Biliary atresia is a condition in which there is an incomplete development of the bile ducts. However, it is a rare disease, but the patient has this disease bile flow from the liver to the gallbladder is blocked. It leads to the situation in which the bile is trapped inside the liver, and cause damage as well as scarring of the liver cells (cirrhosis), and finally, liver failure. In cases where the liver failure occurs then a liver transplant is required and the only option left. Though this disease is the most common reason for liver transplantation in children as children age 1-2 may need liver transplantation for biliary atresia, also, children of 4 to 5 years with the disease will require liver transplant before age 20. In other children above this age group, there is a less severe liver disease but still need medical management and checkups.
Causes of Biliary atresia
The main cause of Biliary atresia is still unknown, and in some children, it may occur because of the incomplete formation of their bile ducts during a pregnancy. In many other children, the bile ducts may be damaged by the immune system as a response to viral infection at birth. However, it is a rare disorder, but girls are more prone to it than boys. It can be in one of the twins or only one child out of two of the same family.there are two types of biliary atresia
- Fetal form– This type of biliary atresia develops during fetal life and is also present at the time of both.
- Perinatal form– It is the most common form of biliary atresia, which cannot be diagnosed until the infant is in the 2nd and 4rth week.
Symptoms of Biliary atresia
Children or infants with Biliary atresia are normal as other babies at birth. The symptoms of the condition typically appear within two weeks to two months after birth. Some common symptoms are:
- Dark urine– If a baby has dark urine, which is caused by a buildup of bilirubin in the blood.
- Jaundice – if the baby has a yellow color of skin and eyes as it is the first stage of Biliary atresia and which is common in newborns.
- Acholic stools– it is a clay coloured stool in babies due to no bile, also abdomen, in this case, swelled up because of an enlarged liver.
Biliary atresia diagnose
The condition has common symptoms that is jaundice, worsening jaundice in the first month after the child’s birth means a liver problem is present. However, jaundice may also be present with other disorders, too; thus, liver specialists will perform several tests to determine correct diagnoses.
- X rays
- Blood tests
- Abdominal ultrasound
- Liver biopsy
- Diagnostic surgery, which is done after the confirmation of condition to know that is there any injured piece of bile ducts going from the liver.
- Operative cholangiogram, which is performed at the time of surgery to confirm the diagnosis of the condition.
Treatment of Biliary atresia
When the Biliary atresia is diagnosed, and it is confirmed that the bile ducts are not open. Then for infants in this condition have to experience the Kasai procedure as it is the only treatment to this also known as hepatoportoenterostomy as there is medication to treat it. If the doctor confirms that there is not enough bile flow with the Kasai procedure also than the liver transport; the plant is the final and the only option left. Experts perform a liver transplant in Turkey, and it is the surgery that is done to remove the damaged liver and to replace it with a new healthy liver from a donor.
If the Kasai procedure is performed by an experienced surgeon than its success rates are up to 85 percent of the patients. The successful procedure means that bile drains from the liver, and the jaundice level decreases. if this process i.e. Kasai procedure does not work, then Liver Transplant is the only option left. Liver transplant in Turkey is a highly successful treatment for biliary atresia and has excellent success rates, and it has increased dramatically in recent years. Because of its success children with biliary atresia are now surviving well.