Necrotizing enterocolitis in full-term neonates: is it aganglionosis?

Area of Science:

• Neonatal Surgery
• Pediatric Gastroenterology
• Clinical Neonatology

Background:

• Necrotizing enterocolitis (NEC) is predominantly a disease of premature infants, but approximately 10% of cases occur in full-term neonates.
• The co-occurrence of Hirschsprung's disease and NEC in full-term infants is rarely reported, prompting this investigation.

Purpose of the Study:

• To investigate the association between Hirschsprung's disease and NEC in full-term neonates.
• To highlight a previously underreported connection in neonatal surgical cases.

Main Methods:

• Retrospective analysis of surgically treated neonates with NEC from 1999 to 2007.
• Categorization into preterm (Group 1) and full-term (Group 2) neonates based on weight (>2400 g for Group 2).
• Rectal biopsies for Hirschsprung's disease diagnosis, followed by Duhamel's pull-through surgery for confirmed cases.

Main Results:

• Aganglionosis, indicative of Hirschsprung's disease, was identified in 4 of 7 rectal biopsies from full-term infants (Group 2).
• Of the 20 neonates with NEC, 3 died (17.6%), with survivors generally developing normally, though one experienced growth retardation.
• One infant in the full-term group presented with cholestatic jaundice.

Conclusions:

• A substantial proportion of term neonates with NEC may have coexisting long-segment or total colonic Hirschsprung's disease.
• This finding contrasts with existing literature and suggests a need for increased awareness and diagnostic consideration.
• The study underscores the importance of considering Hirschsprung's disease in full-term infants diagnosed with NEC.