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Intestinal Failure: Etiologies and Outcomes and Decision-Making Between Rehabilitation and Transplantation

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Book cover Solid Organ Transplantation in Infants and Children

Part of the book series: Organ and Tissue Transplantation ((OTT))

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Abstract

Intestinal failure (IF) is a condition in which severe intestinal malabsorption requires parenteral nutrition (PN). Causes of protracted intestinal failure include short bowel syndrome (SBS), congenital diseases of enterocyte development (CDED), and severe motility disorders [total or subtotal aganglionosis (TIA) or chronic intestinal pseudo-obstruction syndrome (CIPOS)]. IF can result in “nutritional failure,” defined as the incapacity to continue to feed a child by using PN. Today, intestinal failure-associated liver disease (IFALD) is the most common cause of nutritional failure, but catheter-related sepsis and extensive vascular thrombosis may also jeopardize the use of long-term PN. For a child with nutritional failure, intestinal transplantation (ITx), often in the form of a composite visceral graft, offers the only chance option for long-term survival. The management of IF requires a multidisciplinary approach. There have been a number of recent advances in both medical and surgical treatments of IF. In particular, new intestinal lengthening techniques and the use of parenteral nutrition formula rich in fish oil have both resulted in decreased rates of severe complications of IF and its treatment. In addition, improved awareness of the risks and benefits of ITx have resulted in better patient selection and ultimately in improved patient survival, leading to restrict the indication to ITx only to patients with nutritional failure with no other chance to survive.

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Authors and Affiliations

  1. Division of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Paris Cité Sorbonne Paris Descartes Medical School, Paris, France

    Olivier Goulet, Florence Lacaille & Cécile Lambe

Authors
  1. Olivier Goulet
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  2. Florence Lacaille
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  3. Cécile Lambe
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Corresponding author

Correspondence to Olivier Goulet .

Editor information

Editors and Affiliations

  1. Jefferson Medical College, AI Dupont Hospital for Children Department of Surgery, Wilmington, Delaware, USA

    Stephen P. Dunn

  2. Liver & Intestine Transplantation, Seattle Children’s Hospital Liver & Intestine Transplantation, Seattle, Washington, USA

    Simon Horslen

Section Editor information

  1. Hillman Center for Pediatric Transplantation, Children’s Hospital of Pittsburgh of UPMC, 4401 Penn Avenue (FP, Room 6137), 15224, Pittsburgh, PA, USA

    George Mazariegos

  2. Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Dale Zecca

  3. Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Jennifer Melvin

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Goulet, O., Lacaille, F., Lambe, C. (2017). Intestinal Failure: Etiologies and Outcomes and Decision-Making Between Rehabilitation and Transplantation. In: Dunn, S., Horslen, S. (eds) Solid Organ Transplantation in Infants and Children. Organ and Tissue Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-08049-9_19-1

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  • DOI: https://doi.org/10.1007/978-3-319-08049-9_19-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-08049-9

  • Online ISBN: 978-3-319-08049-9

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