Intestinal failure

Abstract

Purpose of review Intestinal failure (IF) has became a specifically defined condition that may be irreversible in some patients. IF requires the use of parenteral nutrition (PN) as long as it persists. As a consequence of both underlying digestive disease and unadapted PN, liver disease may develop with subsequent end-stage liver cirrhosis. Death will occur if combined liver–intestine transplantation is not performed. Catheter-related sepsis and/or extensive vascular thrombosis may impede the continuation of a safe and efficient PN and require intestinal transplantation (ITx). Thus, management of IF patients requires an early recognition of IF and the analysis of its risk of irreversibility. Recent findings Recent literature covered the field of IF management, including therapeutic approaches aiming to avoid extensive intestinal resection, to restore or to develop intestinal function with nutritional or pharmacological treatment and nontransplant surgery in short-bowel patients. Children with extreme short gut, congenital diseases of enterocyte development, or severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome) can be diagnosed early as having irreversible IF and are candidates for ITx. Efficient and uncomplicated PN and home PN remain the mainstay of therapy, independent of the nature of IF. When PN limits are reached, ITx is the logical approach because its outcome improved during the last decade. Summary Timing of referral for ITx remains a crucial issue. As a consequence, IF management should include therapies adapted to each stage of IF based on a multidisciplinary approach in centers involving pediatric gastroenterology, PN expertise, home-PN program, pediatric surgery, and liver-intestinal transplantation program. Promotion of basic and applied research in the field should prevent the liver and vascular consequences of IF and long-term PN, respectively.