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Hirschsprung's Disease and Allied Disorders pp 427–438Cite as

Intestinal Transplantation for Total Intestinal Aganglionosis

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Abstract

Intestinal transplantation is a successful treatment for children with irreversible intestinal failure who experience life-threatening complications of parenteral nutrition. Total or near-total intestinal aganglionosis is a rare form of Hirschprung’s disease (HD) with high morbidity and mortality and near-certain dependence on parenteral nutrition. Advances in surgical techniques, immunosuppressive regimens, and posttransplant surveillance and management have led to improvements in patient and graft survival following intestinal transplantation. Intestinal transplantation may provide a life-saving treatment option for children with total or near-total intestinal aganglionosis.

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References

  1. Abu-Elmagd KM, Costa G, Bond GJ, Wu T, Murase N, Zeevi A, Simmons R, Soltys K, Sindhi R, Stein W, Demetris A, Mazariegos G. Evolution of the immunosuppressive strategies for the intestinal and multivisceral recipients with special reference to allograft immunity and achievement of partial tolerance. Transpl Int. 2009;22(1):96–109.

    Article  Google Scholar 

  2. Abu-Elmagd KM, Kosmach-Park B, Costa G, Zenati M, Martin L, Koritsky DA, Emerling M, Murase N, Bond GJ, Soltys K, Sogawa H, Lunz J, Al Samman M, Saefer N, Sidhi R, Mazariegos GV. Long-term survival, nutritional autonomy, and quality of life after intestinal and multivisceral transplantation. Ann Surg. 2012;256(3):494–508.

    Article  Google Scholar 

  3. Ashokkumar C, Soltys K, Mazariegos G, Bond G, Higgs BW, Ningappa M, Sun Q, Brown A, White J, Levy S, Fazzolare T, Remaley L, Dirling K, Harris P, Hartle T, Kachmar P, Nicely M, O’Toole L, Boehm B, Jativa N, Stanley P, Jaffe R, Ranganathan S, Zeevi A, Sindhi R. Predicting cellular rejection with a cell-based assay: pre-clinical evaluation in children. Transplantation. 2017;101(1):131–40.

    Article  CAS  Google Scholar 

  4. Avitzur Y, Grant D. Intestine transplantation in children: update 2010. Pediatr Clin North Am. 2010;57(2):415–31.

    Article  Google Scholar 

  5. Berney T, Kato T, Nishida S, Tector AJ, Mittal NK, Madariaga J, Nery JR, Cantwell GP, Ruiz P, Tzakis AG. Portal versus systemic drainage of small bowel allografts: comparative assessment of survival, function, rejection, and bacterial translocation. J Am Coll Surg. 2002;195(6):804–13.

    Article  Google Scholar 

  6. Bodian M, Carter CO, Ward BC. Hirschprung’s disease. Lancet. 1951;1(6650):302–9.

    Article  CAS  Google Scholar 

  7. Bond GJ, Reyes JD. Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction. Semin Pediatr Surg. 2004;13(4):286–92.

    Article  Google Scholar 

  8. Celik N, Stanley K, Rudolph J, Al-Issa F, Kosmach B, Ashokkumar C, Sun Q, Brown-Bakewell R, Zecca D, Soltys K, Khanna A, Bond G, Ganoza A, Mazariegos G, Sindhi R. Improvements in intestine transplantation. Semin Pediatr Surg. 2018;27(4)267–72.

    Article  Google Scholar 

  9. Cheng EY, Everly MJ, Kaneku H, Banuelos N, Wozniak LJ, Venick RS, Marcus EA, McDiarmid SV, Busuttil RW, Terasaki PI, Farmer DG. Prevalence and clinical impact of donor-specific alloantibody among intestinal transplant recipients. Transplantation. 2017;101(4):873–82.

    Article  CAS  Google Scholar 

  10. Cober MP, Killu G, Brattain A, Welch KB, Kunisaki SM, Teitelbaum DH. Intravenous fat emulsions reduction for patients with parenteral nutrition-associated liver disease. J Pediatr. 2012;160(3):421–7.

    Article  CAS  Google Scholar 

  11. DeStefano CB, Desai SH, Shenoy AG, Catlett JP. Management of post-transplant lymphoproliferative disorders. Br J Haematol. 2018;182(3):330–43.

