Biliary Atresia Liver Care Africa (BALCA)

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Biliary Atrisia

Biliary Atresia Information

What is Biliary Atresia?

Biliary atresia is a rare but serious liver disease in infants.

It happens when the bile ducts inside or outside the liver are missing, blocked, or abnormally narrow. Because of this, bile (the digestive fluid made by the liver) cannot flow into the intestine as it should. Instead, it builds up in the liver, causing liver damage and scarring (cirrhosis).

What to Look Out For (Signs & Symptoms)

Stool Color Chart

balca liver illustration (1)

Diagnosis Process

Treatment Process

What to Look Out For (Signs & Symptoms).

Watch your baby closely in the first 2–8 weeks of life. If you notice any of these signs, seek medical help immediately.

    • Jaundice that lasts beyond 2 weeks
      (Yellow skin and eyes that do not clear).
    • Pale / clay-colored stools
      (Gray, white, or very light stools instead of yellow or brown).
    • Dark urine
      (Tea- or cola-colored).
  • Swollen tummy (from enlarged liver/spleen).
  • Poor weight gain or slow growth.
  • Frequent irritability or crying.
  • Easy bruising or bleeding.
  • Itchy skin (from bile buildup).
Our Care System
balca SICK CHILD2

Stool Color Chart

Stool Color Chart

Normal, healthy stool color

Possible liver or bile duct problem; see a doctor immediately

Types of Biliary Atresia

  1. Perinatal (Acquired) Type
    • Develops after birth.
    • Thought to be triggered by viral infections or the immune system attacking bile ducts.
    • Accounts for about 80–90% of cases.
  2. Embryonic (Fetal) Type
    • Present before birth (congenital).
    • Often linked with other malformations such as heart defects, spleen abnormalities, or intestinal malrotation.
    • Less common (10–20% of cases).
  1. Extrahepatic Biliary Atresia (Most Common)
    • The bile ducts outside the liver are blocked or absent.
    • Prevents bile from draining into the intestines.
    • This is the classic and most frequent form.
  2. Intrahepatic Biliary Atresia (Rare)
    • The bile ducts inside the liver are affected.
    • Very uncommon and often harder to treat, sometimes overlapping with other liver diseases.
  • Most babies have the perinatal, extrahepatic type.
  • The fetal type is rarer but often comes with other birth defects.
  • Intrahepatic form is very rare.

Regardless of type, early diagnosis and treatment (Kasai or transplant) are critical.

Step 1: Clinical Observation

    • Jaundice lasting more than 2 weeks.
    • Pale/clay-colored stools.
    • Dark urine.
    • Enlarged liver or swollen tummy.

Step 2: Blood Tests

  • Liver function tests → check bilirubin (especially direct/conjugated bilirubin) and liver enzymes.
  • Clotting tests & vitamin levels → show how well the liver is working.

Step 3: Imaging Studies

  • Ultrasound → looks for absent/abnormal gallbladder or bile ducts.
  • Hepatobiliary (HIDA) scan → dye is injected to see if bile flows into the intestine. Lack of flow suggests blockage.

Step 4: Liver Biopsy

  • A small piece of liver tissue is examined under the microscope.
  • Shows scarring, bile buildup, and absence of bile ducts — strong evidence of biliary atresia.

Step 5: Intraoperative Cholangiogram (Final Confirmation)

  • During surgery, a special dye is injected directly into the bile ducts.
  • If dye cannot pass into the intestine, biliary atresia is confirmed.

If confirmed, the surgeon often proceeds with the Kasai procedure or liver transplantation immediately

Step 1: Initial Management & Support

  • Baby is evaluated and stabilized.
  • Nutrition support: high-calorie feeds, MCT oil, and fat-soluble vitamin supplements (A, D, E, K).
  • Medications: to improve bile flow and prevent infections (e.g., antibiotics, ursodeoxycholic acid).

Step 2: Kasai Procedure (Hepatoportoenterostomy)

  • Timing: Best done before 2–3 months of age.
  • Procedure: The blocked bile ducts outside the liver are removed, and a loop of the intestine is attached to the liver to drain bile.
  • Goal: Restore bile flow, slow down liver damage.
  • Outcomes:
    1. Works well in some babies (especially if done early).
    2. Others may still develop liver damage over time.

