Biliary atresia

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Overview

Biliary atresia is a rare but serious liver disease that affects newborn infants. It is found in about one in 10,000 children and is most commonly manifested by girls than boys, especially in Asian and African Americans than Caucasian ones.

Biliary atresia cause is unknown and treatments are only partially successful. Biliary atresia is the most common reason for liver transplantation in children in many parts of the world.


Contents

1. Overview
2. Liver damage
3. Symptoms of biliary atresia
4. Causes of biliary atresia
5. Diagnosis of biliary atresia
6. Treatment
7. What happens after surgery?


Liver damage

Liver damage caused by biliary atresia is caused by injury and bile duct obstruction which are responsible for draining bile from the liver. Bile is produced by the liver and travels through bile ducts and intestines where it helps digest food, fats and cholesterol.

Because bile ducts dysfunction, bile remains in the liver. When bile builds up, the liver can suffer damage, may occur scarring and loss of liver tissue.

Eventually the liver will not be able to function properly and cirrhosis occurs. If the liver fails, liver transplantation will be required. Biliary atresia can cause liver failure and need for liver transplantation in the first 1-2 years of life.


Symptoms of biliary atresia

The first sign of biliary atresia is jaundice that causes yellow skin and sclera of the eye. Jaundice is caused by liver dysfunction, not impossible to eliminate bilirubin, a yellow pigment in the blood. Usually bilirubin is taken up by the liver and released into the bile.

Due to the bile ducts blockage results bilirubin and other elements that accumulate in the blood. Jaundice can be difficult to detect both parents and physicians. Many healthy newborns have jaundice in the first weeks of life due to liver immaturity. This type of jaundice is normal and disappears within one month of life, while in biliary atresia case the jaundice is prolonged.

Infants who experience jaundice after the first weeks of life should be investigated by a doctor to remove a possible liver problem. Other signs of jaundice are a darkening in color of urine and a lightening in color of feces.

Urine darkens as a result of high levels of bilirubin in the blood flow that are discharged into urine, while stool lightens as they never get high amounts of bilirubin in the intestines.

White, gray or another light color of the feces, after the age of two weeks, is probably the surest sign that there is a liver problem and requires a visit to the doctor.


Causes of biliary atresia

Biliary atresia cause is not known. The two types of biliary atresia existing seem to be the fetal form, which occurs during fetal life, is present during delivery and perinatal in a form that is more typical and do not become apparent until the second – fourth week of life.

Some children, especially those with biliary atresia fetal form, often have congenital heart defects, spleen or intestines disorders.

Biliary atresia is not an inherited disease. Cases of biliary atresia are not seen in the same family, and in the identical tween’s case just one of them will have the disease. Biliary atresia is most likely caused by an event that took place during fetal life or during birth.

Possibility of triggering the disorder may include one or a combination of the following factors:
- Infection by a virus or bacterium
- A problem with the immune system
- An abnormal component of the immune system
- A defect in the liver and bile ducts development.

Research related to biliary atresia’s cause are very important. Progress in biliary atresia management and prevention of this condition is caused by a better understanding of its causes.


Diagnosis of biliary atresia

Worsening jaundice in the first month of life means that a problem is present in the liver. Specific diagnosis of biliary atresia requires blood tests and X-ray radiography and sometimes liver biopsy.

If biliary atresia is suspected, the newborn will need a special investigation to a pediatric gastroenterologist doctor who is an expert in children’s liver disease or a pediatric surgeon who specializes in surgery of the liver and biliary tract.

A pediatrician will examine your child to determine his medical history. Several diagnostic procedures will be needed. These could include:

1. Blood Tests

- Liver enzymes – increased levels of liver enzymes can alert physicians related to liver injury or damage because of liver enzymes in the blood flowing in these circumstances.

- Bilirubin – is produced by the liver and excreted in bile. Elevated levels of bilirubin often indicate an obstruction of bile flow or an abnormality in the processing of bile by the liver.

- Albumin and total protein – low levels of proteins produced by the liver are associated with chronic liver disease.

2. Coagulation tests such as prothrombin time and partial activated thromboplastin time – they measure type required for blood clotting. For blood clotting are necessary vitamin K and proteins that are produced by the liver. Liver cell damage and bile flow obstruction can interfere with normal blood clotting.

3. Viral tests, including tests for hepatitis and HIV – tests for viruses in blood flow can help minimize the causes that may be caused by liver problems.

4. Blood cultures – will do tests to detect if there is bacterial infection in the blood, which can affect the liver, indicated for the detection of biliary atresia.

