Biliary Atresia

What is Biliary atresia ?

Biliary atresia is a cholestatic disease seen in infancy.  It presents as prolonged conjugated hyperbilirubinemia in the newborn period beyond two weeks.

Bile is a fluid made by the liver. Normally the bile ducts take bile to the small intestine. Bile helps with digestion and also carries waste products. In biliary atresia, the bile ducts in the liver gets  blocked and bile becomes backed up in the liver. This ultimately damages the liver and Many vital body functions also are affected.

What causes biliary atresia in kids ?

The definitive cause of biliary atresia is still unknown but various theories have been proposed for the same. Some of the possible aetiologies are -Viral or bacterial infections, immune system problems, genetic mutation and antenatal exposure to toxins.

What are the symptoms of biliary atresia ?

Persistent jaundice, light colored stools, dark urine, abdominal distension

How is biliary atresia diagnosed in children ?

Most important blood investigation is LFT which reveals conjugated hyperbilirubinemia and elevated GGT

Ultrasound upper abdomen reveals absent gall bladder or triangular cord sign but is often operator dependent

HIDA scan(radionuclide scan) has a high sensitivity which reveals normal uptake, no excretion into the bowel. Sometimes failure to excrete bile can be seen inneonatal hepatitis. Priming with phenobarbitone is done before conducting HIDA scan.

Liver biopsy is also helpful in raising suspicion of biliary atresia and ruling out other causes

How can one confirm the diagnosis of Biliary atresia ?

Biliary atresia requires surgery for confirmation. The surgeon can see the liver and bile ducts by opening a child’s abdomen. A special intraoperative X ray called as cholangiogram is done to confirm the diagnosis.  If biliary atresia is diagnosed, the surgeon may treat it with surgery at the same time.

How is Biliary atresia treated ?

The child with biliary atresia can not survive without surgery. The surgery of choice for biliary atresia is Kasai procedure. Patients who present late or who have failed Kasai surgery or who develop liver failure few years after Kasai may benefit from Liver transplant surgery.

What is Kasai Surgery ?

Kasai surgery connects the bile drainage from the liver directly to the intestinal tract. It bypassesthe blocked bile ducts. This surgery can allow a child to grow and be in fairly good health for a few years.At some point, bile might back up into the liver. This causes liver damage. A liver transplant may also be needed over time.

What is the prognosis of the child after Kasai Surgery and what factors affect the prognosis ?

The 5- and 10-year native liver survival are about 45%–50%. Rest of the patients benefit from Liver transplant.

The chances of survival are more if the surgery is done within 90 days.

What are the complications of Kasai procedure ?

Post Kasai , child can develop cholangitis (infection of biliary tract), portal hypertension, intestinal obstruction and upper GI bleeding.

What is the role of minimal invasive surgery/laparoscopy in treatment of biliary atresia ?

The role is not well defined as the surgery requires meticulous dissection which can be difficult in laparoscopy.