Step 3: Ongoing Care After Kasai

  • Regular follow-ups with a liver specialist.
  • Continued nutrition and vitamins.
  • Watch for complications (cholangitis, cirrhosis, portal hypertension).
  • Some children do well for years; others may progress to liver failure.

Step 4: Liver Transplant (If needed)

  • If the Kasai fails or the liver is too damaged:
    1. A liver transplant is performed.
    2. Can be from a living donor (parent donates part of their liver) or deceased donor.
  • Transplant is the only permanent cure for biliary atresia.
  • With good follow-up, children can live long, healthy lives after a transplant.

Biliary Atresia

Biliary Atresia

 tortor hendrerit

Head Start

Purpose

  • To restore bile flow from the liver into the intestine when the bile ducts are blocked or absent.
  • It does not cure biliary atresia but can delay or reduce liver damage.

The Procedure (Step by Step)

  1. Surgery under general anesthesia – the baby is asleep and pain-free.
  2. Exploration of the liver – the surgeon confirms the diagnosis and assesses the extent of blockage.
  3. Removal of damaged bile ducts – the non-functioning extrahepatic bile ducts outside the liver are cut away.
  4. Creating a new pathway – a loop of the baby’s small intestine is brought up and surgically connected directly to the liver at the porta hepatis (the place where bile normally drains).
  5. Bile drainage – bile produced in the liver can now flow into the intestine through this new pathway.

Purpose

  • A liver transplant is the only permanent cure for biliary atresia.
  • It replaces the baby’s damaged liver with a healthy one from a donor.

When It’s Needed

  • If the Kasai procedure fails (bile flow doesn’t improve).
  • If the liver becomes severely scarred (cirrhosis) or goes into liver failure.
  • Signs that a transplant is needed include:
    • Worsening jaundice.
    • Poor growth.
    • Swollen belly (ascites).
    • Frequent infections or bleeding problems.
  1. Living Donor Liver Transplant
    • A healthy adult (often a parent) donates part of their liver.
    • The liver can regenerate in both the donor and the child.
  2. Deceased Donor Liver Transplant
    • The liver (whole or part) comes from someone who has passed away.

The Procedure (Step by Step)

  1. Baby is put under general anesthesia.
  2. The surgeon removes the diseased liver.
  3. A healthy piece (or whole) donor liver is connected to the baby’s blood vessels and bile ducts.
  4. The new liver takes over all the functions immediately.

The exact cause is not fully known, but research shows it is not inherited and not passed from parent to child. Possible causes include:

  1. Immune System Problems
    • The baby’s immune system may mistakenly attack the bile ducts, causing inflammation and scarring.
  2. Viral Infections
    • Some babies may get infections (like reovirus, rotavirus, or CMV) before or shortly after birth that damage the bile ducts.
  3. Problems During Development (Embryonic Type)
    • In some babies, the bile ducts don’t form properly during pregnancy.
    • Often linked with other birth defects (heart problems, spleen abnormalities, intestines not positioned correctly).
  4. Environmental Triggers
    • Exposure to toxins or other unknown environmental factors might play a role.

Key Point for Parents

  • Biliary atresia is not caused by anything the mother did or did not do during pregnancy.
  • It is a complex disease with multiple possible triggers, but the exact cause remains unknown.

After Kasai Procedure

  • Antibiotics → to prevent infections in the bile ducts (cholangitis).
  • Vitamins (A, D, E, K) → because the sick liver can’t absorb fat-soluble vitamins well.
  • Ursodeoxycholic acid (Urso/Ursodiol) → helps bile flow and protects liver cells.
  • Steroids (sometimes) → reduce inflammation after surgery.

After Liver Transplant

  • Anti-rejection drugs (immunosuppressants) → e.g., tacrolimus, cyclosporine, steroids.
  • Antibiotics & antifungals → to prevent infections (since the immune system is weakened).
  • Vitamins & supplements → support growth and nutrition.

Aftercare at Home

  • Frequent follow-ups with liver specialists.
  • Growth monitoring → check weight and height often.
  • Nutrition support → high-calorie foods, special formula (if needed).
  • Watch for warning signs: fever, worsening jaundice, pale stools, swelling tummy → seek medical help immediately.
  • Good hygiene → prevent infections, especially after transplant.
  • Vaccinations → very important to protect against infections.

Welcome to the Billiary Atrisia Community!

For every child, every liver, every life.

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