Biliary atresia blocks the bile ducts of a newborn, and the Kasai operation restores bile flow in about half of babies, best when done in the first weeks of life. This guide covers the warning signs of pale stools and lasting jaundice, diagnosis, the Kasai, results in expert centers, liver transplant, stool color screening and the stay.
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Pediatric Surgery
Biliary atresia blocks the bile ducts of a newborn and, untreated, destroys the liver within the first years. The Kasai operation restores bile flow in half of babies. It works best in the first weeks of life.
Biliary atresia is rare, one in every five to six thousand births, and it is the commonest reason children need a liver transplant. A baby with yellow skin beyond the first two weeks of life, pale stools and dark urine needs tests within days. The Kasai portoenterostomy joins a loop of bowel directly to the liver so bile can drain. In a French national study of 743 children, the earlier the operation, the longer children lived with their own liver, and in England and Wales, after care was concentrated in three expert centers, nine in ten children were alive at ten years, with or without a transplant. Send us your baby's blood tests, ultrasound and stool photographs, and a pediatric surgeon will tell you what is needed and how quickly.
55%
Babies whose jaundice cleared after Kasai in England and Wales
89%
Children alive at ten years, with or without a transplant
2 to 3 weeks
In Istanbul after the Kasai operation
Free
Pediatric surgeon's same-day review of your baby's tests
Many newborns turn yellow in the first week, and almost all of them are healthy. The difference lies in timing, stool color and the type of bilirubin.
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Ordinary newborn jaundice against signs that need tests
Feature
Ordinary jaundice
Possible biliary atresia
Timing
Starts in the first days, fades by two weeks
Persists beyond two weeks, or three in breastfed babies
Stool color
Yellow or green
Pale yellow, clay, white or gray
Urine
Pale
Dark yellow, staining the diaper
Blood test
Mostly unconjugated bilirubin
Raised conjugated or direct bilirubin
Pale stools and jaundice after two weeks
A baby who is still yellow after two weeks of age, with pale stools or dark urine, needs a blood test for conjugated bilirubin within days, and if it is raised, urgent referral to a pediatric liver team. Waiting to see whether it fades costs time that the liver cannot recover.
Good outcomes for infants depend on early referral and timely Kasai portoenterostomy, and thus a high index of suspicion is needed for investigation of infants with persistent jaundice.
Review of biliary atresia, The Lancet, 2009
The Kasai operation
1
Exploring the bile ducts
Through an incision in the upper abdomen, the surgeon confirms the diagnosis, often with a dye study, and removes the scarred bile ducts outside the liver.
2
Joining bowel to liver
A loop of the small intestine, called a Roux loop, is brought up and stitched to the cut surface at the base of the liver, where tiny bile channels remain open.
3
Waiting for bile
Over the following weeks, bile begins to drain into the bowel. Stools turn yellow again and jaundice fades in the babies in whom the operation works.
Blood tests for conjugated bilirubin, liver enzymes and clotting, with vitamin K given at once if clotting is prolonged, since these babies bleed easily.
Fasting ultrasound.
A liver scan or biopsy when the diagnosis is still unclear.
Confirmation at surgery with a cholangiogram, an X-ray of the bile ducts.
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Survival with the native liver after Kasai, France, 695 children
Years after surgery
Living with their own liver
2 years
57.1 percent
5 years
37.9 percent
10 years
32.4 percent
15 years
28.5 percent
Increased age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence.
French national study of 743 children, Pediatrics, 2009
Why every week counts
In the French study, survival with the native liver fell steadily with each step in age at surgery, at 30 days or less, 31 to 45, 46 to 60, 61 to 75 and 76 to 90 days, and the effect lasted into adolescence. Earlier surgery would save livers. Operating on every baby before 46 days, the authors estimated, would spare 5.7 percent of French pediatric liver transplants each year.
Experienced centers do better
Biliary atresia is rare, and a surgeon who performs a Kasai once a year cannot match a team that performs dozens. Since 1999, England and Wales have sent every case to three centers that offer both the Kasai and liver transplantation, and their national results became a benchmark.
Results in England and Wales
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Biliary atresia in England and Wales, 1999 to 2009, 443 infants
Measure
Result
Median age at Kasai
54 days
Jaundice cleared after Kasai
55 percent
Living with own liver at 5 and 10 years
46 and 40 percent
Alive at 5 and 10 years, with or without transplant
90 and 89 percent
National outcome measures in BA appear better than those from previously published series from comparable countries and may be attributed to centralization of surgical and medical resources.
National results, Journal of Pediatric Surgery, 2011
Steroids after surgery
The START trial
Steroids were once given routinely after the Kasai, in the hope of reducing inflammation in the liver. The American START trial randomized 140 infants to high-dose steroids or placebo. Bile drainage at six months was 58.6 against 48.6 percent, a difference that did not reach significance. Survival with the native liver at two years was the same, 58.7 against 59.4 percent. Serious side effects appeared earlier with steroids. Many centers have since stopped using them routinely.
