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Background Cardiac anomalies occur frequently in patients with congenital duodenal obstruction (DO). However, the exact occurrence and the type of associated anomalies remain unknown. Therefore, the aim of this systematic review is to aggregate the available literatures on cardiac anomalies in patients with DO. Methods In July 2022, a search was performed in PubMed and Embase.com. Studies describing cardiac anomalies in patients with congenital DO were considered eligible. Primary outcome was the pooled percentage of cardiac anomalies in patients with DO. Secondary outcomes were the pooled percentages of the types of cardiac anomalies, type of DO, and trisomy 21. A meta-analysis was performed to pool the reported data. Results In total, 99 publications met our eligibility data, representing 6725 patients. The pooled percentage of cardiac anomalies was 29% (95% CI 0.26–0.32). The most common cardiac anomalies were persistent foramen ovale 35% (95% CI 0.20–0.54), ventricular septal defect 33% (95% CI 0.24–0.43), and atrial septal defect 33% (95% CI 0.26–0.41). The most prevalent type of obstruction was type 3 (complete atresias), with a pooled percentage of 54% (95% CI 0.48–0.60). The pooled percentage of Trisomy 21 in patients with DO was 28% (95% CI 0.26–0.31). Conclusion This review shows cardiac anomalies are found in one-third of the patients with DO regardless of the presence of trisomy 21. Therefore, we recommend that patients with DO should receive preoperative cardiac screening. Level of evidence II.

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 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 four phases: (1) an updated systematic review identifying all previously reported outcomes, (2) a two-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 (eight countries) and 245 surgeons (10 countries), the second-round response rates were 61% and 85% respectively, with ten outcomes emerging with consensus. After two young peoples’ focus groups, two additional outcomes were added to the final COS (12): mortality, bowel obstruction, intra-abdominal 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 of simple appendicitis in children, to reduce heterogeneity between studies and facilitate data synthesis and evidence-based decision-making.

Currently, accurate biomarkers differentiating simple (phlegmonous) from complex (gangrenous and/or perforated) appendicitis in children are lacking. However, both types may potentially require different treatment strategies, and the search for diagnostic modalities remains warranted. Previously, we demonstrated a distinct microbiota (both an increased bacterial diversity and abundance) in the appendix of children with complex compared to simple appendicitis. From the same cohort of patients we have collected 35 rectal swabs under general anesthesia prior to appendectomy and microbiota analysis was performed by IS-pro, a 16S-23S rDNA–based clinical microbiota profiling technique. Using the obtained IS-profiles, we performed cluster analyses (UPGMA), comparison of diversity (Shannon Diversity Index) and intensity (abundance in relative fluorescence units) on phylum level, and comparison on species level of bacteria between simple and complex appendicitis. Regarding these analyses, we observed no clear differences between simple and complex appendicitis. However, increased similarity of the microbial composition of the appendix and rectal swab was found within children with complex compared to simple appendicitis. Furthermore, PLS-DA regression analysis provided clear visual differentiation between simple and complex appendicitis, but the diagnostic power was low (highest AUC 0.65). Conclusion: Microbiota analysis of rectal swabs may be viable to differentiate between simple and complex appendicitis prior to surgery as a supervised classification model allowed for discrimination of both types. However, the current diagnostic power was low and further validation studies are needed to assess the value of this method. What is Known:• Simple and complex appendicitis in children may require different treatment strategies, but accurate preoperative biomarkers are lacking.• Clear differentiation can be made between both types in children based upon the microbial composition in the appendix. What is New:• Increased similarity was found between the microbial composition of the appendix and rectal swab within children with complex compared to simple appendicitis.• Using a supervised classification model rectal swabs may be viable to discriminate between simple and complex appendicitis, but the diagnostic power was low.

Background: Several clinical prediction rules have been developed for preoperative differentiation between simple and complex appendicitis in children, as potential treatment strategies differ. This study aimed to externally validate applicable clinical prediction rules that could be used to differentiate between simple and complex appendicitis in children. Methods: Potential clinical prediction rules were identified by a scoping review of the literature. Clinical prediction rules applicable in our daily practice were subsequently externally validated in a multicenter historical cohort consisting of 1 tertiary center and 1 large teaching hospital. All children (0.7 were considered acceptable and potentially useful. Results: In total, 31 clinical prediction rules were identified, of which 12 could be evaluated in our cohort consisting of 550 children. The main reason to exclude clinical prediction rules was the use of variables that were not routinely measured in our cohort. In our cohort, 208/550 (38%) were diagnosed with complex appendicitis according to the gold standard. Clinical prediction rules with areas under the receiver operating characteristic curve >0.7 were: Gorter (0.81), Bogaard (0.79), Bröker (0.79), Graham (0.77), Hansson (0.76), BADCF (0.76), and Eddama (0.75). Conclusion: In this study, clinical prediction rules consisting of a combination of clinical and objective variables had the highest discriminative ability. External validation showed that 7 clinical prediction rules were potentially useful. Integration of these clinical prediction rules in daily practice is proposed to guide decision making regarding treatment strategies.