5. Imaging tests

- Abdominal ultrasound is a diagnostic imaging technique which uses high-frequency ultrasound and a computer that provides images of blood vessels, tissues and organs. Ultrasounds are used to view the liver, gallbladder and bile ducts

- Gall scanning – A radioactive isotope (technetium) is injected into a vein of the child. The liver and intestine are scanned by a nuclear medicine camera. If the isotope passes through the liver into the intestine, bile ducts are open and the child is suffering from biliary atresia

- Liver biopsy – tests to help determine a final diagnosis is liver biopsy. A tissue sample is taken from the baby’s liver and possible abnormalities are examined. In this way biliary atresia is differentiated from other liver problems.


Treatment

- Surgery – If biliary atresia is the cause of jaundice in newborns, the next step is surgery. During surgery, the bile ducts are examined and the diagnosis confirmed.

For this procedure, the child is sedated. While the child is asleep, the surgeon makes an incision in the abdomen to directly examine the bile ducts and liver. When the surgeon confirms that biliary atresia is the problem, a procedure called Kasai will be made.

Kasai procedure – When confirmed the diagnosis of biliary atresia, the surgeon will do surgery called the Kasai procedure, named after the Japanese surgeon who was the promoter of this operation.

During the Kasai procedure, the bile ducts are removed and an intestinal loop is used to replace the bile ducts and liver drainage. As a result, the flow of bile from the small bile ducts is directed to the intestine, and will completely pass through large bile ducts.

Through Kasai procedure will restore normal bile flow and will correct many of the problems triggered by biliary atresia. Surgery is not a treatment for the condition, although the result can be great.

Without this surgery, it is unlikely that a child diagnosed with biliary atresia live longer than two years. With surgery the best results are obtained if the baby is under 90 days. The good results obtained after surgery make very important the early diagnosis of biliary atresia before a child suffered permanent liver damage.

A child with biliary atresia who underwent a successful Kasai surgery will have a good health and have a normal life without major liver problems or jaundice. Unfortunately, however Kasai procedure is not always successful.

If bile flow is restored, the child will suffer serious illness and even liver cirrhosis and transplantation will be necessary in the first 1-2 years of life. In addition, even when the Kasai operation is initially successful, can’t completely restore liver function and there will not be a normal development. Child with biliary atresia can slowly develop cirrhosis and complications related to it and will require a liver transplant later in childhood.

Although the Kasai procedure has contributed to progress in the management and control of biliary atresia, operational improvements and clinical management to obtain better results in children suffering from biliary atresia are needed.

- Liver transplant – If the Kasai procedure fails, the child will need a liver transplant within the first 1-2 years of life. Children with biliary atresia in the fetal form rather need liver transplantation and usually much earlier than children who have typical perinatal form of biliary atresia.

Level of impairment of biliary ducts and their degree of deterioration are factors that determine the emergency of liver transplantation. Liver transplantation is a beneficial treatment in biliary atresia’s case. This has helped increase survival rates in recent years.

Children with biliary atresia are now living into adulthood, even having their own children. Because biliary atresia is not an inherited disease, in survivor children of this disease there isn’t any risk of developing this condition. In the past it was used only liver transplantation from early age donors for transplantation to the children diagnosed with biliary atresia.

Recently, advanced methods have been developed to use part of a child’s liver for transplantation in a child with biliary atresia. Besides the development following surgery, it is possible to use part of adult living liver donor for the transplant to be done.

Thus parents or relatives of children with biliary atresia can donate a portion of their liver for transplant. Because healthy liver tissue grows rapidly, if a child receives a portion of liver from a living donor, both the child and the donor’s liver will regenerate completely over time.


What happens after surgery?

Both before and after the Kasai procedure, the children will follow a specific diet with the right combination of nutrients and vitamins in a form that does not need bile to be absorbed.

Inadequate nutrition can lead to developmental problems, so doctors can monitor a child’s nutritional intake closely. Some children may accumulate fluid in the abdomen after the Kasai procedure, which causes inflammation of the child’s belly. This condition is called ascites and usually lasts a few weeks.

If ascites persists for more than 6 weeks, it may mean that cirrhosis is present and liver transplantation is required.

Quite common after the Kasai procedure is the infection of the biliary left in the liver, called cholangitis. Doctors may prescribe antibiotics to prevent cholangitis or to treat an infection that might install. Children with biliary atresia may continue to have problems with their liver after Kasai procedure.

Even a successful surgery and return to normal bile flow may cause injury and damage to small bile ducts within the liver, cirrhosis and scarring.

Cirrhosis can trigger skin problems related to nutrition, bruising, bleeding and itching. Itching is caused by the buildup of bile in the blood and nerve ends irritation on the skin. Doctors can prescribe medicines for itching, including antihistamines to reduce itching skin.

After liver transplantation, the doctor will recommend medications that are designed to help the immune system to not reject the new liver. Doctors can still prescribe special diets, vitamins, blood pressure medications and antibiotics.

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