Dukleska N, et al. - Researchers identified studies reporting outcomes in children with necrotizing enterocolitis totalis using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to capture the definition of necrotizing enterocolitis totalis, along with length of follow-up, patient demographics, and outcomes. On screening a total of 766 articles, they selected 166 for full article review; data on 414 patients with necrotizing enterocolitis totalis was included in 32 of these articles. Thirty-two patients (7.7%) underwent aggressive surgical therapy (defined as bowel resection or fecal diversion), with a mortality rate of 68.8%. In contrast, 382 patients (92.3%) underwent nonaggressive surgical therapy, with a mortality rate of 95%. Rare pursuing of aggressive surgical therapy seems to be the driver of overall high mortality rate. No accepted definition of necrotizing enterocolitis totalis was identified. Standardizing the definition of necrotizing enterocolitis totalis and capturing short and long-term outcomes prospectively are considered important.