RESUMO
BACKGROUND: Jejuno-ileal atresia is one of the main causes of intestinal obstruction in neonates. The origin is vascular accidents in the fetal intestine. It is an entity that requires early and specialist management. OBJECTIVE: to know the factors related to mortality in neonates with jejunoileal atresia. METHODS: Case-control nested in a cohort design, comparative study during ten years, between deceased and survivors analyzing factors related to mortality before surgery and during surgery and in the postoperative course. RESULTS: We analyzed 70 patients in 10 years, there were 10 deaths (14.2%). No one had a prenatal diagnosis. Factors related to mortality were: intestinal perforation with a relative risk (RR) of 4.4, peritonitis (RR: 5.6), the need of stomas (RR: 4.9), the presence of sepsis (RR: 4.6) and when the residual small bowel length was below 1 meter (RR: 7.4). CONCLUSION: The delay in diagnosis causes late intervention and increased mortality delayed diagnosis promotes late transport of the neonate and enhances mortality, factors associated with mortality related to intestinal perforation. It is necessary to spread this disease in the medical community to improve prenatal and postnatal diagnosis.