Necrotizing enterocolitis in a patient with Microvillous Inclusion Disease: A case report.

BACKGROUND

Microvillous Inclusion Disease (MVID) is a rare congenital disorder causing severe diarrhea from early infancy, typically requiring long-term parenteral nutrition (PN). It results from MYO5B gene mutations, disrupting brush border protein trafficking. Necrotizing enterocolitis (NEC) is not typically linked to MVID, with only two cases reported so far.

CASE REPORT: A 5-month-old male with MVID developed worsening diarrhea after switching from breast milk to polymeric formula, leading to dehydration and metabolic acidosis. Amino acid-based formula did not improve his condition. Imaging confirmed NEC, showing pneumatosis intestinalis and gas in the portal system. Conservative treatment stabilized his condition, and stool output improved after switching to donated breast milk.

CONCLUSION

This case underscores the potential risk of NEC in MVID patients, emphasizing the need for careful management of enteral feeding. Clinicians should maintain a high index of suspicion for NEC due to these patients' susceptibility to hypoperfusion and mucosal integrity issues.