Multivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases.

Chronic intestinal pseudo-obstruction (CIPO) in children may be life-threatening due to the complications of parenteral nutrition (PN) or catheter-related sepsis. Multivisceral transplantation (MVTx) is a lifesaving option but limited experience is available. We report our experience with MVTx in pediatric CIPO patients. Sixteen children with CIPO underwent MVTx at median age of 4 years. Indications for MVTx were liver failure (n = 10), loss of venous access (n = 3), or sepsis (n = 3). Modified MVTx without the liver was performed in six patients. Induction immunosuppression included tacrolimus, steroid with adjunctive agent in period I (April 1996 to December 2000), namely, OKT3 (n = 1), mycophenolate mofetil (n = 4), or daclizumab (n = 2); and in period II (January 2001 to present), Campath 1H (n = 4) or daclizumab (n = 5). The grade of rejection was severe in 12.5% and mild to moderate in 87.5% of cases. Isolated rejection of the transplanted stomach or pancreas was not diagnosed during clinical course or on autopsy. Actuarial patient survival for 1 year/2 years for period, I and II were 57.1%/42.9% and 88.9%/77.8%. None of the long-term survivors is on PN and all tolerate enteral feedings. Pancreatic enzyme supplementation or insulin therapy is not needed in survivors. Gastric emptying was substantially affected in one case. Bladder function did not improve in those with urinary retention problems. MVTx for CIPO offers a lifesaving option with excellent function of the transplanted pancreas and stomach among survivors.