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Biliary problem in child

What is biliary atresia?

Bile is a liquid which passes into the gut through small tubes called bile ducts and is necessary for the proper digestion of fat within the diet.

Biliary atresia is a condition in which inflammation develops within the bile ducts around the time of birth. The inflammation can occur in any of the bile ducts both inside and outside the liver. This leads to bile duct damage, reducing the flow of bile, which causes scarring of the liver.

What causes biliary atresia?

The cause of biliary atresia is currently unknown. Research is being carried out, but it has not yet provided any answers.

What are the signs and symptoms of biliary atresia?

In the first weeks of life babies with biliary atresia often seem well, apart from being jaundiced (yellowing of the skin and whites of the eyes). Jaundice is very common in babies and mostly clears within the first two weeks of birth.

In addition to prolonged jaundice, other signs to look out for are:

  • Yellow coloured urine
  • Pale stools
  • Bleeding from the umbilicus or frequent nosebleeds may be an indication of liver disease

How is biliary atresia diagnosed?

The signs above are non-specific so there will need to be a number of investigations. Tests need to be carried out in hospital and include blood tests, scans and liver biopsy. Once other causes of liver disease have been excluded and investigations indicate biliary atresia your baby will be scheduled for surgery.

Treatment options for biliary atresia

Initial treatment is an operation called the ‘Kasai Procedure’ after the Japanese surgeon who pioneered the operation. The aim of the Kasai procedure is to allow bile to drain from the liver into the gut.

Babies with biliary atresia will often need a special milk formula so they can absorb nutrients more fully. The hospital dietitian will advise you. The doctor may prescribe extra vitamins.

Are there any other problems to be aware of?

Even after a successful Kasai operation a number of problems may occur:
  • Cholangitis (an infection of the bile ducts in the liver, resulting in inflammation)
  • Ascites (an abnormal collection of fluid in the abdomen)
  • Portal hypertension (increased blood vessel pressure caused by scarring of the liver, which may cause blood being vomited or passed in the stools)
  • Pruritus (itching)
  • Failure to thrive
The future for biliary atresia

Your baby or child’s growth and development will be followed up by periodic visits to the hospital. Blood tests will be needed to monitor liver function and periodically an ultrasound will be performed to assess the size of the liver and other structures in the abdomen. Routine endoscopy may be undertaken to monitor the development of oesophageal varices. Biliary atresia surgery if fails then child may need liver transplant.

Choledochal cyst
About choledochal cysts

A choledochal cyst is a swelling/widening or dilation of the bile ducts. It usually affects the bile ducts outside the liver, but sometimes it also affects those inside the liver. The exact cause is unknown but it arises because of a malformation of the bile duct when the baby is developing in early pregnancy.

What are the signs and symptoms of choledochal cysts?

Most choledochal cysts are detected in childhood and some are found before birth during ultrasound scans. Every child is different, but they may have some or all of the following:

  • Jaundice
  • Intermittent abdominal pain
  • Cholangitis
  • Peritonitis
  • An abdominal swelling

How is a diagnosis of choledochal cysts made?

A choledochal cyst is most often suspected if an abdominal ultrasound scan shows a dilated bile duct in a baby or child with jaundice or severe abdominal pain. Extra tests are carried out to get more information. These may include:

  • A more detailed ultrasound scan
  • MRCP (Magnetic Resonance Cholangio-Pancreatography)
  • ERCP (Endoscopic Retrograde Cholangio-Pancreatography)

Treatment options for choledochal cysts

Common types of choledochal cysts should be treated by a surgeon experienced in this kind of operation. The best operation involves the cyst being completely removed, which means removing most of the bile ducts outside the liver, together with the gall bladder.

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