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Isolated liver transplantation in an infant with ultrashort gut
1Department of Pediatric Gastroenterology, Hepatology and Clinical Nutrition, University of Torino, Turin, Italy.
Insights
Isolated liver transplantation can improve intestinal function in children with short bowel syndrome and intestinal failure. This approach provided time for intestinal adaptation, potentially avoiding early intestinal transplantation.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Transplantation Surgery
Background:
- Very short bowel syndrome (VSBS) and intestinal failure (IF) pose significant challenges in pediatric patients.
- Total parenteral nutrition (TPN) is often required from birth for infants with VSBS.
- Early development of severe liver disease can complicate the management of IF.
Observation:
- An infant with VSBS and IF developed severe cholestatic liver disease requiring liver transplantation.
- The infant received a living donor left segment liver graft at 8 months of age.
- Post-transplant, oral alimentation improved, but TPN-independence was not achieved.
Findings:
- Isolated liver transplantation led to improved digestive and absorption functions.
- The patient required 50-60% of energy needs via TPN at 20 months post-transplant, with satisfactory growth.
- Reduced stool volume and frequency were noted post-transplantation.
Implications:
- Isolated liver transplantation can be a viable strategy to manage liver failure in infants with VSBS and IF.
- This intervention may provide crucial time for intestinal adaptation, potentially delaying or avoiding the need for intestinal transplantation.
- Further research is needed to optimize outcomes and long-term management in these complex pediatric cases.
Abstract:
Intestinal function in children with very short bowel syndrome and related intestinal failure may improve after isolated liver transplantation. An infant with an ultrashort gut, ileo-cecal valve, and whole colon received total parenteral nutrition from the first days of life. Enteral feeding failed because of the progressive dilatation of the jejunal portion and motility disorders. He developed early severe cholestatic liver disease (aspartate transferase 186, alanine transferase 103 U/L, serum bilirubin 8.4 mg/dL) and subsequent liver failure. At 8 months of age, he benefited from isolated liver transplantation (left segment graft from living donor). His early posttransplant evolution was characterized by recovery of oral alimentation, improvement of digestive and absorption functions, but he did not achieve TPN-independence. At 20 months, 50% to 60% of his energy needs were covered by parenteral nutrition and he has satisfactory growth indices (3rd percentile for weight and height), reduced stool volume, and frequency. Isolated liver transplantation allowed, in this particular case, time for further intestinal adaptation thereby avoiding the need for intestinal transplantation early in life.
