Neonatal intestinal failure and transplantation

Abstract

Neonatal intestinal failure is a devastating condition which carries a significant morbidity and mortality. The most important causes of intestinal failure include short bowel syndrome, motility disorders and rare mucosal enteropathies. The management of these complex patients is challenging and requires a multidisciplinary approach for optimal outcomes. Multidisciplinary coordinated efforts are aimed to optimize nutritional, surgical and medical therapy. The main goals of the treatment include assuring adequate growth and development, optimal utilization of the adaptation potential of the remaining bowel in order to achieve intestinal autonomy and effective prevention and treatment of complications. Survival and successful adaptation depends on an early institution of intestinal rehabilitation which consist of liver-sparing parenteral nutrition, careful central venous feeding line management to avoid and control infections and to maintain venous access sites, individualized enteral nutrition, optimal medical management and carefully planned surgical procedures as well as social integration. Recent refinements in surgical, nutritional and medical treatment have been associated with significant reductions in morbidity and improvements in survival. Autologous gastrointestinal reconstruction is a valuable option in selected patients from the very beginning in the planned management of these patients. The resultant reduction in parenteral nutrition requirement limits the extent of the side effects improving both patients' prognosis and quality of life. The great majority of neonates with short bowel syndrome can be weaned from parenteral nutrition while liver-intestinal transplantation serves as a salvage therapy for those who develop life-threatening complications such as liver failure, central vein thrombosis or recurrent catheter-associated bloodstream infections.