Autoimmune and Rare Diseases,  First Aid and Emergency

Bile Duct Obstruction

Biliary atresia is a rare but serious condition that affects the liver and bile duct system in infants. The condition shows symptoms within a few weeks after birth, and if not recognized in time, it can be fatal. During the disease, the bile ducts become damaged, obstructing the normal flow of bile and potentially leading to liver cirrhosis in the long term. Early diagnosis and appropriate treatment are crucial for saving the child’s life.

Biliary atresia can be caused by several factors, including viral infections and genetic predisposition. However, the causes of the disease are not always clear, making diagnosis and treatment challenging. Parents and doctors need to be aware of the symptoms of the disease to intervene in a timely manner. The aim of this article is to provide a detailed overview of the diagnosis, symptoms, and possible treatment options for biliary atresia.

Symptoms of Biliary Atresia

The most characteristic symptom of biliary atresia is jaundice, which can persist more than two weeks after the infant’s birth. The child may appear healthy at birth, but the prolonged presence of jaundice can raise suspicion and necessitate thorough examination. In addition, biliary atresia may be accompanied by acholia, or pale stools, dark urine in the diaper, and skin paleness. The cholestasis resulting from the disease can be confused with other conditions, such as cystic fibrosis, making diagnosis time-consuming.

Timely recognition of biliary atresia is vital. For any neonatal jaundice lasting longer than two weeks, it is important to rule out the possibility of biliary atresia. Therefore, it is essential for parents and doctors to be aware of the symptoms and seek medical help immediately if the infant exhibits the aforementioned signs.

Diagnostic Options

The diagnosis of biliary atresia involves several steps. The first important step is a blood test, during which doctors examine the levels of liver enzymes and signs of obstruction. Elevated enzyme levels often indicate the presence of the disease. Additionally, hepatobiliary scintigraphy, an imaging procedure, can help confirm the diagnosis as it may show reduced radioisotope uptake by the bile.

During an ultrasound examination, doctors evaluate the condition of the gallbladder and the structures surrounding the liver. If a shrunken gallbladder or abnormal vascular placement is found during the examination, it may raise suspicion of biliary atresia. Furthermore, a cholangiography may be performed, where a dye is injected into the bile ducts to make them visible.

Finally, a liver biopsy may also be performed, where a tissue sample is taken from the liver to obtain further information about the state of the disease. Establishing the diagnosis is a complex process, and multiple tests may be necessary to achieve accurate results.

How is Biliary Atresia Treated?

The treatment of biliary atresia focuses on ensuring the earliest possible bile flow. The Kasai procedure performed in infancy is the most common first step in treating the disease. The goal of the surgery is to create a new connection between the liver and the small intestine during the healing procedure, allowing for normal bile flow. It is important to note that the Kasai procedure can only be applied if the intrahepatic bile ducts are intact; otherwise, direct liver transplantation is necessary.

During the Kasai procedure, surgeons attach a Y-shaped segment made from the small intestine to the liver hilum to ensure bile drainage. A special valve is created during the procedure to reduce the risk of cholangitis caused by bacteria from the small intestine. It is crucial to continuously monitor the child’s condition after the surgery, as complications such as cholangitis or portal hypertension may still occur despite a successful intervention.

Although medication currently does not improve the outcome of the disease, research is ongoing for new agents that may help treat biliary atresia by acting at the receptor level. The medical community is continually working to provide the best treatment options for patients and improve the quality of life for children living with biliary atresia.