[The first Danish child with small bowel transplantation]

Area of Science:

• Gastroenterology
• Pediatric Surgery
• Transplantation Immunology

Background:

• Hirschsprung's disease can necessitate extensive small bowel resection, leading to short bowel syndrome.
• Long-term parenteral nutrition (PN) is often required but carries risks of complications like liver failure.
• Intravenous access issues can further complicate management in pediatric patients with short bowel syndrome.

Observation:

• A pediatric patient with extensive Hirschsprung's disease developed short bowel syndrome after surgery, requiring long-term PN.
• Progressive liver failure and central venous access problems emerged as significant complications.
• At 5.5 years old, the patient underwent a complex small bowel, liver, and pancreas transplantation.

Findings:

• The patient received immunosuppressive therapy, ursodeoxycholic acid, and antibiotic prophylaxis post-transplantation.
• 32 months after the transplant, the patient is clinically well and tolerating enteral nutrition.
• Successful multi-organ transplantation resolved the complications associated with short bowel syndrome and PN.

Implications:

• This case highlights the potential for successful multi-organ transplantation in pediatric patients with severe short bowel syndrome and associated complications.
• It underscores the importance of considering transplantation as a viable option for end-stage liver disease secondary to PN.
• Long-term follow-up demonstrates the feasibility of weaning from PN to enteral nutrition after complex intestinal and liver transplantation.