Medanta is one of the leading hospital groups in India for pediatric disease management, including pediatric liver transplant procedures, and has a highly experienced team of pediatric hepatologist..
Medanta is one of the leading hospital groups in India for pediatric disease management, including pediatric liver transplant procedures, and has a highly experienced team of pediatric hepatologists, transplant surgeons, and critical care specialists. The following are some reasons why Medanta is preferred by many for pediatric liver transplants:
Expertise: Medanta has a dedicated team of pediatric hepatologists and transplant surgeons with extensive experience performing pediatric liver transplant surgeries. They have successfully carried out nearly 4000 complex liver transplant surgeries, with nearly 500 in small children with excellent outcomes. The team led by Dr. Arvinder Singh Soin (Chairman , Institute of Liver Transplantation and Regenerative Medicine) and Dr. Neelam Mohan, (Director - Paediatric Gastroenterology, Hepatology & Liver Transplantation) has been recognized with several credits at international and national levels.
Advanced facilities: Medanta has a modern and well-equipped liver transplant unit with cutting-edge facilities for liver transplant surgeries, including dedicated operation theaters, intensive care units, and high-tech diagnostic and imaging equipment.
Multidisciplinary approach: Medanta follows a multidisciplinary approach for children with liver diseases, involving a team of specialists, including pediatric hepatologists, transplant surgeons, anesthesiologists, critical care specialists, nutritionists, physiotherapists, and highly specialized team of interventional radiologists, histopathologists. Our labs are accredited by JCI and NABH.
Comprehensive pre and post-transplant care: Medanta provides comprehensive care for children undergoing liver transplant surgery, including evaluation, preparation, and post-transplant follow-up care.
Success rates: In India, Medanta has one of the highest success rates for pediatric liver transplant surgeries.
A pediatric liver transplant is a surgical procedure where the surgeon replaces diseased or damaged liver in a child with a healthy liver from a donor. This procedure is the treatment of choice in children with end-stage liver disease or liver failure and those who have exhausted all other treatment options. Dr. Thomas Starzl and his team at the University of Colorado in 1967 performed the first successful pediatric liver transplant on a 19-month-old girl. Since then, scientists, researchers, and clinicians have made significant advancements in pediatric liver transplantation, including improvements in surgical techniques, critical care, immunosuppression medications, and post-operative care.
In India, the doctors performed the first successful pediatric liver transplant with end-stage liver disease in 1998. Over the last two and half decades, there has been a tremendous progress in the field of liver transplant in India.
The following are some indications for pediatric liver transplant:
• Biliary Atresia:
Biliary atresia is a rare pediatric liver disease that affects the bile ducts. It is a condition where the bile ducts (tubes that carry bile from the liver to the small intestine) become inflamed, scarred, and eventually destroyed. It may lead to blockage of the bile flow from the liver to the intestine, causing bile to accumulate in the liver, which can cause damage to the liver cells and eventually lead to liver failure.
The exact cause of biliary atresia is still unknown. It is more common in infants of Asian or African descent. There is a slightly higher female predilection for biliary atresia.
Symptoms of biliary atresia usually appear within the first few weeks to months of life and may include:
o Jaundice (yellowing of the skin and eyes)
o Dark-colored urine
o Pale or clay-colored stools
o Difficulty in gaining weight
o Delay growth
o Enlarged liver and spleen
o Itching
Biliary atresia can lead to cirrhosis and even liver failure if not treated early. The diagnosis of biliary atresia includes physical examinations, blood tests, imaging tests (ultrasound or MRI),HIDA and a liver biopsy.
The treatment of choice for biliary atresia is surgery, the Kasai procedure. In this procedure, the healthcare specialist removes the damaged bile ducts and attaches a piece of the small intestine directly to the liver, allowing bile to flow out of the liver into the intestine. The Kasai procedure has a higher success rate when done within few weeks of age. Results are inferior after 3months of age.
Many children with biliary atresia after Kasai surgery require a liver transplant, either because the Kasai procedure fails to restore normal bile flow or because of progressive liver disease. In addition, if the diagnosis is delayed then one may not perform Kasai surgery but proceed with liver transplant. Regular monitoring by a pediatric hepatologist is essential to ensure that any liver problems are detected early and treated promptly.
• Metabolic liver diseases:
Metabolic liver diseases are a group of genetic disorders that affect the liver's ability to break down and process certain substances in the body. These substances then accumulate in the liver and other organs, damaging the liver cells and leading to liver failure. Metabolic liver diseases are mostly inherited, meaning they are passed down from parents to their children through genes.
