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Paediatric Liver Transplant Indications

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Medically Reviewed by Dr. Aman Priya Khanna
Written by Sangeeta Sharma, last updated on 27 December 2023| min read
Paediatric Liver Transplant Indications

Quick Summary

Here are three reasons why a liver transplant may be necessary for children:

  • Congenital liver conditions
  • Severe liver disease
  • Liver failure

Understanding paediatric liver transplant indications is crucial for parents and caregivers, as it helps ensure early detection and timely intervention. It can ultimately improve the chances of a successful transplant and a healthier future for the little ones in need.

Knowing why a liver transplant may be necessary for children is important. Knowing the critical indications from congenital liver conditions to severe liver disease may warrant this life-saving procedure.

Understanding paediatric liver transplant indications is crucial for parents and caregivers, as it helps ensure early detection and timely intervention. It can ultimately improve the chances of a successful transplant and a healthier future for the little ones in need. 

The blog highlights the vital factors that prompt such interventions, from congenital liver conditions to severe liver diseases. 

What is a Paediatric Liver Transplant?

A paediatric liver transplant is a procedure where the non-functioning liver is replaced with a fully functioning liver or a portion of a healthy liver.
This healthy liver is taken from a living or deceased donor.

  1. About 1500 to 2000 liver transplants occur in India annually, of which 10% are paediatric transplants.

  2. India, with its expert team of doctors and medical facilities, has been highly successful in surpassing a success rate of 90%.

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Indications of Paediatric Liver Transplant

Whether it's an adult or a child, anyone experiencing a life-threatening condition of the liver may require a liver transplant.
Recognising these signs and considering seeking appropriate medical and surgical help is essential.

The following are the primary causes of liver transplantation in children:

  1. Biliary Atresia: The most common indication for liver transplantation is biliary atresia.
    It is a chronic condition that damages the liver and causes cirrhosis when the bile ducts are absent or obstructed. 

    When surgical procedures, such as the Kasai operation (surgical treatment for biliary atresia), fail to restore adequate bile flow or when the kid develops end-stage liver disease, paediatric liver transplants for autoimmune liver disease are often considered.
  1. Metabolic Disorders: Children with specific inborn metabolic abnormalities may experience increasing liver dysfunction and injury. Examples are:

  1. Wilson's Illness: This hereditary ailment leads to copper build-up in the liver and other organs.

  2. Alpha-1 Antitrypsin Deficiency: A hereditary condition that results in improper protein synthesis and liver damage.

  3. Glycogen Storage Diseases: The ability of the liver to process glycogen is compromised.

  4. Maple Syrup Urine Disease: A metabolic condition that affects the metabolism of branched-chain amino acids and causes liver damage.

  1. Acute Liver Failure (ALF): The function of the liver is severely weakened in acute liver failure.
    There can be different causes, which include drug toxicity, metabolic disorders, viral infections (like hepatitis), and autoimmune hepatitis. 

    When supportive care is ineffective, and a high risk of death from irreparable liver disease exists, criteria for paediatric liver transplants are a possibility.
  1. Chronic Liver Disease: Several chronic liver illnesses can develop in children and proceed to end-stage liver disease, requiring liver transplantation. These comprise the following:

  1. Chronic Hepatitis B or C: Viral infections that result in continuous liver damage and inflammation.

  2. Alagille Syndrome: A hereditary disease that affects the growth of the bile ducts and harms the liver.

  3. Autoimmune Hepatitis: Achronic autoimmune disease can damage the liver, causing inflammation.

  4. Progressive Familial Intrahepatic Cholestasis (PFIC): It is a hereditary condition that decreases bile flow, damaging the liver.

  5. Cystic Fibrosis: This multisystem disorder can cause progressive liver damage.

  1. Liver Tumour: Paediatric liver transplant eligibility is crucial for children diagnosed with an incurable or recurrent liver tumour like hepatoblastoma or hepatocellular carcinoma.
    A liver transplant provides the best hope for long-term survival and improved outcomes.

Contraindications with Paediatric Liver Transplantation

Contraindications like absolute contraindications are medical conditions or situations where a liver transplant cannot be performed under any circumstances.
It can be due to extremely high risks or incompatibility.

