Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children

Liver dysfunction is a well-recognized complication of intestinal failure i n children. Advances in total parenteral nutrition (TPN) have allowed these children to survive while their intestinal tract gradually adapts. Unfortu nately TPN may lead to cholestatic liver disease particularly in the young children. Progression of liver disease is associated with a poor prognosis and is an indication for small bowel transplantation. We report our experie nce of orthotopic liver transplantation in four children with short gut and sequential liver and small bowel transplantation in one child. All childre n had TPN-related liver failure. Causes of intestinal failure included necr otising enterocolitis (n=2), gastroschisis (n=1), intestinal atresia (n=1), and megacystic, microcolon syndrome (n=1), At the time of liver transplant ation the children's mean age was 10.9 months (2.5-24) and weight 6.7 kg (4 .8-10.1), The mean serum bilirubin was 522 mu mol/liter (299-823), aspartat e transaminase 423 IU/liter (49-1024) and international normalized ratio 2. 8 (2-3.9). There were two deaths both from respiratory failure secondary to adenovirus pneumonia including the child who received a sequential small b owel transplant, Three children with isolated liver grafts are alive and of f TPN at 20 months (mean) follow up (range 6-35). Isolated orthotopic liver transplantation has a role in selected children with intestinal failure, p articularly those with short but normally functioning gut and progressing w ith satisfactory intestinal adaptation but developing liver disease, Those children with TPN-related liver disease and unadapted gut or irreversible i ntestinal disease need combined liver and small bowel transplantation. Sequ ential small bowel transplantation is feasible after orthotopic liver trans plantation and may provide an option for the child with terminal liver and small bowel failure.