What is it?

Biliary atresia is a condition in which the ducts that carry bile from the liver to the gallbladder are blocked. It is a congenital disorder that results in an abnormal development of bile ducts. either inside or outside the liver. The reason for this condition is not known, it has been linked to some maternal infections like rotavirus infection during pregnancy or a congenital CMV infection. Exposure to aflatoxin was also found linked to the disorder. There are some associations between biliary atresia and some genes. In babies who are born with this condition, bile flow is blocked, leading to liver damage and chirossis, which are deadly if not treated.

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Additional names

This group contains additional names:
- Atresia of bile duct
- Biliary obstruction congenital
- Carolis disease
- Congenital dilatation of lobar intrahepatic bile duct

Signs & symptoms

Jaundice in babies with biliary atresia develops by the second or third week of life. At the age of one month, the infant will start losing weight, become irritable and the jaundice will get worse. Other symptoms may include enlarged spleen, dark urine, pale, floating and foul-smelling stools, and slow growth.

Diagnosis

If biliary atresia is suspected and on physical examination an enlarged liver is palpated, other tests may be performed such as abdominal X-RAY and ultrasound, blood tests for bilirubin levels, hepatobiliary iminodiacetic acid (HIDA) scan, cholangiogram which is an X-RAY of the bile ducts. If necessary, liver biopsy may be performed to rule out other conditions.

Treatment

Biliary atresia treatment is a surgical procedure named the Kasai (or hepatoportoenterostomy) is performed. The earlier this procedure is done, it is more likely to succeed. In this procedure, the damage ducts are removed, and an intestinal loop is connected to a healthy part of the liver. The kasai procedure is not always successful, and for these children they will need liver transplantation. Children with biliary atresia cannot digest fat, and have to eat more calories than other children at their age. They have to receive vitamin supplements and medium-chain- triglyceride oil.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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