Management of Pediatric Intestinal Failure

Abstract

Patients and families suffering from intestinal failure are exposed daily to physical, emotional, and fiscal burdens that result in an immeasurable amount of distress [1]. Intestinal failure occurs in these patients because of an inability of their bowel to meet fluid and/or nutritional needs required to sustain normal physiology and growth without parenteral nutritional support. In children, short-bowel syndrome (SBS) is the major cause of intestinal failure and results from both congenital disorders and extensive surgical resection. The common causes of SBS include intestinal atresia, abdominal wall defects (primarily gastroschisis), intestinal volvulus, long-segment Hirschsprung disease, complicated meconium ileus, and necrotizing enterocolitis (30% of cases and the most common cause). Of all pediatric SBS, 80% occurs during the neonatal period. Data on the incidence and mortality related to SBS are sparse. A recent cohort of very low and extremely low birth weight neonates at 16 tertiary centers in the United States demonstrated incidences of SBS at 0.7% and 1.1%, respectively, although this excluded cases in term infants [2]. Given the increase in the overall number and survival of these at-risk patients, it is logical to assume that the overall number of pediatric SBS will continue to increase as well. Mortality in this population of patients occurs in a bimodal fashion, with the first peak corresponding to infants who undergo massive initial bowel resections and a later peak corresponding to complications of SBS, namely central venous catheter sepsis and intestinal failure–associated liver disease (IFALD). Survival rates for children with SBS have been quoted as 73% to 89%, with lower rates in patients requiring chronic total parenteral nutrition (TPN) [3–6]. Recently, the development of formal multidisciplinary programs has greatly improved the care provided to these patients by reducing both the morbidity and mortality associated with intestinal failure [7]. In general, these programs