Intestinal and Multivisceral Transplantation Guidelines

Updated: Feb 22, 2021
  • Author: Richard K Gilroy, MBBS, FRACP; Chief Editor: Stuart M Greenstein, MD  more...
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Guidelines

Guidelines Summary

A 2001 position paper by the American Society of Transplantation on indications for pediatric intestinal transplantation recommends intestinal transplantation for the subset of children with intestinal failure remaining dependent on parenteral nutrition who develop life‐threatening complications arising from therapy. [92] Life‐threatening complications warranting consideration of intestinal transplantation include the following:

  • Parenteral nutrition–associated liver disease
  • Recurrent sepsis
  • Threatened loss of central venous access

The guidelines note that because a critical shortage of donor organs exists, waiting times for intestinal transplantation are prolonged, so it is essential that children with life‐threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early (ie, in time to receive suitable donor organs before they become critically ill). Recommendations are as follows:

  • Children with liver dysfunction should be considered for isolated intestinal transplantation before irreversible, advanced bridging fibrosis, or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary.
  • Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present.

European Society for Clinical Nutrition and Metabolism (ESPEN) guidelines on chronic intestinal failure in adults, published in 2016, include the following recommendations and suggestions on intestinal transplantation [93] :

ESPEN recommends home parenteral nutrition (HPN) as the primary treatment for patients with chronic intestinal failure CIF and the early referral of patients to intestinal rehabilitation centers with expertise in both medical and surgical treatment for CIF, to maximize the opportunity of weaning off HPN, to prevent HPN failure, and to ensure timely assessment of candidacy for intestinal transplantation.

ESPEN recommends assessment for candidacy for intestinal transplantation when there is failure of HPN, high risk of death attributable to the underlying disease, or intestinal failure with high morbidity or low acceptance of HPN.

Failure of HPN may be indicated by any of the following:

  • Impending liver failure (total bilirubin above 3-6 mg/dL [54-108 mmol/L], progressive thrombocytopenia, and progressive splenomegaly) or overt liver failure (portal hypertension, hepatosplenomegaly, hepatic fibrosis, or cirrhosis) because of intestinal failure-associated liver disease (IFALD).
  • Central venous catheter (CVC)–related thrombosis of two or more central veins (internal jugular, subclavian, or femoral).
  • Frequent central line sepsis: two or more episodes per year of systemic sepsis secondary to line infections requiring hospitalization; a single episode of line-related fungemia; septic shock and/or acute respiratory distress syndrome.
  • Frequent episodes of severe dehydration despite intravenous fluid in addition to HPN.

High risk of death attributable to the underlying disease may be indicated by any of the following:

  • Invasive intra-abdominal desmoid tumors
  • Congenital mucosal disorders (ie, microvillus inclusion disease, tufting enteropathy)
  • Ultra-short bowel syndrome (gastrostomy, duodenostomy, residual small bowel < 10 cm in infants and< 20 cm in adults)

Intestinal failure with high morbidity or low acceptance of HPN may be indicated by any of the following:

  • Need for frequent hospitalization
  • Narcotic dependency
  • Inability to function (ie, pseudo-obstruction, high-output stoma)
  • Patient's unwillingness to accept long-term HPN (ie, young patients)

Other guideline statements are as follows:

  • ESPEN recommends that patients with impending or overt liver failure due to IFALD and those with an invasive intra-abdominal desmoid tumor be listed for a life-saving intestinal transplantation (with or without liver transplantation).
  • ESPEN suggests that patients with CVC-related thrombosis of two or more central veins (internal jugular, subclavian or femoral) be listed for a life-saving intestinal transplantation on a case-by-case basis.
  • ESPN does not recommend listing for a life-saving intestinal transplantation of patients with CIF having any of the indications for assessment of candidacy other than IFALD-related liver failure, intra-abdominal desmoids, or CVC-related multiple vein thrombosis.
  • ESPEN suggests that patients with CIF with high morbidity or low acceptance of HPN might be listed for a rehabilitative intestinal transplantation on a careful case-by-case basis.
  • ESPEN recommends that, whenever possible, patients listed for intestinal transplantation undergo the procedure while they are in stable clinical condition, as represented by being able to stay at home and not requiring hospitalization while waiting for transplant. For patients listed for a combined intestinal and liver transplantation, mechanisms to prioritize patients on the waiting list for liver transplantation should be adopted in order to minimize the risk of mortality while on waiting list  and after transplantation.