Background: A Core Outcome Set for Inguinal Hernia repair (COS-IH) will facilitate adequate comparison of treatment strategies and the interpretation and implementation of clinical trial results. To develop such a COS-IH, this systematic review is performed to determine which outcomes are reported in RCTs and MA in children aged 0-16 years undergoing inguinal hernia repair. Methods: A systematic review was performed according to the PRISMA guidelines using PubMed, EMBASE, MEDLINE and the Cochrane Library databases (Prospero registry: CRD42021281422). This systematic review included all randomized trials (RCTs) and meta-analysis (MA) that assessed inguinal hernia repair in children. Results: A total of 96 unique outcomes were identified in the 47 included articles. These outcomes were mapped into the 62 terms as shown in table 2. Conclusion: The development of this IH-COS is crucial for outcome data comparison and will enable adequate and efficient comparison of treatment strategies, and will aid the interpretation and implementation of clinical trial results.

Purpose: This study aimed to determine the complications after video assisted thoracoscopic surgery (VATS) for congenital lung lesions in children. Methods: All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Both early (Results: In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, less resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof (p=0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. Conclusion: VATS lobectomy is a relatively safe procedure in children, even under one year of age. A preoperative lung infection increases the risk of conversion to open surgery.

Rectal atresia (RA) affects only 1 to 2% of all children with anorectal malformations. No consensus on optimal treatment strategy is yet achieved. Therefore, the aim of this systematic review is to summarize all surgical interventions for RA and outcomes described in the current literature. A literature search was conducted in PubMed, Embase, Web of Science, and Cochrane Library on January 24, 2022. All studies describing treatment for RA in children (< 18 years) were included. Operation technique and postoperative complications were listed. Only descriptive analysis was anticipated. Quality of the studies was assessed using Johanna Briggs Institute critical appraisal checklist for case reports and series. The search yielded 6,716 studies of which, after duplicate removal, 4,028 were excluded based on title and abstract screening. After full-text assessment, 22 of 90 studies were included, yielding 70 patients. Posterior sagittal anorectoplasty (PSARP) and pull-through were most performed (43/70 and 18/70 patients, respectively). Four patients experienced postoperative complications: anal stenosis (n = 1), anastomotic stenosis (n = 2), and death due to a pulmonary complication (n = 1). In the low-quality literature available, most patients with RA are treated with PSARP or pull-through technique. A low complication rate of both has been described but follow-up was often not mentioned. Larger well-designed studies should be performed to determine optimal treatment strategy for children with RA. This study reflects level of evidence V.

Background: In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period. Methods: A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions. Results: In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000–2006 vs. 82% in 2007–2013 vs. 86% in 2014–2020). Median age at follow-up was 7.0 years (IQR 3.0–12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found. Conclusions: The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening. Level of evidence: III.

While clinical guidelines are essential for decision-making based on the latest evidence, they are not all-encompassing for any given patient or context. As such, deviating from guideline recommendations is common practice and can be attributed to contextual, ethical, clinical, or scientific factors. In the setting of diagnostic testing deviating from recommended diagnostic work-up may result in both over- and under-testing. In this Clinical Lesson we discuss two scenarios in primary care, one involving high-sensitivity troponin testing in a patient with episodes of chest discomfort, and the other involving CRP testing in a pediatric patient with acute-onset abdominal pain.

Background: Anterior cutaneous nerve entrapment syndrome (ACNES) is a frequently overlooked cause of chronic abdominal pain in children. Currently, both nonsurgical and surgical treatment options are available to treat this disease. The objective was to give insight into the success rate of different treatment strategies for children with ACNES, and provide treatment recommendations for physicians based on the published evidence. Method: A literature search of PubMed, Embase.com and the Wiley/Cochrane Library was conducted for studies published up to 25 February 2020. Randomized controlled trials, prospective or retrospective cohort studies, meta-analyses and literature reviews describing the outcome of different treatment strategies for children (< 18 years old) with ACNES with a follow-up duration of at least four weeks were included. Results: Six studies, involving 224 patients, were included with an overall quality reported to be between fair and poor. Treatment success of local injections with an anesthetic agent into the trigger point ranged from 38% to 87% with a follow-up ranging from 4 weeks to 39 months. In addition, treatment success of anterior neurectomy ranged from 86% to 100%, with a follow-up duration ranging from 4 weeks to 36 months. Conclusion: A step-up treatment strategy should be applied when treating pediatric patients with ACNES. This strategy starts with an injection with a local anesthetic agent, reserving surgery (anterior neurectomy) as a viable option in case of persistent pain. Level of evidence: II.

PurposeIn general, an appendectomy is presumed to have a limited burden of disease. However, in current literature, reported complication rates vary. This study aims to provide additional insights in the incidence of post-appendectomy complications in children with acute appendicitis.MethodsThis retrospective cohort study included children (0–17 years old) that underwent appendectomy at our tertiary referral centre for suspected acute appendicitis (January 2011–December 2018). Children referred to our centre, and those that underwent non-operative treatment were excluded. Post-appendectomy complications were recorded from electronic medical charts using predefined definitions and classified as severe (Clavien-Dindo III–IV) or less severe (Clavien-Dindo I–II).ResultsA total of 131 children were included. Simple and complex appendicitis was diagnosed in 66 (50%) and 60 (46%) children, respectively. A non-inflamed appendix was seen in five (4%) children. One or more complications were identified in 33 (25%) patients. Eight (12%) children with simple appendicitis developed a complication, three of these were severe. In children with complex appendicitis, 23 (38%) children developed a complication, 14 of these were severe.ConclusionThis study shows a high rate of complications compared with current literature, both in children with simple and complex appendicitis. This is probably the result of our definition of complications and being a tertiary referral centre receiving more severe appendicitis cases. However, these results still show that appendectomy is not always a routine procedure with only few complications. Substantiating the need to keep optimizing treatment for children with appendicitis.

