Cardiac anomalies in children with congenital duodenal obstruction: a systematic review with meta-analysis.

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.

Implementing Nonoperative Treatment Strategy for Simple Pediatric Appendicitis: A Qualitative Study.

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.

Outcomes after appendectomy in children with acute appendicitis treated at a tertiary paediatric centre: results from a retrospective cohort study.

PURPOSE: In 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. METHODS: This 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). RESULTS: A 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. CONCLUSION: This 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.

Laparoscopic versus open pediatric inguinal hernia repair: state-of-the-art comparison and future perspectives from a meta-analysis.

BACKGROUND: Laparoscopic inguinal hernia repair in children is increasingly performed as it allows contralateral inspection and potentially results in shorter operation time and less complications. Evidence from meta-analyses of randomized controlled trials (RCTs) regarding the superiority of laparoscopic versus open hernia repair is lacking. METHODS: A systematic literature search was performed querying PubMed, Embase, MEDLINE, and the Cochrane Library databases. RCTs comparing laparoscopic with open hernia repair in children were considered eligible, without year and language restrictions. Cochrane Risk of Bias tool was used for quality assessment. Data were pooled using a random-effects model. Subgroup analyses were performed according to the laparoscopic suturing technique (i.e., intracorporeal or extracorporeal). RESULTS: Eight RCTs (n = 733 patients; age range 4 months-16 years) were included in this meta-analysis. Laparoscopic (LH) and open (OH) hernia repair was performed in 375 and 358 patients, respectively. Complications (seven RCTs, n = 693; pooled OR 0.50, 95% CI 0.14 to 1.79), recurrences (seven RCTs, n = 693; pooled OR 0.88, 95% CI 0.20 to 3.88), and MCIH rates (four RCTs, n = 343; pooled OR 0.28, 95% CI 0.04 to 1.86) were not different between the groups. LH resulted in shorter bilateral operation time (Five RCTs, n = 194; weighted mean difference (WMD) - 7.19, 95% CI - 10.04 to - 4.34). Unilateral operation time, length of hospital stay, and time to recovery were similar. There was insufficient evidence to assess postoperative pain and wound cosmesis, and evidence of substantial heterogeneity between the included studies. Subgroup analyses demonstrated less complications and shorter unilateral operation time for extracorporeal suturing and shorter length of hospital stay for intracorporeal suturing. CONCLUSIONS AND RELEVANCE: No definite conclusions to decide on the superiority of one of either treatment strategies can yet be drawn from the available literature. There was evidence of substantial heterogeneity and the clinical relevance of most estimated effects is very limited.

Esophageal mucosal integrity improves after laparoscopic antireflux surgery in children with gastroesophageal reflux disease.

BACKGROUND: Esophageal intraluminal baseline impedance reflects the conductivity of the esophageal mucosa and may be an instrument for in vivo evaluation of mucosal integrity in children with gastroesophageal reflux disease (GERD). Laparoscopic antireflux surgery (LARS) is a well-established treatment option for children with proton pump inhibitory (PPI) therapy resistant GERD. The effect of LARS in children on baseline impedance has not been studied in detail. The aim of this study was to evaluate the effect of LARS on baseline impedance in children with GERD. METHODS: This is a prospective, multicenter, nationwide cohort study (Dutch national trial registry: NTR2934) including 25 patients [12 males, median age 6 (range 2-18) years] with PPI-resistant GERD scheduled to undergo LARS. Twenty-four hour multichannel intraluminal impedance pH monitoring (MII-pH monitoring) was performed before and 3 months after LARS. Baseline impedance was evaluated during consecutive 2-h intervals in the 24-h tracings. RESULTS: LARS reduced acid exposure time from 8.5 % (6.0-16.2 %) to 0.8 % (0.2-2.8 %), p < 0.001. Distal baseline impedance increased after LARS from 2445 Ω (1147-3277 Ω) to 3792 Ω (3087-4700 Ω), p < 0.001. Preoperative baseline impedance strongly correlated with acid exposure time (r -0.76, p < 0.001); however, no association between symptomatic outcome and baseline impedance was identified. CONCLUSIONS: LARS significantly increased baseline impedance likely reflecting recovery of mucosal integrity. As the change in baseline impedance was not associated with the clinical outcome of LARS, other factors besides mucosal integrity may contribute to symptom perception in children with GERD.

