Abstract
Intestinal failure (IF) is characterized by the inability of the digestive tract to absorb nutrients in order to cover metabolic needs. The main etiologies in children are short bowel (mainly after intestinal atresia, gastroschisis, necrotizing enterocolitis, and midgut volvulus), intestinal motility disorders (Long segment Hirschsprung’s disease and intestinal pseudo-obstruction), and congenital enterocyte disorders causing intractable diarrhea (tufting enteropathy and microvillus inclusion disease). The first-line treatment of intestinal failure is parenteral nutrition (PN), which provides excellent long-term results (90% patient survival, with close to a normal quality of life on home PN). In short bowel syndromes, intestinal rehabilitation programs, including intestinal lengthening techniques, may help to partially or totally reverse the dependency on PN.
Intestinal transplantation is indicated in cases of irreversible intestinal failure and severe complications of PN: loss of venous access due to large vessel thrombosis, life-threatening line infections, liver disease, and poor quality of life in some patients, due to chronic intestinal obstruction and/or water and electrolyte losses. Other rare indications include retransplantations and (exceptionally) tumors. Intestinal transplantation is classified into four surgical subtypes, according to the organs needed together with the small bowel +/− right colon: isolated intestinal transplantation, liver and intestinal transplantation, modified multivisceral transplantation (all digestive organs without the liver), and multivisceral transplantation (all digestive organs with the liver). A renal transplantation may be added if needed.
Adequate preparation for the procedure is very important, since the short-term results correlate with the general condition of the child at surgery. The intestine is a highly immunogenic organ, requiring a high level of immunosuppression. After intestinal transplantation, the child is exposed to rejection and early to late graft loss and to complications of immunosuppression, including infections, tumors, and drug toxicity (mainly renal function impairment). Current research aims at improving graft survival and reducing the complications of immunosuppression, the transplant Graal being induced tolerance.
References
Abu-Elmagd KM, Armanyous SR, Fujiki M, et al. Management of five hundred patients with gut failure at a single center: surgical innovation versus transplantation with a novel predictive model. Ann Surg. 2019;270(4):656–74.
Beath S, Pironi L, Gabe S, Horslen S, Sudan D, Mazeriegos G, et al. Collaborative strategies to reduce mortality and morbidity in patients with chronic intestinal failure including those who are referred for small bowel transplantation. Transplantation. 2008;85(10):1378–84.
Benedetti E, Holterman M, Asolati M, Di Domenico S, Oberholzer J, Sankary H, et al. Living related segmental bowel transplantation: from experimental to standardized procedure. Ann Surg. 2006;244(5):694–9.
Celik N, Stanley K, Rudolph J, et al. Improvements in intestine transplantation. Semin Pediatr Surg. 2018;27(4):267–72.
Colomb V, Fabeiro M, Dabbas M, Goulet O, Merckx J, Ricour C. Central venous catheter-related infections in children on long-term home parenteral nutrition: incidence and risk factors. Clin Nutr. 2000a;19(5):355–9.
Colomb V, Jobert-Giraud A, Lacaille F, Goulet O, Fournet JC, Ricour C. Role of lipid emulsions in cholestasis associated with long-term parenteral nutrition in children. JPEN J Parenter Enteral Nutr. 2000b;24(6):345–50.
Colomb V, Dabbas-Tyan M, Taupin P, Talbotec C, Revillon Y, Jan D, et al. Long-term outcome of children receiving home parenteral nutrition: a 20-year single-center experience in 302 patients. J Pediatr Gastroenterol Nutr. 2007;44(3):347–53.
de Ville de Goyet J, Mitchell A, Mayer AD, Beath SV, McKiernan PJ, Kelly DA, et al. En block combined reduced-liver and small bowel transplants: from large donors to small children. Transplantation. 2000;69(4):555–9.
Delriviere L, Muiesan P, Marshall M, Davenport M, Dhawan A, Kane P, et al. Size reduction of small bowels from adult cadaveric donors to alleviate the scarcity of pediatric size-matched organs: an anatomical and feasibility study. Transplantation. 2000;69(7):1392–6.
Duclaux-Loras R, Berthiller J, Ferroni A, et al. Clostridium difficile: a frequent infection in children after intestinal transplantation. Transplantation. 2019; https://doi.org/10.1097/TP.0000000000002795.
Fishbein TM. Intestinal transplantation. N Engl J Med. 2009;361(10):998–1008.
Frongia G, Kessler M, Weih S, Nickkholgh A, Mehrabi A, Holland-Cunz S. Comparison of LILT and STEP procedures in children with short bowel syndrome – a systematic review of the literature. J Pediatr Surg. 2013;48(8):1794–805.
Goulet O, Ruemmele F, Lacaille F, Colomb V. Irreversible intestinal failure. J Pediatr Gastroenterol Nutr. 2004;38(3):250–69.
Goulet O, Antebi H, Wolf C, Talbotec C, Alcindor LG, Corriol O, et al. A new intravenous fat emulsion containing soybean oil, medium-chain triglycerides, olive oil, and fish oil: a single-center, double-blind randomized study on efficacy and safety in pediatric patients receiving home parenteral nutrition. JPEN J Parenter Enteral Nutr. 2010;34(5):485–95.
Goulet O, Abi Nader E, Pigneur B, et al. Short bowel syndrome as the leading cause of intestinal failure in early life: some insights into the management. Pediatr Gastroenterol Hepatol Nutr. 2019;22(4):303–29.
