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European Journal Of Pediatric Surgery[JOURNAL]

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The CDH Study Group: Past, Present, and Future.

Holden KI, Ebanks AH, Lally KP … +1 more , Harting MT

Eur J Pediatr Surg · 2024 Apr · PMID 38242150 · Publisher ↗

The Congenital Diaphragmatic Hernia Study Group (CDHSG) is an international consortium of medical centers actively collecting and voluntarily contributing data pertaining to live born congenital diaphragmatic hernia (CDH... The Congenital Diaphragmatic Hernia Study Group (CDHSG) is an international consortium of medical centers actively collecting and voluntarily contributing data pertaining to live born congenital diaphragmatic hernia (CDH) patients born and/or managed at their institutions. These data are aggregated to construct a comprehensive registry that participating centers can access to address specific clinical inquiries and track patient outcomes. Since its establishment in 1995, 147 centers have taken part in this initiative, including 53 centers from 17 countries outside the United States, with 95 current active centers across the globe. The registry has amassed data on over 14,000 children, resulting in the creation of over 75 manuscripts based on registry data to date. International, multicenter consortia enable health care professionals managing uncommon, complex, and diverse diseases to formulate evidence-based hypotheses and draw meaningful and generalizable conclusions for clinical inquiries. This review will explore the formation and structure of the CDHSG and its registry, outlining their functions, center participation, and the evolution of data collection. Additionally, we will provide an overview of the evidence generated by the CDHSG, with a particular emphasis on contributions post-2014, and look ahead to the future directions the study group will take in addressing CDH.

Anatomical Variations of the External Genitalia in Posterior Cloaca: Clinical Consequences of Misdiagnosis-A Systematic Review of the Literature and the ARM-Net Consortium Experience.

Carvalho C, Morandi A, Samuk I … +4 more , Gine C, Gorter R, Martinez-Urrutia MJ, Vilanova-Sánchez A

Eur J Pediatr Surg · 2024 Dec · PMID 38216143 · Publisher ↗

PURPOSE:  All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in... PURPOSE:  All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in posterior cloacas (PCs). If a careful physical examination is not performed, patients may be misdiagnosed with ambiguous genitalia (AG), leading to subsequent unnecessary testing, surgeries, or even wrong gender assignment. The aim was to analyze data of patients with PC within the ARM-Net registry, focusing on the description of the genitalia, gender assignment, and its consequences. Additionally, we investigated the presence of AG diagnosis in utero or at birth in patients with PC in the literature. METHODS:  The ARM-Net registry was scanned for PC cases and data on diagnosis were collected. A systematic literature search was conducted using the PubMed, EMbase, and Web-of-Science databases. Descriptive statistics was used to report data. RESULTS:  Nine patients with PC were identified in the ARM-Net registry. Five patients (55%) were diagnosed with AG, two (22%) were assigned as males and only two patients were correctly assigned as females and diagnosed with PC with respective variations of external genitalia. All patients diagnosed with AG had extensive blood testing including karyotype and hormonal studies. One of the patients who was diagnosed as a male, had surgery for pelvic cystic mass removal, which ultimately led to unaware salpingo-oophorectomy, hysterectomy, and vaginectomy. In the literature we identified 60 patients, 14 (23%) with AG, 1 with clitorolabial transposition and 1 with undeveloped vulva and vagina; 4 patients had normal anatomy. In 40 (67%) patients the anatomy of genitalia was not mentioned. CONCLUSION:  Patients with PC are at high risk of being diagnosed with AG or even assigned the wrong gender at birth. In our series two patients were assigned as males, and consequently one of them underwent a highly mutilating surgery. A thorough physical examination together with a high index of suspicion and laboratory workup are mandatory to identify these variations, avoiding further investigations, unnecessary surgeries, and parental stress.

Thoracoscopic Stage Internal Traction Repair Reduces Time to Achieve Esophageal Continuity in Long Gap Esophageal Atresia.