Patient-Reported Defecation and Micturition Problems Among Adults Treated for Sacrococcygeal Teratoma During Childhood--The Need for New Surveillance Strategies.

BACKGROUND: To evaluate defecation and micturition complaints in adults treated for sacrococcygeal teratoma (SCT) during childhood and to identify risk factors for soiling, urinary incontinence, and constipation beyond childhood. PROCEDURE: Records of patients aged ≥18 treated for SCT during infancy in the Netherlands were retrospectively reviewed. Frequency and severity of soiling, constipation, and urinary incontinence were evaluated using questionnaires designed in line with the Krickenbeck classification. Problems during childhood were compared to outcomes at adult age in part of the cohort. Associations between patient- and disease-related factors with complaints beyond childhood were analyzed with the chi-square test or Fisher's exact test, when appropriate. RESULTS: Of 47 included patients (mean age 26.2 years, SD ±6.5), 49% reported at least one defecation or micturition complaint. Urinary incontinence was present in 30% and had a greater negative impact than soiling (24%). Ten patients (21%) reported constipation; five found this severely bothering. Three patients reported social restrictions due to defecation or micturition complaints (6.4%). While sex and tumor histology were not identified as risk factors, a tumor diameter of >10 cm and Altman type I or type II SCT were associated with constipation during adulthood. CONCLUSIONS: One-third of the patients treated for SCT during childhood reported urinary and defecation problems beyond childhood. In only a minority of cases, these led to social restrictions. A greater tumor diameter was associated with a higher risk of constipation during adulthood. Prolonged surveillance strategies are advised for all patients with SCT.

Normothermic machine perfusion of the kidney: better conditioning and repair?

Kidney transplantation is limited by hypothermic preservation techniques. Prolonged periods of cold ischaemia increase the risk of early graft dysfunction and reduce long-term survival. To extend the boundaries of transplantation and utilize kidneys from more marginal donors, improved methods of preservation are required. Normothermic perfusion restores energy levels in the kidney allowing renal function to be restored ex vivo. This has several advantages: cold ischaemic injury can be avoided or minimized, the kidney can be maintained in a stable state allowing close observation and assessment of viability and lastly, it provides the ideal opportunity to add therapies to directly manipulate and improve the condition of the kidney. This review explores the experimental and clinical evidence for ex vivo normothermic perfusion in kidney transplantation and its role in conditioning and repair.

Children With Anorectal Malformations, Hirschsprung Disease, and Their Siblings: Proxy Reports and Self-Reports.

OBJECTIVES: The aim of the present study was to compare parent proxy reports with that of self-reports of children with anorectal malformations (ARMs) or Hirschsprung disease (HD) and healthy siblings and thereafter was examine whether these comparisons differed between patients and their siblings. METHODS: Parents (n = 98) of either children with ARM (n = 44) or HD (n = 54) and a healthy sibling (n = 98) recruited from the 6 Dutch pediatric surgical centers and from the ARM and HD patient societies were included in this cross-sectional multilevel study. Agreement between child self-reports and parent proxy reports was compared through mean differences and through (intraclass) correlations. We conducted multilevel analyses to take dependencies between assessments within families into account. RESULTS: All of the children (children with ARM or HD and their siblings) reported more pain and symptoms than their parents reported. We also found that only children with ARM or HD reported less positive emotions than their parents. Furthermore, higher correlations were found between parent proxy reports and patient-self reports than between parent proxy reports and sibling self-reports on cognitive functioning and social interaction. CONCLUSIONS: Parents tend to overestimate the physical functioning of both their ill and healthy children, and overestimate the emotional functioning of only their children with ARM or HD. Furthermore, children with ARM or HD and parents agree more on health-related quality of life domains than healthy children and parents.

Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease.