There are several metabolic liver diseases, each with its specific causes and symptoms. The following are some common types:
- Wilson's disease: In this rare genetic disorder, copper starts accumulating in the liver and other organs, which can lead to liver disease, neurological symptoms, psychiatric problems, renal issues and rickets.
- Glycogen storage disease: In this genetic disorder, the body can not break down and store glycogen properly, leading to a buildup of glycogen in the liver and other organs, causing liver disease and other symptoms.
- Tyrosinemia: In this condition, the body cannot break down the amino acid tyrosine, leading to a buildup of toxic substances in the liver and other organs, causing liver disease, kidney problems, and other complications.
- Progressive familial intrahepatic cholestasis (PFIC)
- Aagille’s syndrome
- Hemochromatosis: The patient's body absorbs too much iron from the diet. A higher amount of iron can cause iron overload in the liver and other organs, which can cause liver disease and other complications.
- Alpha-1 antitrypsin deficiency: Your body may poorly produce a protein called alpha-1 antitrypsin, leading to abnormal alpha-1 antitrypsin buildup in the liver and other organs, causing liver damage and respiratory problems. This is seen in West and rarely reported in India.
The diagnosis of metabolic liver disease includes physical exams, blood tests, imaging tests, and genetic testing. Treatment varies depending on the specific type of metabolic liver disease but may include medications, dietary changes, and in some cases, liver transplantation.
• Acute liver failure (ALF):
It is a rare but life-threatening condition in which the liver suddenly and rapidly loses its ability to function, usually within a few days to weeks. ALF can occur due to various factors, including viral infections, drug overdoses, autoimmune disorders, and genetic disorders.
The symptoms of ALF can vary, but the following are some common manifestations:
o Jaundice (yellowing of the skin and sclera)
o Abdominal pain and swelling
o Nausea and vomiting
o Confusion and disorientation
o Fatigue and weakness
o Easy bruising or bleeding
The diagnosis of ALF consists of blood tests to evaluate liver function and imaging tests, such as ultrasound or CT scan, which help assess the liver and other organs. Treatment for ALF depends on the underlying cause of the condition. Sometimes, the liver may recover with only supportive medical care, while in others, more aggressive treatment, such as liver transplantation may be necessary. There are certain criteria for liver transplantation post vitamin K like King’s College Criteria, Cliche Criteria. Often a persistent INR more than 4 necessiates liver transplant.
• Chronic liver disease:
In this condition, the slow deterioration over a long period, often several years or decades, can lead to liver damage. The liver is a vital organ that performs many functions, including processing nutrients, removing toxins, and producing bile to help digestion. Chronic liver disease can interfere with these functions and lead to serious health complications.
The following are the most common causes of chronic liver disease in children:
- Cholestatic liver disease
- Autoimmune hepatitis: In this disease, the body's immune system attacks the liver cells, causing inflammation and damage.
- Genetic conditions: Various genetic conditions, such as tyrosinemia and glycogen storage disease and Wilson's disease, can lead to liver damage over time.
- Nonalcoholic fatty liver disease (NAFLD): In this condition, fat accumulates in the liver, causing inflammation and scarring.
- Chronic viral hepatitis: Chronic hepatitis B and C infections can lead to liver inflammation and scarring over time.
Although, the symptoms of chronic liver disease may differ in each person and depend on the severity of the condition and the underlying cause. But the following are some common symptoms of this condition:
o Extreme fatigue and weakness
o Abdominal pain and swelling
o Jaundice (yellowing of the skin and sclera)
o Itchiness
o Dark-colored urine
o Pale stools
o Nausea and vomiting
o Easy bruising or bleeding
o Spider-like blood vessels on the skin
Generally, the diagnosis of chronic liver disease includes:
o Blood tests to evaluate liver functions
o Imaging tests such as ultrasound or CT scan
o Liver elastography such as fibroscan
o Liver biopsy to confirm the diagnosis
Treatment for chronic liver disease includes:
o Medications to manage symptoms and slow the progression of the disease
o Treatment of portal hypertension
o Nutritional support
o Lifestyle modifications: a healthy diet, and regular exercise
o Liver transplantation is the answer to treat end-stage liver disease.
• Liver tumors:
Liver tumors are abnormal and uncontrolled growth of the liver cells. Liver tumors can be benign (non-cancerous) or malignant (cancerous).
Benign liver tumors are relatively common, and persons do not show any visible symptoms. The following are the most common types of benign liver tumors:
- Hemangiomas: These growths are abnormal clusters of blood vessels that form a mass in the liver.
