Current status of pediatric intestinal transplantation in the United States

Abstract

Purpose of review The present review aims to describe in detail the characteristics, outcomes, and recent trends in the field of pediatric intestinal transplantation in the United States. It will examine the route cause and future implications of these developments. The review will draw from recent publications in the field, the Intestinal Transplant Registry, and contemporary data from large U.S. single centers. Recent findings More than 1500 pediatric intestinal transplants have been performed in the United States since 1985, however, over the past decade there have been fewer than 50 transplants/year nationwide. This trend is largely a result of stagnant long-term ITx outcomes and advancements in intestinal rehabilitation programs. Nationally the overall 1-year and 5-year graft survival are 68 and 50% respectively, whereas certain high-volume centers have experienced significantly better results. Sepsis is the leading cause of death following pediatric ITx, whereas rejection is the leading cause of graft loss. Chronic kidney disease and posttransplant lymphoproliferative disorder are significant and relatively prevalent long-term complications. The majority of pediatric ITx recipients receive T-cell depleting induction agents and are on Tacrolimus-based immunosuppression. Most recipient are off parenteral nutrition, but may require supplemental tube feeds. Many pediatric ITx recipients require special education, and in certain domains some report lower health related quality of life. Summary As intestinal rehabilitation has improved in the modern era, the volume of pediatric ITx in the United States has decreased. Although pediatric ITx results have room for improvement nationwide, successful outcomes have been reported at experienced American centers.

Paediatric intestinal failure and transplantation

“…49,50 The frequency and severity of comorbidities remain an important factor influencing the decision to list individual patients for transplantation. [48][49][50] Conclusion IF is a complex and devasting condition. Children with IF have improved outcomes when managed at a paediatric centre with specific expertise.…”

“…Intestinal transplantation has been restricted to children with irreversible IF suffering from life-threatening complications of PN. [48][49][50] Transplant options include an isolated small intestine, combined small intestine and liver, and variations involving the stomach, colon, pancreas, kidney or a multi-visceral transplant.…”

“…48 The leading cause of death post-transplantation is sepsis although other long-term complications, such as chronic renal disease and lymphoproliferative disease, are not uncommon in transplant recipients. 48 Most children with a successful intestinal transplant are able to cease PN although supplemental enteral tube feeding is often still required. 48 Intestinal transplant listing criteria were recently revised to consider additional factors including home PN outcomes, quality of life and early referral to a transplant programme and cost.…”

“…48 Most children with a successful intestinal transplant are able to cease PN although supplemental enteral tube feeding is often still required. 48 Intestinal transplant listing criteria were recently revised to consider additional factors including home PN outcomes, quality of life and early referral to a transplant programme and cost. 48,49 New disease indications include invasive intra-abdominal desmoids in adolescents and adults, acute diffuse intestinal infarction with hepatic failure and failure of first intestinal transplant.…”

“…48 Intestinal transplant listing criteria were recently revised to consider additional factors including home PN outcomes, quality of life and early referral to a transplant programme and cost. 48,49 New disease indications include invasive intra-abdominal desmoids in adolescents and adults, acute diffuse intestinal infarction with hepatic failure and failure of first intestinal transplant. Refinements specific to children are persistent hyperbilirubinemia >75 micromol/L despite use of Omegaven lipid emulsion, presence of non-cholestatic IFALD with portal hypertension, thrombosis of three of four upper body central veins or occlusion of one brachiocephalic vein in children and life-threatening sepsis or other complications of IF necessitating two or more admissions to an intensive care unit.…”