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  • Title: Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children.
    Author: Sauvat F, Grimaldi C, Lacaille F, Ruemmele F, Dupic L, Bourdaud N, Fusaro F, Colomb V, Jan D, Cezard JP, Aigrain Y, Revillon Y, Goulet O.
    Journal: J Pediatr Surg; 2008 Oct; 43(10):1833-8. PubMed ID: 18926216.
    Abstract:
    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.
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