Fact-sheet: Congenital biliary atresia


Updated on 08/31/2023 at 5:23 PM

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Definition

Obstruction of the bile ducts occurring in the perinatal period due to fibrous obliteration of the extrahepatic bile duct, with constant associated involvement of the intrahepatic bile ducts, which are NOT DILATED.
Most common cause of neonatal cholestasis (biliary retention)
Rare condition: Incidence 1/19,500 births in France
Approximately 40 cases per year

Its cause remains unknown and is not unique
2 forms:

  • Non-syndromic (90%)
  • Syndromic (10%)

In 60% of cases, involvement of the extrahepatic bile duct is complete
In 10% of cases: cyst on the fibrous remnant of the extrahepatic bile duct, ranging from 2 mm to 4 cm
In 10% of cases, biliary atresia is described as "syndromic," associated with a malformation syndrome: situs inversus, polysplenia or asplenia, preduodenal portal vein, IVC anomaly, intestinal malrotation, multiple hepatic artery

Ultrasound

First-line examination !!
Performed after 4 to 6 hours of fasting
Color Doppler: to differentiate the gallbladder from cystic images
Diagnosis ruled out if intrahepatic bile duct dilation is present
Triangular cord sign: visibility of the fibrous zone at the confluence of the right and left hepatic ducts
Very small gallbladder with irregular, hyperechoic contours that does not contract after feeding
Cyst of the porta hepatis or hepatic hilum
Syndromic features: to be looked for

Management

Diagnostic emergency: Clinical findings + ultrasound
Antenatal diagnosis is possible:

  • Cystic forms (syndromic or non-syndromic) of biliary atresia
  • Screening by antenatal ultrasound from 20 weeks of gestation onward
  • Low gamma-GT levels in the amniotic fluid
  • From 18 weeks of gestation onward in fetuses born with biliary atresia
  • Antenatal ultrasound diagnosis is difficult if a cystic structure is detected in the hepatic hilum

Sequential treatment:

  • In the neonatal period, the Kasai procedure (= hepatoportoenterostomy), aiming to restore bile flow to the intestine.
  • Liver transplantation subsequently, in case of failure to restore bile flow and/or complications of biliary cirrhosis.

Classification

Classification of biliary atresia:
A: complete atresia
B: atresia sparing the gallbladder and common bile duct
C: complete atresia with a pedicular cyst
D: complete atresia with a hilar cyst
E: atresia sparing the gallbladder and common bile duct with a hilar cyst
F: atresia of the common bile duct