TY - JOUR
T1 - Intestinal transplantation for total intestinal aganglionosis
T2 - a series of 12 consecutive children
AU - Sauvat, Frederique
AU - Grimaldi, Chiara
AU - Lacaille, Florence
AU - Ruemmele, Franck
AU - Dupic, Laurent
AU - Bourdaud, Nathalie
AU - Fusaro, Fabio
AU - Colomb, Virginie
AU - Jan, Dominique
AU - Cezard, Jean Pierre
AU - Aigrain, Yves
AU - Revillon, Yann
AU - Goulet, Olivier
PY - 2008/10
Y1 - 2008/10
N2 - Background: Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. Methods: Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon. Results: After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. Conclusion: These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.
AB - Background: Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. Methods: Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon. Results: After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. Conclusion: These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.
KW - Fecal continence
KW - Hirschsprung's disease
KW - Intestinal transplantation
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U2 - 10.1016/j.jpedsurg.2008.03.028
DO - 10.1016/j.jpedsurg.2008.03.028
M3 - Article
C2 - 18926216
AN - SCOPUS:51349084503
SN - 0022-3468
VL - 43
SP - 1833
EP - 1838
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 10
ER -