Biliary Atresia

What is Biliary Atresia?

Biliary Atresia is a rare disease of the liver and bile ducts mostly occurring in infants. The early symptoms of the disease start developing anytime between 2-8 weeks after birth. Bile is a liquid produced by the cells in the liver which helps digest fat. It also carries the waste from the liver to the small intestine for excretion. When an infant is suffering from biliary atresia, the bile flow from liver to gallbladder is blocked causing the bile to be trapped inside the liver, and damaging the liver cells.

There are three main types of extrahepatic biliary atresia:

Type I: Atresia is restricted to the common bile duct.

Type II: Atresia of the common hepatic duct.

Type III: Atresia involves the most proximal part of the bile ducts (>95% of all cases).

Symptoms

Initially, the symptoms of this disease are non-distinguishable to neonatal jaundice. Children and infants with biliary atresia begin to show particular symptoms of the disease from 2-8 weeks of their birth onwards. When the bile fails to drain from the liver due to blockage, it starts to build up inside and causes damage and scarring of liver cells.
The symptoms include:

Jaundice.
Itchiness.
Pale stool.
Dark or orange urine.
Swollen abdomen.
Poor absorption of nutrients causing delay in growth and weight loss.

Causes

The causes and reasons of biliary atresia are not completely understood. But in some cases, the incomplete development of bile duct in the mother’s womb could be a reason, or the bile ducts could be affected due to a virus infection in perinatal period. Also, there are no links to medications taken during pregnancy. It is evident that 10-15% of infants with the disease are born with abnormalities in the:

Heart.
Blood vessel (inferior vena canal anomalies).
Intestinal (malrotation).
Spleen (Polysplenia)

Risks

The main risk factors are:

Infection.
Smoking.
Genetic factors.
Environmental toxins.

Prevention

To prevent the occurrence of this disease, one should take care of:

Health.
Sleep hours.
Physical fitness.
Eating habits.

How is it diagnosed?

At Medanta, diagnosis is made based on symptoms, physical examination, and medical history of the patient using blood tests, biopsy of liver, and imaging for a better and clearer picture.

X-rays
Ultrasound
Blood tests
Biopsy
Surgery

How is it treated?

Biliary Atresia cannot be treated with medication. Other treatment option include:

Removal of damaged ducts
The surgeon removes the damaged ducts outside the liver and identifies smaller ducts still open and draining bile. A loop of intestine is then attached to these open ducts for proper drainage and flow of bile from healthy ducts.
Long-term antibiotic therapy
It is prescribed for avoiding any infection along with additional medications to maximize the success of the operation. In cases where Kasai procedure is not successful, liver transplant is the only solution. Success of the procedure is dependent on:
- Age: Younger the infant, greater the success rates of operation.
- Extent of Liver damage.
- Number and size of functional and healthy ducts.
- Experience of the surgical and medical team

When do I contact the doctor?

A person should immediately consult a doctor, if he/she experiences any vitamin deficiencies, weak bones, decreased mental function, enlarged veins or cirrhosis. Our expert doctor with the assistance of advanced equipments and technology, shall thoroughly take care of your health.

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