Borselle D, Davidson J, Loukogeorgakis S … +2 more , De Coppi P, Patkowski D

Eur J Pediatr Surg · 2024 Feb · PMID 38154482 · Publisher ↗

OBJECTIVE:  Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers. METHODS:  We included patients with LGOA (type A and B)... OBJECTIVE:  Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers. METHODS:  We included patients with LGOA (type A and B) between 2008 and 2022. Demographics, surgical methods, and outcomes were collected and compared. RESULTS:  The study population involved 28 patients in center A and 24 patients in center B. A surgical approach was thoracoscopic in center A, only for one patient was open for final procedure. In center B, 3 patients were treated only thoracoscopically, 2 converted to open, and 19 as open surgery. In center A primary esophageal anastomosis concerned 1 case, two-staged esophageal lengthening using external traction 1 patient, and 26 were treated with the multistaged internal traction technique. In 24 patients a full anastomosis was achieved: in 23 patients only the internal traction technique was used, while 1 patient required open Collis-Nissen procedure as final management. In center B primary anastomosis was performed in 7 patients, delayed esophageal anastomosis in 8 patients, esophageal lengthening using external traction in 1 case, and 9 infants required esophageal replacement with gastric tube. Analyzed postoperative complications included: early mortality, 2/28 due to accompanied malformations (center A) and 0/24 (center B); anastomotic leakage, 4/26 (center A) treated conservatively-all patients had a contrast study-and 0/24 (center B), 1 case of pleural effusion, but no routine contrast study; recurrent strictures, 13/26 (center A) and 7/15 (center B); and need for fundoplication, 5/26 (center A) and 2/15 (center B). Age at esophageal continuity was as a median of 31 days in center A and 110 days in center B. Median time between initial procedure and esophageal anastomosis was 11 days in center A and 92 days in center B. CONCLUSION:  Thoracoscopic internal traction technique reduces time to achieve esophageal continuity and the need for esophageal substitution while maintaining a similar early complication rate.

The Timing of Surgery for Congenital Diaphragmatic Hernia in Infants, on or after Weaning from Extracorporeal Membrane Oxygenation: A Meta-Analysis.

Lin M, Liao J, Li L

Eur J Pediatr Surg · 2024 Oct · PMID 38092047 · Publisher ↗

OBJECTIVES:  We conducted a meta-analysis of trials to determine the optimal time to conduct surgery for congenital diaphragmatic hernia (CDH) in infants, on or after weaning from extracorporeal membrane oxygenation (ECM... OBJECTIVES:  We conducted a meta-analysis of trials to determine the optimal time to conduct surgery for congenital diaphragmatic hernia (CDH) in infants, on or after weaning from extracorporeal membrane oxygenation (ECMO). METHODS:  We searched the PubMed, Embase, Scopus, and Cochrane Library databases to identify relevant articles published prior to May 2023 in which surgery was performed to treat CDH in infants. Data were collected, and continuous data were represented by the mean difference (MD) and 95% confidence interval (CI). Dichotomous data were represented by the odds ratio (OR) and 95% CI. Review Manager V.5.4 and Stata were used to synthesize results and to assess publication bias. RESULTS:  The results showed that infants undergoing surgery after being weaned from ECMO had reduced mortality (OR, 2.40; 95% CI, 1.23-4.69;  = 0.01) and postoperative bleeding rates (OR, 16.20; 95% CI, 5.73-45.76;  < 0.00001) and reduced ECMO duration (MD, 3.47; 95% CI, 1.89-5.05;  < 0.0001) compared with those who underwent surgery while on ECMO. There was no statistically significant difference in hospital duration (MD, 5.48; 95% CI, -8.66 to 19.62;  = 0.45) or ventilator duration (MD, -1.93; 95% CI, -8.55 to 4.68;  = 0.57). CONCLUSION:  We recommend weaning patients with CDH from ECMO before performing surgery.

Selection of Quality Indicators to Evaluate Quality of Care for Patients with Esophageal Atresia Using a Delphi Method.

