The Kasai procedure, or hepatoportoenterostomy, is the first operation for babies with biliary atresia. It removes the blocked bile ducts and joins a loop of bowel to the liver so bile can drain again, with the best results before 46 days of age. At Biruni Hospital in Istanbul, our pediatric surgeons confirm the diagnosis, perform the operation, manage cholangitis and follow each child, with liver transplantation available on site if needed.
About This Department
Pediatric Surgery
In biliary atresia, the bile ducts of a newborn baby are blocked and scarred. The Kasai procedure opens a new path for bile from the liver into the bowel.
The Kasai procedure, also called hepatoportoenterostomy or Kasai portoenterostomy, is the first operation for babies with biliary atresia, a rare disease in which the bile ducts outside the liver close in the first weeks of life and bile stays trapped in the liver. The surgeon removes the scarred ducts and joins a loop of the baby's own small bowel to the underside of the liver, where tiny channels still carry bile. Age at surgery shapes the result. In 695 French infants, survival with their own liver fell step by step as age at operation rose, and the best results came before 46 days of age (Serinet and colleagues, Pediatrics, 2009). After care was concentrated in three expert centers in England and Wales, jaundice cleared in 55 percent of 424 babies, and 89 percent were alive at ten years, with or without a later liver transplant (Davenport and colleagues, Journal of Pediatric Surgery, 2011). This page explains warning signs, diagnosis, the operation, medicines, cholangitis, how success is measured, long-term life with the native liver, the link with liver transplantation, and how families from abroad arrange the Kasai procedure at Biruni Hospital in Istanbul.
46
Days of age, the threshold below which Kasai results were best in French national data
55
Percent of babies who cleared jaundice after Kasai in centralized national care
49
Percent of children still living with their own liver 20 years after Kasai in Japan
Free
Review of your baby's blood tests and scans by our pediatric surgery team
Bile is made in the liver and flows through ducts into the bowel, where it helps digest fat and absorb vitamins. In biliary atresia, inflammation destroys the ducts outside the liver in the first weeks after birth, bile backs up and the liver scars, and without treatment cirrhosis develops within the first year.
Biliary atresia is the most common reason children need a liver transplant worldwide. It is no one's fault, and it is not passed on from parents in a simple way.
Forms of biliary atresia
Form
What it means
Share of babies
Isolated
Only the bile ducts are affected
Around eight in ten
With splenic malformation
Extra or absent spleen, heart or vessel differences
Around one in ten
Cystic form
A cyst forms along the blocked duct
Under one in ten
It affects around one in 15,000 to 20,000 babies in Europe and more in East Asia.
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Why surgery works
At the base of the liver, a small area called the porta hepatis still holds microscopic bile channels. Joining bowel directly to this area lets bile drain again.
The procedure carries the name of Morio Kasai, the Japanese surgeon who described it in 1959.
Warning signs in a newborn
Jaundice that lasts beyond two weeks of age, or beyond three weeks in a premature baby, needs a blood test for conjugated bilirubin.
Ordinary newborn jaundice fades in the first two weeks and comes with yellow or green stools. In biliary atresia the yellow skin stays or returns, while stools turn pale. Babies feed and gain weight in the first weeks, so parents and doctors can miss the problem.
Signs that need checking the same week
Sign
What you see
Why it matters
Pale stools
Chalky white, pale yellow or clay colored
Bile is not reaching the bowel
Dark urine
Yellow or brown urine in the diaper
Bilirubin passes into the urine
Lasting jaundice
Yellow skin or eyes after two weeks
Possible blocked bile flow
Firm, large liver
Felt by the doctor
Scarring has begun
Could breastfeeding jaundice explain it?
Breast milk jaundice is common and harmless, but it raises unconjugated bilirubin and stools stay yellow. A simple blood test separating conjugated from unconjugated bilirubin tells the two apart. Any baby with pale stools needs that test, whatever the feeding.
Every week counts
Time is the enemy here.
In the French national study of 743 babies, the share still living with their own liver at two years was 57 percent and at fifteen years 28.5 percent, and at every point the babies operated at 30 days or younger did better than those operated at 31 to 45 days, who in turn did better than those operated later. The authors estimated that operating on every baby before 46 days of age would remove 5.7 percent of all liver transplants performed in French children each year. Taiwan proved the same point from the other side. After a stool color card was added to every baby's health booklet in 2002, the share of babies operated before 60 days rose from 49.4 to 65.7 percent, and five-year survival with a jaundice-free native liver rose from 27.3 to 64.3 percent (Lien and colleagues, Hepatology, 2011). The card costs almost nothing.
Pale stools at any age are a reason to call a doctor today.
Older babies still benefit. We operate beyond three months after a careful look at liver scarring on biopsy and scans, and we discuss early transplant openly when the liver is already cirrhotic.
Confirming the diagnosis
No single blood test proves biliary atresia. The diagnosis rests on several tests and is confirmed during surgery.
We aim to finish the workup within a few days of arrival, because each test that is delayed pushes the operation later.
