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Liver Transplant For Biliary Atresia
Pediatric Surgery

Liver Transplant For Biliary Atresia

About This Department

 
Pediatric Surgery
Organ Transplantation

Biliary atresia is the commonest reason children need a new liver. With part of a parent's liver, nine in ten transplanted children in the largest registry still had a working graft five years later.

Most children with biliary atresia eventually need a liver transplant, some in the first year when the Kasai operation does not restore bile flow, others years later as scarring progresses. For a small child, a parent or close relative can give the left lateral segment of the liver, a piece small enough for the child and safe for the donor, whose liver regrows within months. Japan's national registry of 2,085 children transplanted this way reported graft survival of 90.4 percent at five years and 79.9 percent at twenty. Early referral, before the child is malnourished or critically ill, gives the best results. Send us your child's reports, scans and growth chart, with the blood group of a possible donor, and a pediatric transplant team will tell you whether a transplant is needed, when, and how to prepare.

90.4%
Graft survival at five years, 2,085 living donor transplants in Japan
85.8%
Ten-year patient survival after transplant for biliary atresia in the US
2 to 3 months
In Istanbul for the child, 4 to 6 weeks for the donor
Free
Transplant team's review of your child's file
Free consultation
Why biliary atresia leads to transplant

Biliary atresia scars the bile ducts outside and inside the liver. The Kasai operation restores bile flow in half of babies. Scarring inside the liver continues in many. Most children with the condition will eventually need a new liver. No other condition leads to as many liver transplants in children worldwide.

Some children need a transplant within the first year, when the Kasai fails and jaundice does not clear. Others live well with their own liver for years before cirrhosis, poor growth or bleeding make a transplant necessary in childhood or adolescence.

When to transplant

Indications

A 2017 review from the University of Colorado listed the reasons. They include a failed Kasai, malnutrition that does not respond to feeding support, repeated cholangitis, the complications of high pressure in the liver's veins such as bleeding varices and fluid in the abdomen, and lung complications called hepatopulmonary syndrome and portopulmonary hypertension. Each of these, alone or together, tips the balance toward transplant, and the team watches for them at every visit because they arrive gradually and are easy to accept as part of the illness until a growth chart or an ultrasound shows how far things have moved.

  1. Jaundice that has not cleared three months after the Kasai.
  2. Poor weight gain or growth despite special feeds and vitamins.
  3. Repeated infections of the bile channels.
  4. Bleeding from swollen veins in the gullet or stomach, or a swollen abdomen.
  5. Low oxygen levels or breathlessness from lung complications.
Timing

Timing needs balance. An early transplant exposes a child to a major operation and lifelong medicines they might not need for years. A late one, when the child is malnourished, infected or in intensive care, raises the risk of the operation. The Japanese registry found that early referral to a transplant center improved short-term results, and the American national data showed that children on life support at the time of transplant did worse. A living donor allows the operation to be planned at the right moment, without waiting for a deceased donor.

Child's assessment
Blood tests, ultrasound of the liver vessels, heart and lung checks, nutrition review, vaccinations brought up to date, and infection screening, over one to two weeks.
Donor's assessment
Run in parallel.
Approval
The transplant team and the hospital ethics committee review both assessments and the family relationship, and the date is set.

The living donor

The graft
For an infant or small child, the donor gives the left lateral segment, a fifth of the adult liver. Older children may need the larger left lobe.
The donor operation
Four to six hours. The donor's remaining liver regrows over the following weeks to near its full size.
The child's operation
The child's scarred liver and the old Kasai loop are removed or adapted, and the graft is joined to the child's vessels and bowel. Six to ten hours.
  • A healthy adult relative, in most cases a parent, with a compatible blood group.
  • Normal liver tests and imaging, no fatty liver and a suitable vessel pattern.
  • A free decision, confirmed in private with an independent physician.

Legal rules

Living donation, never purchase
International patients in Turkey receive a transplant from a living donor, in most cases a relative, approved by the hospital ethics committee, and families travel with official documents proving their relationship. Buying or selling an organ is a crime in Turkey and in the patient's home country, and no legitimate program will take part in it.

Donor safety

We conclude that LDLT for BA is a safe and effective treatment modality that does not compromise living donors.
Japanese registry of 2,085 transplants, American Journal of Transplantation, 2018

The first weeks

Weeks.

After surgery the child spends several days in pediatric intensive care, then two to four weeks on the ward. The donor stays in hospital for a week. Most families remain near the hospital for two to three months while anti-rejection medicines are adjusted and the first infections and rejection episodes, if they occur, are treated.

What are the results?

Very good, and improving.