Teunissen NM, Brendel J, Heurn LWEV … +5 more , Ure B, Wijnen R, Eaton S, EPSA|ERNICA Registry Group, EA Quality of Care Initiative

Eur J Pediatr Surg · 2024 Oct · PMID 38086424 · Publisher ↗

OBJECTIVE:  Survival of neonates with esophageal atresia (EA) is relatively high and stable, resulting in increased attention to optimizing care and longer-term morbidity. This study aimed to reach consensus on a quality... OBJECTIVE:  Survival of neonates with esophageal atresia (EA) is relatively high and stable, resulting in increased attention to optimizing care and longer-term morbidity. This study aimed to reach consensus on a quality indicator set for benchmarking EA care between hospitals, regions, or countries in a European clinical audit. METHODS:  Using an online Delphi method, a panel of EA health care professionals and patient representatives rated potential outcome, structure, and process indicators for EA care identified through systematic literature and guideline review on a nine-point Likert scale in three questionnaires. Items were included based on predefined criteria. In rounds 2 and 3, participants were asked to select the five to ten most essential of the included indicators. RESULTS:  An international panel of 14 patient representatives and 71 multidisciplinary health care professionals representing 41 European hospitals completed all questionnaires (response rate: 81%), eventually including 22 baseline characteristics and 32 indicators. After ranking, 10 indicators were prioritized by both stakeholder groups. In addition, each stakeholder group highly prioritized one additional indicator. Following an additional online vote by the other group, these were both added to the final set. CONCLUSION:  This study established a core indicator set of twenty-two baseline characteristics, eight outcome indicators, one structure indicator, and three process indicators for evaluating (quality of) EA care in Europe. These indicators, covering various aspects of EA care, will be implemented in the European Pediatric Surgical Audit to enable recognition of practice variation and focus EA care improvement initiatives.

Perioperative Histologically Controlled Fistula Resection in Patients with Imperforate Anus and Perineal Fistula.

Skaba R, Dotlacil V, Fuccillo P … +3 more , Rouskova B, Pos L, Rygl M

Eur J Pediatr Surg · 2024 Oct · PMID 38016642 · Publisher ↗

INTRODUCTION:  Postoperative constipation (PC) in patients with imperforate anus and perineal fistula (PF) has been reported in up to 60%. Histological studies of PF revealed innervation anomalies which seem to be one of... INTRODUCTION:  Postoperative constipation (PC) in patients with imperforate anus and perineal fistula (PF) has been reported in up to 60%. Histological studies of PF revealed innervation anomalies which seem to be one of the reasons for PC. Perioperative histologically controlled fistula resection (PHCFR) allows appropriate resection of PF and pull-down normoganglionic rectum at the time of posterior sagittal anorectoplasty (PSARP). MATERIALS AND METHODS:  A total of 665 patients with anorectal malformations underwent surgery between 1991 and 2021. Of these, 364 presented PF; 92 out of them (41 F) were studied. Patients with sacral and spinal cord anomalies, neurological disorders, and cut-back anoplasty were excluded. PSARP was done on all patients. Hematoxylin-eosin staining and NADH Tetrazolium-reductase histochemical method were used. Four and more ganglion cells in the myenteric plexus represented a sufficient length of the resection. The continence was scored according to the modified Krickenbeck scoring system. Final scores ranged from 1 to 7 points. Values are given as median. RESULTS:  A total of 65 (70.7%) patients presented an aganglionic segment in PF, and 27 patients presented hypoganglionosis. The median length of the resected fistula was 25 mm (interquartile range [IQR]: 20-30). The median total continence score was 7 (IQR: 6-7). Post-op constipation was observed in 6/92 (6.5%) patients. CONCLUSION:  PHCFR diminished PC to 6.5% of patients.

European Paediatric Surgeons' Association Survey on the Adherence to EAU/ESPU Guidelines in the Management of Undescended Testes.

