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Multivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases
Insights
Multivisceral transplantation (MVTx) offers a life-saving option for children with chronic intestinal pseudo-obstruction (CIPO). This procedure allows patients to discontinue parenteral nutrition and tolerate enteral feedings, significantly improving survival rates.
Area of Science:
- Pediatric Surgery
- Transplantation Immunology
- Gastroenterology
Background:
- Chronic intestinal pseudo-obstruction (CIPO) in children presents life-threatening risks, including complications from parenteral nutrition (PN) and catheter-related sepsis.
- Multivisceral transplantation (MVTx) is a viable, though less common, life-saving intervention for pediatric CIPO.
- Limited data exists on the outcomes of MVTx in this specific pediatric population.
Purpose of the Study:
- To report the experience and outcomes of multivisceral transplantation (MVTx) in pediatric patients diagnosed with chronic intestinal pseudo-obstruction (CIPO).
- To evaluate the efficacy and long-term functional results of MVTx in improving the quality of life for CIPO patients.
Main Methods:
- Retrospective analysis of sixteen pediatric CIPO patients who underwent MVTx.
- Data collection included indications for transplantation, immunosuppression protocols (Period I vs. II), rejection rates, and patient survival.
- Functional outcomes assessed included tolerance of enteral feeding, need for pancreatic enzyme or insulin therapy, gastric emptying, and bladder function.
Main Results:
- Actuarial survival rates improved significantly from Period I (57.1%/42.9% at 1/2 years) to Period II (88.9%/77.8% at 1/2 years).
- All long-term survivors achieved enteral feeding tolerance, eliminating the need for PN, pancreatic enzymes, or insulin.
- Rejection was predominantly mild to moderate (87.5%), with no isolated gastric or pancreatic rejection diagnosed.
Conclusions:
- Multivisceral transplantation (MVTx) is a crucial life-saving intervention for pediatric patients suffering from chronic intestinal pseudo-obstruction (CIPO).
- Survivors experience excellent graft function, particularly of the transplanted pancreas and stomach, enabling full enteral feeding.
- While overall outcomes are positive, specific functional deficits like gastric emptying or bladder function may persist in some cases.
Abstract:
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.