Background Appendiceal mass and abscess and its treatment are associated with significant morbidity and high costs. Still, the optimal treatment strategy is the point of debate. Therefore, this systematic review and meta-analysis aimed to compare overall complications between initial non-operative treatment (NOT) and early appendectomy (EA) in children with appendiceal mass and/or abscess. Methods Pubmed and Embase were searched. Only randomized controlled trials and prospective or historical cohort studies that compared NOT with EA in children with appendiceal mass or abscess in terms of complications were eligible for inclusion. Risk of bias was assessed. Primary outcome was the overall complication rate. Secondary, length of stay and readmission rate were investigated. A meta-analysis of overall complications associated with both treatment strategies was performed. Results 14 of 7083 screened studies were selected, including 1022 children in the NOT group and 333 in the EA group. Duration of follow-up ranged between four weeks and 12 years. Risk of bias was moderate in four and serious in 10 studies. NOT was associated with a lower overall complication rate (risk ratio (RR) 0.37 [95% confidence interval (CI) 0.21–0.65]). However, NOT led to increased length of stay (mean difference varied between 0.2 and 8.4 days) and higher readmission rate (RR 1.75 [95%CI 0.79–3.89]), although not significantly. Interval appendectomy after NOT was performed as a routine procedure in all but one study. This study found a recurrence rate of 34% in a group of 38 patients during a follow-up period of 3.4 ± 1.7 years. Conclusion NOT may reduce the overall complication rate compared to EA, but the evidence is very uncertain. As evidence is scarce, and of low level, and heterogeneity between studies is substantial, the results should be interpreted with caution. Large prospective studies are needed to determine the optimal treatment strategy for children with appendiceal mass and/or abscess.

Investigator-initiated clinical trials are crucial for improving quality of care for children and pregnant women as they are often excluded from industry-initiated trials. However, trials have become increasingly time-consuming and costly since the EU Clinical Trial Directive entered into force in 2001. This directive made compliance with ICH-Good Clinical Practice Guidelines (ethical and quality standard for conducting human subject research) mandatory for all clinical trials, regardless of its risk-classification. By discussing two investigator-initiated, ‘low-risk’ drug trials, we aim to illustrate that compliance with all GCP requirements makes trials very laborious and expensive, while a clear rationale is missing. This discourages clinical researchers to start and carry out investigator-initiated research. However, the forthcoming EU Clinical Trial Regulation (No 536/2014) seems to provide a solution as it allows for less stringent rules for low-risk trials. We want to raise awareness for these developments in both the clinical research community and the European and national regulatory authorities. Implementation of this forthcoming Regulation regulatory policies should be done in such a way that investigator-initiated trials evaluating standard care interventions will become more feasible. This will allow us to obtain evidence on optimal and safe treatments, especially for groups that are underrepresented in medical research. What is Known• Investigator-initiated trials are indispensable for improving care for children and pregnant women as they are often excluded from industry-initiated trials• Trials have become increasingly time-consuming and costly because of mandatory compliance with ICH-GCP guidelines What is New• The forthcoming EU Clinical Trial Regulation allows less stringent rules for low-risk trials• The national legislator and regulatory authorities should recognize the importance of this opportunity and implement the Regulation in such a way that investigator-initiated trials will become more feasible

IntroductionA stoma will cause nutrients loss which could result in impaired growth. Impaired growth can negatively impact long-term development. This study aims to evaluate: 1) the effect of stomas on growth comparing small bowel stoma versus colostomy, 2) if early closure (within 6 weeks), proximal small bowel stoma (within 50 centimeters of Treitz), major small bowel resection (≥30 centimeters) or adequate sodium supplementation (urinary level ≤30mmol/L) influence growth. MethodsYoung children (≤3 years) treated with stomas between 1998-2018 were retrospectively identified. Growth was measured with weight-for-age Z-scores. Malnourishment was defined using the WHO-definition. Comparison between changes in Z-scores at closure and a year following closure was done by Friedman-test with post-hoc-Wilcoxon-signed-rank-test or Wilcoxon-rank-sum-test when necessary. ResultsIn the presence of a stoma, 61% showed growth decline. Severe malnourishment was seen at time of stoma closure in 51% of the patients treated by small bowel stoma and 16% of those treated by colostomy. Within a year following stoma closure 67% showed a positive growth trend. Having a proximal small bowel stoma and undergoing major small bowel resection led to significantly lower Z-scores at closure. Adequate sodium supplementation and early closure didn’t lead to significant changes in Z-scores. ConclusionStomas have a negative impact on growth in the majority of children. This impact might be decreased by preventing small bowel stomas, specifically proximal stomas, and limiting small bowel resection. Since stoma closure is essential in reversing the negative effect on growth, we opt that early closure might result in an early shift to catch-up growth.

