saudigastro.com/patients/biliary-atresia
Category
Serious
Red flags
5
This article is educational and does not replace your doctor's advice. Seek urgent care for any red-flag symptoms.
What is it?
Bile is a digestive fluid made in the liver that carries waste out of the body and helps digest fat. It normally flows through small tubes called bile ducts into the bowel. In biliary atresia (say "BIL-ee-air-ee AT-ree-zhuh"), the bile ducts outside and inside the liver become inflamed and blocked in the first weeks of life. Bile then backs up and damages the liver. Without treatment, it leads to scarring (cirrhosis) of the liver in the first months or years of life.
How common is it?
Biliary atresia is rare, but it is the most common reason for liver transplant in children in many countries. Because it is rare and early signs can look like normal newborn jaundice, it can be missed unless parents and doctors are alert.
Why does it happen?
The cause is not fully understood. It is not caused by anything the mother did in pregnancy, and it is not contagious. Current thinking is that an abnormal inflammatory process, possibly triggered around the time of birth in a baby with some susceptibility, damages the bile ducts. It is usually not inherited.
Symptoms
- Yellow skin and eyes (jaundice) that lasts beyond about two weeks of age
- Pale, chalky, grey or cream-coloured stools
- Dark, tea-coloured urine that stains the nappy
- A baby who may look otherwise well and feed normally at first
- Later: swollen belly, poor weight gain, itching, easy bruising
Red flags — seek urgent medical care
Jaundice in a baby older than 2 weeks (or 3 weeks if born early)
Needs a blood test for conjugated (direct) bilirubin within a day or two. Do not wait. Early diagnosis matters.
Pale or white stools, or dark urine
Suggests bile is not reaching the bowel. See a doctor urgently, ideally the same day. Take a photo of the stool or the nappy to show them.
Vomiting blood, black or bloody stools, or unusual bleeding or bruising
Bleeding can occur because of low vitamin K or liver injury. Go to the emergency department now.
Fever, chills, or increased yellow colour after surgery
May be an infection of the bile ducts (cholangitis). A baby needing urgent care should be seen immediately.
A baby who is very sleepy, floppy, or feeding poorly
Needs emergency assessment.
How is it diagnosed?
Diagnosis needs a paediatric liver specialist. It starts with blood tests that measure bilirubin and liver tests, followed by an abdominal ultrasound. Other causes of newborn liver problems are excluded. Further tests may include a nuclear bile scan or a liver biopsy. When biliary atresia is strongly suspected, the surgeon may check the bile ducts with an X-ray dye test during an operation and, if confirmed, proceed to the corrective surgery.
Outlook — what to expect
The outlook is best when surgery is done early, ideally in the first weeks or months of life. The operation (the Kasai procedure) connects the liver directly to the bowel so bile can drain. It does not cure the disease: some babies recover good bile flow and live for years with their own liver, while many eventually need a liver transplant. Regular follow-up with a specialist liver team is essential for life.
Treatment
Habits & Lifestyle
Most care is led by the specialist team. Parents can help in several ways.
- Look at your baby's stool colour every day and photograph unusual pale stools.
- Give all medicines at the right times; missed doses can reduce their benefit.
- Keep vaccinations up to date and ask the team about any special vaccines.
- Keep all appointments and blood tests.
- Know whom to call and where to go quickly if your baby has a fever.
Diet
Nutrition is a major part of care because bile is needed to absorb fat and some vitamins.
- Breastfeeding can usually continue unless the team advises otherwise.
- Your baby may need a special high-energy formula or added feeds. A dietitian will guide you.
- Fat-soluble vitamins (A, D, E and K) are usually prescribed because they are poorly absorbed. Give them as directed.
- Do not give herbal products, honey-based remedies or home treatments for jaundice, and do not rely on sunlight to treat this type of jaundice.
Medications
Medicines support the liver and prevent complications; they do not cure biliary atresia.
- After surgery, medicines are often used to improve bile flow, protect against infection of the bile ducts, and reduce itching. The specialist chooses these.
- Vitamin supplements are an important part of treatment.
- Do not give any other medicine, including over-the-counter ones, without asking the liver team, because the liver is affected.
- The doctor decides the medicines, doses and how long they are given.
Procedures & Surgery
Surgery is the main treatment and should be done as early as possible.
- The Kasai procedure is performed in a specialist paediatric liver or surgical centre. The aim is to restore bile drainage.
- If bile drainage does not work, or if the liver becomes severely scarred, a liver transplant may be needed. The transplant team will explain the process and options, including living-donor transplant.
- Your baby will be monitored closely for infection, growth, and liver function after surgery.
Living with the condition
- This is a hard diagnosis for families. Ask about counselling and parent support groups.
- Keep a list of medicines and contacts, and bring your child's records when travelling.
- Many children go to school and take part in normal activities when well. Your team will guide you.
Questions to ask your doctor
Save this list or print it before your appointment — doctors appreciate prepared patients.
- How certain is the diagnosis, and how soon can surgery be done?
- Where is the specialist centre for my baby's care?
- What medicines and vitamins will my baby need?
- What signs mean I should come to hospital straight away?
- What are the chances my child will need a liver transplant, and how do we prepare?
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Status: Approved for publication by SGA; individual clinician review is ongoing · Published 24/04/2026
For general education only; it does not replace your doctor’s advice. Individual clinician review of this guide is ongoing. SGA clinician? Review this guide