OBJECTIVES: A nonrelaxing internal anal sphincter is present in a relatively large proportion of children with surgically treated Hirschsprung disease (HD) and can cause obstructive gastrointestinal symptoms. The short- and long-term outcome and adverse effects of intrasphincteric botulinum toxin (Botox) injections in children with obstruction after surgically treated HD are evaluated. METHODS: The outcome of children with surgically treated HD treated with intrasphincteric Botox injections for obstructive symptoms was analyzed with a retrospective chart review between 2002 and 2013 in the University Medical Centers of Maastricht and Nijmegen. RESULTS: A total of 33 patients were included. The median time of follow-up was 7.3 years (range 1-24). A median of 2 (range 1-5) injections were given. Initial improvement was achieved in 76%, with a median duration of 4.1 months (range 1.7-58.8). Proportion of children hospitalized for enterocolitis decreased after treatment from 19 to 7. A good long-term response was found in 49%. Two children experienced complications: transient pelvic muscle paresis with impairment of walking. In both children symptoms resolved within 4 months without treatment. CONCLUSIONS: Intrasphincteric Botox injections in surgically treated HD are an effective long-term therapy in approximately half of our patients with obstructive symptoms. The possibility of adverse effects should be noticed.

Evaluation of pregnancy and delivery in 13 women who underwent resection of a sacrococcygeal teratoma during early childhood.

BACKGROUND: Sacrococcygeal teratoma resection often brings changes in pelvic anatomy and physiology with possible consequences for defecation, micturition and sexual function. It is unknown, whether these changes have any gynecological and obstetric sequelae. Until now four pregnancies after sacrococcygeal teratoma resection have been described and cesarean section has been suggested to be the method of choice for delivery. We evaluated the pregnancy course and mode of delivery in women previously treated for a sacrococcygeal teratoma. METHODS: The records of all patients who underwent sacrococcygeal teratoma resection after 1970 in one of the six pediatric surgical centers in the Netherlands were reviewed retrospectively. Women aged 18 years and older were eligible for participation. Patient characteristics, details about the performed operation and tumor histology were retrieved from the records. Consenting participants completed a questionnaire addressing fertility, pregnancy and delivery details. RESULTS: Eighty-nine women were eligible for participation; 20 could not be traced. Informed consent was received from 41, of whom 38 returned the completed questionnaire (92.7%). Thirteen of these 38 women conceived, all but one spontaneously. In total 20 infants were born, 17 by vaginal delivery and 3 by cesarean section, in one necessitated by previous intra-abdominal surgery as a consequence of sacrococcygeal teratoma resection. Conversion to a cesarean section was never necessary. None of the 25 women without offspring reported involuntary childlessness. CONCLUSIONS: There are no indications that resection of a sacrococcygeal teratoma in female patients is associated with reduced fertility: spontaneous pregnancy is possible and vaginal delivery is safe for mother and child, irrespective of the sacrococcygeal teratoma classification or tumor histology.

Identifying Preoperative Clinical Characteristics of Unexpected Gastrointestinal Perforation in Infants-A Retrospective Cohort Study.

BACKGROUND: Infants presenting with unexpected pneumoperitoneum upon abdominal X-ray, indicating a gastrointestinal perforation (GIP), have a surgical emergency with potential morbidity and mortality. Preoperative determination of the location of perforation is challenging but will aid the surgeon in optimizing the surgical strategy, as colon perforations are more challenging than small bowel perforations. Therefore, the aim of this study is to provide an overview of preoperative patient characteristics, determine the differences between the small bowel and colon, and determine underlying causes in a cohort of infants with unexpected GIP. METHODS: All infants (age ≤ 6 months) who presented at our center with unexpected pneumoperitoneum (no signs of pneumatosis before) undergoing surgery between 1996 and 2024 were retrospectively included. The differences between the location of perforation were analyzed using chi-squared and t-tests. Bonferroni correction was used to adjust for multiple tests. RESULTS: In total, 51 infants presented with unexpected pneumoperitoneum at our center, predominantly male (N = 36/51) and premature (N = 40/51). Among them, twenty-six had small bowel, twenty-two colon, and three stomach perforations. Prematurity (p = 0.001), birthweight < 1000 g (p = 0.001), respiratory support (p = 0.001), and lower median arterial pH levels (p = 0.001) were more present in patients with small bowel perforation compared with colon perforations. Pneumatosis intestinalis was more present in patients with colon perforation (p = 0.004). All patients with Hirschsprung disease and cystic fibrosis had colon perforation. The final diagnoses were mainly focal intestinal perforations (N = 27/51) and necrotizing enterocolitis (N = 9/51). CONCLUSIONS: Infants with unexpected GIP, birthweight < 1000 g, and prematurity have more risk for small bowel perforation. In case of colon perforation, additional screening (for Hirschsprung and cystic fibrosis) should be considered.

