GAPS phase II: development and pilot results of the global assessment in pediatric surgery, an evidence-based pediatric surgical capacity assessment tool for low-resource settings.

PURPOSE: Pediatric surgical care in low- and middle-income countries is often hindered by systemic gaps in healthcare resources, infrastructure, training, and organization. This study aims to develop and validate the Global Assessment of Pediatric Surgery (GAPS) to appraise pediatric surgical capacity and discriminate between levels of care across diverse healthcare settings. METHODS: The GAPS Version 1 was constructed through a synthesis of existing assessment tools and expert panel consultation. The resultant GAPS Version 2 underwent international pilot testing. Construct validation categorized institutions into providing basic or advanced surgical care. GAPS was further refined to Version 3 to include only questions with a > 75% response rate and those that significantly discriminated between basic or advanced surgical settings. RESULTS: GAPS Version 1 included 139 items, which, after expert panel feedback, was expanded to 168 items in Version 2. Pilot testing, in 65 institutions, yielded a high response rate. Of the 168 questions in GAPS Version 2, 64 significantly discriminated between basic and advanced surgical care. The refined GAPS Version 3 tool comprises 64 questions on: human resources (9), material resources (39), outcomes (3), accessibility (3), and education (10). CONCLUSION: The GAPS Version 3 tool presents a validated instrument for evaluating pediatric surgical capabilities in low-resource settings.

Treatment and Outcomes of Congenital Ovarian Cysts A Study by the Canadian Consortium for Research in Pediatric Surgery (CanCORPS).

OBJECTIVE: We conducted a multicenter study to assess treatments and outcomes in a national cohort of infants with congenital ovarian cysts. SUMMARY BACKGROUND DATA: Wide variability exists in the treatment of congenital ovarian cysts. The effects of various treatment strategies on outcomes, specifically ovarian preservation, are not known. METHODS: Female infants diagnosed with congenital intra-abdominal cysts between 2013 and 2017 at 10 Canadian pediatric surgical centers were retrospectively evaluated. Sonographic characteristics, median time to cyst resolution, incidence of ovarian preservation, and predictors of surgery were evaluated. Subgroup analyses were performed in patients with complex cysts and cysts ≥40 mm in diameter. RESULTS: The study population included 189 neonates. Median gestational age at diagnosis and median maximal prenatal cyst diameter were 33 weeks and 40 mm, respectively. Cysts resolved spontaneously in 117 patients (62%), 14 (7%) prenatally, and the remainder at a median age of 124 days. Intervention occurred in 61 patients (32%), including prenatal aspiration (2, 3%), ovary sparing resection (14, 23%), or oophorectomy (45, 74%). Surgery occurred at a median age of 7.4weeks. Independent predictors of surgery included postnatal cyst diameter ≥40 mm [odds ratio (OR) 6.19, 95% confidence interval (CI) 1.66-35.9] and sonographic complex cyst character (OR 63.6, 95% CI 10.9-1232). There was no significant difference in the odds of ovarian preservation (OR 3.06, 95% CI 0.86 -13.2) between patients who underwent early surgery (n = 22) and those initially observed for at least 3 months (n = 131). CONCLUSIONS: Most congenital ovarian cysts are asymptomatic and spontaneously resolve. Early surgical intervention does not increase ovarian preservation.

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.

A recurrent PDGFRB mutation causes familial infantile myofibromatosis.

