Pediatric liver transplant replaces a child's failing liver, most often from biliary atresia, metabolic disease, acute liver failure or hepatoblastoma, with part of a healthy liver donated by a parent or close relative. Both livers regrow within weeks. Our pediatric transplant team evaluates child and donor, performs both operations, cares for your child in pediatric intensive care and follows you once you are back home.
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
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
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.
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.
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 Prof. Dr. Mustafa YAMAN, Pulmonology.