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Pediatric Liver Transplant - Child Liver Transplant
Organ Transplant

Pediatric Liver Transplant - Child Liver Transplant

About This Department

 
Pediatric Transplantation

A small part of a parent's liver can replace a child's failing one. Both livers grow back to full function within weeks.

Pediatric liver transplant, or child liver transplant, replaces a child's diseased liver with a healthy one, for most children a portion of liver donated by a parent or close relative, called living donor liver transplantation. Biliary atresia, a blockage of the bile ducts present from birth, is the commonest reason. Inherited metabolic diseases, sudden liver failure and tumors such as hepatoblastoma follow. For babies, the donor gives the left lateral segment, about a fifth of the liver, and surgeons perform it even in infants weighing less than 5 kilograms. In the largest registry of living donor transplants for biliary atresia, 2,085 children in Japan, 84.2 percent of patients were alive 20 years later (Kasahara and colleagues, 2017). This page explains why children need a transplant and when to refer, how living donation works and what it means for the donor, evaluation of child and donor, the operation, life after transplant, complications, results, and how families from abroad arrange treatment at Biruni Hospital in Istanbul.

84.2
Percent of children alive 20 years after living donor transplant for biliary atresia, 2,085 patients in Japan
90.5
Percent of transplanted livers still working one year after surgery in the same registry
5
Kilograms, the body weight below which infants can still receive a reduced segment from a living donor
Free
Review of your child's records by our pediatric transplant team
Free consultation

Why children need a liver transplant

Main reasons for liver transplant in children
Condition What happens Typical age at transplant
Biliary atresia Bile ducts blocked from birth, cirrhosis despite the Kasai operation Under two years, some in later childhood
Metabolic and genetic diseases Urea cycle defects, Crigler-Najjar syndrome, PFIC, Wilson disease, tyrosinemia Infancy to adolescence
Alagille syndrome Too few bile ducts, severe itching and poor growth Early childhood
Acute liver failure Sudden loss of liver function from viruses, drugs or unknown causes Any age, within days
Liver tumors Hepatoblastoma that cannot be removed after chemotherapy Early childhood
In some metabolic diseases the liver itself looks healthy but lacks a single enzyme, and the new liver cures the whole-body disease. In others, such as biliary atresia, the liver is scarred and failing. The team weighs the child's growth, symptoms, blood tests and quality of life before recommending transplant.
Biliary atresia accounts for about half of all liver transplants in young children worldwide.
Signs that liver disease is advancing
Sign What it reflects Action
Yellow skin and eyes persisting after Kasai Bile still not draining Early referral to a transplant center
Poor weight gain despite feeding Malnutrition from liver disease Nutrition support and transplant assessment
Swollen belly, vomiting blood Fluid and pressure in the liver veins Assessment within days
Early referral gives the team time to prepare the child and the donor calmly.

When to refer

1
The earlier, the better
Children are referred when the first complication of cirrhosis appears, before the illness becomes an emergency. Transplant in a stable, well-nourished child carries lower risk than in a child already critically ill.
Referral triggers
Situation Timing Reason
Failed Kasai operation, bilirubin still high at three months Immediately Most need transplant within the first two years
Growth failure, severe itching, repeated infections of the bile ducts Within weeks Quality of life and survival at risk
Acute liver failure, confusion, bleeding Same day Transplant may be needed within days
Scores such as PELD, for children under 12, and MELD, for older children, help rank urgency. For a living donor transplant the score matters less than readiness of the child and donor.
2
Acute liver failure
A child with sudden liver failure needs transfer to a transplant center with pediatric intensive care immediately, since the window for transplant can close within days.

Biliary atresia and the Kasai operation

The Kasai operation, done in the first two months of life, connects a loop of bowel directly to the liver to drain bile. When it works, the child may keep the native liver for years, sometimes into adulthood. It buys time. Still, the majority of children with biliary atresia eventually need a transplant, some in infancy and others as teenagers, when scarring slowly catches up. Regular follow-up after the Kasai operation allows referral before complications become severe.

