Cholangitis

Cholangitis is an infection of the bile ducts. It is a common complication after the Kasai procedure and needs urgent treatment. Cholangitis most often happens in the first year or two after the Kasai procedure, but it can happen at any time. It can also happen more than once or keep returning.

The symptoms of cholangitis include fever and jaundice. If your child has a temperature over 37.8ºC or an unknown illness, contact your child’s medical team. Cholangitis can be treated with antibiotics, but they can only be given at your local hospital or the liver unit treating your child. Antibiotics will be given intravenously. This is where the medicine is put straight into your baby’s body through a vein. It is often followed by a long course of oral antibiotics (antibiotics given by mouth).

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Intrahepatic biliary cysts (bile lakes)

Intrahepatic biliary cysts, or “bile lakes” are pools of bile that form in the bile ducts. They can block the bile ducts and increase the risk of cholangitis. Bile lakes are fairly common in biliary atresia, with around 1 in 4 children developing at least one bile lake. If the lakes are large or cause problems, they can be drained.

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Vitamin deficiencies

Babies and children with biliary atresia often have trouble absorbing vitamins A, D, E and K, even after a successful Kasai procedure. They will be monitored for vitamin deficiencies and given extra vitamins if needed.

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Faltering growth

Many babies and children with biliary atresia struggle to gain weight, even after a successful Kasai procedure. They may grow more slowly than other children and may need extra nutritional support. A dietitian will be available to support you with feeding options.

Find out more about nutrition

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Portal hypertension and variceal bleeding

Portal hypertension is high pressure in the portal vein. This is the main vein carrying blood from the gut to the liver. When the liver is damaged, it becomes stiff. This stiffness makes it harder for blood to flow through the liver. This causes the high pressure in the portal vein.

Portal hypertension can make the spleen get bigger (splenomegaly). It can also cause swollen blood vessels in the food pipe (oesophageal varices). The swollen blood vessels have thin walls and may bleed. This may cause your child to vomit blood or pass black tarry stools. Both symptoms need immediate medical attention. It is uncommon for this to happen before a child is 2 years old.

If there are no symptoms or complications from portal hypertension, then treatment may not be needed. But some children will need treatments such as banding or sclerotherapy to the oesophageal varices.

Find out more about portal hypertension

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Fluid in the tummy (ascites)

Ascites is the term for a build-up of fluid in the tummy. A small amount of ascites can be present for up to 6 weeks after the Kasai procedure but should then disappear. If it comes back again and your child’s tummy becomes noticeably bigger, or they have unusual weight gain, contact your medical team.

Treatment for ascites may be started by your GP or local paediatrician and may include medicines or changing your child’s diet. In more serious cases, doctors may need to admit your child to hospital for treatment.

Find out more about ascites

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Hepatopulmonary syndrome

This is an uncommon complication that affects the ability of the lungs to exchange oxygen. It happens in around 5–7% of children with biliary atresia. Symptoms include shortness of breath and low blood oxygen levels.

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Portopulmonary hypertension

This is a rare complication and happens in less than 3% of children and young people with biliary atresia. It usually starts in adolescence and is more common in girls. Symptoms include shortness of breath and tiredness.

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Liver cancer

Liver cancer can develop in anyone with a badly scarred liver, but it is generally very rare in babies and children. It has been reported in less than 2% of babies and children after a Kasai procedure. Every person with biliary atresia will have routine screening for liver cancer using blood tests and ultrasound scans. If picked up early a liver transplantation will be considered.

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