Transplantation of kidneys from paediatric DCD donors: a comparison with DBD donors.

BACKGROUND: Although acceptable outcomes have been reported in kidney transplantation from donation after cardiac death (DCD), little is known about kidney transplantation from paediatric DCD. The objective of this study was to compare the outcome of kidney transplantation using paediatric DCD with the outcome of paediatric donation after brain death (DBD). METHODS: Recipients from DCD and DBD donors <18 years of age transplanted in the Netherlands between January 1981 and July 2006 were included in this study. Ninety-one patients were transplanted with kidneys from paediatric DCD donors and 405 patients received grafts from paediatric DBD donors. RESULTS: Grafts from DCD donors were associated with higher percentage of primary non-function (9 versus 2%, P < 0.01) and delayed graft function (48 versus 8%, P < 0.001) compared with DBD donor grafts. Estimated glomerular filtration rate did not differ between groups (57 ± 17 versus 58 ± 21 mL/min at 1 year and 62 ± 14 versus 57 ± 22 mL/min at 5 years, respectively). After correction for confounding variables, the risk of graft failure was higher in the DCD group [hazard ratio 2.440 (95% confidence interval (CI) 1.280-4.650; P = 0.007]. Patient survival, however, was similar between groups [hazard ratio 1.559 (95% CI 0.848-2.867; P = 0.153)]. CONCLUSIONS: Paediatric DCD kidneys represent a valuable source of donor kidneys that has not been fully utilized. Although transplantation of paediatric DCD kidneys is associated with a higher risk of graft failure than transplantation of paediatric DBD kidneys, results are comparable with adult donors. We therefore conclude that paediatric DCD kidneys can be safely added to the donor pool.

Variability in protocols on donation after circulatory death in Europe.

INTRODUCTION: Organ donation after circulatory death (DCD) has become an accepted strategy to reduce the shortage of organs for transplantation in many European countries. The use and number of DCD donors varies between countries. The purpose of this study was to evaluate the available protocols for DCD in Europe. METHODS: We contacted national transplant societies and responsible transplant co-ordinators in the countries that perform DCD to obtain DCD protocols. We compared information on the protocols and additional data including: inclusion and exclusion criteria for donation, legislation, determination of death and preservation methods. RESULTS: In ten European countries DCD is performed, eight of which describe the methods in protocols. There are large differences in used DCD categories, legislation and the way death is determined. Protocols differ in the detail in which DCD procedures are described and the way methods are supported by additional consensus statements and ethical frameworks. CONCLUSIONS: Although DCD is an established strategy to enlarge the donor pool and to contribute to the reduction of the waiting list for transplantation, its potential has not been fully utilized yet. To further promote DCD transplantation, it is important to share expertise and obtain consensus, so that this can be translated into more uniform and solid protocols supported by the competent authorities, transplant and intensive care professionals, which may eventually result in a further promotion of DCD transplantation in Europe.

Variability in the Reporting of Baseline Characteristics, Treatment, and Outcomes in Esophageal Atresia Publications: A Systematic Review.

