Burden and mortality of congenital gastrointestinal anomalies: insights from a nationwide cohort study
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In brief
In brief
This nationwide Malaysian cohort study examined 228 pediatric patients with congenital gastrointestinal anomalies and abdominal wall defects, revealing an 8.8% mortality rate with congenital diaphragmatic hernia as the strongest predictor of poor outcomes. The research highlights critical factors influencing survival in resource-limited settings.
- CDH accounts for 60% of mortality in congenital GI anomalies, making it the highest-risk diagnosis requiring early recognition and aggressive care.
- Overall mortality for congenital GI anomalies and abdominal wall defects in Malaysia is 8.8%, with CDH being the strongest predictor of death.
- Higher ASA score, need for central venous access, blood transfusion, and ventilation are independent risk factors for mortality in these patients.
- Early identification of CDH and prompt escalation of care are critical interventions to reduce sepsis on arrival and improve survival outcomes.
Written by the GCMD Library team from the article.
Abstract
Purpose
Improved perioperative care has enhanced survival in children with congenital gastrointestinal conditions and abdominal wall defects (AWD). However, epidemiological and surgical outcomes in developing nations are still scarce. Our aim was to assess the burden and mortality of common congenital gastrointestinal anomalies and AWD in Malaysia, and their influencing factors.
Methods
Using the Global PaedSurg study protocol with permission, we performed a prospective cohort study on children presenting for the first time between October 2021 and April 2022 with these conditions: Esophageal atresia (EA), congenital diaphragmatic hernia (CDH), intestinal atresia, gastroschisis, exomphalos, anorectal malformation (ARM) and Hirschsprung’s disease. We compared mortality and 30-day outcome data across different geographical regions in Malaysia.
Results
There were 228 patients with 242 study conditions (EA n = 28, CDH n = 36, intestinal atresia n = 49, gastroschisis n = 12, exomphalos n = 8, ARM n = 77, Hirschsprung’s disease n = 32). Our mortality rate was 8.8%; 60% of these were CDH patients. Factors significantly associated with mortality were CDH diagnosis, central venous access requirement, higher American Society of Anesthesiologists (ASA) score, blood transfusion and ventilation requirement.
Conclusion
Diagnosis of CDH is the most important predictor for sepsis on arrival and mortality, therefore measures should be taken for early recognition and aggressive management.