The Surgical Management of Children with Intestinal Failure

Abstract

Intestinal failure represents an inability to survive and grow on absorption from the autologous bowel. Its management is complex, expensive, and demanding of major long-term commitment to central venous access and total parenteral nutrition to ensure survival, to provide opportunity for mucosal adaptation and growth and for autologous bowel reconstruction or eventual transplantation. It is best guided by a multidisciplinary team based at a specialist centre and delivered through a hub-and-spoke model that allows for home-based care supported by a dedicated outreach team of carers who provide practical, psychological, and financial support for the family.

Gene replacement opens the possibility for future gene ‘correction’, and early antenatal diagnosis and laparoscopic skills offer renewed consideration for prenatal intervention to prevent antenatal volvulus. Much present conventional postnatal management is unsatisfactory, and new approaches based on better understanding are long overdue. Management must commence with ‘prevention and preservation’ based on accurate early diagnosis, understanding, and skilled intervention, with bowel resections limited to absolute necessity. Following extensive bowel loss, there is the option of bowel expansion to grow the residual autologous bowel for enhanced absorption and adaptation and for eventual reconstruction. Finally, there is hope from bowel or liver-bowel transplantation and safer immunosuppression. This chapter attempts to stimulate new thought and practice towards offering the intestinal failure child and family a greater hope for full enteral nutrition on autologous bowel.