Intestinal failure-associated liver disease in a child with short bowel syndrome: management issues

Intestinal failure (IF) refers to a state where the absorptive surface of the small intestine is inadequate to maintain water and electrolyte balance and to meet nutritional demands for optimal growth in children. We describe a 2-month-old infant with short bowel syndrome (SBS) and intestinal failure-associated liver disease (IFALD) following surgical resection for intestinal malrotation and midgut volvulus. He required prolonged total parenteral nutrition (TPN), which resulted in cholestasis and progressive liver dysfunction. Management included ursodeoxycholic acid, amino acid-based continuous feeds, antibiotics, percutaneous transhepatic biliary drainage, and supportive therapies. The illness course was complicated by recurrent diarrhea, hypoglycemia, suspected small intestinal bacterial overgrowth, and Candida sepsis. Gradual clinical improvement was observed, with the resolution of IFALD and appropriate weight gain. On follow-up at 1.5 years, the child was tolerating full oral feeds and had normal liver function. This case highlights the management challenges of SBS with IFALD associated with TPN.