Infantile myofibromatosis (IM) is the most common benign fibrous tumor of soft tissues affecting young children. By using whole-exome sequencing, RNA sequencing, and targeted sequencing, we investigated germline and tumor DNA in individuals from four distinct families with the familial form of IM and in five simplex IM cases with no previous family history of this disease. We identified a germline mutation c.1681C>T (p.Arg561Cys) in platelet-derived growth factor receptor ß (PDGFRB) in all 11 affected individuals with familial IM, although none of the five individuals with nonfamilial IM had mutations in this gene. We further identified a second heterozygous mutation in PDGFRB in two myofibromas from one of the affected familial cases, indicative of a potential second hit in this gene in the tumor. PDGFR-ß promotes growth of mesenchymal cells, including blood vessels and smooth muscles, which are affected in IM. Our findings indicate p.Arg561Cys substitution in PDGFR-ß as a cause of the dominant form of this disease. They provide a rationale for further investigations of this specific mutation and gene to assess the benefits of targeted therapies against PDGFR-ß in aggressive life-threatening familial forms of the disease.

The Canadian Biliary Atresia Registry: Improving the care of Canadian infants with biliary atresia.

Biliary atresia is the most common cause of end-stage liver disease and liver cirrhosis in children, and the leading indication for liver transplantation in the paediatric population. There is no cure for biliary atresia; however, timely diagnosis and early infant age at surgical intervention using the Kasai portoenterostomy optimize the prognosis. Late referral is a significant problem in Canada and elsewhere. There is also a lack of standardized care practices among treating centres in this country. Biliary atresia registries currently exist across Europe, Asia and the United States. They have provided important evidence-based information to initiate changes to biliary atresia care in their countries with improvements in outcome. The Canadian Biliary Atresia Registry was initiated in 2013 for the purpose of identifying best standards of care, enhancing public education, facilitating knowledge translation and advocating for novel national public health policy programs to improve the outcomes of Canadian infants with biliary atresia.

L'atrésie des voies biliaires est la principale cause d'insuffisance hépatique terminale et de cirrhose chez les enfants, et la première indication de transplantation du foie au sein de la population d'âge pédiatrique. Aucun traitement ne guérit l'atrésie des voies biliaires, mais un diagnostic rapide et le jeune âge du nourrisson au moment de l'intervention chirurgicale par hépato-porto-entérostomie de Kasai optimisent le pronostic. L'orientation tardive vers un spécialiste constitue un problème important au Canada et ailleurs. Par ailleurs, il n'existe pas de protocole de soins standardisés dans les centres de traitement du pays. On trouve des registres d'atrésie des voies biliaires en Europe, en Asie et aux États-Unis, lesquels ont fourni de l'information importante fondée sur des données probantes pour susciter des changements aux soins de cette affection dans ces pays et favoriser une amélioration des résultats. Le Registre canadien d'atrésie des voies biliaires a été créé en 2013 pour définir les meilleures normes de soins, améliorer l'éducation publique, favoriser le transfert des connaissances et prôner de nouveaux programmes de politiques en santé publique en vue d'améliorer le sort des nourrissons canadiens présentant une atrésie des voies biliaires.

Dextranomer hyaluronic acid copolymer effects on gastroesophageal junction.

OBJECTIVE: The outcomes of fundoplication for gastroesophageal reflux disease are suboptimal in many children, and alternatives are clearly needed. Dextranomer hyaluronic acid (DxHA) copolymer, an agent with proven efficacy in vesicoureteral reflux, was studied with respect to its effects on the gastroesophageal junction (GEJ). METHODS: Twelve New Zealand white rabbits underwent measurement of lower esophageal sphincter pressure followed by laparotomy and injection into the muscular layer of the GEJ (controls, 1.0 mL saline; low-dose DxHA [0.5 mL]; high-dose DxHA [1.0 mL]). After a 12-week survival period, the animals underwent manometry, sacrifice, and necropsy. Organs were examined histologically by pathologists blinded to the injection delivered. RESULTS: All animals survived. Weight gain was equal in the 3 groups. There was no significant difference in mean lower esophageal sphincter pressure from baseline in any group (control 2.3 mmHg [95% confidence interval, CI -3.3 to 7.9]; low-dose group 3.2 mmHg [95% CI -0.8 to 7.2]; high-dose group -4.0 mmHg [95% CI -18.95 to 10.95]). Histologically, DxHA injection produced an intramural implant, with a foreign body giant cell reaction, and fibroblastic infiltration with collagen deposition. High-dose injection did not consistently result in a qualitative increase in the magnitude of the reaction. There was no mucosal injury or luminal stenosis. CONCLUSIONS: In this first study evaluating the effects of DxHA injection at the GEJ, a histologic bulking effect was observed without obvious functional complications. The agent may have a role in the treatment of gastroesophageal reflux disease.

