Kasai Procedure - Hepatoportoenterostomy

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.

What biliary atresia is

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.

It affects around one in 15,000 to 20,000 babies in Europe and more in East Asia.

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.

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.

Ruling out other causes

How the Kasai operation works

The operation takes three to four hours under general anesthesia, and one parent stays with the baby until sleep.

• 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.

Open or laparoscopic surgery

The first weeks after surgery

The first sign of success is stool color. Yellow or green stools in the days after surgery mean bile has started to flow.

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.

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

Measuring success

Clearance at three to six months is a good sign, though it does not guarantee a native liver for life.

Results and center experience

England improved its national results after limiting the Kasai procedure to three centers that also perform liver transplants.

Living with the native liver

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

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.

Cost

Compare quotes for the same scope of care, and check whether transplant is available on site if the Kasai does not drain bile.