INTRODUCTION: As survival rates of infants born with esophageal atresia (EA) have improved considerably, research interests are shifting from viability to morbidity and longer-term outcomes. This review aims to identify all parameters studied in recent EA research and determine variability in their reporting, utilization, and definition. MATERIALS AND METHODS: Following PRISMA guidelines, we performed a systematic review of literature regarding the main EA care process, published between 2015 and 2021, combining the search term "esophageal atresia" with "morbidity," "mortality," "survival," "outcome," or "complication." Described outcomes were extracted from included publications, along with study and baseline characteristics. RESULTS: From 209 publications that met the inclusion criteria, 731 studied parameters were extracted and categorized into patient characteristics (n = 128), treatment and care process characteristics (n = 338), and outcomes (n = 265). Ninety-two of these were reported in more than 5% of included publications. Most frequently reported characteristics were sex (85%), EA type (74%), and repair type (60%). Most frequently reported outcomes were anastomotic stricture (72%), anastomotic leakage (68%), and mortality (66%). CONCLUSION: This study demonstrates considerable heterogeneity of studied parameters in EA research, emphasizing the need for standardized reporting to compare results of EA research. Additionally, the identified items may help develop an informed, evidence-based consensus on outcome measurement in esophageal atresia research and standardized data collection in registries or clinical audits, thereby enabling benchmarking and comparing care between centers, regions, and countries.

Transition Zone Pull-through in Patients with Hirschsprung Disease: Is Redo Surgery Beneficial for the Long-term Outcomes?

INTRODUCTION: Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients. METHODS: We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up. RESULTS: Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12-260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63). CONCLUSION: Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.

Development of an international core outcome set for treatment trials in paediatric inguinal hernia: protocol for a three-phase study including a systematic review and Delphi survey.

INTRODUCTION: In children, open inguinal hernia repair has been the gold standard for treatment, but with recent technical advancements in laparoscopy, laparoscopic hernia repair is gaining popularity. Despite available results from comparative studies, there is still no consensus regarding the superiority of open versus laparoscopic treatment strategy. An important reason for lack of consensus is the large heterogeneity in the trials' reported outcomes and outcome definitions, which limits comparisons between studies and precludes conclusions regarding the superiority of treatment strategies. The development and implementation of a core outcome set (COS) is a solution for this heterogeneity in the selection, measurement and reporting of trial outcome measures across studies. Currently, there is no COS for the treatment of paediatric inguinal hernia. METHODS AND ANALYSIS: The aim of this project is to reach international consensus on a minimal set of outcomes that should be measured and reported in all future clinical trials investigating inguinal hernia repair in children. The development process comprises three phases. First, we identify outcome domains associated with paediatric inguinal hernia repair from a patient perspective and through a systematic review of the literature using EMBASE, MEDLINE and the Cochrane Library databases. Second, we conduct a three-step Delphi study to identify and prioritise 'core' outcomes for the eventual minimal set. In the third phase, an expert meeting is held to establish the final COS and develop implementation strategies with participants from all stakeholder groups: healthcare professionals, parents and patients' representatives. The final COS will be reported in accordance with the COS-Standards for Reporting statement. ETHICS AND DISSEMINATION: The medical research ethics committee of the Amsterdam UMC confirmed that the Dutch Medical Research Involving Human Subjects Act (WMO) does not apply to this study and that full approval by the committee is not required. Electronic informed consent will be obtained from all participants. Results will be presented in peer-reviewed academic journals and at relevant conferences. PROSPERO REGISTRATION NUMBER: CRD42021281422.

Routine contrast enema prior to stoma reversal seems only required following treatment for necrotizing enterocolitis: An evaluation of the diagnostic accuracy of the contrast enema.

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.

Appendicitis and its associated mortality and morbidity in infants up to 3 months of age: A systematic review.