Grant D, Abu-Elmagd K, Reyes J, Tzakis A, Langnas A, Fishbein T, et al. 2003 report of the intestine transplant registry: a new era has dawned. Ann Surg. 2005;241(4):607–13.
Gura KM, Duggan CP, Collier SB, Jennings RW, Folkman J, Bistrian BR, et al. Reversal of parenteral nutrition-associated liver disease in two infants with short bowel syndrome using parenteral fish oil: implications for future management. Pediatrics. 2006;118(1):e197–201.
Intestinal-Transplant-Association. 2013 Intestinal transplant registry update. 2013.; Available from: http://www.intestinaltransplant.org/itr/.
Jones BA, Hull MA, Potanos KM, Zurakowski D, Fitzgibbons SC, Ching YA, et al. Report of 111 consecutive patients enrolled in the international Serial Transverse Enteroplasty (STEP) data registry: a retrospective observational study. J Am Coll Surg. 2013;216(3):438–46.
Jugie M, Canioni D, Le Bihan C, Sarnacki S, Revillon Y, Jan D, et al. Study of the impact of liver transplantation on the outcome of intestinal grafts in children. Transplantation. 2006;81(7):992–7.
Lacaille F, Vass N, Sauvat F, Canioni D, Colomb V, Talbotec C, et al. Long-term outcome, growth and digestive function in children 2 to 18 years after intestinal transplantation. Gut. 2008;57(4):455–61.
Lopushinsky SR, Fowler RA, Kulkarni GS, Fecteau AH, Grant DR, Wales PW. The optimal timing of intestinal transplantation for children with intestinal failure: a Markov analysis. Ann Surg. 2007;246(6):1092–9.
Mazariegos GV, Steffick DE, Horslen S, Farmer D, Fryer J, Grant D, et al. Intestine transplantation in the United States, 1999–2008. Am J Transplant. 2010;10(4 Pt 2):1020–34.
Modi BP, Javid PJ, Jaksic T, Piper H, Langer M, Duggan C, et al. First report of the international serial transverse enteroplasty data registry: indications, efficacy, and complications. J Am Coll Surg. 2007;204(3):365–71.
Norsa L, Artru S, Lambe C, et al. Long term outcomes of intestinal rehabilitation in children with neonatal very short bowel syndrome: parenteral nutrition or intestinal transplantation. Clin Nutr. 2019;38(2):926–33.
Pironi L, Hebuterne X, Van Gossum A, Messing B, Lyszkowska M, Colomb V, et al. Candidates for intestinal transplantation: a multicenter survey in Europe. Am J Gastroenterol. 2006;101(7):1633–43; quiz 79.
Pironi L, Joly F, Forbes A, Colomb V, Lyszkowska M, Baxter J, et al. Long-term follow-up of patients on home parenteral nutrition in Europe: implications for intestinal transplantation. Gut. 2011;60(1):17–25.
Pironi L, Goulet O, Buchman A, Messing B, Gabe S, Candusso M, et al. Outcome on home parenteral nutrition for benign intestinal failure: a review of the literature and benchmarking with the European prospective survey of ESPEN. Clin Nutr. 2012a;31(6):831–45.
Pironi L, Baxter JP, Lauro A, Guidetti M, Agostini F, Zanfi C, et al. Assessment of quality of life on home parenteral nutrition and after intestinal transplantation using treatment-specific questionnaires. Am J Transplant. 2012b;12(Suppl 4):S60–6.
Raghu VK, Beaumont JL, Everly MJ, et al. Pediatric intestinal transplantation: analysis of the intestinal transplant registry. Pediatr Transplant. 2019;23:e13580.
Sarnacki S, Revillon Y, Cerf-Bensussan N, Calise D, Goulet O, Brousse N. Long-term small-bowel graft survival induced by a spontaneously tolerated liver allograft in inbred rat strains. Transplantation. 1992;54(2):383–5.
Sauvat F, Grimaldi C, Lacaille F, Ruemmele F, Dupic L, Bourdaud N, et al. Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children. J Pediatr Surg. 2008;43(10):1833–8.
Stanger JD, Oliveira C, Blackmore C, Avitzur Y, Wales PW. The impact of multi-disciplinary intestinal rehabilitation programs on the outcome of pediatric patients with intestinal failure: a systematic review and meta-analysis. J Pediatr Surg. 2013;48(5):983–92.
Sudan D, Thompson J, Botha J, Grant W, Antonson D, Raynor S, et al. Comparison of intestinal lengthening procedures for patients with short bowel syndrome. Ann Surg. 2007;246(4):593–601; discussion –4.
Tzakis AG, Kato T, Levi DM, Defaria W, Selvaggi G, Weppler D, et al. 100 multivisceral transplants at a single center. Ann Surg. 2005;242(4):480–90; discussion 91–3.
Acknowledgments
As part of their publication requirements, the authors acknowledge: The pioneers Claude Ricour (pediatrician) and Yann Révillon (surgeon) who launched the pediatric intestinal rehabilitation and transplantation program at Necker -Enfants Malades Hospital in the 1980s. The colleagues of this team, including hepatogastroenterology and nutrition, surgery, anesthesiology, intensive care, radiology, pathology, immunology, psycho-social, and support services.
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Chardot, C. (2020). Intestinal Transplantation. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_118-1
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DOI: https://doi.org/10.1007/978-3-642-38482-0_118-1
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