Eur J Pediatr Surg 2008; 18(6): 368-371
DOI: 10.1055/s-2008-1038928
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Is Intestinal Transplantation the Future of Children with Definitive Intestinal Insufficiency?

F. Sauvat1 , F. Fusaro1 , F. Lacaille1 , L. Dupic1 , N. Bourdaud1 , V. Colomb1 , J. P. Hugot2 , Y. Aigrain1 , 2 , O. Goulet1 , Y. Revillon1
  • 1UFR Necker-Enfants Malades, University René Descartes Paris V, FAMA de Transplantation Intestinale, AP‐HP and the , National Reference Centre for Rare Digestive Diseases, Paris, France
  • 2Hôpital Robert Debré, University Paris VII, FAMA de Transplantation Intestinale, AP‐HP and the National Reference Centre for Rare Digestive Diseases, Paris, France
Weitere Informationen

Publikationsverlauf

received June 23, 2008

accepted after revision July 7, 2008

Publikationsdatum:
20. November 2008 (online)

Abstract

Intestinal transplantation (IT) is the newest and most difficult of organ transplantations. The first ever (1987) and the longest surviving (1989) IT were performed in our institution. However, IT still has to demonstrate its benefit to children on long-term parenteral nutrition (PN). We tried to clarify this aspect by looking back at our 13 years' experience. Patients: From 1994 to December 2007, 74 IT were performed in 69 children, 39 with an isolated small bowel (IT), 35 combined with a liver transplant (LITx). The indications were: short bowel syndrome (n = 25), congenital mucosal diseases (n = 22), and motility disorders (n = 22). Median age at transplantation was 5 years (1 – 17 years). Follow-up was 1 to 12 years (median 5 years). Results: Thirty-one children have a functioning graft (42 %), 15/39 IT, 16/35 LITx. They are at home without PN, with a good quality of life. One child is PN-dependent 1.5 years post IT. Post IT, 16 children were detransplanted: 12 early on (1 for mechanical complications, 11 because of resistant rejection; 3 less than 3 years, one 9 years post SBT (chronic rejection). In 2 noncompliant teenagers, PN was reintroduced (one was detransplanted later on). Several years post LITx, 2 children underwent bowel detransplantation due to an acute viral infection complicated with rejection. Twenty-two children died (32 %, 8 IT, 14 LITx), 18 early on from infectious or surgical complications, 4 more than 1 year post IT, 3 after retransplantation (1 in another unit). Bad prognostic factors are multiple previous surgeries, an older age (> 7 y), and chronic intestinal pseudo-obstruction. Discussion: Complications post IT are frequent and life-threatening, especially early on: rejection (IT), infections (LITx). Later on, the rate of complications decreases but remains significant, especially in noncompliant patients. However we describe here a 13-year learning curve; the recent results are encouraging with regard to control of rejection and viral infections. Conclusion: Intestinal transplantation is indicated only in selected patients in whom long-term PN cannot be performed safely any more. In every child with intestinal insufficiency, the therapeutic strategy must be discussed early on in order to perform IT at the right time under optimal conditions. IT should evolve from being a “rescue” procedure to becoming a true therapeutic option.

References

  • 1 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;  44 347-353
  • 2 Encinas J L, Luis A, Avila L F, Hernandez F, Sarria J, Gamez M. et al . Nutritional status after intestinal transplantation in children.  Eur J Pediatr Surg. 2006;  16 403-406
  • 3 Fouquet V, De Lagausie P, Faure C, Bloch J, Malbezin S, Ferkhadji L. et al . Do prognostic factors exist for total colonic aganglionosis with ileal involvement?.  J Pediatr Surg. 2002;  37 71-75
  • 4 Goulet O, Ruemmele F. Causes and management of intestinal failure in children.  Gastroenterology. 2006;  130 (2 Suppl 1) S16-S28
  • 5 Goulet O, Salomon J, Ruemmele F, de Serres N P, Brousse N. Intestinal epithelial dysplasia (tufting enteropathy).  Orphanet J Rare Dis. 2007;  2 20
  • 6 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;  241 607-613
  • 7 Ishii T, Mazariegos G V, Bueno J, Ohwada S, Reyes J. Exfoliative rejection after intestinal transplantation in children.  Pediatr Transplant. 2003;  7 185-191
  • 8 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;  57 455-461
  • 9 Ruemmele F M, Sauvat F, Colomb V, Jugie M, Jan D, Canioni D. et al . Seventeen years after successful small bowel transplantation: long term graft acceptance without immune tolerance.  Gut. 2006;  55 903-904
  • 10 Sauvat F, Dupic L, Caldari D, Lesage F, Cezard J P, Lacaille F. et al . Factors influencing outcome after intestinal transplantation in children.  Transplant Proc. 2006;  38 1689-1691
  • 11 Sudan D L, Iyer K R, Deroover A, Chinnakotla S, Fox Jr I J, Shaw Jr B W. et al . A new technique for combined liver/small intestinal transplantation.  Transplantation. 2001;  72 1846-1848
  • 12 Sudan D. Cost and quality of life after intestinal transplantation.  Gastroenterology. 2006;  130 (2 Suppl 1) S158-S162
  • 13 Ueno T, Kato T, Revas K, Gaynor J, Velasco M, Selvaggi G. et al . Growth after intestinal transplant in children.  Transplant Proc. 2006;  38 1702-1704

Dr. F. Sauvat

Pediatric Surgery
Hôpital Necker-Enfants Malades

149 rue de Sèvres

75015 Paris

France

eMail: frederique.sauvat@nck.aphp.fr

    >