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Indications for and Management of Pediatric Intestinal Transplant Patients

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Pediatric Solid Organ Transplantation

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Abstract

Replacement of the small intestine with or without other parts of the digestive system such as the stomach, liver, pancreas, and colon represents the ultimate treatment for intestinal failure, the state of permanent dependence on parenteral nutrition for support of life with an anatomically or functionally inadequate gastrointestinal tract [1]. Intestinal transplantation was first performed in humans as an experimental, end-of-life procedure by Lillihei and coworkers in 1968 [2]. The operation was consistently unsuccessful at that time because of the failure of then available immunosuppressive agents to prevent rejection of a solid organ with the largest population of lymphocytes of any transplanted organ [3, 4], as well as the overwhelming infectious complications observed from the augmented immunosuppression required. Interest in intestinal transplantation faded over the next two to three decades, which were without meaningful improvements in the immunosuppressive armamentarium, when parenteral nutrition became increasingly available for use in the home setting. Interest in intestinal transplantation returned during the late 1980s and early 1990s as limitations of extended parenteral nutrition therapy for intestinal failure became increasingly apparent and as immunosuppressive agents with markedly increased efficacy, specifically the calcineurin inhibitor tacrolimus, became available for clinical use [3, 5]. Despite improved immunosuppressive therapy, morbidity and mortality were extremely high during the first decade of clinical intestinal transplantation, because severe allograft rejection and secondary post-operative sepsis and multi-organ failure were frequent. The often desperately ill state of patients, the scarcity of suitable donor organs, the non-sterile nature of the intestine itself, and the limitations of available laboratory and imaging methods contributed to poor outcomes during these early years. In fact, 5-year patient survival through the 1990s was markedly inferior to contemporaneous outcomes following most other solid organ transplants [6]. Subsequent refinements in patient selection, operative techniques, immunosuppressive therapy, and post-operative rehabilitation comprehensively managed by integrated teams of transplant surgeons, physicians, nurses, dieticians, social workers, and others have improved outcomes. Data from the 2019 Intestinal Transplant Registry indicate steady improvement in long-term pediatric intestinal transplant survival in each 5-year era increment from 1995 to 2015 [7]. Despite these improvements in survival, the decision to pursue intestinal transplantation in infants and children continues to remain a complex one. Once relegated as a second-tier therapy for intestinal failure, the improvements in outcome for intestinal transplantation in infants, children, and adolescents may outpace parallel improvements in intestinal rehabilitation, thus offering an attractive treatment alternative for those suffering from intestinal failure. The current approach to pediatric intestinal transplantation is summarized in this chapter.

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Authors and Affiliations

  1. Pediatric Intestinal Rehabilitation and Transplantation, MedStar Georgetown Transplant Institute, Washington, DC, USA

    Stuart S. Kaufman

  2. Center for Intestinal Care and Transplantation, MedStar Georgetown Transplant Institute, Washington, DC, USA

    Cal S. Matsumoto

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  1. Stuart S. Kaufman
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Correspondence to Cal S. Matsumoto .

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Editors and Affiliations

  1. Recanati/Miller Transplantation Institute, Mount Sinai Hospital, NY, USA

    Ron Shapiro

  2. Division of Transplant Surgery, University of California, San Francisco, CA, USA

    Minnie M. Sarwal

  3. Department of Nephrology, Akron General Medical Center at Cleveland Clinic Foundation and Akron Children’s Hospital, OH, USA

    Rupesh Raina

  4. Kidney and Urology Institute, Medanta, The Medicity, Gurugram, Haryana, India

    Sidharth Kumar Sethi

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Kaufman, S.S., Matsumoto, C.S. (2023). Indications for and Management of Pediatric Intestinal Transplant Patients. In: Shapiro, R., Sarwal, M.M., Raina, R., Sethi, S.K. (eds) Pediatric Solid Organ Transplantation. Springer, Singapore. https://doi.org/10.1007/978-981-19-6909-6_21

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