Characteristics and outcomes of a multi-institution cohort of infants with necrotizing enterocolitis totalis

OBJECTIVE: To characterize the presentation, management and outcomes of infants with necrotizing enterocolitis totalis (tNEC) vs surgical non-totalis NEC (sNEC). STUDY DESIGN: This retrospective study identified infants undergoing surgery for NEC through The Children’s Hospitals Neonatal Database. Demographic, surgical and mortality characteristics were compared. RESULTS: Of 1059 infants, 161 (15.2%) had tNEC. Perinatal characteristics did not differ. tNEC infants were older and were less likely to have pneumoperitoneum at referral (5.6% vs 13.1%, p < 0.001) or intestinal perforation at surgery (38.5% vs 66.7%, p < 0.001). Infants with tNEC were more acidotic preoperatively (7.1, [IQR 7, 7.3] vs 7.3, [IQR 7.2, 7.4], p < 0.001). Mortality was 96.9% for tNEC and 26.5% for sNEC (p < 0.001). tNEC cases varied by center, accounting for 0–43% of all surgical NEC cases. CONCLUSIONS: Mortality is high for tNEC infants, who present at older age, with greater illness severity but are less likely to have intestinal perforation than sNEC infants.