Background and Aims: Although appendicitis is rare in young infants, the reported mortality is high. Primary aim of this systematic review was to provide updated insights in the mortality and morbidity (postoperative complications, Clavien-Dindo grades I-IV) of appendicitis in infants ≤3 months of age. Secondary aims comprised the evaluation of patient characteristics, diagnostic work-up, treatment strategies, comorbidity, and factors associated with poor outcome. Methods: This systematic review was reported according to the PRISMA statement with a search performed in Pubmed, Embase and Web of Science (up to September 5th 2022). Original articles (published in English ≥1980) reporting on infants ≤3 months of age with appendicitis were included. Both patients with abdominal appendicitis and herniated appendicitis (such as Amyand's hernia) were considered. Data were provided descriptively. Results: In total, 131 articles were included encompassing 242 cases after identification of 4294 records. Overall, 184 (76%) of the 242 patients had abdominal and 58 (24%) had herniated appendicitis. Two-hundred (83%) of the patients were newborns (≤28 days) and 42 (17%) were infants between 29 days and ≤3 months of age. Either immediate, or after initial conservative treatment, 236 (98%) patients underwent surgical treatment. Some 168 (69%) patients had perforated appendicitis. Mortality was reported in 20 (8%) patients and morbidity in an additional 18 (8%). All fatal cases had abdominal appendicitis and fatal outcome was relatively more often reported in newborns, term patients, patients with relevant comorbidity, nonperforated appendicitis and those presented from home. Conclusion: Mortality was reported in 20 (8%) infants ≤3 months of age and additional morbidity in 18 (8%). All patients with fatal outcome had abdominal appendicitis. Several patient characteristics were relatively more often reported in infants with poor outcome and adequate monitoring, early recognition and prompt treatment may favour the outcome.

Additional Anomalies in Children with Gastroschisis and Omphalocele: A Retrospective Cohort Study.

BACKGROUND: Congenital abdominal wall defects might be associated with other anomalies, such as atresia in gastroschisis and cardiac anomalies in omphalocele patients. However, in the current literature, an overview of these additional anomalies and potential patient-specific risk factors is missing. Therefore, we aimed to assess the prevalence of associated anomalies and their patient-specific risk factors in patients with gastroschisis and omphalocele. METHODS: A mono-center retrospective cohort study between 1997 and 2023 was performed. Outcomes were the presence of any additional anomalies. Risk factors were analyzed via logistic regression analysis. RESULTS: In total, 122 patients were included, of whom 82 (67.2%) had gastroschisis, and 40 (32.8%) had omphalocele. Additional anomalies were identified in 26 gastroschisis patients (31.7%) and in 27 omphalocele patients (67.5%). In patients with gastroschisis, intestinal anomalies were most identified (n = 13, 15.9%), whereas, in patients with omphalocele, cardiac anomalies were most identified (n = 15, 37.5%). Logistic regression showed that cardiac anomalies were associated with complex gastroschisis (OR: 8.5; CI-95%: 1.4-49.5). CONCLUSIONS: In patients with gastroschisis and omphalocele, intestinal and cardiac anomalies were most identified, respectively. Cardiac anomalies were found to be a risk factor for patients with complex gastroschisis. Therefore, regardless of the type of gastroschisis and/or omphalocele, postnatal cardiac screening remains important.

Machine perfusion or cold storage in deceased-donor kidney transplantation.

BACKGROUND: Static cold storage is generally used to preserve kidney allografts from deceased donors. Hypothermic machine perfusion may improve outcomes after transplantation, but few sufficiently powered prospective studies have addressed this possibility. METHODS: In this international randomized, controlled trial, we randomly assigned one kidney from 336 consecutive deceased donors to machine perfusion and the other to cold storage. All 672 recipients were followed for 1 year. The primary end point was delayed graft function (requiring dialysis in the first week after transplantation). Secondary end points were the duration of delayed graft function, delayed graft function defined by the rate of the decrease in the serum creatinine level, primary nonfunction, the serum creatinine level and clearance, acute rejection, toxicity of the calcineurin inhibitor, the length of hospital stay, and allograft and patient survival. RESULTS: Machine perfusion significantly reduced the risk of delayed graft function. Delayed graft function developed in 70 patients in the machine-perfusion group versus 89 in the cold-storage group (adjusted odds ratio, 0.57; P=0.01). Machine perfusion also significantly improved the rate of the decrease in the serum creatinine level and reduced the duration of delayed graft function. Machine perfusion was associated with lower serum creatinine levels during the first 2 weeks after transplantation and a reduced risk of graft failure (hazard ratio, 0.52; P=0.03). One-year allograft survival was superior in the machine-perfusion group (94% vs. 90%, P=0.04). No significant differences were observed for the other secondary end points. No serious adverse events were directly attributable to machine perfusion. CONCLUSIONS: Hypothermic machine perfusion was associated with a reduced risk of delayed graft function and improved graft survival in the first year after transplantation. (Current Controlled Trials number, ISRCTN83876362.)