Paediatrics: Liver transplantation

Liver transplantation

Indications for liver transplantation

The commonest underlying conditions leading to irreversible liver failure and transplant are:

• Fulminant hepatic failure: e.g. viral, toxic, Wilson’s disease.

• Biliary atresia.

• Chronic end-stage liver disease: e.g. post-viral hepatitis with cirrhosis.

• Liver based metabolic conditions: e.g. Wilson’s disease, α₁-antitrypsin deficiency, Crigler–Najjar syndrome, tyrosinaemia.

·Acute liver failure following a liver transplant: e.g. primary non-function of transplant or hepatic artery thrombosis.

• Neonatal hepatitis.

• Autoimmune hepatitis.

• Unresectable tumour confined to the liver: e.g. hepatoblastoma.

Clinical features requiring consideration for transplantation

• Bleeding varices due to portal hypertension.

• Failure of growth or development.

• Resistant ascites.

• Hepatic encephalopathy.

• Poor quality of life: e.g. pruritis, lethargy.

• Coagulopathy (PTT >2 x normal).

• Multi-organ failure: e.g. hepatorenal syndrome, hepatopulmonary syndrome.

Preparation for transplant

Requires multidisciplinary evaluation to include the following:

• Nutritional support.

• Development and psychological assessment of child and family.

• Education and counseling.

• Ensure vaccinations are current: e.g. MMR, varicella, hepatitis A and B.

• Cardiac evaluation (ECG, echocardiogram).

·Abdominal US (patency of major hepatic blood vessels).

Post-transplant complications

• Primary non-function of the liver (<5%).

• Hepatic artery thrombosis (10–15%).

• Biliary leaks and strictures (20%).

• Acute rejection (50%).

• Chronic rejection (5–10%).

• Sepsis (main cause of death).

Prognosis

Long-term studies indicate normal psychosocial development and quality of life in survivors. Patients require lifelong immunosuppression drug ther-apy, e.g. ciclosporin or tacrolimus.

• 1yr survival is 90%.

•   5yr survival is 80%.