Aubert O, Zaidan H, Garnier H … +2 more , Saxena AK, Cascio S

Eur J Pediatr Surg · 2024 Oct · PMID 38016641 · Publisher ↗

INTRODUCTION:  The aim of this study was to assess the adherence to the European Association of Urology (EAU)/European Society for Pediatric Urology (ESPU) 2016 guidelines in the management of undescended testes (UDT). M... INTRODUCTION:  The aim of this study was to assess the adherence to the European Association of Urology (EAU)/European Society for Pediatric Urology (ESPU) 2016 guidelines in the management of undescended testes (UDT). MATERIALS AND METHODS:  An online questionnaire was sent in 2023 to members of the European Paediatric Surgeons' Association (EUPSA). RESULTS:  Among 157 members, 46 and 44% perform orchidopexy before 12 and 18 months, respectively. In total, 92% recommend conservative management of retractile testes and 58% offer close follow-up. In case of nonpalpable testes, 78% favor laparoscopy and 18% ultrasonography. If a peeping testicle is identified at laparoscopy, 76% perform a single-stage orchidopexy. In case of a high testicle, a staged procedure is preferred (84%). Management of blind-ending spermatic vessel is heterogenous with a majority ending the operation, followed by exploration of the inguinal canal and removal of the testicular nubbin with optional fixation of the contralateral testis. Only a minority recommends hormonal therapy to improve fertility potential in bilateral UDT. A majority (59%) discuss testis removal in UDT in postpubertal boys. In addition, 77% declare following the EAU/ESPU guidelines. Unawareness of guidelines was the most common reason cited for nonadherence. International guidelines were found to have the greatest influence on clinical practice; however, personal experience and institutional practice seem to play an important role. CONCLUSION:  Most recommendations of the EAU/ESPU guidelines are being followed by EUPSA members; however, personal and institutional practice impact decision making. Hormonal therapy in bilateral UDT, management of vanishing testes, and UDT in postpubertal boys could be improved.

The Development of the International Intestinal Failure Registry and an Overview of its Results.

Avitzur Y, Pahl E, Venick R … +1 more , and the International Intestinal Failure Registry

Eur J Pediatr Surg · 2024 Apr · PMID 37973155 · Full text

Pediatric intestinal failure (IF) is a rare disease that represents an evolving field in pediatric gastroenterology and surgery. With only a limited number of multicenter collaborations, much of the research in pediatric... Pediatric intestinal failure (IF) is a rare disease that represents an evolving field in pediatric gastroenterology and surgery. With only a limited number of multicenter collaborations, much of the research in pediatric IF is often confined to single-center reports with small sample sizes. This has resulted in challenges in data interpretation and left many knowledge gaps unanswered. Over the past two decades, five large multicenter collaborations, primarily from North America and Europe, have published their findings. Apart from one ongoing European adult and pediatric registry, these relatively large-scale efforts have been concluded.In 2018, the International Intestinal Failure Registry (IIFR) was initiated by the International Intestinal Rehabilitation and Transplant Association to continue these efforts and answer some of the knowledge gaps in pediatric IF. The IIFR goals are to prospectively assess the natural history of children diagnosed with IF and creating a worldwide platform to facilitate benchmarking and evidence-based interventions in pediatric IF. A pilot phase involving 204 enrolled patients was initiated in 2018 to assess the feasibility of an international IF registry and refine the study protocol and data collection forms. Following the successful completion of this phase, the current phase of the IIFR was launched in 2021. As of May 2023, the registry includes 362 prospectively followed children from 26 centers worldwide. This review provides an overview of the development, structure, and challenges of the IIFR, as well as the main findings from both the pilot and current phase.

Erratum to: The Development and Validation of Artificial Intelligence Pediatric Appendicitis Decision-Tree for Children 0 to 12 Years Old.

Shikha A, Kasem A

Eur J Pediatr Surg · 2023 Oct · PMID 37949436 · Publisher ↗

Abstract loading — click title to view on PubMed.

The French Experience with a Population-Based Esophageal Atresia Registry (RENATO).

Sfeir R, Aumar M, Sharma D … +3 more , Labreuche J, Dauchet L, Gottrand F

Eur J Pediatr Surg · 2024 Apr · PMID 37940126 · Publisher ↗

This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also... This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also detailed. In 2023, more than 2,500 patients with EA are included. Prevalence of EA in France was calculated at 1.8/10,000 live birth. Main clinical results are listed with scientific publications issued directly from the register.

Near-Infrared Fluorescence Imaging with Intravenous Indocyanine Green Method in Segmentectomy for Infants with Congenital Pulmonary Airway Malformation.

