Management of Pediatric Intestinal Failure
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 consist of gastroenterologists, pediatric surgeons, advanced care providers dedicated to intestinal rehabilitation, intestinal failure nutritionists, pharmacists with advanced training in parenteral nutrition (PN), and social workers. As intestinal rehabilitation centers care for children within their institutions as well as accepting numerous referrals both nationally and internationally, administrative support that provides these families and referring physicians easy access to the program is invaluable. This includes access to people who can help provide timely information on how to receive a consult and recommendations from the team, coordinate referrals and transport to the center, and provide continuity of care when they return home. After discharge, ongoing communication with the referring physicians and provision of detailed care plans can avoid many of the complications that contribute to the morbidity associated with intestinal failure.
Feeding strategies
In general, all at-risk patients for intestinal failure should be treated using protocols designed to minimize complications and enhance adaptation. Most of these patients will require initial support by PN. At our institution, we have established a lipid reduction protocol that limits the amount of fat to less than 1 g/kg/d in all at-risk patients and we have noted a dramatic decrease in the incidence of elevated direct bilirubin and liver enzymes. In select cases with ongoing elevation of liver enzymes and direct bilirubin, intravenous omega-3 fatty acids (Omegaven; Fresenius Kabi AG, Bad Homburg, Germany) appear to be beneficial [13]. Ongoing prospective studies are evaluating these 2 treatment options and should provide useful data in the near future to help establish data-driven practical guidelines regarding the use of these strategies.
Early initial feeds should be provided continuously using breast milk (mother’s or donor’s) for all infants when available. There are several studies that support the use of breast milk in this patient population for reducing complications associated with SBS [12]. This benefit is likely because of its high levels of immunoglobulin A, nucleotides, long-chain fats, growth factors, and free amino acids, including glutamine present in breast milk. In cases where protein allergies occur, when breast milk is not available, or in older patients, we initially provide an elemental amino acid–based formula.
Predictors of outcome in intestinal failure
Several retrospective studies have tried to identify certain characteristics that may be useful in predicting outcomes in children with SBS and/or intestinal failure. Given the wide array of diagnoses that can lead to intestinal failure, it is difficult to isolate predictors of outcome, but there are some general trends (Table 1). The length of remaining bowel and its location are the most logical factors to use in predicting a patient’s course with intestinal failure. Neonates have an amazing capacity for adaptation, and this potential is increased in preterm infants [9]. Previous work has shown that patients with less than 10% of their predicted small-bowel length were less likely to wean from PN and they have a higher relative risk of death [5]. This fact makes accurate assessment of small-bowel length at the time of surgery critical for helping to plan a comprehensive treatment regimen and for anticipating complications. The remnant bowel should be measured and documented in the operative notes with each surgical procedure. Using a silk suture along the antimesenteric border of the bowel is a reasonable approach to obtain an accurate length [8].
Table 1 Factors associated with prognosis in intestinal failure
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