    Article  Google Scholar 

  12. El Kasmi KC, Anderson AL, Devereaux MW, Vue PM, Zhang W, Setchell KD, Karpen SJ, Sokol RJ. Phytosterols promote liver injury and Kupffer cell activation in parenteral nutrition-associated liver disease. Sci Transl Med. 2013;5(206):206ra137.

    Article  Google Scholar 

  13. Fiel MI, Wu HS, Iyer K, Rodriguez-Laiz G, Schiano TD. Rapid reversal of parenteral-nutrition-associated cirrhosis following isolated intestinal transplantation. J Gastrointest Surg. 2009;13(9):1717–23.

    Article  Google Scholar 

  14. Fishman JA, Rubin RH. Infection in organ-transplant recipients. N Engl J Med. 1998;338(24):1741–51.

    Article  CAS  Google Scholar 

  15. Florescu DF, Islam MK, Mercer DF, Grant W, Langnas AN, Freifeld AG, Sudan D, Basappa R, Dimaio D, Kalil AC. Adenovirus infections in pediatric small bowel transplant recipients. Transplantation. 2010;90(2):198–204.

    Article  Google Scholar 

  16. Fouquet V, De Lagausie P, Faure C, Bloch J, Malbezin S, Ferkhadji L, Bauman C, Aigrain Y. Do prognostic factors exist for total colonic aganglionosis with ileal involvement? J Pediatr Surg. 2002;37(1):71–5.

    Article  CAS  Google Scholar 

  17. Gonzalez-Hernandez J, Prajapati P, Ogoa G, Channabasappa N, Piper HG. A comparison of lipid minimization strategies in children with intestinal failure. J Pediatr Surg. 2018;53:96–100.

    Article  Google Scholar 

  18. Goulet O, Ruemmele F, Lacaille F, Colomb V. Irreversible intestinal failure. J Pediatr Gastroenterol Nutr. 2004;38(3):250–69.

    Article  Google Scholar 

  19. Goulet O, Sauvat F, Ruemmele F, Caldari D, Damotte D, Cezard JP, Lacaille F, Canioni D, Hugot JP, Berebi D, Sarnacki S, Colomb V, Jan D, Aigrain Y, Revillon Y. Results of the Paris program: ten years of pediatric intestinal transplantation. Transplant Proc. 2005;37(4):1667–70.

    Article  CAS  Google Scholar 

  20. Goulet O, Antebi H, Wolf C, Talbotec C, Alcindor LG, Corriol O, Lamor M, Colomb-Jung V. A new intravenous fat emulsion containing soybean oil, medium-chain triglyceride, olive oil, and fish oil: a single-center double-blind randomized study on efficacy and safety in pediatric patients receiving home parenteral nutrition. J Parenter Enteral Nutr. 2010;34(5):485–95.

    Article  CAS  Google Scholar 

  21. Grant D, Abu-Elmagd K, Reyes J, Tzakis A, Langnas A, Fishbein T, Goulet O, Farmer D, on behalf of the Intestine Transplant Registry. 2003 Report of the Intestine Transplant Registry. Ann Surg. 2005;241(2):607–13.

    Article  Google Scholar 

  22. Grant D, Abu-Elmagd K, Mazariegos G, Vianna R, Langnas A, Mangus R, Farmer DG, Lacaille F, Iyer K, Fishbein T, Intestinal Transplant Association. Intestinal transplant registry report: global activity and trends. Am J Transplant. 2015;15(1):210–9.

    Article  CAS  Google Scholar 

  23. Green M, Michaels MG. Epstein-Barr virus infection and posttransplant lymphoproliferative disorder. Am J Transplant. 2013;13(s3):41–54.

    Article  CAS  Google Scholar 

  24. Gross TG, Orijuela MA, Perkins SL, Park JR, Lynch JC, Cairo MS, Smith LM, Hayashi RJ. Low-dose chemotherapy and rituximab for PTLD: a children’s oncology group report. Am J Transplant. 2012;12(11):3069–175.

    Article  CAS  Google Scholar 

  25. Huard G, Schiano T, Fiel MI, Ward SC, Moon J, Iyer K. Comparative incidence of rejection occurring in small intestinal and colonic mucosal biopsies of patients undergoing intestinal transplantation. Histopathology. 2016;69(4):600–6.