Introduction: Contrast enemas are often made prior to stoma reversal in order to detect distal intestinal strictures distal of the stoma. If untreated these strictures can cause obstruction which might necessitate redo-surgery. However, the value of contrast enemas is unclear. Therefore, we aim to evaluate the contrast enema's diagnostic accuracy in detecting strictures in children with a stoma. Methods: Young children (≤3 years) treated with a stoma between 1998 and 2018 were retrospectively included. The STARD criteria were followed. Patients treated for anorectal malformations and those that died before stoma reversal were excluded. Surgical identification of strictures during reversal or redo-surgery within three months was used as gold standard. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the curve (AUC) reflected diagnostic accuracy. Results: In 224 included children, strictures were found during reversal in 10% of which 95% in patients treated for necrotizing enterocolitis. Contrast enema was performed in 68% of all patients and detected 92% of the strictures. In the overall cohort, the sensitivity was 100%, specificity 98%, PPV 88% and NPV 100% whilst the AUC was 0.98. In patients treated for NEC, the sensitivity was 100%, specificity 97%, PPV 88% and NPV 100% whilst the AUC was 0.98. Conclusion: Strictures prior to stoma reversal seem to be mainly identified in patients treated for NEC and not in other diseases necessitating a stoma. Moreover, the contrast enema shows excellent diagnostic accuracy in detecting these strictures. For this reason we advise to only perform contrast enemas in patients treated for NEC. Level of Evidence: II

Objective: Discussion remains on how to advise women with a past medical history of surgically corrected anorectal malformations (ARMs) regarding vaginal delivery. The aim of this review is to evaluate and review the reported obstetrical complications and outcomes after vaginal delivery for these women.Data sources: A systematic search was performed from inception up to 8 December 2020 in PubMed, Embase.com and Clarivate Analytics/Web of Science Core Collection, with backward citation tracking. Study eligibility criteria / appraisal: All articles reported on the outcomes of interest in women with a past medical history of surgically corrected anorectal malformation and had a vaginal delivery were included with the exception of editorial comments or invitational commentaries. Screening and data extraction was done by two authors independently with a third and fourth reviewer in case of disagreement. Tool for Quality assessment depended on the type of article. As low quality evidence was expected no meta-analysis was performed. Results: Only four of the 2125 articles screened were eligible for inclusion with a total of 12 vaginal deliveries attempts in 7 women. In two patients complications were reported: failed vaginal delivery requiring urgent cesarean section (n=1), vaginal tears (n=1). Conclusion: high quality evidence regarding outcomes and complications after vaginal delivery in women with a history of surgically corrected anorectal malformation is lacking. Therefore, based upon this systematic review no formal recommendation can be formulated regarding its safety. Future studies are essential to address this problem. Prospero registration: CRD42020201390. Date: 28-07-2020s

IntroductionIn daily practice large heterogeneity in the treatment of children with complex appendicitis exists. Complex appendicitis can be divided into two subtypes; complex appendicitis with and without appendiceal mass and/or abscess. As complex appendicitis is associated with high morbidity and costs, identification of the optimal treatment strategy is essential. In this article, we present the study protocol for the CAPP (Complex Appendicitis in the Pediatric Population) study.Methods and analysisThis nation-wide, multi-centre, comparative, non-randomised prospective cohort study includes all children Ethics and disseminationThe Medical Ethics Review Committee of the Amsterdam UMC, location AMC, declared that the Medical Research involving Human Subjects Act (WMO) did not apply to this study. Therefore, no official approval was required by national law. Study results will be presented in peer-reviewed scientific journals and at (inter)national conferences.Trial registration numbersNCT04755179; NL9371.

Aim To evaluate the practice patterns of the European Pediatric Surgeons' Association (EUPSA) members regarding the management of primary spontaneous pneumothorax (PSP) in children. Methods An online survey was distributed to all members of EUPSA. Results In total, 131 members from 44 countries participated in the survey. Interventional approach (78%) is the most common choice of treatment in the first episode, and most commonly, chest tube insertion (71%) is performed. In the case of a respiratory stable patient, 60% of the responders insert chest tubes if the pneumothorax is more than 2 cm. While 49% of surgeons prefer surgical intervention in the second episode, 42% still prefer chest tube insertion. Main indications for surgical treatment were the presence of bullae more than 2 cm (77%), and recurrent pneumothorax (76%). Eighty-four percent of surgeons prefer thoracoscopy and perform excision of bullae with safe margins (91%). To prevent recurrences, 54% of surgeons perform surgical pleurodesis with pleural abrasion (55%) and partial pleurectomy (22%). The responders who perform thoracoscopy use more surgical pleurodesis and prefer shorter chest tube duration than the surgeons performing open surgery (p Conclusion Most of the responders prefer chest tube insertion in the management of first episode of PSP and perform surgical treatment in the second episode in case of underlying bullae more than 2 cm and recurrent pneumothorax. The surgeons performing thoracoscopy use more surgical pleurodesis and prefer shorter chest tube duration than the responders performing open surgery. The development of evidence-based guidelines may help standardize care and improve outcomes in children with PSP.

Introduction: A nonoperative treatment strategy (NOT) with antibiotics for children with simple appendicitis could reduce anesthesia and surgery-related complications. As the implementation of a new treatment in routine clinical practice may take years, this study aims to identify barriers and facilitators for implementation of NOT for children with simple appendicitis. Materials and methods: To identify barriers and facilitators for its implementation, we conducted 14 semistructured interviews and a focus group with health insurance/hospital policymakers, surgical clinicians, and young people—together with their parents—who have been treated surgically or with antibiotics. Transcripts were open coded and categorized as per the framework for healthcare innovations by Fleuren et al. Results: We identified four main themes: (1) Appendicitis is a well-known disease. Children, parents, and clinicians regard appendectomy as routine and safe, making them reluctant toward other treatment options. (2) Clinicians regard uptake of NOT in the national appendicitis guideline necessary for its implementation. (3) For identification of patients best suited for NOT more experience and scientific evidence is needed. (4) Appendectomy and NOT have different risk and benefits making the treatment choice depended on individual patient preferences. Conclusions: By addressing how NOT and operative treatment are regarded by patients and surgeons could have a substantial impact on the implementation of NOT for children with simple appendicitis. Furthermore, the individual preferences of patients need to be taken into account when choosing between operative and NOT. In other words, offering NOT in a shared decision-making model seems the first appropriate step in its implementation.

