Intestinal Failure

Intestinal failure (IF) is defined as insufficient gut function requiring parenteral nutrition (PN) for ≥60 days [e296-c1, e4741-c2]. Short bowel syndrome—most commonly from necrotizing enterocolitis, gastroschisis, volvulus, or atresia—accounts for the majority of pediatric IF [e296-c10, e4741-c4, e4741-c5]. Survival in modern intestinal rehabilitation programs now exceeds 90%, with 60–80% achieving enteral autonomy [e5141-c18, e5141-c19, e10183-c8]. Residual bowel length remains predictive, but the 50% autonomy threshold has shifted from 30–40 cm (2001) to 10–20 cm (2014) . Preserved ileum and colon confer the greatest adaptive advantage via GLP-2 secretion, bile reclamation, and short-chain fatty acid production [e4993-c7, e4993-c8, e5141-c10, e5141-c27]. Necrotizing enterocolitis patients paradoxically fare best, likely because enteral feeding was established before disease onset [e929-c25, e5141-c30, e10183-c12]. Multidisciplinary teams, lipid-sparing strategies (SMOF at 2–2.5 g/kg/day), aggressive enteral feeding, and ethanol/EDTA line locks have driven dramatic reductions in IF-associated liver disease and catheter sepsis [e1036-c1, e1036-c8, e6734-c5, e6734-c11, e6734-c12, e13892-c12, e13892-c13]. Teduglutide (GLP-2 analog, 0.05 mg/kg/day) produces ≥20% PN reduction in 69% of children and complete autonomy in 10% [e6180-c8, e6180-c9, e13892-c42]. Surgical lengthening (STEP, Bianchi) is reserved for patients plateaued on maximal medical therapy with dilated bowel (≥4–5 cm); early procedures in the first year often fail because motility has not matured [e927-c23, e927-c26, e5909-c19, e10183-c47]. Intestinal transplant—now indicated primarily for end-stage liver disease or unreconstructable anatomy—carries 5-year survival of ~65%, inferior to rehabilitation (>90%) [e927-c43, e10271-c12, e10271-c13, e13892-c46].