- Hepatic adenomas: Adenomas are rare tumors that develop from liver cells. Sometimes they can grow to a large size.
- Focal nodular hyperplasia (FNH): A non-cancerous tumor that occurs when normal liver tissue starts growing and forms a mass
Malignant liver tumors are cancerous and can be life-threatening if not diagnosed and treated promptly. The most common type of malignant liver tumor is hepatocellular carcinoma (HCC). Other types of the liver are cholangiocarcinoma (bile duct cancer) and angiosarcoma (blood vessel cancer).
The symptoms of liver tumors depend on the size and location of the mass, but the common manifestations are:
o Pain or discomfort in the upper right quadrant of the abdomen
o Unexplained weight loss
o Loss of appetite
o Fatigue and weakness
o Jaundice
o Nausea and vomiting
o Abdominal swelling
o Swelling in the legs
o Enlarged liver or spleen
The liver tumors diagnosis includes imaging tests such as ultrasound, CT scan, or MRI. In some cases, doctors recommend a liver biopsy to confirm the diagnosis.
Treatment for liver tumors includes surgery to remove the mass and some surrounding healthy tissues, radiation therapy, chemotherapy, targeted therapy, or liver transplantation.
There are two main types of pediatric liver transplants, such as:
• Orthotopic liver transplant (OLT):
The orthotopic liver transplant (OLT) is the most common type of liver transplant. In this procedure, the healthcare specialist removes the entire diseased liver and replaces it with a healthy liver from a deceased or living donor. The new liver is positioned as the original site and attached to the blood vessels and bile ducts. The OLT procedure can take several hours. After the surgery, the patient is closely monitored in the intensive care unit (ICU) for several days. The child will need to take immunosuppressive medications to prevent rejection of the new liver and undergo regular monitoring and follow-up care.
• Living donor liver transplant (LDLT):
In this procedure, a portion of the liver from a living donor (usually a family member) is removed and transplanted into the child. The liver is a self-regenerating tissue, so the remaining portion of the donor's liver will regenerate within a few weeks, allowing both the donor and the recipient to have functioning livers.
The LDLT procedure can be either a full left or right lobe transplant or a partial transplant, depending on the recipient's physical status and the portion of the donated liver.
Although it is a safe procedure, the living donor liver transplant may have some risks for both the donor and the recipient, including bleeding, infection, and bile leakage. It also offers several advantages over the cadaveric liver transplant, such as reduced waiting time for a suitable donor liver, improved outcomes for the recipient, and the capability to transplant a smaller liver into a small child.
The type of liver transplant depends on several factors, including the availability of a suitable donor liver, the child's overall health and medical history, and the preferences of the child's healthcare providers and family. Both liver transplants require careful evaluation, planning, and post-operative care for better possible outcomes for the child.
A pediatric liver transplant is a major surgery that requires careful planning, evaluation, and post-operative care. After the surgery, the child may need to stay in the hospital for several weeks to recover and receive appropriate medical care. The following are the steps:
• Pre-operative preparation: A child will undergo extensive medical investigations before surgery to assess their overall health and determine procedure type. This evaluation includes blood tests, imaging, and other diagnostic tests to evaluate the liver's function and the health of other organs. Sometimes, the child may need additional testing, such as a biopsy, to further confirm the diagnosis and severity of the liver disease.
• Anesthesia: Generally, the liver transplant procedure requires general anesthesia. The anesthesia team will monitor the child's vital signs throughout the surgery to ensure their safety.
• Liver removal: In the case of a deceased donor transplant, the diseased liver is removed and replaced with a healthy liver from a deceased donor. The surgeon makes an incision in the abdomen to access the liver and carefully remove it while ensuring that blood vessels and other organs are intact. Similarly, in the living donor transplant procedure, a portion of the liver from a living donor is removed and transplanted into the child. The surgeon removes a part of the healthy donor's liver, ensuring enough remaining liver tissue for the donor's liver to regenerate.
• Liver transplantation: After removal, the surgeon transplants the healthy liver into the child and attaches the blood vessels and bile ducts to it. The surgeon will carefully monitor blood flow to ensure that the donated part of the liver is functioning. The surgeon may perform additional procedures, such as repairing or replacing damaged blood vessels or bile ducts.
• Wound closure: Once the liver transplantation procedure is complete, the surgeon closes the abdominal incision with stitches or staples. The child may also have drain pipes placed to help remove excess fluid or blood, which may accumulate around the liver.