Purpose Accurate differentiation between simple and complex appendicitis is important since differences in treatment exist. This study aimed to assess the accuracy of ultrasonography in differentiating between simple and complex appendicitis. Methods Data from children aged Results 176 children were eligible for inclusion. The mean age at the time of operation was 10.1 ± SD 4.6 years. 84 (47.7%) children had simple appendicitis and 92 (52.3%) had complex appendicitis. The use of ultrasonography yielded a sensitivity: 46%, specificity: 90%, PPV: 84%, and NPV: 60%. Conclusion Ultrasonography as standalone modality is not suitable for differentiating between simple and complex appendicitis in children. To improve preoperative differentiation, other variables such as clinical signs and laboratory data are necessary in conjunction with ultrasonography findings.

Background Little is known about stoma related morbidity in young children. Therefore, the aim of this study is to assess major morbidity after stoma formation and stoma closure and its associated risk factors. Methods All consecutive young children (age ≤ three years) who received a stoma between 1998 and 2018 at our tertiary referral center were retrospectively included. The incidence of major stoma related morbidity (Clavien-Dindo grade ≥III) was the primary outcome. This was separately analysed for stoma formation alone, stoma closure alone and all stoma interventions combined. Non-stoma related morbidity was excluded. Risk factors for major morbidity were identified using multivariable logistic regression analysis. Results In total 336 young children were included with a median follow-up of 6 (IQR:2-11) years. Of these young children, 5% (n=17/336) received a jejunostomy, 57% (n=192/336) an ileostomy, and 38% (n=127/336) a colostomy. Following stoma formation, 27% (n=92/336) of the young children experienced major stoma related morbidity, mainly consisting of high output stoma, prolapse and stoma stenosis. The major morbidity rate was 23% (n=66/292) following stoma closure, most commonly comprising anastomotic leakage/stenosis, incisional hernia and adhesive obstructions. For combined stoma interventions, major stoma related morbidity was 39% (n=130/336). Ileostomy was independently associated with a higher risk of developing major morbidity following stoma formation (OR:2.5; 95%-CI:1.3-4.7) as well as following closure (OR:2.7; 95%-CI:1.3-5.8). Conclusions Major stoma related morbidity is a frequent and severe clinical problem in young children, both after stoma formation and closure. The risk of morbidity should be considered when deliberating a stoma.

Background: Umbilical discharge is common in children and mostly attributed to infection or granuloma. However, an underlying congenital abnormality warranting surgery might also be present. Ultrasound is the imaging modality of choice to diagnose the presence of a congenital abnormality. The aim of this study is to investigate diagnostic accuracy of the ultrasound to detect pathology requiring surgical excision. Methods: All patients ≤ 18 years with umbilical discharge from January 2008 to September 2019 were retrospectively included. Diagnostic accuracy, i.e., sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR +) and negative likelihood ratio (LR −), were calculated. Results: Eighty-one patients were included and 56 were operated. The ultrasound was false positive in 10 patients and false negative in 13 patients. The sensitivity of ultrasound was 71.1% (95% CI 55.7–83.6), specificity 72.2% (54.8–85.8), PPV 76.2% (64.7–84.8), NPV 66.7% (54.8–76.8), LR + 2.6 (1.5–4.5) and LR − 0.40 (0.2–0.7). Conclusions: This study shows that the diagnostic accuracy of ultrasound for detecting underlying congenital abnormalities warranting surgery for umbilical discharge in the pediatric population is low, even with experienced pediatric radiologists. Therefore, the role of the ultrasound in the diagnostic workup and value in clinical decision making is limited. Type of study: Study of diagnostic test. Level of evidence: III

Long-term results after non-operative treatment for children with simple appendicitis seem promising, possibly avoiding appendicectomy in 70 per cent of children after a median follow-up of 5 years. The need for delayed appendicectomy more than 2 years after the initial treatment is rare (0–5 per cent) and no complications occurred past 1 year, including children who underwent delayed appendicectomy.

Purpose To compare the outcome of initially nonoperative treatment with immediate appendectomy for simple appendicitis in children. Methods Between September 2012 and June 2014 children aged 7–17 years with a radiologically confirmed simple appendicitis were invited to participate in a multicentre prospective cohort study in which they were treated with an initially nonoperative treatment strategy; nonparticipants underwent immediate appendectomy. In October 2015, their rates of complications and subsequent appendectomies, and health-related quality of life (HRQOL) were assessed. Results In this period, 25 children were treated with an initially nonoperative treatment strategy and 19 with immediate appendectomy; median (range) follow-up was 25 (16–36) and 26 (17–34) months, respectively. The percentage [95%CI] of patients experiencing complications in the initially nonoperative group and the immediate appendectomy group was 12 [4–30]% and 11 [3–31]%, respectively. In total 6/25 children (24%) underwent an appendectomy; none of the 6 patients operated subsequently experienced any postappendectomy complications. Overall, HRQOL in the nonoperative treatment group was similar to that of healthy peers. Conclusions Outcome of initially nonoperative treatment for acute simple appendicitis in children is similar to the outcome in those who undergo immediate appendectomy. Initially nonoperative management seems to be able to avoid appendectomy in 3 out of 4 children. Level of evidence 2 (prospective comparative study). This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.