The living donor liver

The liver is divided into segments, each with its own blood supply and bile drainage. A surgeon can remove part of an adult's liver and transplant it into a child. Both parts regrow, the donor's to nearly full size within six to eight weeks and the child's graft as the child grows.
Which part of the liver is used
Graft Share of donor liver Child who receives it
Reduced or monosegment A tenth or less Infants under about 5 kilograms
Left lateral segment About a fifth Infants and young children
Left lobe About a third Older children and small adolescents
Right lobe About three fifths Adolescents and adult-sized recipients

Results in the largest registry

The 1-, 5-, 10-, 15- and 20-year graft survival rates for biliary atresia patients undergoing living donor liver transplantation were 90.5, 90.4, 84.6, 82.0 and 79.9 percent, and living donor transplantation proved a safe and effective treatment that does not compromise living donors (Kasahara and colleagues, American Journal of Transplantation, 2018).
1
Why living donation suits children
Deceased donor livers small enough for young children are scarce. Waiting can be fatal. A living donor allows the operation to be planned at the best moment, with a healthy graft and a short cold time.

The donor

The donor is usually a parent. Any healthy adult relative with a compatible blood group can also be assessed. Donating the left lateral segment for a small child removes less liver than donation for an adult, and recovery is faster.
Who can be a donor
Criterion Usual requirement Note
Age 18 to about 55 Older donors assessed individually
Blood group Compatible with the child Incompatible transplants possible with extra treatment
Health No liver disease, heart disease or diabetes Fatty liver may need weight loss first
Relationship Family member, with documents Required by Turkish law for foreign patients
1
Donor evaluation
Blood tests, heart and lung checks, CT or MRI scans of the liver to map vessels and measure volume, and a psychological assessment. An independent doctor who is not part of the child's team confirms the donor is fit and acting freely.
How risky is it for the donor?
Donation is major surgery. Complications such as bile leak, infection or wound problems occur in a minority, and most resolve fully. The worldwide rate of donor death is very low, around 2 in 1,000 across all living liver donations, most of which were adult-to-adult donations with larger grafts. Donation of the smaller left lateral segment for a child carries less risk. The surgeon discusses these figures with each donor in detail before consent.
The donor mortality rate was 0.2 percent across 11,553 donor operations in 71 programs, with the majority of deaths occurring within 60 days (Cheah and colleagues, Liver Transplantation, 2013).
Donor recovery
Time Stage Activities
Days 1 to 7 Hospital stay Walking from day one or two
Weeks 2 to 6 Home recovery Light activity, no heavy lifting
Months 2 to 3 Liver regrown Most return to full work and sport

Evaluation of the child

1
One to two weeks of tests
Pediatric hepatologists, surgeons, anesthetists, cardiologists and dietitians assess the child. The evaluation also checks vaccinations, which are completed before transplant wherever possible, since live vaccines cannot be given afterwards.
Tests before transplant
Test Purpose Note
Blood tests and infections screen Liver function, blood group, viruses such as CMV and EBV Guides medicines after transplant
Ultrasound, CT or MRI Vessels, size of the abdomen, earlier surgery Plans the graft and the connections
Heart ultrasound Heart function and congenital defects Common in Alagille syndrome
Nutrition assessment Weight, muscle, vitamins Feeding support improves recovery

The operation and intensive care

1
Surgery day
The donor and child operations run in parallel in two theaters. The donor operation takes four to six hours, the child's six to ten. The child's diseased liver is removed and the new graft connected to the child's blood vessels and bile drainage.
After surgery the child goes to the pediatric intensive care unit, where breathing, blood flow to the new liver and fluid balance are watched closely. Most children come off the breathing machine within a day or two and move to the transplant ward within a week. Daily ultrasound checks the flow in the new liver's vessels during the first days.

A typical hospital course for the child
Period Where Main focus
Days 1 to 5 Pediatric intensive care Breathing, blood flow, early rejection signs
Weeks 1 to 3 Transplant ward Feeding, walking, medicine levels
Weeks 3 to 4 Discharge to nearby accommodation Clinic visits two to three times a week

Preparing your child and family

Children cope better when they know what is coming.

Explain the operation in simple words suited to the child's age, using the picture books and dolls the play specialist provides, and let the child bring a favorite toy and blanket into hospital. Brothers and sisters need an explanation too, and a plan for their care while both a parent and the child are in hospital, since the donor parent will need days of rest after surgery and cannot lift the child for several weeks. Families who arrange a second adult to stay with them in Istanbul find the weeks after surgery far easier, because one parent recovering from donation and another caring for a child in intensive care is too much for one household to carry alone.

Write your questions down. Bring them. The team answers every one.

