Abstract
AimThe aim of this study was to analyze outcomes of severe short bowel syndrome (SBS) treated with autologous intestinal reconstruction (AIR) surgery to facilitate independence of parenteral nutrition (PN). MethodsPN dependence, growth, nutritional status, liver function, and survival were comparatively assessed in SBS children treated with (n=10) or without (n=18) AIR surgery. ResultsMedian follow-up was 9.2 (4.9–14) years. Residual small intestinal length was 28cm at birth. A total of 13 AIR procedures were performed at median age of 16 (11–41) months: serial transverse enteroplasty (STEP; n=8), longitudinal lengthening and tailoring (LILT; n=2), and tapering enteroplasty (n=3). Following STEP and LILT, length of the remaining small intestine increased by 19 (15–38) cm or 43% (38%–64%). Two children required repeat STEP due to recurrence of symptoms and bowel re-dilatation. Median duration of PN was 12 (11–17) months before and 14 (0–19) months after AIR. Eight children remain off PN 6.9 (3.6–9.7) years after surgery, and one with ultra short bowel (12cm) receives 12% of energy as PN — all with reassuring growth and nutritional status and normal liver function. Actuarial PN dependence, including those who died on PN, was similar (P=0.19) with or without AIR surgery being 30% and 20% at four years, respectively. Complications of AIR surgery (lymphocele, bowel obstruction, and staple line bleeding) resolved without operative interventions. Survival was 90% with and 83% without AIR procedures. ConclusionsAIR surgery can provide long-term survival, independence of PN, and satisfactory general health in selected children with severe SBS.
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