    Article  Google Scholar 

  26. Huard G, Schiano T, Moon J, Iyer K. Choice of allograft in patients requiring intestinal transplantation: a critical review. Can J of Gastroenterol and Hepatol. 2017;1–10.

    Article  Google Scholar 

  27. Huard G, Schiano TD, Moon J, Iyer K. Severe acute cellular rejection after intestinal transplantation is associated with poor patient and graft survival. Clin Transplant. 2017;31(5):e12956.

    Article  Google Scholar 

  28. Hukkinen M, Koivusalo A, Merras-Salmio L, Rintala RJ, Pakarinen MP. Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatr Surg. 2015;50(11):1859–64.

    Article  Google Scholar 

  29. Iyer K, Horslen S, Iverson A, Sudan D, Fox I, Shaw B, Langnas A. Nutritional outcome and growth of children after intestinal transplantation. J Pediatr Surg. 2002;37(3):464–6.

    Article  Google Scholar 

  30. Jeppesen PB, Pertkiewicz M, Messing B, Iyer K, Seidner DL, O’keefe SJ, Forbes A, Heinze H, Joelsson B. Gastroenterology. 2012;143(6):1473–81.

    Article  CAS  Google Scholar 

  31. Kato T, Tzakis AG, Selvaggi G, Gaynor JJ, David AI, Bussotti A, Moon JI, Ueno T, DeFaria W, Santiago S, Levi DM, Nishida S, Velasco ML, McLaughlin G, Hernandez E, Thompson JF, Cantwell P, Holliday N, Livingstone AS, Ruiz P. Intestinal and multivisceral transplantation in children. Ann Surg. 2006;243(6):756–64.

    Article  Google Scholar 

  32. Kato T, Selvaggi G, Gaynor JJ, Takahasi H, Nishida S, Moon J, Levi D, Smith L, Hernandez E, Ruiz P, Tzakis A. Inclusion of donor colon and ileocecal valve in intestinal transplantation. Transplantation. 2008;86(2):293–7.

    Article  Google Scholar 

  33. Kaufman SS, Atkinson JB, Bianchi A, Goulet OJ, Grant D, Langnas A, SV MD, Mittal N, Reyes J, Tzakis AG, American Society of Transplantation. Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. Pediatr Transplant. 2001;5(2):80–7.

    Article  CAS  Google Scholar 

  34. Matsumoto CS, Fishbein TM. Modified multivisceral transplantation with splenopancreatic preservation. Transplantation. 2007;83(2):234–6.

    Article  Google Scholar 

  35. Lacaille F, Irtan S, Dupic L, Talbotec C, Lesage F, Colomb V, Salvi N, Moulin F, Sauvat F, Aigrain Y, Revillon Y, Goulet O, Chardot C. Twentyeight years of intestinal transplantation in Paris: experience of the oldest European center. Transpl Int. 2017;30(2):178–86.

    Article  Google Scholar 

  36. Millar AJW, Gupte G, Sharif K. Intestinal transplantation for motility disorders. Semin Pediatr Surg. 2009;18:258–62.

    Article  Google Scholar 

  37. Nakamura H, Henderson D, Puri P. A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis. Pediatr Surg Int. 2017;33:837–41.

    Article  Google Scholar 

  38. Parizhskaya M, Redondo C, Demetris A, Jaffe R, Reyes J, Ruppert K, Marin L, Abu-Elmagd K. Chronic rejection of small bowel grafts: pediatric and adult study of risk factors and morphologic progression. Pediatr Dev Pathol. 2003;6(3):240–50.

    Article  Google Scholar 

  39. Parrish CR, DiBaise JK. Managing the adult patient with short bowel syndrome. Gastroenterol Hepatol. 2017;13(10):600–8.

    Google Scholar 

  40. Premkumar MH, Carter BA, Hawthorne KM, King K, Abrams SA. Fish oil-based emulsions in the treatment of parenteral nutrition-associated liver disease: an ongoing positive experience. Adv Nutr. 2014;5(1):65–70.

