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.
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 ITxpatients. 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.
Authors: Fereshteh Salimi Jazi; Tiffany J Sinclair; Chad M Thorson; Ricardo Castillo; Andrew C Bonham; Carlos O Esquivel; Matias Bruzoni Journal: Pediatr Surg Int Date: 2017-11-23 Impact factor: 1.827
Authors: E Bruder; A-L Rougemont; R I Furlano; J F Schneider; J Mayr; F-M Haecker; K Beier; J Schneider; P Weber; T Berberich; G Cathomas; W A Meier-Ruge Journal: Pathologe Date: 2013-03 Impact factor: 1.011
Authors: Fereshteh Salimi Jazi; Julia M Chandler; Chad M Thorson; Tiffany J Sinclair; Florette K Hazard; John A Kerner; Sanjeev Dutta; James C Y Dunn; Stephanie D Chao Journal: BMC Pediatr Date: 2019-04-05 Impact factor: 2.125