Abstract
Background: Short bowel syndrome (SBS) secondary to intestinal resection is the most common cause of intestinal failure (IF) in children and is associated with prolonged parenteral support (PS), impaired growth, and substantial morbidity. SBS outcomes have improved dramatically over the past ten years due to advancements in intestinal rehabilitation programs, nutritional approaches, pharmaceutical treatments, and surgical restoration. Objective: With an emphasis on surgical decision-making, intestinal rehabilitation, nutritional optimization, pharmacologic advancements and predictors of enteral autonomy, the goal is to thoroughly review and synthesize the most recent data on managing pediatric SBS after intestinal resection. Methods: This systematic review was conducted in accordance with the PRISMA 2020 statement using PubMed and Embase. Eligible primary clinical studies evaluated management strategies or clinically relevant outcomes in pediatric patients with SBS secondary to intestinal resection. Secondary evidence sources, including review articles and evidence-based position papers, were considered separately and were used to contextualize the primary findings and contemporary clinical recommendations. The methodological quality and risk of bias of primary studies were assessed using design-appropriate critical appraisal tools. Owing to substantial clinical and methodological heterogeneity, the evidence was synthesized qualitatively. Results: Multidisciplinary intestinal rehabilitation programs have become the mainstay of therapy, increasing the rates of enteral autonomy and improving survival. While optimal PS administration reduces intestinal failure–associated liver disease (IFALD) and catheter-related bloodstream infections (CRBSIs), early, organized enteral feeding encourages intestinal adaptation. Significant advances in decreasing PS requirements have been achieved by pharmacological therapies such as glucagon-like peptide-2 (GLP-2) analogs. Advances in multidisciplinary intestinal rehabilitation have reduced the need for intestinal transplantation and have refined patient selection for autologous bowel reconstruction. Consequently, procedures such as Serial Transverse Enteroplasty (STEP) and Longitudinal Intestinal Lengthening and Tailoring (LILT) are now reserved for highly selected patients with persistent bowel dilatation, dysmotility, and inadequate intestinal adaptation despite optimized nutritional and medical therapy. Conclusions: Following intestinal resection, pediatric SBS is now managed through multidisciplinary, protocol-driven intestinal rehabilitation. Outcomes have improved through the integration of nutritional, pharmacological, and surgical strategies tailored to each patient’s residual anatomy and adaptive potential. Future prospective pediatric studies are needed to standardize outcome measures and clarify the optimal timing and sequencing of nutritional, pharmacological, and surgical interventions during intestinal rehabilitation.