Paediatric Liver Transplantation
September 1, 2019
Liver diseases are increasingly being diagnosed in children due to increased awareness among primary care physicians and advancement and widespread availability of various diagnostic modalities. Many of the pediatric liver diseases may require liver transplantation as a part of their treatment. Some of the common diseases affecting the liver in children are as follows:
1. Cholestatic liver disease:
- Biliary atresia
- Progressive Familial Intrahepatic Cholestasis (PFIC types 1-5)
- Alagille Syndrome
2. Metabolic liver disease:
- Wilson disease (including fulminant Wilson disease)
- Disorders of amino acid metabolism (including urea cycle defects)
- Alpha 1 antitrypsin deficiency
3. Liver tumours:
- Hepatocellular carcinoma
4. Budd Chiari Syndrome
5. Acute Liver Failure
The above mentioned diseases may require liver transplantation as a part of their treatment at various stages of their natural history.
Liver transplantation in children:
Liver transplantation in children is a challenging endeavour. The small size of a child’s hepatic vasculature, need for implantation of a small sized partial liver graft, severe adhesions secondary to previous operations etc., make the operation technically complex. Further, the clinical and biochemical changes that occur in the perioperative period in children with metabolic liver disease are highly complex need experienced pediatric hepatologists and transplant surgeons for proper management.
Liver grafts for transplantation in children can be procured from related living donors or from cadaver organs which are split into two parts for transplantation into one adult and one child (Split Liver Transplantation). Children usually require the left lateral segment (20-25% of whole liver) or the left lobe (30-40% of whole liver) to compensate for their metabolic demands. The retrieved partial liver is then implanted into the child after the removal of the child’s own native liver. The blood vessels (hepatic veins, portal vein, hepatic artery) and bile ducts of the new liver graft are connected to their counterpart structures in the recipient using fine sutures. Following the operation, children are managed in the dedicated liver ICU initially and later transferred to a private room once medically suitable. Typical hospital stay lasts for about 2 weeks. Recipients need to be on lifelong immunosuppression to maintain a healthy liver graft.
Dr. Rela Hospital is an International Quaternary Care Multi-Superspeciality Hospital located in Chennai, India, and is one of the leading centres for multiorgan transplantation in Asia. Professor Mohamed Rela is an internationally recognized pioneer in liver disease and transplantation with an experience of more than 25 years. He is the foremost authority in paediatric transplantation in the world and was instrumental in the development and progress of many innovative techniques in liver transplantation including Auxiliary Partial Orthotopic Liver Transplantation (APOLT) and Split Liver Transplantation. With a large team of experts including transplant surgeons, adult and paediatric hepatologists, liver intensivists and other supporting specialties, Dr. Rela Hospital provides comprehensive care for paediatric and adult patients suffering with liver disease.