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. MethodsWe 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. FindingsWe 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•4...
Gastrointestinal stromal tumors (GIST) in children are rare and their behavior has been regarded as difficult to predict on pathological criteria. We report our experience with two gastric GISTs in children aged 10 and 11 years. Both remain alive and free of disease at 5 years and 2 years respectively. Comparison of the pathological features in the resected specimens with a recently proposed guidelines for predicting outcome in this group of tumors is reported.
Background Neonatal gastric perforation (NGP) is a rare, serious, and life-threatening condition which needs early recognition with prompt intervention for better prognosis. Its etiology is not yet well established, but multiple speculations have been put forth for its etiopathogenesis. Few most considered are traumatic, spontaneous, or secondary to inflammatory process like necrotizing enterocolitis. This study describes the etiological and prognostic factors for patients with NGP in our experience at a single center. Results A total of 46 neonates were included. Male gender predominated with M:F being 1.7:1. Most (n=36) neonates presented within 10 days of life whereas 8 neonates presented within 15 days. At presentation, gas under diaphragm was the most common radiologic finding in 25 (54.3%) neonates. On exploratory laparotomy, it was found that greater curvature was involved in maximum number of cases (n=27), followed by lesser curvature and anterior and posterior walls of the stomach. Most of perforations were within 1–5 cm in size. Gastrorrhaphy was done in all cases, and in two cases, feeding jejunostomy was done along with repair for feeding purpose. Finally, spontaneous NGP was diagnosed in 30 (60.8%), and NEC patches on other parts of the intestine were seen in 11 patients. Postoperatively, 28 neonates developed complications in the form of sepsis (n=13), wound infection (n=10), and burst abdomen (n=5). Regarding clinical outcome, 27 (58.7%) were discharged from the hospital whereas 19 (39.3%) patients died. Conclusion Our results show that spontaneous NGP is most commonly associated with NEC in our population, usually affecting the greater curvature. We observed a high mortality rate; however, good ICU care may improve the survival.
Background Perforation of Meckel Diverticulum (MD) is a rare cause of pneumoperitoneum in neonates. We hereby report six cases of perforation of MD in neonates, with addition of 53 cases from systematic review of the literature. A systematic review was performed using Mesh terms “Neonate, Meckel Diverticulum, Perforation, Pneumoperitoneum.” All reports of perforated MD in the English literature were identified. Details of our 6 cases were analyzed in similar fashion. Results A total of 3027 manuscripts were screened and 59 cases including 6 of our own were identified. The vast majority (78%) were female. Fifty patients (84.7%) presented in the newborn period. Half of the cases (52.5%) had associated anomalies and 13 neonates (22%) required oxygen supplementation including CPAP or ventilatory support before surgery. In 73% of the cases, a resection of gut was undertaken. Histopathological assessment in 44 cases (74.6%) revealed no ectopic gastric mucosa. Three cases demised prior to treatment. The outcome in the vast majority was excellent with 84.7% surviving and discharged well. Conclusion Perforated MD is an unusual cause of a pneumoperitoneum in the newborns. Diagnosis is established at laparotomy and it rare to find ectopic mucosa histopathologically. The overall outcome is excellent.
Survival for children with TOF/OA is not dependent on factors including birth weight, gestational age, pre/postoperative ventilation and a major cardiac anomaly taken independently. In the modified prognostic classification for OA/TOF, a low birth weight combined with cardiac malformations is associated with a poor prognosis. This alternate prognosticator offers benefits for appropriately advising parents of babies with such anomalies taking the current standards of care into consideration. It should also serve as a foundation stone for further prospective studies.
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