    Article  CAS  Google Scholar 

  41. Rahhal R, Abu-El-Haija MA, Fei L, Ebach D, Orkin S, Kiscaden E, Cole CR. Systemic review and meta-analysis of the utilization of ethanol locks in pediatric patients with intestinal failure. J Parenter Enteral Nutr. 2018;42(4):690–701.

    Google Scholar 

  42. Sauvat F, Dupic L, Caldari D, Lesage F, Cezard JP, Lacaille F, Ruemmele F, Hugot JP, Colomb V, Jan D, Hubert P, Revillon Y, Goulet O. Factors influencing outcome after intestinal transplantation in children. Transplant Proc. 2006;38(6):1689–91.

    Article  CAS  Google Scholar 

  43. Sauvat F, Grimaldi C, Lacaille F, Ruemmele F, Dupic L, Bourdaud N, Fusaro F, Colomb V, Jan D, Cezard J, Aigran Y, Revillon Y, Goulet O. Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children. J Pediatr Surg. 2008;43(10):1833–8.

    Article  Google Scholar 

  44. Saxton ML, Ein SH, Hoehner J, Kim PC. Near-total intestinal aganglionosis: long-term following of a morbid condition. J Pediatr Surg. 2000;35(5):669–72.

    Article  CAS  Google Scholar 

  45. Sharif K, Beath SV, Kelly DA, McKiernan P, van Mourik I, Mirza D, Mayer AD, Buckels JA, de Ville de Govet J. New perspective for the management of near-total or total intestinal aganglionosis in infants. J Pediatr Surg. 2003;38(1):25–8.

    Article  CAS  Google Scholar 

  46. Soltys KA, Bond G, Sindhi R, Rassmussen SK, Ganoza A, Khanna A, Mazariegos G. Pediatric intestinal transplantation. Semin Pediatr Surg. 2017;26(4):241–9.

    Article  Google Scholar 

  47. Squires RH, Duggan C, Teitelbaum DH, Wales PW, Balint J, Venick R, Rhee S, Sudan D, Mercer D, Martinez JA, Carter BA, Soden J, Horslen S, Rudolph JA, Kocoshis S, Superina R, Lawlor S, Haller T, Kurs-Lasky M, Belle SH, Pediatric Intestinal Failure Consortium. Natural history of pediatric intestinal failure: initial report from the Pediatric Intestinal Failure Consortium. J Pediatr. 2012;161(4):723–8.

    Article  Google Scholar 

  48. Todo S, Tzakis AG, Abu-Elmagd K, Reyes J, Fung JJ, Casavilla A, Nakamura K, Yagihashi A, Jain A, Murase N, et al. Cadaveric small bowel and small bowel-liver transplantation in humans. Transplantation. 1992;53(2):369–76.

    Article  CAS  Google Scholar 

  49. Todo S, Reyes J, Furukawa H, Abu-Elmagd K, Lee RG, Tzakis A, Rao AS, Starzl TE. Outcome analysis of 71 clinical intestinal transplantations. Ann Surg. 1995;222(3):270–82.

    Article  CAS  Google Scholar 

  50. Yann R, Yves A, Jan D, Thierry Y, Goulet O, Lacaille F. Improved quality of life by combined transplantation in Hirschprung’s disease with a very long aganglionic segment. J Pediatr Surg. 2003;38(3):422–4.

    Article  Google Scholar 

  51. Ziegler MM, Ross AJ 3rd, Bishop HC. Total intestinal aganglionosis: a new technique for prolonged survival. J Pediatr Surg. 1987;22(1):82–3.

    Article  CAS  Google Scholar 

  52. Ziegler MM, Royal RE, Brandt J, Drasnin J, Martin LW. Extended myectomy-myotomy: a therapeutic alternative for total intestinal aganglionosis. Ann Surg. 1993;218(4):504–11.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Joanne Lai

  2. Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, NY, USA

    Kishore Iyer

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  1. Joanne Lai
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  2. Kishore Iyer
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Correspondence to Joanne Lai .

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Editors and Affiliations

  1. National Children’s Research Centre, Our Lady’s Childrens Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

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Lai, J., Iyer, K. (2019). Intestinal Transplantation for Total Intestinal Aganglionosis. In: Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-15647-3_30

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  • DOI: https://doi.org/10.1007/978-3-030-15647-3_30

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