Long-term controlled outcomes after autologous intestinal reconstruction surgery in treatment of severe short bowel syndrome.

Objective: The 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).

Methods: PN dependence, growth, nutritional status, liver function, and survival were comparatively assessed in SBS children treated with (n=10) or without (n=18) AIR surgery.

Results: Median follow-up was 9.2 (4.9-14) years. Residual small intestinal length was 28 cm 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 (12 cm) 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.

Conclusions: AIR surgery can provide long-term survival, independence of PN, and satisfactory general health in selected children with severe SBS.