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

Ultrasonographic and Radiographic Diagnosis of Ectopic Ureter in a Dog

Background: Ureteral ectopia (or ectopic ureter) is a congenital anomaly of the urinary system in which the ureter inserts anywhere other than the vesical trigone. This anatomical change may have unilateral or bilateral involvement. The most evident clinical sign, occurring mostly in females, is urinary incontinence, however in some cases the condition may progress to nephritis and dilation of the renal pelvis. The diagnosis is established through imaging, and definitive treatment requires surgical approach. The present study reports a case of ureteral ectopia in a dog which was diagnosed by ultrasound and contrast radiography (excretory urography) and successfully treated by neoureterostomy.Case: A 10-month-old female American Pit Bull Terrier was attended at the Veterinary Hospital of the Federal Rural University of the Semi-Arid (UFERSA), in Mossoró, RN. Her owner reported incontinence of dark, malodorous urine since birth as the chief complaint. After clinical examination, cystitis was suspected, and a complete blood count, urinalysis, and abdominal ultrasound was requested. The blood count and creatinine were within the reference values. The presence of struvite crystals were found on urinalysis. Ultrasound examination revealed a tortuous, dilated right ureter from the renal pelvis to the urinary bladder; no uroliths were identified as a cause of potential obstruction, but the ipsilateral kidney showed increased cortical echogenicity, loss of corticomedullary definition, and moderate pelvic dilation. These findings supported a presumptive diagnosis of ectopic ureter. For the purpose of confirming this suspicion, excretory urography was performed, revealing unilateral ureteral dilation and radiopaque contrast uptake following the path of the urethra. Once the diagnosis was confirmed, surgery was performed to correct the ureteral ectopia using the standard neoureterocistostomy technique. Considering the unilateral involvement, location of the insertion, and preserved renal function, the decision was made to perform a neoureterostomy. During the surgery it was possible to identify that the ectopic ureter was found to be intramural. At 2-month follow-up, urinary incontinence had resolved and control ultrasound showed significant improvement in the inflammatory appearance of the right renal parenchyma, with no signs of dilation of the renal pelvis or ureter.Discussion: Different from what happens in male dogs, females with an ectopic ureter will often present with urinary incontinence as the main (and, often, only) symptom, usually in the first months of life. As pollakiuria suggests a wide range of diseases of the urinary tract, ultrasound was considered the first-line imaging modality of choice, indispensable for ruling out other differential diagnoses such as a severe urinary tract infection, urolithiasis, or even malignancy. Despite the literature reporting that urinary incontinence persists in 44 to 67% of cases of ureteral ectopia, even after surgery in this case there was complete recovery of the patient after two months. Accessible techniques like ultrasonography and contrast radiography (excretory urography) supplemented one another in the elucidation of this case, with both demonstrating an excellent contribution to the diagnosis of ectopic ureter as well as served as support for surgical planning, enabling effective repair and consequent recovery of the patient.