Huang JX, Chen Q, Hong SM … +2 more , Hong JJ, Cao H

Eur J Pediatr Surg · 2024 Oct · PMID 37940125 · Publisher ↗

BACKGROUND:  Video-assisted thoracoscopic surgery is a commonly used procedure for treating congenital pulmonary airway malformation (CPAM) in infants, particularly when performing segmentectomy for segmental lesions. An... BACKGROUND:  Video-assisted thoracoscopic surgery is a commonly used procedure for treating congenital pulmonary airway malformation (CPAM) in infants, particularly when performing segmentectomy for segmental lesions. An innovative technique employing near-infrared fluorescence (NIRF) imaging with intravenous indocyanine green (ICG) has been utilized to delineate the intersegmental demarcation during surgery. However, no previous reports have investigated this method's application, specifically in infants. The primary aim of this study was to assess the safety and efficacy of the NIRF imaging with ICG approach in this context. METHODS:  Between January 2021 and April 2022, a total of 19 consecutive segmentectomies were conducted using the NIRF imaging with ICG method to precisely identify the intersegmental plane. The results were concurrently compared with those obtained using the modified inflation-deflation technique. Comprehensive imaging and clinical data were gathered and analyzed to assess the safety and accuracy of the NIRF imaging with ICG approach. RESULTS:  The study involved infants with a median age of 5.12 months (mean body weight of 8.08 g). All segmentectomies were performed successfully without any ICG-related complications. The mean operating time for the surgeries was 88.47 ± 7.94 minutes. Notably, no intraoperative conversions or significant complications were observed in any of the patients. The average hospital stay after surgery was 4.0 ± 0.82 days. During the follow-up period, extending beyond 1-year of postoperation, all patients exhibited excellent recovery with no cases of recurrence. CONCLUSIONS:  Based on our experience, the NIRF imaging with intravenous ICG method proved to be both safe and effective when performing segmentectomy for infants with CPAM. Low doses of ICG did not hinder the accurate identification of the intersegmental plane.

Antegrade Continence Enema Alone for the Management of Functional Constipation and Segmental Colonic Dysmotility (ACE-FC): A Pediatric Colorectal and Pelvic Learning Consortium Study.

Ahmad H, Smith C, Witte A … +14 more , Lewis K, Reeder RW, Garza J, Zobell S, Hoff K, Durham M, Calkins C, Rollins MD, Ambartsumyan L, Rentea RM, Yacob D, Lorenzo CD, Levitt MA, Wood RJ

Eur J Pediatr Surg · 2024 Oct · PMID 37940124 · Publisher ↗

OBJECTIVE:  The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS:  A re... OBJECTIVE:  The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS:  A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. -Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States). RESULTS:  A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males  = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% ( = 0.023) with pelvic dyssynergia underwent subsequent colonic resection. CONCLUSION:  In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection.

Unlocking the Power of Data: Standardized Data Collection in Pediatric Surgery.

Teunissen NM, Wijnen RMH

Eur J Pediatr Surg · 2024 Apr · PMID 37940123 · Publisher ↗

Abstract loading — click title to view on PubMed.

Bridging the Gap: A Systematic Review on Reporting Baseline Characteristics, Process, and Outcome Parameters in Rectosigmoid Hirschsprung's Disease.

Rossi D, Löf Granström A, Teunissen NM … +3 more , Wijnen RMH, Wester T, Sloots CEJ

