Pediatric liver transplant

Pediatric liver diseases 

Biliary atresia

Biliary atresia, also known as extrahepatic biliary atresia, is an inflammatory condition of the biliary system outside the liver, leading to fibrosis and blockage of bile duct. It is congenital and presents within few days of birth with progressive jaundice. The diagnosis is confirmed by blood tests, and imaging studies like abdominal ultrasonography, MRCP, and HIDA scan.

The two surgical interventions which can benefit the child are kasai’s portoenterostomy and liver transplantation. If not treated early, the progression to liver cirrhosis is generally inevitable and such children usually require liver transplantation. The success rate of kasai’s procedure is approximately 50-60%. In the long term, approximately 60-70% of the children require liver transplantation.  

Metabolic liver diseases

Metabolic liver diseases are generally hereditary syndromes with deficiency of specific enzymes that are important in metabolism of certain compounds. They may present either as life-threatening conditions in neonatal period or as chronic liver disease in adolescence or adulthood, which may progress to liver failure, cirrhosis, or hepatocellular carcinoma. They are an important cause of pediatric liver disease. The common metabolic liver diseases in children are alpha-1 antitrypsin deficiency, glycogen storage diseases, congenital disorders of glycosylation, porphyria, tyrosinemia, cystic fibrosis, and others. The treatment of these disorders, when progressed to liver cirrhosis, is liver transplantation.

Pediatric liver transplant

Pediatric liver transplant is a complex surgery and usually requires extensive surgical experience and a good set-up comprising of pediatric ICU and pediatric intensivist besides other routine requirement for an adult liver transplant.

A pediatric recipient requires only a small portion of donor liver. Usually left lobe, or even smaller part of liver is sufficient for the child. The challenges in a pediatric liver transplant are due to small abdominal size of the child, smaller sized blood vessels, and difficult closure of the abdomen. Post-operative management of the child is also challenging and requires an experienced pediatric intensive care. The long-term outcomes of pediatric liver transplant are generally good and comparable to adult liver transplant.