Short Bowel Syndrome in Children

Abstract

Short bowel syndrome (SBS) reflects a state of malabsorption that occurs due to loss of a significant portion of the small bowel. The pathophysiology of SBS is determined largely by the process of adaptation, which is the innate attempt by the remnant portions of the intestine to increase fluid and nutrient reabsorption. In recent years, emphasis has been placed on intestinal rehabilitation with multidisciplinary teams as a comprehensive approach to the management of patients with SBS. In our institution, the multidisciplinary team members include pediatric gastroenterologists, pediatric surgeons, pediatric dieticians, physical therapists, occupational therapists, neonatologists (especially for patients still under their care), transplant surgeons, transplant coordinators and social workers. Parenteral nutrition plays a significant role in the management of SBS, but its use is associated with many potential complications, including cholestatic liver disease. Fish oil-based lipid emulsions have shown promise in their ability to reverse and also prevent the development of cholestasis in these patients. Clinical trials have shown that growth factors and other trophic hormones facilitate the process of adaptation. The most significant impact has been shown with the use of glucagon-like peptide-2 and its analog (teduglutide). Surgical interventions remain an important part of the management of SBS to facilitate adaptation and treat complications. Intestinal transplantation is a last resort option when the process of adaptation is unsuccessful. This review article is intended to provide an overview of the conventional and emerging therapies for pediatric SBS.