Introduction: Children who develop intestinal failure-associated liver disease (IFALD) may be referred for combined liver intestinal transplantation with an aim to becoming parenteral nutrition (PN)-independent and free from liver disease. Isolated liver transplantation (ILT) in these patients is avoided, as most patients who had this procedure developed disease in the graft liver due to the ongoing need for PN. However, with the current paradigm of successful long-term PN managed in an expert centre, ILT may be an option for children at risk of death due to end-stage IFALD where no intestinal graft is available.

Methods: A retrospective review of case notes of three patients with IFALD who underwent ILT in a single centre between 2014 and 2018.

Results: Case 1 is an 8 year-old with short bowel syndrome (SBS) secondary to gastroschisis. Following resection she had 21cm of small bowel, and developed life-threatening IFALD. She was referred for combined liver intestinal transplant, but her parents declined the small bowel so she underwent ILT at age 3. She remains PN-dependent, despite pre-transplant bowel lengthening surgery. She is well and growing along the <0.4th centile with no liver disease at 4 years post-transplant.

Case 2 also developed SBS secondary to gastroschisis, with 32cm of small bowel after resection. She was listed for combined liver intestine transplant at 9 months of age, but developed end-stage liver disease with severe stomal bleeding necessitating a life-saving ILT in 2015. Following transplant she remains PN-dependent with some oral feeds and no signs of liver disease at 2 years post-transplant[J1] [J2] .

Case 3 is a 2 year-old with SBS due to antenatal bowel perforation requiring removal of a large portion of small bowel as a neonate. He underwent ILT at 13 months due to development of end-stage liver disease and coagulopathy, with no available intestinal graft. Recovery was complicated with life-threatening line infections, but he has achieved stability, and is growing well with 70% of his calorie and fluid requirement coming from PN and no signs of liver disease at 9 months post-transplant.

Conclusion: In the era of successful modern PN, ILT in children with end-stage IFALD may be life-saving for patients in whom size-matched organs are not available, or intestinal transplantation is declined.