Background It is now well established that there are two types of appendicitis: simple (nonperforating) and complex (perforating). This study evaluates differences in the composition of the immune cellular infiltrate in children with simple and complex appendicitis. Materials and methods A total of 47 consecutive children undergoing appendectomy for acute appendicitis between January 2011 and December 2012 were included. Intraoperative criteria were used to identify patients with either simple or complex appendicitis and were confirmed histopathologically. Immune histochemical techniques were used to identify immune cell markers in the appendiceal specimens. Digital imaging analysis was performed using Image J. Results In the specimens of patients with complex appendicitis, significantly more myeloperoxidase positive cells (neutrophils) (8.7% versus 1.2%, P

Background Anterior cutaneous nerve entrapment syndrome (ACNES) has been described as a possible cause for chronic pain in the pediatric population. However, the exact pathophysiology of ACNES is unknown. It may be caused by compression or traction of cutaneous nerve branches of intercostal nerves, or it may be the result of an infection. Therefore, we present histopathological evidence to determine the pathophysiology of ACNES. Methods A total of seven pediatric patients underwent a neurectomy for ACNES. All specimens were sent for histopathological evaluation, including immunohistochemical staining, to evaluate if there were any signs of infection, inflammation or compression. Results Seven out of seven (100%) histopathological specimens showed non-specific nerve degeneration. Immunohistochemical evaluation showed there were several CD68-positive macrophages present in the specimens. Four out of seven (57%) specimens showed the presence of a few CD3-positive T-cells, however, this was not suggestive for inflammation or infection. Conclusion Our study supports the hypothesis that ACNES is caused by compression of the nerves rather than inflammation. Level of evidence III

BACKGROUND: Two types of appendicitis are hypothesized, simple and complex, with potential different treatment strategies. To improve differentiation, underlying pathogeneses need to be further unraveled. AIM: To determine if the microbial composition in the appendix differs between children with simple and complex appendicitis. METHODS: Two-center, prospective cohort study including 40 children (0-17 years old) undergoing appendectomy for suspected appendicitis. Appendix tissue was used for IS-pro analysis to identify bacterial species by their length of 16S-23S rDNA interspacer (IS) region. Cluster analysis, based on IS-profiles, and correspondence with type of appendicitis, using Fisher exact test, was performed. Simple and complex appendicitis were compared regarding bacterial presence, intensity and diversity, using Fisher exact test and Mann-Whitney U test, respectively. RESULTS: Appendicitis was confirmed in 36 of 40 patients (16 simple, 20 complex). Cluster analysis identified 2 clusters, encompassing 34 patients. Distribution of simple and complex appendicitis was 12 (80%) and 3 (20%) versus 3 (16%) and 16 (84%) patients for clusters 1 and 2, respectively (P < 0.001). Complex appendicitis was on phylum level characterized by an increased intensity (Bacteroidetes P = 0.001, Firmicutes, Actinobacteria, Fusobacteria and Verrucomicrobia (FAFV) P = 0.005 and Proteobacteria P < 0.001) and diversity (Bacteroidetes P = 0.001 and Proteobacteria P = 0.016) and an increased abundance of 5 species (Alistipes finegoldii P = 0.009, Bacteroides fragilis P = 0.002, Escherichia coli P = 0.014, Parvimonas micra P = 0.022 and Sutterella spp P = 0.026). CONCLUSIONS: The microbial composition of the appendix differs between children with simple and complex appendicitis, regarding both composition and diversity. Future research should focus on the role of these bacteria in the pathogenesis of both types and its implications for preoperative diagnostics.

IntroductionAppendicitis is a global disease affecting roughly 1 in every 12 people in the world, with the highest incidence between ages 10 and 19 years. To date, a wide variety of health outcomes have been reported in randomised controlled trials and meta-analyses evaluating treatments for appendicitis. This is especially the case in studies comparing non-operative treatment with operative treatment. A set of standard outcomes, to be reported in all future trials, is needed to allow for adequate comparison and interpretation of clinical trial results and to make data pooling possible. This protocol describes the development of such a global core outcome set (COS) to allow unified reporting of treatment interventions in children with acute uncomplicated appendicitis.Methods and analysisWe use current international standard methodology for the development and reporting of this COS. Its development consists of three phases: (1) an update of the most recent systematic review on outcomes reported in uncomplicated paediatric appendicitis research to identify additional outcomes, (2) a three-step global Delphi study to identify a set of core outcomes for which there is consensus between parents and (paediatric) surgeons and (3) an expert meeting to finalise the COS and its definitions. Children and young people will be involved through their parents during phase 2 and will be engaged directly using a customised face-to-face approach.Ethics and disseminationThe medical research ethics committee of the Academic Medical Center Amsterdam has approved the study. Each participating country/research group will ascertain ethics board approval. Electronic informed consent will be obtained from all participants. Results will be presented in peer-reviewed academic journals and at (international) conferences.Trial registration numberCOMET registration: 1119

