Paediatrics: Liver transplantation

2021-03-04 12:00 AM

Indications for liver transplantation, Clinical features requiring consideration for transplantation, Post-transplant complications.

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 varies 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, hepato pulmonary syndrome.

Preparation for transplant

Requires multidisciplinary evaluation to include the following:

  • Nutritional support.
  • Development and psychological assessment of child and family.
  • Education and counselling.
  • 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 (the main cause of death).

Prognosis

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

  • 1yr survival is 90%.
  • 5yr survival is 80%.