Eur J Pediatr Surg · 2024 Apr · PMID 37884061 · Publisher ↗

The variation in standardized, well-defined parameters in Hirschsprung's disease (HSCR) research hinders overarching comparisons and complicates evaluations of care quality across healthcare settings. This review address... The variation in standardized, well-defined parameters in Hirschsprung's disease (HSCR) research hinders overarching comparisons and complicates evaluations of care quality across healthcare settings. This review addresses the significant variability observed in these parameters as reported in recent publications. The goal is to compile a list of commonly described baseline characteristics, process and outcome measures, and to investigate disparities in their utilization and definitions. A systematic review of literature on the primary care process for HSCR was performed according to PRISMA guidelines. Relevant literature published between 2015 and 2021 was obtained by combining the search term "Hirschsprung's disease" with "treatment outcome," "complications," "mortality," "morbidity," and "survival" in Medline, Embase, and the Cochrane Library. We extracted study characteristics, reported process and outcome parameters, and patient and disease characteristics. We extracted 1,026 parameters from 200 publications and categorized these into patient characteristics ( = 226), treatment and care process characteristics ( = 199), and outcomes ( = 601). A total of 116 parameters were reported in more than 5% of publications. The most frequently reported characteristics were sex (88%), age at the time of surgery (66%), postoperative Hirschsprung-associated enterocolitis (64%), type of repair (57%), fecal incontinence (54%), and extent of aganglionosis (51%). This review underscores the pronounced variation in reported parameters within HSCR studies, highlighting the necessity for consistent, well-defined measures and reporting systems to foster improved data interpretability. Moreover, it advocates for the use of these findings in the development of a Core Indicator Set, complementing the recently developed Core Outcome Set. This will facilitate quality assessments across pediatric surgical centers throughout Europe.

Benchmarks for Pediatric Surgical Registries: Recommendations for the Assessment and Grading of Complications.

Madadi-Sanjani O, Ure BM

Eur J Pediatr Surg · 2024 Apr · PMID 37871645 · Publisher ↗

Procedure-related registries in general surgical practice offer a platform for prospective trials, the pooling of data, and detailed outcome analysis. Recommendations by the Idea, Development, Exploration, Assessment, an... Procedure-related registries in general surgical practice offer a platform for prospective trials, the pooling of data, and detailed outcome analysis. Recommendations by the Idea, Development, Exploration, Assessment, and Long-term follow-up (IDEAL) collaboration and Outcome4Medicine have further improved the uniform reporting of complications and adverse events.In the pediatric surgical network, disease-specific registries for rare and inherited congenital anomalies are gaining importance, fostering international collaborations on studies of low-incidence diseases. However, to date, reporting of complications in the pediatric surgical registries has been inconsistent. Therefore, the European Reference Network for Rare Inherited and Congenital Anomalies (ERNICA) recently endorsed the validation of the first severity grading system for children. The planned reform of the European Paediatric Surgical Audit (EPSA) registry, which includes the implementation of the Clavien-Madadi classification, represents a further effort to establish uniform outcome reporting.This article provides an overview of experiences with surgical registries and complication reporting, along with the potential application of this knowledge to future pediatric surgical practice.

Development Methodology, Availability, and Implementation of Core Outcome Sets in Pediatric Surgery.

Allin BSR, Bethell GS, Hall NJ

Eur J Pediatr Surg · 2024 Apr · PMID 37871644 · Publisher ↗

Core outcome sets (COSs) provide a mechanism to guide researchers and clinicians when deciding which outcomes to report in research related to a specific clinical condition or intervention. The intention behind creating... Core outcome sets (COSs) provide a mechanism to guide researchers and clinicians when deciding which outcomes to report in research related to a specific clinical condition or intervention. The intention behind creating a COS for a specific condition is to improve the reporting of important and meaningful outcomes, thus enhancing the relevance of research. Additionally, a COS helps facilitate comparison of outcomes between different clinical studies and reduces research waste. In this paper, we discuss the availability of COSs in the field of pediatric general surgery. We provide an overview of the methodologies used to develop a COS, including common pitfalls, and finally, we discuss COS uptake and implementation. An understanding of all these aspects is important for researchers considering developing a new COS and for those reading research where a COS has been developed or used within a study. Failure to adequately appreciate the nuances of COS development, in particular, risks fundamental flaws that may jeopardize COS validity and subsequently hinder COS uptake and implementation.

Risk Factors for Dehiscence of Operative Incisions in Newborns after Laparotomy.

