Abstract
Short bowel syndrome (SBS) is the most common cause of intestinal failure in the pediatric population. Functionally, SBS is defined as the spectrum of malabsorption features occurring after extensive surgical resection. Congenital SBS is a rare entity, with an estimated prevalence of 0.02-0.1% among all live births. The mainstay of treatment consists of parenteral nutrition and intestinal rehabilitation, comprising surgical reconstruction. The two main procedures are Longitudinal Intestinal Lengthening and Tailoring procedure (LILT) and Serial Transverse Enteroplasty Procedure (STEP). Transplantation is reserved as treatment of last resort, when intestinal rehabilitation has been unsuccessful. Short bowel syndrome is a debilitating disease, whose management stretches over the course of years. However, with the innovation of surgical techniques such as LILT and STEP, as well as advances in multidisciplinary intestinal rehabilitation centers, long-term survival rates are approaching 90%. In this review we discuss the current and future treatment options for SBS.
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