Experience in enteral and parenteral nutrition schemes for familial congenital short bowel syndrome: Two case reports.

BACKGROUND

Congenital short bowel syndrome (CSBS) is a rare disorder characterized by a congenital shortage in the length of the small intestines, resulting in compromised intestinal functionality, frequently accompanied by congenital intestinal malrotation. This study summarizes the experience of enteral and parenteral nutrition (PN) schemes for two cases of CSBS accompanied by intestinal malrotation to provide a reference for this condition.

CASE SUMMARY: Case 1 underwent surgical intervention 23 days after birth, but experienced postoperative intolerance to oral feeding. Consequently, the patient was transitioned to nasal feeding for 6 months while concurrently receiving round-the-clock PN. Despite ongoing adjustments to the caloric intake of enteral and PN throughout the treatment, cholestatic liver injury persisted. Furthermore, this child developed malnutrition by the age of 1 year. Case 2 received surgical intervention on 2 days after birth, demonstrating favorable tolerance for enteral feeding and intermittent PN. The child exhibited satisfactory growth and development without any associated complications.

CONCLUSION

CSBS is rarely encountered in clinical practice and is often accompanied by congenital intestinal malrotation. It can be improved with early diagnosis and active surgical intervention. Thus, we recommend early initiation of total PN, which is a highly effective measure in promoting early growth and development.