Medical and surgical management of the pediatric patient with intestinal failure

Until recently, extreme short bowel due primarily to massive resection in the neonatal period had been considered incompatible with long-term survival. Indeed, parents of infants with midgut volvulus or other causes of very extensive intestinal necrosis still may be informed that resection is futile. The advent of intestinal transplantation as a potential therapy and its evolution into a standard therapy for irreversible intestinal failure have led to changing attitudes regarding these catastrophic gastrointestinal events. The experience gained from aggressively maintaining infants with little if any functional small bowel while awaiting transplantation has led to the increasing recognition that long-term survival is possible in many of these children with and often without intestinal transplantation. Even children with very small lengths of residual intestine ultimately may adapt and grow sufficiently to allow enteral autonomy. Achievement of these outcomes requires early referral to a dedicated multidisciplinary intestinal care team well versed in the management options for such children. Initial assessment often involves an inpatient evaluation followed by very close outpatient follow-up. Aggressive management is imperative for all patients with intestinal failure, allowing time for full enteral adaptation before complications become life-threatening; those with no possibility of significant adaptation can achieve optimal growth while awaiting transplantation. Along with medical and nutritional therapy and nontransplant surgery, intestinal transplantation should be seen as one of many modalities available for the optimal management of this population of patients. Thus, patients with irreversible intestinal failure and those with indications for transplantation (even those for whom hope remains that sufficient enteral adaptation still may occur) should be evaluated by the transplant team. If there is no intestinal transplant program at the center undertaking the intestinal failure management, strong links and regular communication with an intestinal transplant program that can partner in the care of these patients should be established. Multicenter collaborative and interventional studies are necessary to clearly demonstrate outcomes and to move the field forward.