Background: Laparoscopic appendectomy is the predominant method of treatment of acute appendicitis. There is insufficient evidence on the most effective management of the appendix stump. The aim of this study was to investigate the relative effectiveness and provide a treatment ranking of different options for securing the appendix stump. Methods: Electronic databases were searched to identify randomized controlled trials comparing ligation methods of the appendix. The primary outcomes were organ/space infection and superficial operative site infection. We performed a network meta-analysis and estimated the pairwise relative treatment effects of the competing interventions using the odds ratio and its 95% confidence interval. We obtained a hierarchy of the competing interventions using rankograms and the surface under the cumulative ranking curve. Results: Forty-three randomized controlled trials were eligible and provided data for >5,000 patients. Suture ligation seemed to be the most effective treatment strategy, in terms of both organ/space infection and superficial operative site infection. Statistical significance was reached for the comparisons of clip versus endoloop (odds ratio 0.56, 95% confidence interval, 0.32-0.96) for organ/space infection; and suture versus clip (odds ratio 0.20, 95% confidence interval 0.08-0.55) and clip versus endoloop (odds ratio 2.22, 95% confidence interval 1.56-3.13) for superficial operative site infection. The network was informed primarily by indirect treatment comparisons. Conclusion: The use of suture ligation of the appendix in laparoscopic appendectomy seems to be superior to other methods for the composite parameters of organ/space and superficial operative site infection.

Background To determine if non-operative treatment is safe in children with acute appendicitis, we evaluated the incidence of unexpected findings after an appendectomy in children, and the influence they have on subsequent treatment. Methods A historical cohort study (January 2004–December 2014) was performed including children, aged 0–17 years, who underwent an appendectomy for the suspicion of acute appendicitis. Patients were divided based upon histopathological examination. Unexpected findings were reviewed, as well as the subsequent treatment plan. Results In total 484 patients were included in this study. In the overall group, unexpected findings were noted in 10 (2.1%) patients of which two patients intra-operatively with a non-inflamed appendix (Ileitis terminalis N = 1 and ovarian torsion N = 1) and in 8 patients on histopathological examination. The latter group consisted of 4 patients with concomitant simple appendicitis (parasitic infection N = 3 and Walthard cell rest N = 1), two with concomitant complex appendicitis (carcinoid N = 1 and parasitic infection N = 1) and two patients with a non-inflamed appendix (endometriosis N = 1 and parasitic infection N = 1). Treatment was changed in 4 patients ( Conclusions Results from this study corroborate the safety of non-operative strategy for acute simple appendicitis, as the occurrence of unexpected findings was low, with extremely few necessary changes of the treatment plan because of serious findings. Type of study Prognosis study. Level of evidence Level 2 (retrospective cohort study).

Background: To compare the risk of complications between initial nonoperative treatment and appendectomy of uncomplicated (simple) appendicitis in children. Methods: Systematic literature search. Eligible for inclusion were both and randomized controlled trials and cohort studies including children inwhich the outcome of nonoperative treatment of uncomplicated appendicitis was reported with a minimum follow-up period of one year. Two authors extracted data independently and assessed quality. Primary outcome parameter was the percentage of children experiencing complications. Secondary outcomes were early failures, recurrent appendicitis and appendectomies, for all indications and on request. Results: Five of the 2051 articles screenedwere eligible for inclusion, including 147 children (nonoperative treatment) and 173 children (appendectomy) with one year follow-up. Percentage of children experiencing complications ranged from 0 to 13% versus 0-17% for nonoperative and appendectomy, respectively. Nonoperative treatment avoided an appendectomy in 62-81% of the children after one year follow-up. Conclusion: The evidence base for initial nonoperative treatment of acute uncomplicated appendicitis in children is by far insufficient. It suggests that the percentage of patients experiencing complications in the initial nonoperative treatment group is comparable to the appendectomy group, and it may avoid an appendectomy in the large majority of children after one year follow-up. Type of study: Systematic review. Level of evidence: 1. (C) 2017 Elsevier Inc. All rights reserved

BACKGROUND: Trimodality treatment leads to improved survival for superior sulcus tumor (SST) patients. Not much is known about the impact of this treatment on arm function and patient quality of life. OBJECTIVE: To analyze arm function and quality of life in SST patients undergoing trimodality treatment. METHODS: This was a prospective cohort study of consecutive SST patients treated with trimodality treatment that was conducted between April 1, 2010 and October 31, 2012. We obtained informed consent for 20 of 22 eligible patients. The 36-item Short Form Health Survey (SF-36) and disabilities of the arm, shoulder, and hand (DASH) questionnaires were used to asses patient quality of life and subjective arm function at 0 (preoperative day), 3, and 12 months after trimodality treatment. RESULTS: DASH scores were significantly lower at 3 and 12 months ( d443; = .024 and d443; = .011) compared with preoperative scores. Significantly lower scores were reported for the SF-36 domains of physical functioning at 12 months ( d443; = .020) and of physical role functioning at 3 months ( d443; = .041), and significantly more pain was reported at 3 and 12 months ( d443; = .006 and d443; = .019, respectively). Patients who underwent T1 nerve root resection had lower scores for the SF-36 domain health change at 3 months ( d443; = .037) compared with those in whom the T1 root was spared. For all other domains no differences were found. LIMITATIONS: Small sample size; patient pre-chemoradiation function and quality of life unknown. CONCLUSION: Subjective arm function and patient quality of life is reduced following trimodality treatment. Resection of the T1 nerve root has no significant long-term effect on the subjective arm function and quality of life.