• Post-operative care: The child will need close monitoring in the ICU for several days to weeks after surgery. In this period, the healthcare experts ensure the proper function of the new liver and manage any post-operative complications. The doctor may prescribe immunosuppressive medications to prevent rejection of the new liver. After discharge from the hospital, the doctor may schedule numerous appointments for regular liver function monitoring and follow-up care.
The eligibility criteria for pediatric liver transplant depends on the specific transplant center and the child's health status. However, the followings are some eligibility criteria for pediatric liver transplant:
• Diagnosis criteria: The child must have a diagnosis of a liver disease that is not responding to medication or other interventions. End-stage liver disease, acute liver failure, or certain genetic and metabolic disorders can cause fatal liver damage, therefore considered for a liver transplant. Other liver diseases for which your doctor may suggest liver transplants are biliary atresia, primary sclerosing cholangitis, and metabolic liver conditions such as glycogen storage disease. Common indications for liver transplantation are biliary atresia, acute liver failure, autoimmune hepatitis, primary sclerosing cholangitis and metabolic liver conditions such as Wilson’s disease, progressive familial intrahepatic cholestasis, Langerhans cell histiocytosis, glycogen storage disease etc.
• Age: Most pediatric liver transplants are for children under 18. However, some transplant centers may consider transplanting young adults up to 21.
• Overall health status: The child must be in generally good health and not have any other medical conditions which may increase the risk of complications during or after the transplant. Children with critical medical conditions, such as heart or lung disease, may not be eligible for a liver transplant.
• Absence of active infections or cancers: Another essential criterion for liver transplant is that the child must not have any active infectious diseases or cancers, as these conditions could increase the risk of post-transplant complications. Children with a recent infection or cancer may need to wait until they fully recover before the liver transplant procedure.
• Support system: The child must have a sound support system that helps with post-operative care and transportation to follow-up appointments. Children who do not have adequate support may not be eligible for a liver transplant.
Compliance with the medical regimen: The child and their family must be willing and able to comply with the post-operative care regimen, including taking immunosuppressive medications and attending regular follow-up appointments. Failure to comply with the post-operative care regimen can increase the risk of complications and decrease the chances of a successful transplant.
Generally, a pediatric liver transplant is a safe procedure with minimal complications. The liver transplant team may decide to proceed with a liver transplant after careful consideration of the child's individual health status and overall well-being and weighing the potential benefits of the transplant against the potential risks. Some of the potential risks are:
• Bleeding: Like other surgeries, the risk of bleeding during the transplant surgery is generally low. It can occur due to surgical complications or underlying bleeding disorders. Excessive bleeding can require blood transfusions or additional surgery to control it.
• Infection: After the transplant, the child is at high risk for getting infections due to a weakened immune system. This risk is highest in the first few months following the transplant when the immune system is significantly weak and immunosuppressive drugs are at higher dosage. It can be bacterial, viral, or fungal and require antibiotics or antifungal medications based on the disease-causing pathogen.
• Rejection: Rejection occurs when the child's immune system recognizes the transplanted liver as a foreign substance and attacks it to get rid. The rejection risk is highest in the first few months following the transplant but can occur at any time. Rejection symptoms may include fever, abdominal pain or discomfort, and jaundice. The treatment involves increased doses of immunosuppressive medications or other drugs.
• Bile duct complications: Sometimes, the bile ducts may damage during the transplant surgery, leading to bile leaks or blockages (stricture). Bile duct stricture can cause abdominal pain, fever, and jaundice. Treatment may include surgical intervention or placement of stents to help drain the bile.
• Renal impairment: The surgery and the immunosuppressive medications can affect the kidneys which need constant monitoring and management.
• Cardiovascular complications: The transplant procedure and post-operative immunosuppressive drugs may affect the heart and blood vessels, leading to hypertension, high cholesterol, or other cardiovascular complications. Close monitoring and management of these conditions is crucial to prevent long-term complications.
• Cancer: Immunosuppressive medications can increase the risk of certain cancers, such as PTLDor lymphoma. Therefore, close monitoring and screening for cancer is crucial after the transplant.
Neurological complications: In rare cases, the transplant surgery and the immunosuppressive medications can cause neurological complications, such as seizures or encephalopathy.
The recovery is a gradual process and varies from child to child, including:
• Hospital Stay: After the transplant surgery, the child may have to remain hospitalized for several weeks. During this time, the medical staff will closely monitor the child for any signs of complications, and their medications and treatment plan will be adjusted as needed.
• Medications: The child will need to take immunosuppressive drugs for the rest of their life to prevent rejection of the transplanted liver. The doctors may recommend other medicines to manage pain, prevent infections, or treat various conditions.