Miholjcic TBS, Baud O, Iranmanesh P … +1 more , Wildhaber BE

Eur J Pediatr Surg · 2024 Aug · PMID 37816380 · Full text

BACKGROUND:  Surgical wound dehiscence (SWD) in neonates is a life-threatening complication. The aim was to define risk factors of postoperative incision dehiscence in this population. METHODS:  Data of 144 patients from... BACKGROUND:  Surgical wound dehiscence (SWD) in neonates is a life-threatening complication. The aim was to define risk factors of postoperative incision dehiscence in this population. METHODS:  Data of 144 patients from 2010 to 2020 were analyzed retrospectively. All full-term newborns or preterm newborns up to 42 weeks of amenorrhea (adjusted) who had a laparotomy within 30 days were included. Descriptive patient information and perioperative data were collected. SWD was defined as any separation of cutaneous edges of postoperative wounds. RESULTS:  Overall, SWD occurred in 16/144 (11%) patients, with a significantly increased incidence in preterm newborns (13/59, 22%) compared with full-term newborns (3/85, 4%;  < 0.001). SWD was significantly associated with exposure to postnatal steroids (60% vs. 4%,  < 0.001) and nonsteroidal anti-inflammatory drugs (25% vs. 4%,  < 0.01), invasive ventilation duration before surgery (median at 10 vs. 0 days,  < 0.001), preoperative low hemoglobin concentration (115 vs. 147 g/L,  < 0.001) and platelet counts (127 vs. 295 G/L,  < 0.001), nonabsorbable suture material (43% vs. 8%,  < 0.001), the presence of ostomies (69% vs. 18%,  < 0.001), positive bacteriological wound cultures (50% vs. 6%,  < 0.001), and relaparotomy (25% vs. 3%,  < 0.01). Thirteen of 16 patients with SWD presented necrotizing enterocolitis/intestinal perforations (81%,  < 0.001). CONCLUSION:  This study identified prematurity and a number of other factors linked to the child's general condition as risk factors for SWD. Some of these can help physicians recognize and respond to at-risk patients and provide better counseling for parents.

Point of Care Ultrasound in Pediatric Surgery across the European Region-European Pediatric Surgery Association Endorsed Survey.

Lindert J, Rolle U, Naidoo G

Eur J Pediatr Surg · 2024 Feb · PMID 37793426 · Publisher ↗

INTRODUCTION:  Our purpose was to assess the state of training, clinical practice, and barriers to use point-of-care ultrasound (POCUS) in pediatric surgery in Europe. METHODS:  An electronic survey was disseminated amon... INTRODUCTION:  Our purpose was to assess the state of training, clinical practice, and barriers to use point-of-care ultrasound (POCUS) in pediatric surgery in Europe. METHODS:  An electronic survey was disseminated among European pediatric surgeons utilizing the European Pediatric Surgery Association network and other existing networks. RESULTS:  There were 186 respondents from 27 European countries and 7 non-European countries. In most countries (86.6%;  = 161), the initial ultrasound for acute admissions is performed by radiologists, with 1 to 6 hours turnover in 62.9% ( = 117) of urgent cases. Ultrasound by pediatric surgeons (point-of-care ultrasound/POCUS) is performed by 48.4% ( = 90) of respondents, with 29% ( = 54) using it at least once per week. The most common indications for POCUS include abdominal focused abdominal sonography in trauma (53.8%;  = 100), diagnosis of appendicitis (41.9%;  = 78), and intussusception (44.6%;  = 84). In malrotation-volvulus, 28.5% ( = 53) used ultrasound for its diagnosis, while 27.5% ( = 51) would not see an indication here. Training in POCUS occurred informally for 55.4% ( = 103) of participants, while 31.2% ( = 58) attended formal training courses. Almost all respondents wanted to attain further POCUS training (89.3%;  = 166), only 7% ( = 13) did not think this would be useful. For 73.1% ( = 136), POCUS is not currently part of the pediatric surgery training curriculum in their country. Perceived barriers to POCUS use include a lack of training opportunities (26.3% [ = 49]) and a paucity of portable ultrasound machines (17.8% [ = 33]). CONCLUSION:  There is a wide spectrum of POCUS use in pediatric surgery across Europe. For those surgeons who practice POCUS, it is most used for the diagnosis of abdominal conditions. There are differing views among clinicians concerning the most useful applications of POCUS. The extent to which ultrasound is taught during pediatric surgery training differs substantially across European curricula.

The Irish Experience with Sacrococcygeal Teratomas: Are Type IV Lesions More Common than We Think?

