysis to empirically derive key areas for quality improvement from a sample of 3601 narrative comments about paediatric surgery from patients and families at British Columbia Children's Hospital. STUDY DESIGN: Thematic content analysis conducted on a total of 3601 patient and family narratives received between 2011 and 2018. RESULTS: Overall satisfaction with care was high and experiences with healthcare providers at the hospital were positive. Areas for improvement were identified in the themes of health outcomes, communication and surgery timelines. Results informed follow-up interprofessional quality improvement initiatives. CONCLUSIONS: Recording and analysing patient experience data as part of validated quality improvement programmes such as ACS NSQIP can provide valuable and actionable information to improve quality of care.

Improving the diagnostic accuracy of appendicitis using a multidisciplinary pathway.

BACKGROUND/PURPOSE: Improvement opportunities exist in the accuracy and timeliness of the diagnosis of childhood appendicitis. The purpose of our study was to conduct a post-implementation audit of a diagnostic pathway for children with suspected appendicitis presenting to our pediatric emergency department. METHODS: We adopted a diagnostic pathway that utilized a validated risk of appendicitis stratification tool (Alvarado Score) with protocolized use of abdominal ultrasound for moderate risk patients. We conducted a 10% convenience sample audit of pathway patients treated over the subsequent 18-month period. Outcome measures included false negative and positive rates, sensitivity, specificity, and overall pathway accuracy. RESULTS: One hundred thirty-four pathway patients, of which 22 (16.4%) had appendicitis confirmed pathologically, were evaluated. The risk group distribution of patients was: low risk (29%), moderate risk (60%), and high risk (11%). The negative appendectomy rate was 4.4% (reduced from 14% pre-pathway), and the false negative (missed appendicitis) rate was 3.0%. No patients received CT scans. Pathway sensitivity was 81.8%% (95% CI 59.7% to 94.8%), specificity-92.9%% (95% CI 86.4%-96.9%), and overall accuracy-91.0% (95% CI 84.9%-95.3%). CONCLUSION: Implementation of a diagnostic pathway achieved a high level of accuracy and reduced our institutional negative appendectomy rate by 67%. The audit identified additional pathway improvement opportunities. LEVELS OF EVIDENCE: Level IV.

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.

Immediate versus staged repair of omphaloceles.

Omphalocele is an abdominal wall defect involving the umbilical ring which results in visceral herniation of small and large intestine, liver, spleen and sometimes gonads. The covering of the herniated viscera by a fused membrane consisting of peritoneum, Wharton's jelly and amnion projects viscera from mechanical injury and exposure to chemical irritants in amniotic fluid. Omphalocele is usually diagnosed before birth, is variable in size, and is frequently associated with chromosomal and somatic anomalies, syndromes, and variable degrees of pulmonary hypoplasia which can be lethal. In this article we examine surgical closure options for omphaloceles ranging from early primary fascial repair for small omphaloceles to a staged repair, often facilitated by an amnion preserving silo, which may be necessary for giant omphaloceles that cannot be closed primarily. We also review some of the adjuncts to abdominal wall reconstruction including tissue expansion and mesh. Conservative management (paint and wait) of giant omphaloceles is described elsewhere.

CAPS at 50: Now comfortably seated with the grownups.

The Canadian Association of Pediatric Surgeons was established 50 years ago, with 30 founding members. Since then, the CAPS membership has grown nearly 10-fold and has developed a global presence with representation from 15 countries. CAPS has 8 pediatric surgery training programs which contribute not only to the North American workforce, but also to profoundly underserved low and middle income countries, particularly in Africa. On the occasion of our 50th Anniversary meeting, we celebrate our diversity and inclusivity as we reflect on our past, on the contributions of our Founding Members, the Women of CAPS, and others who have had a transformational impact on the practice of pediatric surgery. We look forward to our future, which while unknown, holds great promise for future generations of pediatric surgeons and the children and families in need of their care.

Creation of an Enhanced Recovery After Surgery (ERAS) Guideline for neonatal intestinal surgery patients: a knowledge synthesis and consensus generation approach and protocol study.

INTRODUCTION: Enhanced Recovery After Surgery (ERAS) guidelines integrate evidence-based practices into multimodal care pathways designed to optimise patient recovery following surgery. The objective of this project is to create an ERAS protocol for neonatal abdominal surgery. The protocol will identify and attempt to bridge the gaps between current practices and best evidence. Our study is the first paediatric ERAS protocol endorsed by the International ERAS Society. METHODS: A research team consisting of international clinical and family stakeholders as well as methodological experts have iteratively defined the scope of the protocol in addition to individual topic areas. A modified Delphi method was used to reach consensus. The second phase will include a series of knowledge syntheses involving a rapid review coupled with expert opinion. Potential protocol elements supported by synthesised evidence will be identified. The Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) system will be used to determine strength of recommendations and the quality of evidence. The third phase will involve creation of the protocol using a modified RAND/UCLA Appropriateness Method. Group consensus will be used to rate each element in relation to the quality of evidence supporting the recommendation and the appropriateness for guideline inclusion. This protocol will form the basis of a future implementation study. ETHICS AND DISSEMINATION: This study has been registered with the ERAS Society. Human ethics approval (REB 18-0579) is in place to engage patient families within protocol development. This research is to be published in peer-reviewed journals and will form the care standard for neonatal intestinal surgery.

New anthropometric classification scheme of preoperative nutritional status in children: a retrospective observational cohort study.

OBJECTIVE: WHO uses anthropometric classification scheme of childhood acute and chronic malnutrition based on low body mass index (BMI) ('wasting') and height for age ('stunting'), respectively. The goal of this study was to describe a novel two-axis nutritional classification scheme to (1) characterise nutritional profiles in children undergoing abdominal surgery and (2) characterise relationships between preoperative nutritional status and postoperative morbidity. DESIGN: This was a retrospective observational cohort study. SETTING: The setting was 50 hospitals caring for children in North America that participated in the American College of Surgeons National Surgical Quality Improvement Program Paediatric from 2011 to 2013. PARTICIPANTS: Children >28 days who underwent major abdominal operations were identified. INTERVENTIONS/MAIN PREDICTOR: The cohort of children was divided into five nutritional profile groups based on both BMI and height for age Z-scores: (1) underweight/short, (2) underweight/tall, (3) overweight/short, (4) overweight/tall and (5) non-outliers (controls). MAIN OUTCOME MEASURES: Multiple variable logistic regressions were used to quantify the association between 30-day morbidity and nutritional profile groups while adjusting for procedure case mix, age and American Society of Anaesthesiologists class. RESULTS: A total of 39 520 cases distributed as follows: underweight/short (656, 2.2%); underweight/tall (252, 0.8%); overweight/short (733, 2.4%) and overweight/tall (1534, 5.1%). Regression analyses revealed increased adjusted odds of composite morbidity (35%) and reintervention events (75%) in the underweight/short group, while overweight/short patients had increased adjusted odds of composite morbidity and healthcare-associated infections (43%), and reintervention events (79%) compared with controls. CONCLUSION: Stratification of preoperative nutritional status using a scheme incorporating both BMI and height for age is feasible. Further research is needed to validate this nutritional risk classification scheme for other surgical procedures in children.