The largest experience
Japan has performed living donor liver transplants for children since 1989, and its national registry of 2,085 children with biliary atresia is the largest with the longest follow-up in the world. Graft survival depended on the donor's body mass index, blood group compatibility, the type of graft, the child's age, the center's experience and the era of transplantation. Adolescents did worse in the long term than younger children.
Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in the pediatric population.
United States national data on 1,976 children, Liver Transplantation, 2005
Intensive care
Several days of close monitoring, blood flow checks by ultrasound and the first doses of anti-rejection medicine.
The ward
Two to four weeks of feeding, physiotherapy and medicine adjustment, with parents learning every dose and sign to watch.
Clinic visits
Blood tests twice a week at first, then weekly, until levels are stable and the family can fly home.
The optimum timing for LT is crucial for a successful outcome, and early referral to transplantation center can improve the short-term outcomes of LT for BA.
Japanese registry, American Journal of Transplantation, 2018

This table scrolls sideways on a narrow screen. Swipe or drag to see every column.

Survival after liver transplantation for biliary atresia
Measure Japan, living donor, 2,085 children United States, 1,976 children
Graft survival at 5 years 90.4 percent 76.2 percent
Graft survival at 10 years 84.6 percent 72.7 percent
Graft survival at 20 years 79.9 percent Not reported
Patient survival at 5 and 10 years Not reported 87.2 and 85.8 percent

What is life like after transplant?

Close to normal for most.

Children who are 10-year survivors of OLT have excellent graft function and, despite chronic extrahepatic morbidities, a self-reported high quality of life.
Toronto study of 10-year survivors, Transplantation, 2004

Most transplanted children go to school, play sport and grow up, and many reach adulthood with their first graft. Long-term care matters. In a North American study of 167 children alive ten years after transplant, all took daily anti-rejection medicine, 23 percent had impaired growth, 9 percent kidney problems and 5 percent a lymphoma linked to immunosuppression, and quality-of-life scores were lower than in healthy children. Adolescence brings the highest risk, when skipped medicines can cause late rejection, and teenagers need extra support in taking charge of their own care.

How long is the stay, and what decides the cost?

  • The child's reports since birth, including the Kasai operation note, liver tests, ultrasound and growth chart.
  • Blood group and age of possible donors in the family.
  • Documents proving the family relationship, for the ethics committee.

Families plan for months.

Plan two to three months in Istanbul for the child and four to six weeks for the donor. The child flies home once the graft is stable, medicine levels are steady and a pediatric hepatologist at home has agreed to take over. The team helps plan long flights, since a child on anti-rejection medicines must avoid infections.

Cost depends on the assessment of both child and donor, the two operations, intensive care, the hospital stay, medicines and the weeks of follow-up. Your own figure follows the free review of your child's file.

One coordinator supports the family through the whole stay, from the visa invitation letter sent ten days ahead to the transfer and an apartment near the hospital where the family can live for the months of recovery. A parent stays with the child throughout, and the donor parent is cared for on the adult ward. The team speaks seven languages, halal meals and a prayer room are available, and a female doctor is assigned on request when the schedule allows. Back home, the transplant team reviews blood results sent by your local doctors for life and stays reachable on the same WhatsApp number. Before you leave, the team writes a full plan for your local pediatrician and hepatologist, with the medicine doses and target levels, the schedule of blood tests and ultrasound, the vaccinations allowed and those to avoid, the signs of rejection and infection, and what to do if the child develops fever, diarrhea or vomiting that could upset medicine levels, so that care at home continues exactly where it stopped in Istanbul.

Liver transplant for biliary atresia FAQ

When does a child with biliary atresia need a liver transplant?
When the Kasai fails to clear jaundice, or later when cirrhosis causes poor growth, repeated cholangitis, bleeding or fluid in the abdomen, or lung complications.
Can a parent donate part of the liver to the child?
Yes. A parent with a compatible blood group can give the left lateral segment, a fifth of the liver, which regrows within months.
What is the success rate?
In Japan's registry of 2,085 living donor transplants, graft survival was 90.4 percent at five years and 79.9 percent at twenty.
Will my child need medicines for life?
Yes. Anti-rejection medicines are taken every day, and regular blood tests keep the doses right.
How long will we stay in Istanbul?
Two to three months for the child and four to six weeks for the donor parent.
Can the whole family come?
Yes. The international patients office arranges an apartment near the hospital for the months of the stay.

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. American Journal of Transplantation. 2018;18(3):659-668.
  2. Sundaram SS, Mack CL, Feldman AG, Sokol RJ. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transplantation. 2017;23(1):96-109.
  3. Barshes NR, Lee TC, Balkrishnan R, et al. Orthotopic liver transplantation for biliary atresia: the U.S. experience. Liver Transplantation. 2005;11(10):1193-1200.
  4. Ng VL, Alonso EM, Bucuvalas JC, et al. Health status of children alive 10 years after pediatric liver transplantation performed in the US and Canada: report of the studies of pediatric liver transplantation experience. Journal of Pediatrics. 2012;160(5):820-826.
  5. Avitzur Y, De Luca E, Cantos M, et al. Health status ten years after pediatric liver transplantation, looking beyond the graft. Transplantation. 2004;78(4):566-573.

Editor's note

Written by the Biruni Hospital medical editorial team. Reviewed by Prof. Dr. Hilmi APAK, Pediatric Hematology and Oncology.

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