These conditions make the person ineligible for a liver transplant, as attempting the procedure could jeopardize their health and well-being.
Below are a few absolute contraindications:

  1. Extrahepatic Active Cancer: People with cancer actively spreading outside of the liver, such as cancer in an advanced stage, may be prohibited from receiving a liver transplant.
    After transplantation, there is a substantial chance of cancer recurrence or metastasis, which may outweigh any potential advantages.

  1. Advanced Cardiac or Pulmonary Disease: Absolute contraindications may exist for severe cardiac or pulmonary disorders that increase the risk of surgical complications or reduce life expectancy.
    Transplantation might be too dangerous in severe coronary artery disease, severe pulmonary hypertension, or irreversible lung disease.

  1. Observable Neurological Impairment: Certain cognitive impairments or impairing neurologic disorders are included in this.
    It can cause severe and irreversible neurological impairment.

Consequences of Untreated Paediatric Liver Transplants

Untreated paediatric liver transplant indications can have many adverse effects on the child's overall condition and lead to further damage.
Some of the common consequences of delaying paediatric liver transplant indications include the following:

  1. Increased Disease Progression: Delaying a paediatric liver transplant can lead to the progression of the underlying liver condition.
    It can worsen the child's health and potentially complicate the transplant procedure.

  2. Reduced Quality of Life: The child may experience a diminished quality of life.
    Liver diseases can cause discomfort, fatigue, and limitations in daily activities.

  3. Risk of Complications: As the liver disease progresses, the child becomes more vulnerable to complications such as infections, bleeding, and fluid buildup. This can be life-threatening.

  4. Limited Treatment Options: Delaying the transplant may limit the effectiveness of alternative treatments, as liver transplantation is often the best option for certain liver conditions.

  5. Organ Availability: Paediatric liver donors are relatively scarce, so delaying the transplant may result in a longer wait for a suitable donor match.

  6. Long-term Health Effects: It can impact the child's future health and potential complications.

  7. Overall Risk to Life: Ultimately, delaying a liver transplant can increase the risk of life-threatening complications.
    It can reduce the chances of a successful transplant and a healthier life post-transplant.

Prevention of Paediatric Liver Transplant

Preventing the need for paediatric liver transplants is of utmost importance to ensure the well-being of children. 

Focusing on proactive measures and early intervention can reduce the risk of liver diseases and the necessity for transplant procedures. Below are some key points on how to prevent paediatric liver transplant:

  1. Vaccinations: Ensuring children receive all recommended vaccinations can protect them from liver infections like Hepatitis A and B.
    This reduces the likelihood of liver disease progression.

  2. Healthy Diet: Encouraging a balanced and nutritious diet can help prevent obesity and fatty liver disease in children, promoting optimal liver function.

  3. Regular Exercise: Engaging in regular physical activity promotes a healthy weight and overall well-being.
    It reduces the risk of liver-related complications.

  4. Hygiene Practices: Promoting good hygiene habits, such as washing hands, can prevent the spreading of infections.

  5. Early Detection and Treatment: Regular health check-ups and timely medical intervention can identify liver problems early on, allowing for prompt treatment and minimizing disease progression.

  6. Prevention of Mother-to-Child Transmission: Preventing transmission of viral infections from mother to child, such as Hepatitis B, can protect infants from developing liver diseases.

  7. Safe Medication Use: Ensuring that children take medications as prescribed and avoiding over-the-counter drug misuse can prevent drug-induced liver damage.

  8. Management of Chronic Conditions: Properly managing chronic health conditions like diabetes and autoimmune diseases can reduce their impact on liver health.

Treatment of Paediatric Liver Transplant Indications

A paediatric liver transplant is a safe and successful procedure. However, in some cases, indications of problems may arise.
These problems can be addressed and treated with the help of the following surgical and non-surgical treatments. These are: 

  1. Immunosuppressants: These are medicines that are used to control the body's immunity.
    It stops the body's defence from attacking the new liver in the body. 

  2. Antibiotics: Medicines are prescribed to patients suffering from problems with infections.
    With the help of antibiotics, one can get rid of bacterial infections and felicitate healing.