1
Blood and ultrasound first
Conjugated bilirubin, liver enzymes, clotting and an ultrasound by a pediatric radiologist come first. A small or absent gallbladder and a thick band at the base of the liver, the triangular cord sign, point to biliary atresia.
Tests before the Kasai procedure
Test
What it shows
Time needed
Conjugated bilirubin and GGT
Blocked bile flow pattern
Same day
Abdominal ultrasound
Gallbladder, triangular cord sign, spleen
Same day
Liver biopsy
Duct proliferation, degree of scarring
Two to three days
Operative cholangiogram
Dye shows whether the ducts are open
During surgery
Ruling out other causes
Conditions that can look similar
Condition
How it is found
Treatment
Alpha-1 antitrypsin deficiency
Blood level and genetic type
Medical care
Alagille syndrome
Heart, spine and eye findings, genetics
Medical care
Choledochal cyst
Ultrasound shows a dilated duct
Different operation
Infection or metabolic disease
Targeted blood and urine tests
Specific treatment
How the Kasai operation works
The operation takes three to four hours under general anesthesia, and one parent stays with the baby until sleep.
1
Confirming and opening
The surgeon confirms the diagnosis with a dye study and a frozen section, then frees the liver to expose the porta hepatis.
The scarred bile duct remnant and gallbladder are removed.
The tissue at the porta hepatis is trimmed level with the liver capsule to open the microscopic channels.
A loop of small bowel around 40 centimeters long is brought up and stitched to the porta hepatis.
The other end of the loop is joined back into the bowel, a Roux-en-Y connection that keeps food away from the liver.
A small liver biopsy taken during the operation records the degree of scarring, which guides follow-up. No tubes or bags stay outside the body, and the wound heals as a single scar across the upper abdomen.
Open or laparoscopic surgery
Laparoscopic against open Kasai, 23 studies with 1,507 babies
The open approach gives the surgeon direct touch and a magnified view of the porta hepatis, the step on which bile flow depends. A 2015 meta-analysis found better two-year survival with the native liver after open surgery, and the 2023 meta-analysis of 23 studies found no advantage in bile drainage for the laparoscopic route (Okur and colleagues, Pediatric Surgery International, 2023). A smaller scar does not justify any trade in bile flow.
The open operation uses a transverse incision in the upper abdomen. It fades and grows with the child, and it does not complicate a later transplant beyond the adhesions any abdominal surgery leaves.
The first weeks after surgery
A typical recovery in hospital
Stage
Timing
What happens
Intensive or high dependency care
First one to two days
Pain relief, fluids, monitoring
First feeds
Day two to four
Breast milk or special formula
Antibiotics and medicines start
First week
Through a drip, then by mouth
Discharge
Day seven to ten
Feeding well, no fever
Pain is controlled with an epidural or a continuous infusion in the first days. Parents stay in the room, hold the baby and continue breastfeeding as soon as feeds restart.
The first sign of success is stool color. Yellow or green stools in the days after surgery mean bile has started to flow.
1
Feeding and growth
Babies with poor bile flow absorb fat poorly. A formula rich in medium chain triglycerides, extra calories and vitamins A, D, E and K protect growth while breastfeeding continues.
We check bilirubin before discharge and weekly in the first month.
Medicines after Kasai
Antibiotics by mouth for several months reduce the risk of cholangitis.
Ursodeoxycholic acid thins bile and helps it flow.
Fat soluble vitamins A, D, E and K, with doses adjusted to blood levels.
Special formula and, when needed, extra feeds through a fine nasal tube.
Steroids after Kasai, the START randomized trial in 140 infants
Outcome
Steroids
Placebo
Good bile drainage at six months
58.6 percent
48.6 percent, difference not significant
Survival with native liver at two years of age
58.7 percent
59.4 percent
Serious side effect within 30 days
37.2 percent
19.0 percent
High dose steroids did not improve bile drainage or survival in the trial and brought side effects earlier (Bezerra and colleagues, JAMA, 2014). We decide on steroids case by case and explain the reasons to parents.
Cholangitis and its warning signs
Cholangitis is an infection of the bile channels inside the liver. It occurs in a large share of babies in the first two years, mostly in the first months. Each episode harms bile flow.
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Signs to act on
Fever without another cause. Poor feeding or irritability. Stools turning pale again and returning jaundice.
Treatment is intravenous antibiotics in hospital for one to two weeks, started as soon as the infection is suspected, before culture results return. Repeated episodes lead to longer courses of preventive antibiotics.
What should we do if our baby has a fever at home?
Treat any fever in the first two years after Kasai as cholangitis until proven otherwise. Go to the nearest children's emergency department the same day, show the Kasai letter we give you, and ask the team to contact us. Do not wait to see whether the fever settles.
Measuring success
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Clearance of jaundice
Bilirubin returning to normal, below 20 micromoles per liter, within six months is the strongest early sign that the Kasai has worked.