Non-operative treatment of uncomplicated appendicitis in children is gaining ground. Pending definitive evidence regarding its effectiveness, there is a call to evaluate clinical recovery after non-operative treatment. In this study, we analyzed data collected during initial admission of a multicenter prospective cohort in which children, 7–17 year, were treated non-operatively for uncomplicated appendicitis. During admission clinical parameters (pain and gastro-intestinal symptoms), inflammation parameters and sequential abdominal ultrasound were recorded. In total, 45 children were included, 42(93%) were discharged without the need for appendectomy; median [IQR] pain scores on admission were 5 [4–7], decreasing to 2 [0–3] after 1 day of treatment. Initially, 28/42 (67%) reported nausea and 19/42 (45%) vomiting; after 1 day, this was 3/42 (7%) and 1/42 (2%), respectively. White blood cell count declined from a median [IQR] of 12.9 [10.7–16.7] 10E9/L on admission to 7.0 [5.8–9.9] 10E9/L on day 1. Median [IQR] C-reactive protein levels increased from 27.5 [9–69] mg/L on admission to 48 [22–80] mg/L on day 1, declining to 21.5 [11–42] mg/L on day 2. Follow-up ultrasound showed no signs of complicated appendicitis in any of the patients. Conclusion: Clinical symptoms resolved in most children after 1 day of non-operative treatment. This suggests that non-operative treatment is a viable alternative to appendectomy regarding clinical recovery. Trail registration: NCT01356641 What is Known: • Non-operative treatment of uncomplicated appendicitis in children is safe and its use around the world is gaining ground, however high quality evidence from adequately designed randomized trials is still lacking.• Concerns have been raised regarding the potentially prolonged clinical recovery associated with non-operative treatment. What is New: • Most clinical symptoms resolve after 1 day of non-operative treatment in the majority of children. Electronic supplementary material The online version of this article (10.1007/s00431-018-3277-9) contains supplementary material, which is available to authorized users.

PURPOSE: To identify available clinical prediction rules (CPRs) and investigate their ability to rule out appendicitis in children presenting with abdominal pain at the emergency department, and accordingly select CPRs that could be useful in a future prospective cohort study.METHODS: A literature search was conducted to identify available CPRs. These were subsequently tested in a historical cohort from a general teaching hospital, comprising all children (RESULTS: Twelve CPRs were tested in a cohort of 291 patients, of whom 87 (29.9%) suffered from acute appendicitis. The Ohmann score, Alvarado score, modified Alvarado score, Pediatric Appendicitis score, Low-Risk Appendicitis Rule Refinement, Christian score, and Low Risk Appendicitis Rule had a negative likelihood ratio 0.1. Three CPRs were excluded because the score could not be calculated for at least 50% of patients.CONCLUSION: This study identified seven CPRs that could be used in a prospective cohort study to compare their ability to rule out appendicitis in children and investigate if clinical monitoring and re-evaluation instead of performing additional investigations (i.e., ultrasound) is a safe treatment strategy in case there is low suspicion of appendicitis.

This study aims to provide an overview of both our own experience and the available literature on the treatment of post-appendectomy abscess (PAA) in children. We performed a historical cohort study encompassing all children aged 0–17 years old treated for a radiologically confirmed PAA between 2007 and 2013. Their medical charts were reviewed and descriptive analyses were performed. A literature search on the treatment of PAA in children was performed. In our cohort, 25 out of 372 (7 %) children developed a PAA. Thirteen were treated with a noninvasive strategy and 12 with an invasive strategy (percutaneous or surgical drainage). The immediate success rate was 9/13 (69 %) and 8/12 (67 %) for the noninvasive and invasive strategy, respectively. In both groups, four children (31 and 33 % resp.) required delayed interventions after their initial treatment. In the literature review, six studies were included which reported a median (range) frequency of persistent or recurrent abscess of 9 % (0–30 %), 50 % (0–100 %) and 24 % (0–33 %) for the antibiotic (noninvasive), percutaneous drainage (invasive) and surgical drainage strategies, respectively. Conclusion: Although confounding by indication cannot be excluded, we recommend noninvasive treatment as a safe strategy for PAA in children with stable condition. What is known: • Post-appendectomy abscess is a well-known and feared complication, occurring in up to 24 % of the children treated surgically for appendicitis.• Several strategies are available to treat this condition, all with advantages and disadvantages. What is new: • Noninvasive strategy is a safe strategy for children with a PAA in a stable condition. • An overview of the literature (the first to our knowledge) supports the above-mentioned statement.

Although a national guideline has been implemented, the optimal approach for appendectomy in children remains subject of debate in the Netherlands. Opponents of laparoscopy raise their concerns regarding its use in complex appendicitis as it is reported to be associated with an increased incidence of intra-abdominal abscesses. The aim of this study was to evaluate the outcome of surgical approaches in both simple and complex appendicitis in paediatric patients. A 10-year retrospective cohort study was performed (2001-2010) in paediatric patients treated for suspected acute appendicitis. Patients were divided into either simple or complex appendicitis and into different age groups. Primary outcome parameters were complication rate (intra-abdominal abscess (IAA), superficial surgical site infection (SSI) and readmission) and hospital stay. In total, 878 patients have been treated (median age 12, range 0-17 years). Two-thirds of the patients younger than 6 years had complex appendicitis, compared to one quarter in the group aged 13-18. In the complex appendicitis group, LA was associated with more IAA and early readmissions. In the simple appendicitis group, the complication rate was comparable between the two approaches. Significantly more IAAs were seen after LA in the youngest age group. This study demonstrates the unfavourable outcome of LA in the youngest age group and in patients with complex appendicitis. Therefore, we advise to treat these patients with an open approach