• Follow-up Appointments: Your child may need frequent follow-up visits so that the doctor can monitor their recovery and adjust their treatment plan with time. During follow-up appointments, the doctor may prescribe blood tests, imaging studies, and other tests to monitor the transplanted liver functions and detect any signs of rejection or other complications.
• Diet and Exercise: The doctor and the transplant team may suggest a special diet and exercise plan to help promote healing and prevent complications. These may include avoiding certain foods or activities and following a specific exercise plan to help rebuild strength and endurance.
• Emotional Support: The recovery process can be physically and emotionally stressful for the child and their family. Therefore, it is crucial to have a support system. You may also take help for counseling or other forms of emotional support.
• Long-Term Care: The child will need long-term health care to prevent and manage long-term complications. These may include regular blood tests, imaging studies, and tests to monitor the transplanted liver functions.
The following are some studies that support the efficacy of recovery after a pediatric liver transplant:
• Survival rates: According to data from the Scientific Registry of Transplant Recipients, the overall survival rate for pediatric liver transplant recipients has improved over time. In 2020, the 1-year survival rate was 93.8%, and the 5-year survival rate was 85.4%.
• Quality of life: A study published in the journal Pediatric Transplantation in 2017 found that children who underwent liver transplants had a significantly improved quality of life compared to those who did not receive a transplant. The study used a validated questionnaire to assess the quality of life in pediatric liver transplant recipients.
• Physical and cognitive function: A study published in the journal Pediatric Transplantation in 2019 found that children who underwent liver transplants experienced a significant improvement in physical and cognitive function compared to before the transplant.
• Growth and development: A study published in the journal Liver Transplantation in 2021 found that pediatric liver transplant recipients show improvements in growth and development after transplant. The study found that height and weight increased significantly after transplant and that children achieved developmental milestones at rates similar to the general population.
What is a pediatric liver transplant?
A pediatric liver transplant is a surgical procedure in which the surgeon replaces a diseased or damaged liver with a healthy liver from a living donor or cadaveric organ.
What are some indications for pediatric liver transplants?
Common indications for pediatric liver transplant include biliary atresia, metabolic liver diseases, acute liver failure, chronic liver disease, and liver tumor occasionally done to replace liver enzyme which cause predominantly extra hepatic damage such as urea cycle defects, organic academias, primary hyperoxaluria, protein C, protein S, factor 7 deficiency etc.
What is the success rate of pediatric liver transplants?
The success rate of pediatric liver transplants has improved significantly over time, with a 1-year survival rate of 93 to 95% and a 10-year survival rate nearing 90% .
How long does the transplant surgery typically take?
The transplant surgery typically takes between 6 to 12 hours to complete.
How long does the recovery process usually take?
The recovery process can take several weeks to months and may involve several follow-up appointments and ongoing medications.
Can a child receive a liver from a deceased (brain dead) donor?
Yes, a child can receive a liver from a deceased or a living donor.
What are the risks associated with pediatric liver transplants?
The risks associated with pediatric liver transplants include infection, bleeding, rejection, biliary leak / stricture and side effects from immunosuppressive medications.
How long does my child need immunosuppressive medications after a liver transplant?
A child may have to take immunosuppressive medications for the rest of their life to prevent rejection of the transplanted liver.
Can a child participate in physical activities after a liver transplant?
Yes, the children can participate in physical activities after a liver transplant but with proper care and guidance,
Will a child need to follow a special diet after a liver transplant?
A child may need to follow a special diet after a liver transplant, depending on the individual medical needs and preferences. Subsequently a child is motivated to have healthy eating habits.
How often will a child need to attend follow-up appointments after a liver transplant?
A child will need regular follow-up appointments with their medical team for the rest of their life to monitor their health and the transplanted liver functions. The visits are gradually reduced to 2 to4 per year unless some issue.
Can a child go back to school after a liver transplant?
Yes, most children can return to school after a liver transplant within a couple of months.
Can a child have children after a liver transplant?
Yes, with proper care and guidance from their doctor, most people conceive after a liver transplant.
How long will a child's liver take to function to return to normal after a liver transplant?
It can take few weeks for a child's liver function to return to normal after a liver transplant.
Can a child develop complications from immunosuppressive medications?
Yes. Some children may develop complications from immunosuppressive medications, such as infections or side effects like weight gain or increased blood pressure.
Can a child receive a liver transplant if they have other medical conditions?
It depends on the specific medical conditions and their severity, but in most cases, a child with other medical problems which are not life threatening and still be eligible for a liver transplant unless they are not live threatening.
