Biliary Atresia - Causes, Symptoms, and Treatment

Introduction:

Biliary atresia is a rare gastrointestinal disease characterized by either absence or destruction of a portion of the entire bile duct located outside the liver (extrahepatic). Due to this, there is an abnormal accumulation of bile in the liver, causing symptoms like jaundice in infants, fibrosis of the liver and heart defects, kidney malformations, and intestinal disorders. The cure for the disease is only surgery after a timely diagnosis. Special monitoring of nutritional needs and diet should be done for children suffering from biliary atresia. It is one of the common causes of end-stage liver disease and liver transplantation.

What Is a Bile Duct?

It is a tube-like structure that carries bile from the liver to the gallbladder, where it is stored. Further, this bile reaches the duodenum (the first part of the small intestine) for use in the digestion of food. The bile duct can be intrahepatic (inside the liver) and extrahepatic (outside the liver).

What Are the Causes of Biliary Atresia?

The exact cause of the disease is not established, but several possibilities can lead to biliary atresia. Factors such as immunological, genetic, infectious, or toxic can contribute to the disorder's development and progression. Some of the phenomena which can lead to disease are:

• Several viruses are studied, which can be possible causative agents. These viruses include reovirus type 3, rotavirus, and cytomegalovirus.

• In the majority of cases, bile ducts may be normal at the time of birth, but contributing factors such as genetic factors, immunological, or toxic factors can initiate epithelial degeneration leading to rapid production of fibrous tissue, which causes sclerosis and, finally, obstruction of bile ducts.

• A few cases can be seen due to developmental defects of the liver and biliary tree. These defects can be during pregnancy.

What Signs and Symptoms Do Biliary Atresia Show?

Infants at two to six weeks of age develop symptoms such as:

Jaundice:

• Yellowish discoloration of the mucous membrane, sclera of the eyes, and skin.

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• Swollen stomach or hepatomegaly (abnormal enlargement of the liver).

• Dark urine and abnormal pale stool.

• Poor weight gain.

Portal Hypertension:

• Elevation in blood pressure in the veins that carry blood from the intestine to the liver.

• Intrahepatic bile ducts (bile ducts inside the liver) are also involved.

• If left untreated, it may result in permanent fibrosis of the liver (cirrhosis) and eventually hepatic failure.

Some children suffering from biliary atresia may develop congenital abnormalities like:

• Situs Inversus: Rare condition with abnormal positioning of the heart.

• Ventricular Septal Defects: Abnormal holes formed in the ventricles of the heart.

• Polysplenia: More than one spleen is present.

• Asplenia: Absence of spleen from the body.

How Does Biliary Atresia Affect Diet?

Since children affected with biliary atresia have low bile flow, the following digestive effects take place:

• Loss of appetite - The absence of feeling of hungriness.

• A very fast metabolism leads to needing more calories and energy.

• Difficulty in digesting fat-rich foods.

• Fat-soluble vitamins are not absorbed properly.

• Decreased concentration of protein, vitamins, and minerals in the body.

Which Disorders Are Similar to Biliary Atresia?

• Primary Sclerosing Cholangitis: A rare progressive disease in which inflammation, abnormal formation, and thickening of fibrous tissue within the bile duct and liver take place.

• Neonatal Cholestasis: A group of disorders along with jaundice as one. There is a reduced flow of bile from the liver.

How to Diagnose Biliary Atresia?

Direct examination of bile ducts and tissue is necessary. The procedures performed for this are:

• Laparotomy: Small incision made in the abdomen to examine internal organs.

• Intraoperative Cholangiogram: A special dye is injected into the gallbladder, and the filling of bile ducts is outlined using multiple X-rays. The movement or obstruction of dye through bile ducts and into the small intestine is traced.

• Liver Biopsy: Tissue from the liver is excised surgically to investigate any malignancies.

• Blood Tests: The presence of viral agents, the concentration of bilirubin, and elevated levels of liver enzymes can be monitored.

• Ultrasound: It can help to check for the absence of organs like the gallbladder and spleen.

What Are Treatment Options for Biliary Atresia?

Surgical intervention is the only treatment option for biliary atresia. Along with this, attention to nutritional needs and diet is also essential for children.

• Kasai Hepatoportoenterostomy: Surgical removal of obstruction from bile ducts to allow bile to flow to the intestine. In the procedure, the extrahepatic bile ducts are cut and removed and replaced with a portion of the small intestine of the same infant. In most cases, bile drainage gets established by this surgery. However, in some children, there can be liver dysfunction of a small degree still observed. The Kasai surgery can also be used as an early intermediate support procedure for a child’s growth.

• In some cases, in spite of the Kasai procedure, there can be chances of liver transplantation. Liver transplantation is the removal of an infected or injured liver and replacement with a healthy liver from a different person called a donor.

How to Regulate Diet in Biliary Atresia?

Depending on the symptoms and treatment performed, a proper diet should be planned for the affected child.

• Different food supplements like fat-soluble vitamins and medium-chain triglyceride (MCT) oil should be advised. MCT is easy to digest and adds calories to food.

• If the child is not getting sufficient nutrition from the food, a nasogastric feeding tube is inserted to provide more caloric liquid directly into the stomach.

• In some cases, total parenteral nutrition (intravenously) is also administered.

Conclusion:

Biliary disease, the absence or destruction of the bile duct, is a serious digestive issue in children. Proper diagnosis and cure of the symptoms, along with surgical treatment of the disease, are very important. Maintenance of diet and regulation of nutritional balance is important for adequate growth and health of the affected children.

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