Synonym
Atresia of the bile ducts, English: biliary atresia, BA classification according to ICD-10 ? Q44. 2General Biliary atresia is a congenital malformation of the bile ducts.
The bile ducts are closed (occlusion = atresia). The disease is found exclusively in newborns and is a common indication for a liver transplant in infancy. Causes As with many human diseases, the aetiology of biliary atresia is not fully understood.
However, there are genetic and inflammatory infectious components that most likely lead to such a dislocation of the bile ducts. There is most likely a connection between this malformation and a viral infection during pregnancy with the Epstein-Barr, cytomegalovirus, respiratory syncitial and human papilloma viruses. Furthermore, genetic abnormalities are found in the affected children.
If the bile ducts are viewed as fine tissues, sclerosis (obstruction with connective tissue) and inflammatory changes can be seen. Girls are more frequently affected by bile duct atresia than boys. The disease occurs with a prevalence of 1:20,000 and is more frequent in Asia and the Pacific region.
A distinction is made between a syndromal and a non-syndromal form. In the non-syndromal form of the disease, only the bile ducts are obstructed, whereas the syndromal form is associated with other malformations such as heart defects. Symptoms In newborns with a biliary atresia, there is a prolonged jaundice (in contrast to the physiological newborn jaundice), a discoloration of the stool (acholic stool) and a brown coloration of the urine.
Furthermore, the liver may be enlarged, which is called hepatomegaly. The general condition of the children then deteriorates over time, so that weight loss can be observed. The result is an enlargement of the spleen, which can be extremely painful, and ascites in the abdomen.
Digestive disorders and thus pain in the intestinal area can result from the lack of bile acids. There is also an increased tendency to bleed, since vitamin K absorption is disturbed due to the lack of bile acids. Diagnosis An ultrasound examination may reveal anatomical abnormalities such as a reduced gallbladder or deformities of the liver.
If the findings are uncertain, cholangiography is an option, in which the bile ducts are visualized using a contrast medium. It is also possible to perform a liver biopsy. Treatment Biliary atresia requires treatment in any case, since untreated liver cirrhosis develops, which leads to early death. Infants are usually treated surgically. Liver transplantation is usually necessary until the second year of life.