Cholangitis and follow-up
Infection of the bile channels, called cholangitis, is the commonest problem in the first year after a Kasai. Fever, irritability, pale stools or returning jaundice signal it. Intravenous antibiotics start at once, since each episode can damage the liver. Most centers give preventive antibiotics for months after surgery, and vitamin supplements, since fat-soluble vitamins are poorly absorbed while bile flow is reduced.
When is a liver transplant needed?
For many children, eventually.
1
Kasai fails to clear jaundice
When jaundice persists three months after surgery, the liver is likely to fail, and assessment for transplant begins.
2
Later liver failure
Some children who clear jaundice develop cirrhosis, poor growth, bleeding from swollen veins or fluid in the abdomen years later, and need a transplant then.
3
Living donor transplant
A parent or relative can donate part of the liver. In experienced centers, children transplanted for biliary atresia do well, and most reach adulthood.
How does stool color screening help?
A card in the baby's health booklet shows normal and abnormal stool colors.
Parents compare.
Any pale color is reported, and the baby is tested within days.
Taiwan's experience
Taiwan added a stool color card to every newborn's health booklet in 2004. In its second year, the cards detected 97.1 percent of babies with biliary atresia before 60 days of age, the share having a Kasai before 60 days rose from 60 to 74.3 percent, and the rate of clearing jaundice three months after surgery reached 59.5 percent, against 37.0 percent before screening. Parents anywhere can use the same idea by looking at their baby's stool every day in the first weeks.
What is recovery like after the Kasai?
The first months matter most.
Babies spend the first days in a surgical ward or intensive care, with a drip and a tube to rest the bowel. Feeding starts within days. Parents learn to watch stool color, temperature and feeding, give medicines and recognize cholangitis before discharge, and a nurse specialist goes through each step with them until they feel confident.
Feeding resumes within a few days, often with special milk that is easier to absorb.
Preventive antibiotics, vitamins A, D, E and K and medicine to help bile flow.
Blood tests every week or two at first, then monthly, with weight and length checked each time, because good growth is one of the clearest signs that bile is reaching the bowel and fat is being absorbed.
Daily stool color checks.
How long is the stay, and what decides the cost?
Planning the visit
Speed matters more than planning for this condition. The team reviews tests on the same day they arrive and, if biliary atresia is likely, arranges admission as soon as the family can travel. The baby stays in hospital for ten days to two weeks after the operation, and the family remains in Istanbul for two to three weeks in all, until the baby feeds well, has no fever and the first blood tests show the trend. A baby is fit to fly once feeding is established, the wound has healed and the surgeon has given a letter for the airline and a plan for antibiotics, vitamins and blood tests at home.
Cost depends on the tests, the operation, the hospital days and any intensive care. The coordinator discusses it with the family once the baby's plan is clear.
One coordinator handles every practical step, the visa invitation letter sent the same day in urgent cases, the transfer and a family hotel near the hospital. A parent stays with the baby throughout, breastfeeding mothers are supported, and the team works in seven languages, with halal meals and a prayer room available. Back home, the coordinator and the pediatric liver team stay reachable on WhatsApp, review blood results and stool photographs, and work with your local pediatrician on preventive antibiotics, vitamins and the signs of cholangitis, and if the liver later begins to fail, the same team plans the transplant assessment early, so that a living donor in the family can be evaluated well before the child becomes seriously ill.
Biliary atresia FAQ
What are the first signs of biliary atresia?
Jaundice lasting beyond two weeks of age, pale or white stools and dark urine. The blood test shows raised conjugated bilirubin.
When should the Kasai operation be done?
As early as possible. Results in France fell with every two weeks of delay, and outcomes are best when surgery is done within the first 45 to 60 days.
Does the Kasai operation cure biliary atresia?
It restores bile flow in about half of babies and lets many live with their own liver into adolescence. Others will need a liver transplant later.
Do steroids help after the Kasai?
The START trial of 140 infants found no significant improvement in bile drainage or survival with steroids, and earlier side effects.
How long will we stay in Istanbul?
Two to three weeks in all, with ten days to two weeks in hospital after the operation.
Can a parent stay with the baby?
Yes. A parent stays with the baby throughout, and breastfeeding mothers are supported.
References
Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet. 2009;374(9702):1704-1713.
Serinet MO, Wildhaber BE, Broué P, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics. 2009;123(5):1280-1286.
Davenport M, Ong E, Sharif K, et al. Biliary atresia in England and Wales: results of centralization and new benchmark. Journal of Pediatric Surgery. 2011;46(9):1689-1694.
Bezerra JA, Spino C, Magee JC, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014;311(17):1750-1759.
Hsiao CH, Chang MH, Chen HL, et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology. 2008;47(4):1233-1240.
Editor's note
Written by the Biruni Hospital medical editorial team. Reviewed by Assoc. Prof. Dr. Aysel TAKTAK, Pediatric Nephrology.
