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

Métastase choroïdienne compliquée d’une occlusion d’une branche veineuse rétinienne : à propos d’un cas

Introduction    Les métastases choroïdiennes sont des tumeurs malignes oculaires qui apparaissant généralement dans un contexte défavorable de cancer multi-métastasé. Cependant, seul un cas de mélanome choroïdien compliqué d’occlusion de la veine rétinienne centrale a été rapporté.Nous rapportons le cas d’une métastase choroidienne compliquée d’une occlusion d’une branche veineuse rétinienne. Matériels et méthodesNous rapportons le cas d'un patient de 47 ans, tabagique chronique à 20 paquet année, sans aucun antécédent médico-chirurgical, qui se présente aux urgences ophtalmologiques pour baisse de l’acuité visuelle brutale de l’œil droit depuis 24 heures. ObservationL’examen ophtalmologique du patient avait retrouvé une acuité visuelle à compte les doigts au niveau de l’œil droit avec au segment antérieur une cataracte corticale. Au fond d’œil, on retrouve une masse parapapillaire supérieure noyée dans des hémorragies et des exsudats dans l’hémirétine supérieure. L’examen de l’œil gauche était normal. L'angiographie à la fluorescéine a confirmé le tableau d’occlusion de branche veineuse rétinienne. L’échographie retrouve une tumeur choroïdienne hypoéchogène de 7/10mm sans excavation choroïdienne. Au bilan radiologique, un processus médiastino-pulmonaire gauche associé à une lymphangite carcinomateuse et des adénopathies médiastinales ont été retrouvés. La biopsie du processus révèle un carcinome pulmonaire peu différencié et invasif. Le bilan d’extension montre la présence de lésions secondaires hépatiques et cérébrales. ConclusionLes métastases choroidiennes sont de mauvais pronostic dans leur contexte général. Chez notre patient, c’est la complication vasculaire qui a révélé sa pathologie néoplasique. Cette étiologie tumorale est donc à rechercher chez les patients présentant une occlusion veineuse rétinienne