K. Dukleska1, A. E. Martin1,2, J. Miller3,4, K. M. Sullivan3,4, C. Levy3,4, S. Prestowitz2, K. Flathers2, C. D. Vinocur1,2, L. Berman1,2 1Thomas Jefferson University,Surgery,Philadelphia, PA, USA 2Nemours/Alfred I. duPont Hospital for Children,Surgery,Wilmington, DE, USA 3Nemours/Alfred I. duPont Hospital for Children,Pediatrics,Wilmington, DE, USA 4Thomas Jefferson University,Pediatrics,Philadelphia, PA, USA
Introduction:
Necrotizing enterocolitis totalis (NEC-T) was once thought to be uniformly fatal, and is generally accepted to have poor outcomes. This perception may lead to limitation of the options that are offered for infants with this condition. Therefore, we sought to better understand how NEC-T is defined in the literature and quantify reported mortality in these patients.
Methods:
A systematic literature review was conducted in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A web-based search was performed to identify studies reporting outcomes on necrotizing enterocolitis. Identified abstracts were screened independently in triplicate and a consensus was required for inclusion. Results were pooled, definitions used for NEC-T were captured, and outcomes were described.
Results:
A total of 766 abstracts were screened, of which 166 met inclusion criteria for review of the full article. From the reviewed articles, 35 included data on a total of 501 patients with NEC-T. There was no consensus regarding the nomenclature or definition of NEC-T. The most common terms used were total involvement of the small bowel (29.4%) and pan-intestinal involvement, which denoted involvement of small and large bowel (20.6%). Length of viable bowel or whether or not the ileocecal valve remained was not standardly captured, even in more contemporary reports. Operative interventions performed for NEC-T included exploratory surgery, placement of peritoneal drains, and, very infrequently, bowel resections. The aggregate reported mortality rate was 345/400 patients (86.3%), with limited details on long-term outcomes.
Conclusion:
NEC-T mortality is high, and the aggressiveness of surgical intervention appears to be low. As advances are made in maintaining long-term durable intravenous access and preventing parenteral nutrition-related liver failure, it is likely that survival prospects for these patients will improve. This study highlights the importance of establishing a standardized definition for NEC-T and prospectively tracking outcomes in these patients. A better understanding of the range of possible outcomes will facilitate more informed decision-making for patients with NEC-T.