Determinants of postoperative abscess occurrence and percutaneous drainage in children with perforated appendicitis.

PURPOSE: Postoperative abscesses after perforated appendicitis have no clear risk factors or indications for percutaneous drainage. Our study addressed these two issues. METHODS: A logistic regression model was used to delineate risk factors for postoperative abscess in children with perforated appendicitis treated during a recent 5-year period. Drainage of abscess was compared to antibiotic treatment. RESULTS: Postoperative abscess occurred in 42 (14.8%) of 284 patients. Higher WBC count, presence of bowel obstruction at presentation, diffuse peritonitis with a dominant abscess at surgery, and one specific surgeon were significantly associated with postoperative abscess, while fever or pain requiring narcotics at the time of abscess diagnosis was significantly associated with drainage. Compared to non-drainage, those drained had longer hospital stay including readmissions (15.9 ± 5.3 vs. 12.2 ± 4.6 days, p < 0.005) and less readmissions (9.5 vs. 33.3%, p = 0.06). Over the 5-year period, there was no increased trend in abscess occurrence (p = 0.56), but there was an increased trend in the use of percutaneous drainage (p = 0.02). CONCLUSIONS: The risk of a postoperative abscess can be predicted by specific clinical characteristics, surgical findings, and treatment-related factors. Percutaneous drainage was associated with longer hospital stays, but less readmissions.

Satisfaction levels of paediatric surgeons in Canada.

BACKGROUND: Surgeons' satisfaction levels may affect patient care and the stability of the surgical workforce. METHODS: A detailed Internet-based satisfaction survey was administered to paediatric surgeons in Canada in 2005 and 2012. Satisfaction was rated 1 (most) to 5 (least), in five areas: quality of life, financial compensation, work environment, academics and patient care. RESULTS: Responses were received from 21 surgeons in 2005 and 61 in 2012, representing 43% and 98% of practicing paediatric surgeons in Canada, respectively. Satisfaction levels were generally moderate to high in most areas during both years. In academics, surgeons were more satisfied in 2012 with the amount of teaching they provided (1.8 versus 2.2; P=0.02), and clinical research they performed (2.5 versus 3.0; P=0.04). In patient care, there was higher satisfaction with the ability to provide elective services without impediment (2.5 versus 3.0; P=0.02). Over the seven-year period, surgeons increasingly preferred the Canadian health care system over that of the United States (1.7 versus 2.2; P=0.02). In the 2012 survey, no differences in levels of satisfaction were found between male and female surgeons. CONCLUSIONS: During the recent seven-year period, satisfaction levels of paediatric surgeons in Canada have been stable with regard to quality of life, compensation and work environment, and improving in areas of academics and patient care. Male and female surgeons are equally satisfied. The Canadian health care system is preferred over that of the United States.

