What is biliary atresia?
Bile duct atresia is a disease where children are born.
In healthy children, there is a connection between the liver and the intestines. That connection is called the bile duct. By biliary go waste from the liver to the intestines. Children with biliary atresia have no bile duct or bile duct is closed by their inflammation. As a result, the waste products remain trapped in the liver. The liver gets damaged thereby.
An important feature of biliary atresia in newborn babies is jaundice. Babies with biliary atresia often do not grow well. Furthermore, they suffer from lethargy, fatigue and itching.
A child with biliary atresia need surgery as soon as possible. The more time passes before surgery, the worse the bile duct damage. Then, a liver transplant is needed.
Biliary atresia is a rare disease. In the Netherlands, an average of ten babies a year are born with this disease. The cause of biliary atresia is not known. It's certainly not a hereditary disease.
Symptoms of biliary atresia
When biliary atresia, the bile can no longer get rid of waste. Which remain in the liver. Thereby damaging the liver and the bile enters the bloodstream. This gives your child the following symptoms:
- jaundice;
- dark urine;
- light colored stool.
Later, the child may develop these symptoms:
- A greasy, sticky stools, often thinner than normal. This is because dietary fat is not digested properly.
- Itching. This is because there is too much bilirubin (yellow bile pigments) in the body and is in the skin.
- The biliary atresia important nutrients your child may not record properly. Thereby not grow well and is tired. Also, the blood does not clot properly.
- Your child may get liver cirrhosis.
- There esophageal varices can occur, especially in the lower part of the esophagus.
- There is too much fluid in the abdomen. Babies will receive a swollen abdomen.
Biliary atresia diagnosis
It is important that the doctor biliary atresia quickly detected. They can then take action quickly.
An important clue for biliary atresia is jaundice within a few days after birth. The jaundice is getting worse. Jaundice is seen in a healthy babies. Since biliary atresia is rare, the doctor will be there with jaundice so might not immediately think of. It is often quite difficult for doctors to diagnose biliary atresia.
To be able to diagnose with certainty, the doctor does different studies:
- She looks at how much bilirubin in the blood and in the urine of the child seat. Children with biliary atresia need a lot of bilirubin.
- In addition, they can view the bile ducts with an echo.
- Perhaps there is also an exploratory surgery in the abdomen required (laparoscopy).
Treatment of biliary atresia
Children with damaged bile duct to be operated on as soon as possible. The doctor removes the damaged biliary way. Then she makes a new connection between the liver and the intestine. This operation called the Kasai operation.
Most children with biliary atresia may after surgery Kasai a number of years with their own liver life. But even after successful surgery, there is a fairly high probability of problems. Namely the bile duct can easily ignite again. Many children should therefore regularly to the hospital for monitoring. If the bile duct re-ignites, the child needs surgery again. Eventually getting almost all children with biliary atresia, a liver.
Children who do not have bile duct, always get a liver transplant. Most children get the transplant between their fourth and eighth year of life. The physician typically uses a portion of an adult liver. Indeed, there are almost no children's livers for transplantation. The surgery takes about six hours.
The results of liver transplantation in children are often good. Five years after surgery survives eight out of ten children are transplanted. In children under two years the operation more difficult. This is because the organs are still very small. The chance of a successful transplant in young children, therefore unfortunately smaller.