Biliary Atresia
Congenital Progressive Extrahepatic Cholestatic Obstruction
Primary risk age: 2 to 8 Weeks (Newborn/Early Infancy)
- Urgency
- Severe
- Typical age
- 2 to 8 Weeks (Newborn/Early Infancy)
- Body system
- Neonatal (Newborns)
Typical course: Surgical recovery is 1-2 weeks; clinical management of biliary flow is lifelong.
Reviewed against AAP · CDC · WHO · NHS guidance Last reviewed 2026-06-13
1. Summary & Pathophysiology
Congenital Progressive Extrahepatic Cholestatic Obstruction
Pathophysiology (Development Path)
Progressive, inflammatory sclerosis of the extrahepatic biliary tree prevents bile from draining from the liver to the duodenum. The accumulation of toxic bile acids causes rapid, progressive hepatocyte necrosis, portal tract fibrosis, biliary cirrhosis, and portal hypertension.
Primary Causes & Etiology
Unknown; believed to be an immune-mediated or infectious inflammatory process causing progressive damage to the bile ducts shortly after birth.
2. Symptom Continuum
- Early Onset Signs
Persistent neonatal jaundice beyond 14 days of life; dark yellow urine that stains the diaper.
- Progressive Phase
Acholic (pale, clay-colored or white) stools; hepatomegaly (enlarged, firm liver) and splenomegaly.
- Severe Indicators
Ascites (abdominal fluid), poor weight gain, bruising or bleeding tendencies due to malabsorption of vitamin K, and hepatic failure.
3. Clinical Verification
Direct/conjugated bilirubin measurement; abdominal ultrasound demonstrating small/absent gallbladder; liver biopsy showing ductular proliferation. Confirmed via intraoperative cholangiogram.
4. Care & Elements Plan
Primary Care Treatment Plan
Urgent surgical hepatoportoenterostomy (Kasai procedure) to bypass blocked ducts, ideally before 60 days of age. Supplemental fat-soluble vitamins and MCT formula. Liver transplantation if Kasai fails.
Home Support Elements
Monitor stool color daily using a stool color card; administer fat-soluble vitamins; feed high-calorie medium-chain triglyceride formula; watch for signs of fever/cholangitis.
Generic Active Ingredients (No Brands)
- Ursodeoxycholic acid (generic bile acid active ingredient to promote bile flow)
- Vitamins A, D, E, K (generic fat-soluble vitamins to prevent deficiency).
Lists active elements only. Never administer self-designed therapies.
5. Doctor Critical Lines
Critical Thresholds: When to See a Doctor
Seek immediate pediatric evaluation if a newborn remains jaundiced past 2 weeks of age, passes white or clay-colored stools, or has dark yellow urine.
6. Vaccine & Prevention
Routine Prophylaxis (Prevention)
No known prevention measures exist.
Immunization Context
Standard immunizations are recommended; live vaccines (e.g. Rotavirus) are withheld if liver failure progresses or transplant is planned.
7. Timelines & Outlook
Active Timeline
Surgical recovery is 1-2 weeks; clinical management of biliary flow is lifelong.
Expected Prognosis
Highly dependent on age at Kasai surgery. Even with early surgery, ~70-80% of children eventually require a liver transplant before adulthood.
Potential Untreated Complications
Ascites, cholangitis, portal hypertension, esophageal varices, end-stage liver failure.
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