  3. Interventional Radiology Procedures: These are specialised procedures that regulate blood flow to the new liver.
    This is mostly used in the case of Vascular complications.

  4. Endoscopic Retrograde Cholangio-pancreatography (ERCP): Under this procedure, an endoscope is used to treat problems with bile leakage.

Takeaway

Paediatric liver transplantation is a challenging and life-saving treatment for kids with end-stage liver disease, acute liver failure, or certain metabolic diseases. Identifying the proper indications for liver transplantation in paediatric patients can maximise the success rate. 

Contact HexaHealth to explore the most suitable treatment choices for paediatric liver transplants. We'll be there every step, from understanding the condition to providing post-surgical care as your supportive partner.

Suggested Reads

Top 10 Paediatric Liver Transplant Hospitals Risks and Complications After a Liver Transplant
Liver Transplant Rejection Unknown Facts about Liver Transplant Surgery
Liver Transplant Success Rate Liver Transplant Who can Donate

Frequently Asked Questions

Liver transplants in children are indicated when they have severe liver diseases or conditions that cannot be treated effectively with other medical interventions. These conditions may include:

  1. End-stage liver failure

  2. Congenital liver abnormalities 

  3. Certain liver tumours.

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Each transplant hospital may have slightly different requirements for liver transplant indications in infants. The criteria include: 

  1. A severe liver disease that is resistant to medical treatment

  2. End-stage liver failure

  3. Significant impairment of liver function

  4. The absence of transplant-related contraindications

  5. A reasonable chance of survival and improved quality of life after transplantation

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Following are some of the most common indications for paediatric liver transplantation : 

  1. Paediatric liver transplant by Biliary atresia (a rare congenital condition in which the bile ducts outside or inside the liver are either absent or damaged)

  2. Acute liver failure

  3. Metabolic diseases

  4. Progressive liver cirrhosis

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 Infant liver transplantation may be necessary for several causes. These indications include: 

  1. Biliary atresia

  2. Acute liver failure

  3. Metabolic liver illnesses

  4. Congenital liver diseases

  5. Hereditary liver disorders

  6. Liver tumours

  7. Progressive liver cirrhosis

  8. Certain metabolic abnormalities that cannot be adequately treated by medicinal interventions alone

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Yes, adolescents do have particular liver transplant criteria. These include: 

  1. Acute liver failure

  2. Metabolic liver diseases

  3. Genetic liver disorders

  4. Certain liver tumours that cannot be effectively treated by other means

  5. Autoimmune hepatitis, viral hepatitis, or primary sclerosing cholangitis. 

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A comprehensive strategy is necessary to treat chronic paediatric liver transplants for Wilson disease in children. Typically, this can be done in the following ways: 

  1. Taking medical measures to manage the disease's symptoms

  2. Changes in diet

  3. Regular exercise

  4. Careful monitoring of liver function

  5. Routine follow-up appointments with a paediatric hepatologist 

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Kasai procedure is the main treatment for a paediatric liver transplant for biliary atresia. The damaged bile ducts are removed during this procedure. And a small portion of the small intestine is connected directly to the liver to allow bile drainage.

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Genetic disorders where the liver transplant is required are:

  1. The Alagille syndrome: It is characterised by bile duct abnormalities affecting the heart, liver, and other organs.

  2. Crigler-Najjar syndrome: This is a rare condition where certain enzymes are deficient in the blood.

  3. Glycogen Storage Diseases - A group of genetic disorders affecting the liver and other organs of the body.

  4. Paediatric liver transplant Alpha-1 Antitrypsin Deficiencies - A liver condition that creates problems in the normal functioning of the liver.

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In circumstances where the tumour is unresectable (cannot be totally removed), a paediatric liver transplant may be advised. The tumour spreads outside of the liver in this case. 

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References

All the articles on HexaHealth are supported by verified medically-recognized sources such as; peer-reviewed academic research papers, research institutions, and medical journals. Our medical reviewers also check references of the articles to prioritize accuracy and relevance. Refer to our detailed editorial policy for more information.


