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

Tracheal injury during extraction of an esophageal foreign body: Repair utilizing venovenous ECMO

Extracorporeal membrane oxygenation (ECMO) is a form of life support with an ever-expanding range of indications. Veno-venous (VV) ECMO is often utilized to support children with respiratory compromise, and has been employed successfully in the acute setting of traumatic tracheobronchial injury as well as during elective tracheal surgery. We present a successful case of VV ECMO used in the perioperative management of a tracheal repair for a traumatic laceration caused by attempts to retrieve an esophageal foreign body. While this mechanism of injury appears to be rare, we believe that VV-ECMO allowed for the optimal management of this child and should be considered for other extensive tracheal injuries in children

Congenital diaphragmatic hernia and complete tracheal rings: The value of peri-operative ECMO

A full-term male prenatally diagnosed with left congenital diaphragmatic hernia (CDH) was intubated at birth and scheduled for elective repair. The pre-operative chest radiograph incidentally demonstrated tracheal narrowing and an intraoperative flexible bronchoscopy was performed, suggestive of impending airway occlusion secondary to complete tracheal rings. Given these findings, he was electively cannulated post-operatively to venoarterial extracorporeal membrane oxygenation (ECMO) for stabilization and tracheal repair. Post-operative day one, he underwent further characterization by computed tomography and was taken for slide tracheoplasty on ECMO support. Immediately following tracheal repair, he was decannulated from ECMO and was extubated to nasal CPAP two days after the slide tracheoplasty. This case highlights the rare association of tracheal anomalies associated with CDH and options for perioperative management, including the role of ECMO. Keywords: Congenital diaphragmatic hernia, CDH, Extracorporeal membrane oxygenation, ECMO, Complete tracheal rings, Tracheal stenosis, Tracheoplast

Continuous double volume exchange transfusion is a safe treatment for ECMO-induced hemolysis

Extracorporeal membrane oxygenation (ECMO)-related hemolysis is a recognized complication and remains a source of significant morbidity and mortality in the neonatal population. This is the first description of continuous double-volume exchange transfusion (ET) performed for profound hemolysis that developed in a neonate with congenital heart disease on VA ECMO for sepsis. The aim of this report is to demonstrate the safety and efficacy of performing ET on a physiologically frail patient on ECMO support. Keywords: Veno-arterial extracorporeal membrane oxygenation, Hemolysis, Exchange transfusio