Living Donor Liver Transplant
In a liver transplant, the diseased liver is completely removed and replaced with a new liver. The donor liver can either be from a brain dead person on life support in an ICU, or the right or left half of a living person’s liver (living donor liver transplant). Such a donor is 18-55 years old, blood group compatible, and from the family - either spouse, or a first or second degree relative. Amazingly, both the transplanted part and the donor’s remaining liver grow back by regeneration to their normal size in 2 months. This makes the surgery very safe for the donor. The donor and recipient surgery proceed simultaneously, and the donor is discharged home a week and the recipient 2 weeks after transplant.
Fatty Liver Disease
Fatty liver is the excessive build-up of fat in the liver. This can be due to excess alcohol, or even without alcohol due to diabetes, obesity, deranged lipids and cholesterol, and poor lifestyle choices such as sedentary habits and fast food. Initial stages are reversible. However, if the cause is not corrected, the damage can continue, worsening into inflammation, scarring (cirrhosis), liver failure and/or liver cancer.
Alcoholic Liver Disease
Alcoholic liver disease is liver damage due to alcohol abuse.
How much is too much? Liver disease occurs after years of heavy drinking (over 10 units/week; each unit equals 30ml of whisky/200 ml of wine or equivalent). It initially causes fat deposits in the liver. If the damage continues, it causes inflammation, scarring, cirrhosis, and even cancer. The chances of serious liver disease are higher among women, and go up with the duration and amount of drinking. The damage also depends on individual metabolic capacity and the genetic make up.
Living Donor Liver Transplant In Children (Pediatric)
Liver may fail in children due to Biliary Atresia, Progressive Familial Intrahepatic Cholestasis (PFIC), or metabolic diseases such as Wilson’s Disease (excessive copper), liver enzyme deficiencies in urea cycle defects such as OTC, Citrullinemia, and others. At Medanta, we have done nearly 400 pediatric living donor transplants with a success rate of 97%. One of the parents or an Uncle or Aunt (blood group matched, non-obese and 18-55 years old) usually donates about 20-35% of their liver for this. Both the transplanted part and the donor’s remaining liver grow back to normal size within 2 months. After surgery, the donor is discharged home in a week and the child in 2 weeks. These children grow normally and enjoy a completely normal life in the long-term. We now have several children who are well 15-24 years after transplant.
Living Donor Liver Transplant In Babies (Less Than 8 kg)
Liver failure at this age can be due to Biliary Atresia and metabolic diseases. Transplanting fragile and ill small babies is very challenging. It requires complex reduction of the donor liver to a small enough size to fit the little bellies, as well as special micro-surgery techniques to connect the vital blood vessels of the new liver to the baby’s minute vessels. At Medanta, we have extensive experience in this, having successfully transplanted nearly 200 such babies, the smallest being just 2 kg. The transplants are rewarding, with a 97% success rate, and the babies enjoy an excellent long-term survival and quality of life.
Biliary atresia is the congenital inadequacy of the ducts that drain bile from the liver. It begins soon after birth. Bile is trapped inside the liver and causes damage and scarring of the liver, eventually resulting in cirrhosis.
Symptoms and diagnosis
Jaundice after the age of 2 weeks.
Yellow eyes and skin, light-colored stools and dark urine
Weight loss, irritability
Blood tests: increased bilirubin level
Ultrasound, HIDA scan, Liver biopsy
Treatment: Before 90 days of age, an operation called Kasai’s portoenterostomy can benefit 40-50% children. After that and those who do not improve after this operation need a liver transplant.
Wilson's Disease is a genetic defect (runs in families) that impairs the ability of the liver to clear copper. This results in copper accumulation in and damage to the liver, brain, kidney and other organs. Most people are diagnosed between the ages of 5 and 35.
Earlier stages of the disease can be controlled with medicines (copper binding agents eg., D-pen). However, over time, liver disease usually becomes severe, resulting in cirrhosis. It can also cause acute (sudden) liver failure. These patients often have brain symptoms such as tremors, involuntary movements, clumsy gait and speech difficulties. The only cure is a timely liver transplant which also reverses many of the brain symptoms. The transplant must be done before serious brain damage occurs.
Other Liver Diseases/Causes Of Cirrhosis In Children
Other causes of serious liver diseases in children (all of which can be cured with a liver transplant) include metabolic deseases like Hereditary Hemochromatosis, Alpha-I Antitrypsin (AAT) deficiency, Progressive Familial Intrahepatic Cholestasis (PFIC), Maple Syrup Urine Disease (MSUD), Urea cycle defects such as Ornithine Transcarbamoylase (OTC) Deficiency & Citrullinemia, Hereditary Tyrosinemia.
Hereditary Hemochromatosis: defective liver protein resulting in excessive iron absorption, leading to its overload in and damage to the liver, heart and other vital organs.
Alpha-I Antitrypsin (AAT) Deficiency: a protein deficiency that causes lung and liver damage.
PFIC: defective bile production and secretion from liver cells causing buildup of bile in them leading to liver damage and cirrhosis.
MSUD: These babies are unable to use or break down branched chain amino acids (leucine, isoleucine and valine - all end products of protein digestion). Apart from liver damage and cirrhosis, high levels of these amino acids also damage the brain. While these children can avoid brain toxicity and survive on special low-protein diets in the short-term, a liver transplant is the only permanent cure.
Urea cycle defects: Two such disorders are Ornithine transcarbamoylase (OTC) deficiency and Citrullinemia – in which liver enzyme defects prevent the conversion of ammonia (waste product of protein metabolism) into urea, thus ammonia accumulates. This is toxic to both the liver and the brain. To survive, these children need special diets, which are difficult to sustain. A liver transplant can cure these diseases and restore normalcy.
Hereditary Tyrosinemia is an inherited disorder characterized by an enzyme deficiency resulting in excess tyrosine and its metabolites that are toxic to both the liver and the kidney. While special diets can provide temporary relief, liver transplantation permanently cures the condition.
HCC (Hepatocellular Carcinoma – Primary Liver Cancer)
HCC is the sixth commonest cancer and the third commonest cause of cancer death worldwide. It occurs in persons with pre-existing liver disease due to Hepatitis B, Hepatitis C, Alcoholic Liver Disease or Non-Alcoholic Fatty Liver Disease. While those with early HCC and good liver function can be treated with ‘burning the cancer’ (RFA), or local chemotherapy (TACE), patients with advanced HCC and/or poor liver function are often cured with a liver transplant. Once secondaries develop, palliative immunotherapy or targeted therapy can improve quality of life and survival by a few months.
Swap Liver Transplant
At Medanta, we offer a unique option to patients with liver failure or HCC who have a medically fit family donor having the opposite blood group. An A group patient with a B group donor can exchange livers with another patient whose own group is B and donor’s is A.
We maintain a large database of such donor-recipient pairs and can provide a match for a swap transplant within 2-3 weeks.
Robotic and Key Hole Liver Donor Surgery
At Medanta, we perform key-hole surgery for donors so they can have a relatively painless recovery and avoid a big scar. The operation is conducted using thin instruments inserted via four small 1cm incisions (cuts) in the abdomen, and the part of the liver to be donated is then removed via a 10 cm incision just above the pubic bone (well below the belt area). This way, the main cut is small, hidden, and avoids muscle cutting, thus heals faster. This is much more comfortable and cosmetically appealing for liver donors compared to open surgery with a big incision.
Medanta Institute Of Liver Transplantation And Regenerative Medicine, Sector 38, Gurugram,
Haryana 122001, India
Dr. A. S. Soin
Hepatobiliary and Liver Transplant Surgeon