HISTORIQUE: Le taux de satisfaction des chirurgiens peut influer sur les soins aux patients et sur la stabilité de la main-d'œuvre en chirurgie. MÉTHODOLOGIE: Les chirurgiens en pédiatrie du Canada ont reçu un sondage virtuel détaillé sur leur satisfaction en 2005 et en 2012. La satisfaction était classée de 1 (très satisfait) à 5 (insatisfait), dans cinq domaines : la qualité de vie, la rémunération, le milieu de travail, le milieu universitaire et les soins aux patients. RÉSULTATS: Vingt et un chirurgiens ont répondu en 2005 et 61 en 2012, soit 43 % et 98 % des chirurgiens en exercice en pédiatrie au Canada, respectivement. Dans la plupart des domaines des deux sondages, le taux de satisfaction était généralement modéré à élevé. Dans le milieu universitaire, les chirurgiens étaient plus satisfaits de la quantité d'enseignement qu'ils donnaient (1,8 au lieu de 2,2; P=0,02) et de la recherche clinique qu'ils effectuaient (2,5 au lieu de 3,0; P=0,04) en 2012. En matière de soins aux patients, la satisfaction était plus élevée quant à la capacité d'offrir des services non urgents sans entraves (2,5 au lieu de 3,0; P=0,02). Pendant cette période de sept ans, les chirurgiens ont préféré de plus en plus le système de santé canadien à celui des États-Unis (1,7 au lieu de 2,2; P=0,02). Dans le sondage de 2012, il n'y avait pas de différences dans les taux de satisfaction des chirurgiens et chirurgiennes. CONCLUSIONS: Au cours de la récente période de sept ans, le taux de satisfaction des chirurgiens canadiens est demeuré stable sur le plan de la qualité de vie, de la rémunération et du milieu de travail, et s'est amélioré dans le milieu universitaire et les soins aux patients. Les chirurgiens et les chirurgiennes présentaient la même satisfaction. Le système de santé canadien est préféré à celui des États-Unis.

James Warden and the Global Alliance Partnership: A Pediatric Surgery Giant and His Enduring PAPS Landmark.

The discipline of pediatric surgery has honored many of the early giants through programs that bear their names. One of those programs is the M. James Warden Global Alliance Partnership, a landmark program celebrated at each annual meeting of the Pacific Association of Pediatric Surgeons since 1989. This article describes James Warden and his legacy as a surgeon and humanitarian and provides an update on the past, present, and future of the Global Alliance Partnership that bears his name. LEVEL OF EVIDENCE: 5.

Using Artificial Intelligence to Label Free-Text Operative and Ultrasound Reports for Grading Pediatric Appendicitis.

PURPOSE: Data science approaches personalizing pediatric appendicitis management are hampered by small datasets and unstructured electronic medical records (EMR). Artificial intelligence (AI) chatbots based on large language models can structure free-text EMR data. We compare data extraction quality between ChatGPT-4 and human data collectors. METHODS: To train AI models to grade pediatric appendicitis preoperatively, several data collectors extracted detailed preoperative and operative data from 2100 children operated for acute appendicitis. Collectors were trained for the task based on satisfactory Kappa scores. ChatGPT-4 was prompted to structure free text from 103 random anonymized ultrasound and operative records in the dataset using the set variables and coding options, and to estimate appendicitis severity grade from the operative report. A pediatric surgeon then adjudicated all data, identifying errors in each method. RESULTS: Within the 44 ultrasound (42.7%) and 32 operative reports (31.1%) discordant in at least one field, 98% of the errors were found in the manual data extraction. The appendicitis grade was erroneously assigned manually in 29 patients (28.2%), and by ChatGPT-4 in 3 (2.9%). Across datasets, the use of the AI chatbot was able to avoid misclassification in 59.2% of the records including both reports and extracted data approximately 40 times faster. CONCLUSION: AI chatbot significantly outperformed manual data extraction in accuracy for ultrasound and operative reports, and correctly assigned the appendicitis grade. While wider validation is required and data safety concerns must be addressed, these AI tools show significant promise in improving the accuracy and efficiency of research data collection. LEVELS OF EVIDENCE: Level III.

A minimally invasive hybrid procedure to correct pectus arcuatum.

