Liver transplantation in children: SAQ

This boy with biliary atresia has developed the classic indications for liver transplantation after a failing Kasai: intractable pruritus, recurrent cholangitis, growth failure, and early synthetic dysfunction. The fall in his growth centile is itself a marker of disease severity that, uniquely in children, is built into the allocation score. The family's question about a living donor is timely, because the smallest children face the longest waits for a size-matched deceased organ. [10]

Question 1 (10 marks)

Outline the indications for liver transplantation in this child, describe how he would be prioritised on the deceased-donor waiting list, and discuss the role of living-donor transplantation. [1]

The accepted indications for transplantation in a child with a failing Kasai are synthetic failure (a falling albumin and rising international normalised ratio, which he shows with an albumin of 28 grams per litre and an international normalised ratio of 1.7), intractable pruritus that disrupts sleep and quality of life, growth failure despite nutritional support (his fall from the 25th to below the 3rd centile), recurrent cholangitis (his two episodes), and portal hypertension that is difficult to control. He meets several of these, and referral to a paediatric transplant centre is indicated. [10]

He would be prioritised on the deceased-donor waiting list by the Paediatric End-stage Liver Disease score, introduced for allocation in the United States from 2002. The score is calculated from five variables: serum bilirubin, the international normalised ratio, serum albumin, growth failure defined as height or weight less than two standard deviations below the mean, and age at listing, with additional weight given to age under one year. Growth failure is deliberately weighted because malnutrition reflects disease severity in children in a way that no adult variable captures, and his marked growth failure will raise his score and his priority. His bilirubin of 140 micromoles per litre, his international normalised ratio of 1.7, his low albumin, and his growth failure will all contribute to a high PELD score. [3]

I would counsel the family that living-donor transplantation is an established option in which a parent typically donates a left lateral segment. It was developed because the smallest infants have the highest waiting-list mortality, as size-matched deceased donors are scarce, and a living-donor graft shortens waiting time and reduces the risk of dying on the list. The donor evaluation is conducted to the same safety standards as a deceased-donor pathway, and the donor hepatectomy, while safe in experienced hands, carries a small but real morbidity and a rare mortality that must be disclosed. The trade-off is a higher vascular and biliary complication rate in the segmental graft, which I would discuss honestly. [1]