  1. Spada M, Riva S, Maggiore G, Cintorino D, Gridelli B. Pediatric liver transplantation. World J Gastroenterol. 2009 Feb 14;15(6):648-74. doi: 10.3748/wjg.15.648. PMID: 19222089; PMCID: PMC2653434.link
  2. Esquivel CO, Iwatsuki S, Gordon RD, Marsh WW Jr, Koneru B, Makowka L, Tzakis AG, Todo S, Starzl TE. Indications for pediatric liver transplantation. J Pediatr. 1987 Dec;111(6 Pt 2):1039-45. doi: 10.1016/s0022-3476(87)80053-7. PMID: 3316578; PMCID: PMC3086407.link
  3. Engelmann G, Schmidt J, Oh J, Lenhartz H, Wenning D, Teufel U, Büchler MW, Hoffmann GF, Meyburg J. Indications for pediatric liver transplantation. Data from the Heidelberg pediatric liver transplantation program. Nephrol Dial Transplant. 2007 Sep;22 Suppl 8:viii23-viii28. doi: 10.1093/ndt/gfm649. Plink
  4. Cox KL, Berquist WE, Castillo RO. Paediatric liver transplantation: indications, timing and medical complications. J Gastroenterol Hepatol. 1999 May;14 Suppl:S61-6. doi: 10.1046/j.1440-1746.1999.01904.x. PMID: 10382641.link
  5. Hackl C, Schlitt HJ, Melter M, Knoppke B, Loss M. Current developments in pediatric liver transplantation. World J Hepatol. 2015 Jun 18;7(11):1509-20. doi: 10.4254/wjh.v7.i11.1509. PMID: 26085910; PMCID: PMC4462689.link
  6. Sundaram SS, Mack CL, Feldman AG, Sokol RJ. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl. 2017 Jan;23(1):96-109. doi: 10.1002/lt.24640. PMID: 27650268; PMCID: PMC5177506.link
  7. Dababneh Y, Mousa OY. Liver Transplantation. [Updated 2023 Apr 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. link
  8. Whitington PF, Emond JC, Black DD, Whitington SH, Boone P, Smith C, Thistlethwaite JR, Broelsch CE. Indications for liver transplantation in pediatric patients. Clin Transplant. 1991 Apr;5(2 part 2):155-60. PMID: 10147638.link
  9. Varma V, Mehta N, Kumaran V, Nundy S. Indications and contraindications for liver transplantation. Int J Hepatol. 2011;2011:121862. doi: 10.4061/2011/121862. Epub 2011 Oct 5. PMID: 22007310; PMCID: PMC3189562.link
  10. Basturk A, Yılmaz A, Sayar E, Dinçkan A, Aliosmanoğlu İ, Erbiş H, Aydınlı B, Artan R. Pediatric Liver Transplantation: Our Experiences. Eurasian J Med. 2016 Oct;48(3):209-212. doi: 10.5152/eurasianjmed.2016.0147. Erratum in: Eurasian J Med. 2018 Feb;50(1):64. PMID: 28149148; PMCID: PMC5268605.link

Last Updated on: 27 December 2023

Disclaimer: The information provided here is for educational and learning purposes only. It doesn't cover every medical condition and might not be relevant to your personal situation. This information isn't medical advice, isn't meant for diagnosing any condition, and shouldn't replace talking to a certified medical or healthcare professional.

Reviewer

Dr. Aman Priya Khanna

Dr. Aman Priya Khanna

MBBS, DNB General Surgery, Fellowship in Minimal Access Surgery, FIAGES

12 Years Experience

Dr Aman Priya Khanna is a well-known General Surgeon, Proctologist and Bariatric Surgeon currently associated with HealthFort Clinic, Health First Multispecialty Clinic in Delhi. He has 12 years of experience in General Surgery and worke...View More

Author

Sangeeta Sharma

Sangeeta Sharma

BSc. Biochemistry I MSc. Biochemistry (Oxford College Bangalore)

6 Years Experience

She has extensive experience in content and regulatory writing with reputed organisations like Sun Pharmaceuticals and Innodata. Skilled in SEO and passionate about creating informative and engaging medical conten...View More

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