Kane GA, Mortell A, Gillick J … +2 more , Thambipillai SP, Cascio S

Eur J Pediatr Surg · 2024 Oct · PMID 37793425 · Publisher ↗

INTRODUCTION:  Sacrococcygeal teratomas (SCTs) are rare tumors occurring in approximately 1 in 35,000 to 40,000 live births. The Altman classification is used to describe SCTs. There are four types, with type 1 predomina... INTRODUCTION:  Sacrococcygeal teratomas (SCTs) are rare tumors occurring in approximately 1 in 35,000 to 40,000 live births. The Altman classification is used to describe SCTs. There are four types, with type 1 predominantly external through to type IV which is a presacral, completely internal mass. As far as the authors are aware, this is the first study to focus on type IV SCT lesions. MATERIALS AND METHODS:  Using ICD-10-AM (The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Australian Modification) codes, we identified all patients in the Republic of Ireland with a diagnosis of SCT from 2004 to 2020. The following information was obtained for each patient: gender, time of diagnosis, clinical presentation, method of diagnosis, Altman classification, biomarkers, age at operation, surgical technique, pathology, recurrence, and age at most recent follow-up. RESULTS:  There were 29 patients in total; 23 females (79%) and 6 males (21%). In total, 16 (55%) were diagnosed antenatally, 4 (14%) at less than 1 month, 4 (14%) less than 1 year, 3 (10%) age 2 to 4 years, and 2 (7%) were aged 5 to 12 years. In addition, 22 (76%) were mature teratomas, 2 (7%) immature teratomas, and 5 (17%) were malignant tumors. There were 6 (21%) type I lesions, 9 (30%) type II, 6 (21%) type III, and 8 (28%) type IV lesions. CONCLUSION:  In Altman's original 1974 study, type IV lesions were present in 10% of cases. As a result, this is the most frequently quoted figure. Type IV lesions were present in 28% of cases in our study. We propose that type IV lesions may be more common than the current literature suggests and consequently a higher index of suspicion of their presence should be entertained.

Impact of BCL-2 Expression on Course of Disease in Neuroblastoma.

Muehling J, Fröba-Pohl A, Muensterer OJ … +2 more , von Schweinitz D, Kappler R

Eur J Pediatr Surg · 2024 Feb · PMID 37774735 · Publisher ↗

OBJECTIVE:  The antiapoptotic BCL-2 protein has implications for maturation and differentiation of neural tissue and acts as a strong modulator of carcinogenesis in different tumors. Recent research focuses not only on i... OBJECTIVE:  The antiapoptotic BCL-2 protein has implications for maturation and differentiation of neural tissue and acts as a strong modulator of carcinogenesis in different tumors. Recent research focuses not only on its benefit as a prognostic factor, but also as a potential therapeutic target. The role of BCL-2 in neuroblastoma, the most common extracranial solid tumor in childhood, remains controversial. The aim of our study was to determine the gene expression level of BCL-2 in a large cohort of neuroblastoma patients and its correlation with clinical parameters. METHODS:  Tumor samples and clinical data were collected from 100 neuroblastoma patients treated according to the NB2004 protocol of the German Society of Pediatric Oncology and Hematology. BCL-2 gene expression levels were measured by quantitative reverse transcription polymerase chain reaction and correlated with clinical parameters. RESULTS:  BCL-2 expression was detected in all tumor samples. Relative BCL-2 expression levels were higher in females versus males (1.839 vs. 1.342;  = 0.0143), in patients with low versus high International Neuroblastoma Staging System stage (2.051 vs. 1.463;  = 0.0206), in nonmetastatic versus metastatic disease (1.801 vs. 1.342;  = 0.0242), as well as in patients without presurgical chemotherapy (2.145 vs. 1.402;  = 0.0016), but was not associated with overall survival and MYCN amplification. CONCLUSION:  Our study demonstrates the ubiquitous expression of BCL-2 in neuroblastoma and suggests the possibility for targeted therapy with BCL-2 inhibitors, even in lower-stage neuroblastoma. It also underlines the need for further research on concomitant genetic alterations for a better understanding of the impact of BCL-2 on this pediatric tumor type.
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