Biliary Atresia in Babies: Kasai & Liver Transplant, Kolkata

Key points

Jaundice that lasts beyond two weeks of age (three weeks in premature babies) needs a blood test for conjugated (direct) bilirubin — not just sunlight or watching.
Pale, chalky or clay-coloured stools and dark yellow urine in a jaundiced baby are warning signs. Compare the stool with a stool colour card.
The Kasai portoenterostomy has the best chance of success when done early — ideally before about 60 days of life.
Even after a successful Kasai, many children eventually need a liver transplant, most often with part of a parent's liver.

What happens in biliary atresia

Bile is made in the liver and flows through the bile ducts into the intestine, where it gives stools their yellow or green colour. In biliary atresia, the ducts are progressively destroyed in the first weeks of life. The exact cause is not known; it is not caused by anything the parents did during pregnancy, and it is not usually inherited.

Trapped bile injures the liver, and scarring advances rapidly — within a few months, untreated babies develop cirrhosis. Without treatment, biliary atresia is fatal in early childhood. With timely surgery and, when needed, transplant, most children now survive into adulthood.

Warning signs parents should know

See a paediatrician within a day or two if your baby has jaundice with any of these:

pale, white, grey or clay-coloured stools; dark yellow urine that stains the nappy; jaundice lasting beyond two weeks of age (three weeks if born early); or bleeding from the umbilical stump, nose or into the skin, or unusual drowsiness. Ask specifically for a conjugated (direct) bilirubin test. Go to hospital immediately if the baby is bleeding, very sleepy or not feeding.

Most newborn jaundice is harmless and settles within two weeks; breastfed babies can stay mildly yellow a little longer with normal-coloured stools. The difference is the stool. Healthy stools are yellow, mustard or green. A baby with biliary atresia often has normal stools at first that become progressively paler. Babies usually look well and gain weight in the early weeks, which is why the condition is often missed.

Several countries give parents an infant stool colour card with photographs of normal and abnormal stool colours. Parents can photograph the nappy and show the doctor; any pale stool in a jaundiced baby should be reported the same week.

How biliary atresia is diagnosed

1
Blood tests
A raised conjugated (direct) bilirubin in a baby is always abnormal and needs urgent investigation. GGT, liver enzymes and clotting tests are done at the same time; vitamin K is given if clotting is prolonged.
2
Ultrasound
Performed after a few hours of fasting. An absent or abnormal gallbladder or a triangular fibrous area near the liver supports the diagnosis.
3
Further tests
Depending on the centre, a nuclear scan (HIDA), liver biopsy and tests for other causes of infant jaundice — infections, metabolic and genetic conditions.
4
Confirmation at surgery
The diagnosis is confirmed in the operating theatre by an X-ray dye study of the bile ducts (cholangiogram), usually followed at the same operation by the Kasai procedure.

These tests should be organised within days, not weeks. Every week of delay reduces the chance that the Kasai operation will work.

The Kasai portoenterostomy

The Kasai operation, performed by a paediatric surgical team, removes the scarred bile ducts and joins a loop of the baby's own intestine directly to the underside of the liver, where tiny remaining bile channels may still drain. It does not cure biliary atresia, but when it works, bile flows again, jaundice clears and liver damage slows — sometimes for many years.

Timing matters most. Results are clearly better when the operation is done early, ideally before about 60 days of age. It may still be worthwhile later in selected babies, but some babies presenting late with established cirrhosis are better served by going directly to transplant assessment.
Outcomes. In published series, jaundice clears after the Kasai in roughly half of babies. Those whose jaundice clears have a much better chance of long-term survival with their own liver.
After surgery. Babies need special feeds or supplements, fat-soluble vitamins (A, D, E and K), and close follow-up. Episodes of fever may signal cholangitis — infection of the bile channels — which needs prompt hospital antibiotics.

When a child with biliary atresia needs a liver transplant

Biliary atresia is the commonest reason for liver transplant in children worldwide. Most children with biliary atresia need a transplant at some point: early in infancy if the Kasai does not restore bile flow, or later in childhood or adolescence if cirrhosis and portal hypertension develop.

Signs a transplant assessment is needed	Why it matters
Jaundice not clearing within about three months of Kasai	The operation has not restored adequate bile flow
Poor weight gain or muscle loss despite feeding support	Malnutrition makes surgery riskier and affects development
Ascites, bleeding from varices, or a very large spleen	Signs of advanced cirrhosis and portal hypertension
Repeated episodes of cholangitis	Recurrent infection damages the liver further
Breathing problems linked to liver disease	Some lung complications of liver disease, such as hepatopulmonary syndrome, improve only after transplant

In India, most children receive a paediatric living donor liver transplant, in which a parent or another near relative donates a small portion of their liver — often the left lateral segment — which grows with the child. A difference in blood group does not always rule out a parent; see ABO-incompatible liver transplant. The donor's own operation and recovery are explained in liver donor surgery, and eligibility in who can be a liver donor.

Dr Bappaditya Har received paediatric liver transplant training at the National Center for Child Health and Development (NCCHD), Tokyo, and performs paediatric living donor liver transplants at Narayana RN Tagore Hospital, Mukundapur, Kolkata. Parents can send the baby's reports on WhatsApp (+91 70420 39420) for an early opinion, or call 080675 06860.

Frequently asked questions

Is jaundice at three weeks normal in a breastfed baby?

Mild breast-milk jaundice can last beyond two weeks in a healthy baby with normal yellow stools. But any baby still jaundiced after two weeks needs a conjugated (direct) bilirubin test to rule out biliary atresia and other liver diseases.

What colour are the stools in biliary atresia?

Pale yellow, cream, grey, white or clay-coloured. Normal baby stools are yellow, mustard or green. Show a photograph of the nappy to your doctor and compare it with a stool colour card.

Can biliary atresia be cured with medicine?

No. Biliary atresia needs surgery. Medicines support the baby after the Kasai operation, but they cannot open the blocked ducts.

What is the success rate of the Kasai operation?

Published results show jaundice clears in roughly half of babies, with better results when surgery is done early. Many children, including some whose Kasai worked, eventually need a liver transplant.

Can a mother or father donate part of their liver to a baby?

Yes. A healthy parent is the most common donor for a child. Only a small part of the liver is needed, and the donor's liver regrows. The donor has a full medical evaluation, and approval is required under India's transplant law.

How do children do after liver transplant for biliary atresia?

Large centres commonly report good long-term survival after paediatric liver transplant, and most children go on to school, grow normally and lead active lives, with lifelong medicines and follow-up.

Biliary Atresia in Babies: Early Signs, Kasai Operation and Liver Transplant in Kolkata