From the cradle to enteral autonomy: The role of autologous gastrointestinal reconstruction

The short bowel state is treatable, with acceptable long-term quality of life. Management during the first 6-12 months of life is critical but, presently, frequently compromises long-term survival and prospects. At first presentation, primary caregivers, working with specialists at designated intestinal failure centers, should develop a structured individual-oriented management plan. Preservation of venous access, "hepatosparing" parenteral nutrition, and avoidance of liver sepsis are crucial to survival. Early surgery should be limited to conservation of autologous bowel, even short bowel lengths having great potential, and to facilitating natural intestinal adaptation. Bowel expansion may be relevant prior to delayed bowel reconstruction with single or combined techniques that include bowel lengthening and/or tailoring, reversed segments, and colon interposition. Bowel transplantation, as yet not recommended for primary management, offers survival and opportunity to those with no prospect of autologous bowel autonomy or following failed autologous gastrointestinal reconstruction. This paper reviews current surgery for the short bowel state and concludes that it is presently appropriate before bowel transplantation to offer autologous gastrointestinal reconstruction, with its prospect of enteral autonomy with quality life. It emphasizes the need for an individual-oriented management plan, developed jointly at the time of first presentation between the primary caregivers and a designated multidisciplinary intestinal failure center, to enhance the prospects for enteral autonomy preferably on autologous bowel. Autologous gastrointestinal reconstruction is in its infancy and requires resources, commitment, and research from dedicated bowel reconstructive surgeons toward a better opportunity for the child and family with short bowel.