Paediatrics Q14

Biliary Atresia

Overview

Biliary atresia is a severe liver disorder that requires early diagnosis. Without timely intervention, the condition can escalate into chronic liver failure, usually resulting in death before the age of 2.

Presentation

1. Jaundice: Yellowing of the skin and eyes.

2. Pale Stools and Dark Urine: Indicative of liver dysfunction.

3. Hepatomegaly: Liver enlargement, usually palpable around 3-4 weeks after onset.

.4. Splenomegaly: Generally, not a feature unless presentation is late.

5. Failure to Thrive: Occurs due to poor nutrient absorption related to liver dysfunction.

Diagnosis

i. Blood Tests: Elevated levels of conjugated bilirubin (CB >50% of total bilirubin) and abnormal liver function tests. Gamma-glutamyl transferase (GGT) is typically high.

ii. Abdominal Ultrasound: Used for initial evaluation.

iii. Cholangiogram: To assess bile ducts.

iv. Liver Histology: Obtained via percutaneous biopsy and provides a definitive diagnosis.

Treatment

Referral to paediatric surgery for surgical repair is required.

 

Further Reading: Biliary Atresia

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