**Background:** Short-bowel syndrome (SBS) is a complex disease resulting from physical loss or loss of function of a portion of the small and/or large intestine, leading to reduced absorption of nutrients, fluids, and electrolytes. In pediatric patients, SBS is the leading cause of intestinal failure (IF) and underlies 50% of cases requiring home parenteral nutrition (HPN). The severity of malabsorption depends on the length and quality of the remaining bowel. Intestinal adaptation—morphologic and functional changes in the remnant gut—gradually improves absorptive capacity. The therapeutic approach requires multidisciplinary evaluation encompassing nutritional, medical, and surgical aspects. Parenteral nutrition (PN) has decreased mortality but is associated with complications including liver disease, catheter malfunction, and bloodstream infections. Intestinal rehabilitation programs (IRPs) focus on complication mitigation and promoting enteral autonomy.
**Methods:** This is a narrative review of literature published from 2010 to December 2022. The authors searched PubMed, Cochrane Library, Embase, Web of Science, Google Scholar, and Scopus using keywords including "short bowel syndrome," "intestinal failure," "pediatric," "parenteral nutrition," "intestinal adaptation," "liver disease," "sepsis," "small intestinal bacterial overgrowth (SIBO)," "catheter-related complications," "D-lactic acidosis," "IFALD," and "intestinal rehabilitation program." Over 1500 manuscripts were identified; only those published in English with original data or systematic reviews were included. Additional papers were identified through reference lists.
**Key Results:** Epidemiological data are fragmentary. In very low birth weight infants from 16 US tertiary neonatal centers, the incidence of surgical SBS was 0.7% (7 per 1000) among 12,316 infants, and 1.1% (11 per 1000) among 5,657 extremely low birth weight infants; 96% were caused by necrotizing enterocolitis (NEC). A Canadian population-based study reported an overall SBS rate of 24.5 per 100,000 live births (95% CI = 12.1–36.9), with NEC (35%) and complicated meconium ileus (20%) as main causes. NEC itself causes approximately one-third of all pediatric SBS cases. Intestinal adaptation involves epithelial hyperplasia (villous lengthening, crypt deepening, microvilli proliferation), bowel dilatation and elongation, and smooth muscle hypertrophy. The ileum demonstrates greater adaptive capacity than the jejunum. Prognostic factors include remnant bowel length (full-term neonates have ~150–250 cm of small bowel), site of resection, preservation of the ileocecal valve (ICV), and colonic continuity. Catheter-related bloodstream infections (CRBSIs) occur at rates of 1.3–10.2 per 1000 catheter days, with higher risk in children under 1 year. Ethanol lock therapy reduces CRBSI risk by 81% in children with IF. IFALD occurs in 40–60% of patients on long-term PN; each septic episode is associated with a 3.2-fold increased risk of developing jaundice. Metabolic bone disease prevalence can reach 80%. Renal function is impaired in up to 54% of SBS patients after intestinal transplantation. D-lactic acidosis is a rare but dramatic complication in patients with an intact colon. SIBO is defined as >10^5 CFU/mm³ of bacterial species on duodenal aspirate culture. Teduglutide, a GLP-2 analogue, at doses of 0.025 or 0.05 mg/kg, was associated with reduced PN requirements in children over 12 months of age.
**Clinical Implications:** SBS management requires early, coordinated multidisciplinary care through intestinal rehabilitation programs. Key strategies include: early initiation of enteral nutrition to promote adaptation; use of human milk or extensively hydrolyzed formula; low-fat parenteral nutrition (<1 g/kg/day) with omega-3-enriched lipid emulsions to minimize IFALD; aggressive prevention and treatment of CRBSIs using ethanol locks and strict catheter care; diagnosis and management of SIBO with individualized antibiotic therapy; monitoring for micronutrient deficiencies (vitamin D, zinc, iron, vitamin B12); and surveillance for metabolic bone disease, renal dysfunction, and neurodevelopmental delays. Teduglutide represents a promising pharmacological option to reduce PN dependence. Intestinal transplantation should be considered when IF is irreversible and severe PN-related complications develop. Multicenter registries and research consortia are needed to personalize management and improve quality of life.