What clearance of jaundice predicts
Result at six months
What it means
Follow-up
Bilirubin normal
Good bile drainage, the native liver is likely to last years
Clinic every three to six months
Bilirubin falling but raised
Partial drainage
Close review, early transplant assessment
Bilirubin rising
Kasai has not drained bile
Transplant evaluation
In the English national series, clearance was lower in babies with other anomalies, 43 against 57 percent, so we set expectations for each baby from the start.
2
Signs at home
Yellow stools, falling jaundice and steady weight gain are encouraging. Pale stools or deepening yellow need a call to us.
Liver stiffness measured by ultrasound elastography and blood counts track scarring and the spleen over time.
Clearance at three to six months is a good sign, though it does not guarantee a native liver for life.
Results and center experience
National results after the Kasai procedure
Series
Babies
Native liver survival
France, 1986 to 2002
695
37.9 percent at five years, 28.5 percent at fifteen
England and Wales, 1999 to 2009
424
46 percent at five years, 40 percent at ten
Japan, 1989 to 2015
3,160 registered
49 percent at twenty years
Overall survival, counting children who later received a transplant, reached 89 percent at ten years in England and Wales and 89 percent at twenty years in Japan (Nio, Pediatric Surgery International, 2017).
Taiwan before and after stool color card screening
Measure
Before screening
After screening
Kasai before 60 days of age
49.4 percent
65.7 percent
Jaundice free three months after surgery
34.8 percent
60.8 percent
Five-year overall survival
55.7 percent
89.3 percent
England improved its national results after limiting the Kasai procedure to three centers that also perform liver transplants.
Points to check with any Kasai team
Point
Why it matters
A strong answer
Kasai operations each year
Practice at the porta hepatis
A steady yearly number
Share of babies who clear jaundice
Measures surgical quality
Figures near national benchmarks
Transplant on site
Seamless care if Kasai fails
Same team, same hospital
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Our setup
Kasai surgery, pediatric hepatology, pediatric intensive care and living donor liver transplantation work as one team at Biruni Hospital.
Living with the native liver
Children whose jaundice clears go to nursery and school, play sport and grow normally. Scarring continues slowly in many of them. Follow-up therefore lasts for life, and each visit checks growth, blood counts, liver tests and an ultrasound of the liver and spleen.
1
Long-term problems we watch for
Portal hypertension with an enlarged spleen and varices in the esophagus, which can bleed. Low platelets, itching, poor growth, and rarely lung complications. Each has a treatment, and band ligation of varices is done through an endoscope.
When transplant is the next step
Kasai and transplant work as a sequence.
Transplant becomes the next step when bilirubin stays high after the Kasai, when the baby stops growing despite feeding support, or later in childhood when portal hypertension, bleeding or repeated cholangitis take over, and in Japan, England and France around half of children had received a new liver by adolescence. A successful Kasai that lets a child reach two, five or ten years with the native liver still counts as a win, because an older and larger child faces a safer transplant, with more donor options and better growth beforehand. At Biruni Hospital the same team that performs the Kasai follows the liver and moves to living donor liver transplantation, usually from a parent, when the time comes, and our pediatric liver transplant page describes the donor evaluation, the operation and life afterward.
No family starts over with strangers.
Primary transplant without a Kasai is an option for babies diagnosed late with established cirrhosis.
For families from abroad
1
Length of stay
Plan for three to four weeks in Istanbul. Tests take two to four days, the hospital stay after surgery is seven to ten days, and we check bilirubin and stool color in clinic before fitness to fly is confirmed.
2
What to send first
Recent bilirubin results, both conjugated and total, liver enzymes, ultrasound reports and photographs of the baby's stools next to a stool color card.
One coordinator from the international patients office handles arrangements from the first message through discharge and answers the same WhatsApp number after you return. 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.
After you return home, blood tests every two to four weeks for six months are done with your local pediatrician, and we review results and stool photographs by video and message.
A baby with pale stools should travel as soon as the review is done. We fast-track the visa letter and the first appointment for suspected biliary atresia.
The price depends on the baby's condition at arrival, the length of intensive care and any extra tests. We send a written quote after reviewing results, before you travel.
Compare quotes for the same scope of care, and check whether transplant is available on site if the Kasai does not drain bile.
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Follow-up
Video follow-up after discharge is included for the first year.
Costs to plan for after discharge
Item
Duration
Note
Antibiotics and ursodeoxycholic acid
Months
Bought locally
Vitamins and special formula
First year
Bought locally
Local blood tests
Every two to four weeks
Results sent to us
References
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. J Pediatr Surg. 2011;46(9):1689-1694.
Lien TH, Chang MH, Wu JF, et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology. 2011;53(1):202-208.
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.
Okur MH, Aydoğdu B, Azizoğlu M, et al. Laparoscopic versus open portoenterostomy for treatment of biliary atresia. A meta-analysis. Pediatr Surg Int. 2023;39(1):148.
Nio M. Japanese Biliary Atresia Registry. Pediatr Surg Int. 2017;33(12):1319-1325.
Editor's note
Written by the Biruni Hospital medical editorial team. Reviewed by Assistant Professor Ömer DOĞRU, Pediatric Hematology and Oncology.
