Ultra-short bowel syndrome during infancy: improving outcomes and novel therapies.

Abstract

Ultra-short bowel syndrome is relatively rare and has not yet been extensively reported. In ultra-short bowel syndrome, poor absorption of nutrients and dysmotility, interfere with fluid, energy, electrolyte and micronutrient balance. Patients with this disorder are managed through prolonged parenteral nutrition with the ultimate goal of achieving enteral autonomy. Overall outcomes of these patients are dependent on postsurgical bowel anatomy (residual length, intact colon, ostomy closure timing), incidence of sepsis, and care by a multidisciplinary specialized team. Over the years, standardization of management has improved outcomes. This includes central line care, lipid alternatives, enteral therapy, medications (antidiarrheal agents, acid suppression medications, bile acid binding salts, and enteral antibiotics) including Teduglutide. Bowel lengthening procedures have also proven beneficial, and finally bowel transplant does remain an option for a patient in whom rehabilitation has failed. Although there are many factors that influence outcomes of ultra-short bowel patients, novel therapies such as Teduglutide have been introduced with the aim of improving intestinal adaptation. Surgical lengthening and transplant are viable options in the setting of failed rehabilitation.