Life after transplant

A liver transplant replaces one disease with a new, manageable condition. Lifelong medicines, a healthy lifestyle and regular follow-up keep the new liver working.
Follow-up after transplant
Item Frequency Purpose
Immunosuppressant medicine, usually tacrolimus Twice daily, for life Prevents rejection
Blood tests Weekly at first, then every one to three months Liver function, drug levels, infections
Ultrasound At intervals set by the team Blood flow and bile ducts
Vaccines Inactivated vaccines on schedule Live vaccines avoided
The dose of immunosuppressant falls over the first year, and many children take a single medicine at low dose in the long term. Adolescents are the group at most risk of missing doses, and the team works with teenagers directly to make the medicines their own responsibility.

Growing up with a new liver

Most children catch up.

Growth resumes, appetite returns and energy lifts within months of a successful transplant. Children return to school three to six months after surgery, and take part in sport once the wound has healed fully. Development can catch up too, especially when the transplant happened early, though some children need extra support at school. Hand washing, food hygiene and prompt attention to fever become habits. As teenagers, children learn to manage their own medicines and appointments before moving to an adult transplant clinic.

Complications

Complications after pediatric liver transplant are common in the first months. Most are treated without losing the new liver.
Complications and their treatment
Complication When Treatment
Acute rejection First weeks to months Higher doses of immunosuppression, usually reversed
Hepatic artery thrombosis First days to weeks Emergency surgery or new transplant
Bile duct narrowing or leak Weeks to years Stent, drainage or surgery
Infections, including CMV and EBV First months Antiviral medicines and monitoring
Lymphoproliferative disease, PTLD Months to years Reduced immunosuppression, rituximab
Parents learn the warning signs before discharge and have a number to call day and night.
Retransplantation is needed in a minority of children, most often because of vessel problems in the first weeks or chronic rejection years later.
Warning signs for parents
Sign Possible cause What to do
Fever Infection or rejection Call the transplant team the same day
Yellow eyes, pale stools, dark urine Bile duct problem or rejection Call the team
Vomiting and unable to keep medicines down Risk of low drug levels Call the team at once

For families from abroad

One coordinator from the international patients office handles arrangements from the first message through discharge and answers the same WhatsApp number once you are back home. The office works in English, Arabic, French, Russian, Serbian, Romanian and Spanish and books interpreters for other languages, and it arranges the visa invitation letter, airport transfers, daily transport and accommodation near the hospital. Halal, vegetarian and diabetic meals, a prayer room on site, and a female physician on request wherever the rota allows.
Children from abroad receive a liver from a living relative, since deceased donor organs in Turkey are allocated through the national system. Turkish law requires the donor to be a relative, and the relationship must be documented before surgery. The coordinator lists the exact documents for your country, and translations are arranged here.
Documents for living donation
Document Purpose Note
Birth certificates or family register Proves the relationship between donor and child Apostilled or consular-certified, translated
Passports of child and donor Identity Valid for the whole stay
Medical reports Diagnosis, earlier surgery, blood group Sent in advance for review
1
Length of stay and fitness to fly
The child and family stay about two to three months in total, covering one to two weeks of evaluation, three to four weeks in hospital and close follow-up afterwards. The donor usually leaves hospital within a week and is fit to fly after about four weeks. The child flies once medicine levels are stable and the team is satisfied with liver function.
Once you are back home, your local pediatrician and hepatologist continue follow-up with our written plan, blood results are shared with us, and our team remains reachable for questions.

Cost

What a quote for pediatric liver transplant covers
Item Included Priced separately
Evaluation Child and donor tests, consultations Additional tests for complex cases
Surgery Both operations, intensive care, hospital stay Retransplantation, extra procedures for complications
After discharge Clinic visits and tests during the stay Medicines after return home, accommodation
The quote is given as a package covering child and donor, based on the child's condition. Complications that extend the stay add to the cost, and the quote explains how these are handled. Compare packages that include the same items, especially intensive care days and the donor's evaluation.

References

  1. Kasahara M, Umeshita K, Sakamoto S, et al. Living donor liver transplantation for biliary atresia. An analysis of 2085 cases in the registry of the Japanese Liver Transplantation Society. Am J Transplant. 2018;18(3):659-668.
  2. Kasahara M, Umeshita K, Sakamoto S, Fukuda A, Furukawa H, Uemoto S. Liver transplantation for biliary atresia. A systematic review. Pediatr Surg Int. 2017;33(12):1289-1295.
  3. Cheah YL, Simpson MA, Pomposelli JJ, Pomfret EA. Incidence of death and potentially life-threatening near-miss events in living donor hepatic lobectomy. A world-wide survey. Liver Transpl. 2013;19(5):499-506.

Editor's note

Written by the Biruni Hospital medical editorial team. Reviewed by Assoc. Prof. Dr. Utku Mahir YILDIRIM, Radiology.

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