Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Neither Carinatum nor Excavatum: Insights Regarding the Novel Postprematurity Thoracic Dysplasia and Traditional Harrison Grooves

Background/purpose: In recent years, a new chest wall malformation (CWM), the postprematurity thoracic dysplasia (PPTD), was reported. It was characterized as an inward deviation of the anterior tips of the 6th ribs and a personal history of prematurity and respiratory distress. At the moment, PPTD's prevalence and etiology are yet to be defined. Thus, we aimed to explore and identify patients with a compatible morphology within a large cohort of patients with CWM. Methods: Retrospective analysis of electronic health records including medical photographs of 4640 consecutive patients with chest wall malformations at our Pectus Clinic between 2002 and 2023. Patients presenting with bilateral inward curvature of the 6th to 8th ribs were included. Among the variables analyzed, the history of preterm birth and ventilatory support requirement were evaluated. Results: From 2069 patients with non-excavated deformities, 29 (1.4%) patients presented a PPTD morphology. After excluding 12 for incomplete records, we found that 9 patients (52.9%) had a positive history of prematurity (group PT) and 8 (47.1%) didn't (group T). However, both groups showed a high incidence of respiratory comorbidities (PT: 89% vs. T: 76%, p = 0.91). The median age of deformity onset was 12 months (IQR 2; 12) and 24 months (IQR 1; 60) for the PT and T groups, respectively (p = 0.37). Both groups showed a low incidence of family history of CWM (PT: 38% and T: 14%, p = 0.57). Conclusions: In this retrospective study including 4640 patients with chest wall malformations, the presence of a phenotype described as postprematurity thoracic dysplasia was similarly distributed among patients with or without a history of prematurity. We hypothesize that this condition, different from pectus excavatum and carinatum, might correspond to the Harrison grooves, a deformity whose etiology was profusely discussed in the medical literature from the first half of the 20th century. Type of study: Comparative retrospective study. Level of evidence: III