Liver Transplantation

The common indications of liver transplant in children include cholestatic liver disease (most common of which is biliary atresia) and metabolic liver disease followed by acute liver failure. Occasionally it is indicated for liver tumor and certain rare condition.

Indications for liver transplantation  are:

• Cholestatic liver disease:  Biliary atresia, Alagille’s, syndrome, Progressive familial  intrahepatic cholestasis(PFIC), Neonatal hepatitis.
• Metabolic liver disease : Wilson’s disease, Tyrosinaemia, Glycogen storage disorder, Galactosemia, Fatty liver disease, Fatty acid oxidation defects, Bile acid disorders, a1 anti trypsin deficiency.
• Hepatitis : Viral hepatitis (A,E,B,C  others), Autoimmune hepatitis, Drugs and poisoning ( including paracetamol poisoning/ toxicity secondary to  antituberculosis/antiepileptic drugs).
• Metabolic and Genetic disorders: Metabolic disease where liver is the calpirt.  Criggler-Najjar syndrome type-1, Organic acidemias,Urea cycle defects like maple syrup urine disease (MSUD) and Primary oxalosis.
• Liver tumors: Benign tumors that have replaced the whole liver, Unresectable malignant, without extrahepatic metastasis.

Certain rare condition such as factor VII deficiency, Protein C and Protein S deficiency and familial hypercholesterolemia.

Management of hepatic complications:

It is important to ensure that specific hepatic complications are appropriately managed while the patient waits for transplant. These include portal hypertension, oesophageal varices, ascities, hypoproteinemia etc.

Nutritional support:

It has been demonstrated in several studies that nutritional status at liver transplant is an important prognostic factor in survival, that is better outcome is seen in patients with good nutritional status. The patient needs to be on a high calorie diet (150- 200% calories with good protein intake) with two times the RDA of multi vitamins and in patients with cholestasis supplementation with fat soluble vitamins like vitamin A,D,E,K is done. In patients with cholestasis,MCT oil as in coconut oil is used for cooking. If a child is not able to feed well orally then tube feed supplementation is done, which could be for overnight feeds or during the day as per the need.

We have completed >200 Pediatric Liver Transplants in small children and are the busiest center in India with success rate of 92% at 1 year and overall success rate of over 90% on a median follow up of >4 years.

Our Experience indications of pediatric liver transplantation-

• Cholestatic liver disease is the commonest cause of Liver Transplantation. This includes Biliary atresia, Carolis disease, Allagille Syndrome, Progressive familial intrahehepatic cholestasis (PFIC)Sclerosing cholangitis including Langerhans cell histiocytosis (LCH). Langerhans cell histiocytosis (LCH) is a rare condition of Liver disease secondary to Sclerosing cholangitis and we have 5 year survival of 100% in these patients
• Metabolic Liver Disease – Wilson Diseases is the commonest cause in Metabolic Liver disease that have been transplanted. We have a large experience of other metabolic conditions such as Tyrosinemia, Gyclogen storage disorder (GSD), Maple Syrup Urine Diseases (MSUD), Progressive familial intrahehepatic cholestasis (PFIC), factor VII deficiency & protein C and S deficiency.
• Our team has a good experience of Combined Liver Kidney transplantation in Primary Hyperoxaluria in children with 100%results.
• We have experience in Liver Transplantation in Metabolic inborn errors of metabolism such as MSUD, Citrullinemia etc.
• Acute Liver failure (ALF) and Acute on Chronic liver failure (ACLF) constitutes one third of our total transplants. We have advanced Management Statergy to care in ALF & ACLF patient like Intracranial Bolt in ALF, hemo-dialysis, CRRT, plasma- pheresis, MARS etc. Common causes of ALF are Viral Hepatitis A/E, drugs (ATT) and ACLF are, Wilson disease, tyrosinemia and autoimmune liver disease.
• We have also large series of liver transplant with autoimmune liver disease including autoimmune sclerosing cholangitis.
• We have done surgical innovations to answer shortage of organs such as

1. ABO incompatible Liver transplantation – where blood group of donor and recipient is not matching.
2. Swap liver transplants– It is a method to match the blood group incompatible donor and recipient couples with another donor and recipient having similar issues.
3. Domino liver transplants– In a domino Liver transplant, the explanted liver from a patient who has an illness curable by liver transplant, is used as domino graft for some other patient, as the explanted liver is otherwise well.

• Hepatopulmonary syndromeis a rare complication of Portal hypertension where there is lung failure in association with liver disease. We have transplanted 8 such cases with excellent results.