Management of short bowel syndrome.

Most intestinal failure in children is due to short bowel syndrome (SBS) where congenital or acquired lesions have led to an extensive loss of intestinal mass. The vast majority of morbidity and mortality of patients with SBS is due to complications secondary to their long term dependence on parenteral nutrition. In response to SBS, the intestine undergoes a process of remodeling termed adaptation. Principles guiding the medical management of SBS include providing adequate parenteral nutrition, fluids and electrolytes for growth and normal development, promoting small bowel adaptation, and preventing and treating complications related to the patient's underlying disease and their parenteral nutrition. Catheter associated blood stream infection (CABSI) is major source of morbidity and mortality in patients with intestinal failure from SBS. Intestinal failure associated liver disease (IFALD)is another major source of morbidity and mortality in patients with SBS. IFALD is the most consistent negative predictor of outcome including death and continued parenteral nutrition dependence. Enteral nutrition is critical for intestinal adaptation and preventing IFALD. Patients with SBS who develop dilated dysmotile segments may benefit from autologous intestinal reconstruction surgery (AIRS) with the goal of decreasing stasis and disordered motility through intestinal narrowing and lengthening. Patients with SBS should be referred for transplantation if they have failed intestinal rehabilitation including AIRS, have no reasonable chance for enteral feeding tolerance, develop irreversible IFALD, have recurrent sepsis, or have exhausted their central venous access sites. With improvements in medical and surgical care, overall survival of patients with SBS now exceeds 90%.