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Biliary Atresia

Obstruction of extrahepatic bile ducts in the newborn; early surgical treatment is life-saving.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Biliary Atresia?

Biliary atresia is one of the most important causes of neonatal cholestasis. The extrahepatic bile duct system progressively obstructs and bile flow is blocked. If untreated, progressive hepatic fibrosis and cirrhosis develop.

Clinically, prolonged jaundice starting at 2-6 weeks of age, dark urine, acholic (pale) stools and hepatomegaly are typical. Direct hyperbilirubinemia, elevated GGT and mild-to-moderate elevation of liver enzymes are seen.

Diagnosis uses ultrasonography (triangular cord sign), hepatobiliary scintigraphy, intraoperative cholangiography and liver biopsy. Kasai portoenterostomy (Roux-en-Y anastomosis from the hepatic hilum to the jejunum) gives the best results when performed before 45-60 days of age. If unsuccessful, liver transplantation is required.

Symptoms

Prolonged jaundice exceeding 2 weeks
Pale (acholic) stools
Dark urine
Hepatomegaly
Poor growth
Edema and ascites (late stage)
Bleeding tendency (vitamin K deficiency)

Risk Factors

Viral infection (CMV, reovirus)
Autoimmune mechanisms
Female sex (slightly more common)
Polysplenia syndrome
Congenital anomalies
Asian ethnicity

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Infant jaundice exceeding 14 days
  • Pale stools and dark urine
  • When liver enlargement is noticed
  • When direct bilirubin predominates on evaluation
  • Growth and developmental delay

Treatment Methods

01
Direct bilirubin and liver function tests
02
Ultrasonography and hepatobiliary scintigraphy
03
Liver biopsy and intraoperative cholangiography
04
Kasai portoenterostomy (before 45-60 days)
05
Fat-soluble vitamin (A, D, E, K) supplementation
06
Liver transplantation (failed cases)

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.