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.

Background: Duodenal obstruction (DO) is a congenital anomaly that is highly associated with other anomalies, such as cardiac anomalies and trisomy 21. However, an overview of additional anomalies and patient-specific risk factors for cardiac anomalies is lacking. Potential association with the vertebral, anorectal, cardiac, trachea-esophageal, renal and limb anomalies (VACTERL) spectrum remains unknown. Therefore, we aim to examine the incidence of associated anomalies, a VACTERL-spectrum association and patient-specific risk factors for cardiac anomalies in patients with DO. Methods: A retrospective cohort study was performed between 1996 and 2021. Outcomes were the presence of any additional anomalies. Risk factors for cardiac anomalies were analyzed using multivariate logistic regression. Results: Of 112 neonates with DO, 47% (N = 53/112) had one associated anomaly and 38% (N = 20/53) had multiple anomalies. Cardiac anomalies (N = 35/112) and trisomy 21 (N = 35/112) were present in 31%. In four patients, VACTERL-spectrum was discovered, all with cardiac anomalies. Trisomy 21 was found to be a risk factor for cardiac anomalies (OR:6.5; CI-95%2.6–16.1). Conclusion: Associated anomalies were present in half of patients with DO, of which cardiac anomalies and trisomy 21 occurred most often, and the VACTERL-spectrum was present in four patients. Trisomy 21 was a significant risk factor for cardiac anomalies. Therefore, we recommend a preoperative echocardiogram in patients with DO. In case a cardiac anomaly is found without trisomy 21, VACTERL-screening should be performed.

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.

Necrotizing enterocolitis (NEC) is a leading cause of death in very preterm born infants. The most severe variant is NEC totalis (NEC-T), where necrosis of the small intestines is so extensive that curative care is often withdrawn. Mortality and NEC-T are difficult to predict before surgery, complicating counseling and decision-making. This study's aim was to identify preoperative risk factors for overall 30-day mortality and NEC-T in preterm born infants with surgical NEC. This multicenter retrospective cohort study included preterm born infants (<35 weeks) surgically treated for NEC between 2008 and 2022. NEC-T was defined as necrosis of the majority of small intestine, leading to a surgical open-close procedure without curative treatment. Preoperative risk factors for 30-day postoperative mortality, NEC-T, and mortality without NEC-T were assessed using multivariable logistic regression analyses. Among the 401 patients included, the 30-day mortality rate was 34.2% (137/401), of which 18.7% (75/401) involved NEC-T. Significant risk factors for mortality were male sex (odds ratio [OR]: 2.53; 95% confidence interval [CI]: 1.54–4.16), lower birthweight (OR: 0.91; 95% CI: 0.86–0.96/100 g), portal venous gas (PVG) on abdominal radiograph (OR: 1.89; 95% CI: 1.11–3.20), need for cardiovascular support between NEC diagnosis and surgery (OR: 3.26; 95% CI: 2.02–5.24), and shorter time between diagnosis and surgery (OR: 0.74; 95% CI: 0.65–0.84). Similar risk factors were found for NEC-T. In patients without NEC-T, the need for cardiovascular support (OR: 2.33; 95% CI: 1.33–4.09) and time between diagnosis and surgery (OR: 0.77; 95% CI: 0.64–0.91) were significant. Male sex, lower birthweight, PVG, cardiovascular support, and a short interval between NEC diagnosis and surgery are preoperative risk factors for 30-day mortality and NEC-T. Preoperative cardiovascular support and a shorter time interval between diagnosis and surgery are also risk factors for mortality without NEC-T. II ^* These authors contributed equally. Received: 05 September 2024 Accepted: 09 February 2025 Article published online: 21 March 2025 © 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/) Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

Journal of Clinical Research in Pediatric Endocrinology