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Biliary atresia

Specialties > Child health

Background knowledge 🧠

Definition

  • Biliary atresia is a rare congenital disorder characterized by the obstruction or absence of the extrahepatic bile ducts.
  • Leads to cholestasis, progressive liver damage, and cirrhosis if untreated.
  • Typically presents in the neonatal period.

Epidemiology

  • Incidence: 1 in 10,000 to 20,000 live births.
  • Slight female predominance.
  • More common in East Asian populations.
  • Most common cause of neonatal jaundice requiring surgery.

Aetiology and Pathophysiology

  • Exact cause is unknown; likely multifactorial (genetic, environmental).
  • Biliary ducts become inflamed, leading to fibrosis and obliteration.
  • Leads to bile accumulation in the liver, causing cirrhosis.
  • Associated with other congenital anomalies in some cases.

Types

  • Type I: Obstruction at the common bile duct.
  • Type II: Obstruction at the hepatic ducts.
  • Type III: Most common (85-90%); obstruction at the porta hepatis.
  • Isolated biliary atresia vs. syndromic (with other anomalies).

Clinical Features 🌡️

Symptoms

  • Prolonged neonatal jaundice beyond 2 weeks of age.
  • Dark urine due to conjugated bilirubin.
  • Pale stools (acholic stools).
  • Poor weight gain or failure to thrive.
  • Irritability and feeding difficulties.
  • Hepatomegaly may be noted.

Signs

  • Jaundice (persistent beyond physiological neonatal jaundice).
  • Hepatomegaly, often firm on palpation.
  • Splenomegaly in later stages (due to portal hypertension).
  • Failure to thrive and growth retardation.
  • Ascites and peripheral oedema in advanced disease.

Investigations 🧪

Tests

  • Initial blood tests: Elevated conjugated bilirubin, raised ALT/AST, GGT.
  • Ultrasound: Absence of gallbladder or triangular cord sign.
  • Hepatobiliary scintigraphy (HIDA scan): Lack of bile excretion into intestine.
  • Liver biopsy: Portal tract fibrosis, bile duct proliferation, and bile plugs.
  • Intraoperative cholangiography: Gold standard for diagnosis.

Management 🥼

Management

  • Kasai portoenterostomy: Surgical procedure to restore bile flow, ideally performed before 60 days of age.
  • Liver transplantation: Required in cases where Kasai fails or in advanced disease.
  • Nutritional support: High-calorie diet, fat-soluble vitamin supplementation.
  • Management of complications: Treatment of cholangitis, portal hypertension.
  • Regular follow-up: Monitoring for liver function, growth, and development.

Complications

  • Cholangitis: Infection of the bile ducts.
  • Liver cirrhosis: Irreversible scarring of the liver.
  • Portal hypertension: Increased pressure in the portal venous system.
  • Hepatic failure: End-stage liver disease.
  • Post-Kasai complications: Recurrent cholangitis, biliary strictures.

Prognosis

  • Prognosis depends on early diagnosis and success of the Kasai procedure.
  • Approximately 80% of patients develop cirrhosis by adolescence without liver transplantation.
  • 5-year survival post-transplant is 85-90%.
  • Lifelong monitoring is required for complications and liver function.

Key Points

  • Early detection and intervention are critical for improving outcomes.
  • Kasai portoenterostomy is most effective if performed before 60 days of life.
  • Liver transplantation is often required and has good long-term outcomes.
  • Lifelong follow-up is essential to manage complications.

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Child health