BACKGROUND: Pectus arcuatum, also known as horns of steer anomaly or Currarino-Silverman Syndrome, is a distinct chest wall anomaly characterized by severe manubriosternal angulation, a shortened sternum, and mild pectus excavatum. The anomaly is typically repaired using open techniques, employing orthopedic fixation devices. Here, we report the results of a minimally invasive hybrid procedure to repair pectus arcuatum. METHODS: The procedure combines a standard Nuss procedure to correct the depressed sternum with a short upper chest (in boys) or inter-mammary (in girls) incision for bilateral subperichondrial resection of the upper costal cartilages, osteotomy, and correction of the manubrial angulation. The medical records of all patients who underwent the procedure over the last 10 years were reviewed. RESULTS: Five patients, 3 boys and 2 girls, aged 14 to 17 years, underwent the procedure. Three patients had their pectus bars removed 3-4 years after repair. Follow-up after correction ranged from 6 months to 7 years. Good correction resulted in all patients achieving recovery without complications and recurrence. To date, all patients have been satisfied with their results. CONCLUSIONS: The minimally invasive hybrid procedure adequately corrects pectus arcuatum with minimal scarring and high satisfaction.

The Canadian Pediatric Surgery Workforce: A 10-year Prospective Assessment.

BACKGROUND: The Canadian Association of Paediatric Surgeons launched a 10-year prospective assessment of the Canadian pediatric surgery workforce and training environment, beginning in 2013. The results of the first 5 years (2013-2017) were previously published. Here, we present the results of the last 5 years (2018-2022), and the cumulative results of the past decade. METHODS: With IRB approval, a web-based survey was sent to all pediatric surgery division chiefs in Canada each year (2013-2022). The survey gathered workforce data on pediatric surgery practices, as well as data regarding fellowship graduates from Canadian training programs. RESULTS: Complete responses were received from all 18 divisions (100% response rate). Over the decade studied, the number of pediatric surgeons and full-time equivalent positions increased from 73 to 81, and 65 to 82, respectively. Thirty positions were vacated (15 retirement, 6 new Canadian practice, 8 leaving Canada, 1 other), and 38 were filled (20 new Canadian fellowship graduates, 8 Canadian surgeons moving from other sites in Canada, 10 surgeons coming from outside Canada). Seventy-five fellows completed training eligible for North American certification, including 34 Canadians, 31 Americans, and 10 non-North American foreign nationals (9 of whom left North America after training). The proportion of Canadian graduates who desired, but could not find, a Canadian position improved from 44% in the first 5 years to 20% in the second 5 years. CONCLUSIONS: The Canadian pediatric surgery workforce has experienced a modest increase over a decade. A mismatch still exists between Canadian pediatric surgery graduates and attending staff positions, but the situation has improved during the last 5 years. TYPE OF STUDY: Survey.

The Impact of Educational Materials on Parental Anxiety and Productivity: A Clinical Trial in Pediatric Appendicitis.

OBJECTIVES: There is limited literature on how acute appendicitis, the most common acute children's surgical illness, affects the family. We conducted a prospective study to assess the impact of educational materials on parents' anxiety and productivity during the child's illness. METHODS: A quasi-experimental clinical trial was conducted among parents of children undergoing laparoscopic appendectomy. In Phase I, parents received the standard explanations at diagnosis and throughout the postoperative period. In Phase II, parents also received a comprehensive educational brochure on pediatric appendicitis at diagnosis. The primary outcome, parental preoperative anxiety, was assessed using the Amsterdam Preoperative Anxiety and Information Scale (APAIS). The secondary outcome, parental productivity, was evaluated through a post-recovery online questionnaire based on the Productivity and Disease Questionnaire (PRODISQ). Baseline characteristics and outcomes were compared between the two cohorts using t-tests, Mann-Whitney, chi-square, or Fischer's exact test as appropriate. RESULTS: Phases I and II included 67 and 66 families, respectively. Patient demographics and disease severity were similar between both groups. Of the 53 parents (80.3%) in Phase II who answered the postoperative questionnaire, most recommended the booklet (96.2%), as it decreased their stress (78.0%) and enhanced their understanding of appendicitis (94.1%). However, the two groups showed similar preoperative anxiety levels and postoperative productivity loss. CONCLUSIONS: Educational materials increased satisfaction with surgical care but did not mitigate the high parental preoperative anxiety levels and postoperative productivity loss. Additional research is required to elucidate interventions that may improve these important patient and family-centered outcomes. TYPE OF STUDY: Non-Randomized Clinical Trial. LEVEL OF EVIDENCE: II.

A comparison of techniques for laparoscopic gastrostomy placement in children.

BACKGROUND: The insertion of gastrostomy tube (GT) for children is typically accomplished using a minimally invasive approach. There is considerable variability in the technical details of this operation, depending on how much of the procedure is performed intracorporeal. The purpose of this study is to compare the outcomes and resource utilization of two differing techniques for laparoscopic GT insertion in the pediatric population. MATERIALS AND METHODS: A single-center retrospective review of all patients who underwent a laparoscopic GT insertion from 2001-2011 was conducted and analyzed based on technique of insertion. This was laparoscopy plus either an intracorporeal Seldinger technique, or an extracorporeal insertion approach, (mini-open technique; [MOT]). Outcomes investigated included short-term complications within the first mo (dislodgement, infection), long-term complications (infection, need for revision, dislodgement), and measures of resource utilization (operative time, material cost, and GT-related hospital visits). RESULTS: A total of 129 insertions were performed; 87 (67.4%) done using the Seldinger technique, and 42 underwent MOT. Overall, complication rates did not differ between the two groups. Of all patients who underwent a GT placement, 38% were treated for granulation tissue, 27.1% experienced dislodgement, and 23.3% were reported to have a GT-related infection. The MOT approach was associated with a 29% reduction in disposable operating room costs and a 57% reduction in emergency department visits (P < 0.05). CONCLUSIONS: Pediatric patients undergoing laparoscopic gastrostomy tube insertion via the Seldinger or MOT method have similar morbidity risks, although MOT was associated with less overall resource utilization in this study.

Prenatal and postnatal features of mesenchymal hamartoma of the chest wall: case report and literature review.

Mesenchymal hamartoma of the chest wall is a rare, benign chondro-osseous tumor of the bone. Although it most commonly presents at birth or soon after, prenatal detection is rare. We report a case of prenatally detected mesenchymal hamartoma, and provide the rationale, details, and outcomes of our management. The literature is reviewed, with particular attention to prenatal detection and postnatal management options.

"Your child needs surgery": A survey-based evaluation of simulated expert consent conversations by key stakeholders.

BACKGROUND: Consent conversations in pediatric surgery are essential components of pre-operative care which, when inadequate, can lead to significant adverse consequences for the child, parents, surgeon, and others in the healthcare system. The aim of this study is to explore expert consenting practice from the key stakeholders' perspective. METHODS: Four senior attending pediatric surgeons obtained consent from a standardized mother of a child requiring surgery in two scenarios: a low-risk elective surgery (inguinal hernia repair - Video 1), and a high-risk emergency surgery (intestinal atresia - Video 2). All sessions were recorded. Families of children who had undergone minor or major surgery, families without medical or surgical background, and healthcare professionals were invited to view and evaluate the videos using a semi-structured questionnaire. RESULTS: Out of 251 distributed surveys, 56 complete responses were received. Thirty two participants (57.1%) evaluated video 1 and 24 (42.9%) evaluated. Overall, 22 (69%) respondents to video 1 and 20 (84%) respondents to video 2 were "very satisfied" with the recorded consent conversation. Qualitative responses shared common themes of valuing surgeon empathy, good surgeon communication, patient engagement, and adequate time and information. Suggestions for improvement included additional resources and visual aids, improved patient engagement, and discussion of post-operative expectations. CONCLUSION: Our data identifies strengths and gaps in the current consent process from the perspective of patient families and providers. Identified areas for improvement in the informed consent process based on multi-stakeholder input will guide the planned development of a consenting educational video resource. LEVEL OF EVIDENCE: IV.