Antenatal presentation of biliary atresia
Section snippets
Patients and methods
King's College Hospital is the largest tertiary referral center for children with liver disease in the United Kingdom. Over the period of last 25 years, more than 500 infants have been diagnosed with BA. To identify infants who presented with antenatal BCM, the King's College Hospital BA database between 1991 and 2002 was reviewed. This period was chosen to encompass the era of routine antenatal ultrasonography in this country. The medical records were reviewed of those infants in whom
Results
During the study period, 194 patients were diagnosed with BA. Nine infants (4.6% of the total) had presented antenatally with intra-abdominal cysts on routine ultrasonography (Figure). There were six female infants and three male infants. All of the infants were born after 37 weeks' gestational age and presented to our unit with prolonged conjugated hyperbilirubinemia. Median age at referral was 4 weeks (range, 1-14 weeks). None of the mothers of the affected infants had positive viral serology
Discussion
The cause of BA remains uncertain.2 It is generally accepted that there are two types: embryonic (congenital, syndromic) and perinatal (acquired, nonsyndromic).3 This study, which is the largest series to date investigating antenatal presentation of BA, demonstrates that both nonsyndromic and syndromic forms (BASM syndrome) can present before birth.
Antenatal diagnosis of biliary malformations is not an easy one.6 Furthermore, histological distinction between BA and obstructed choledochal cyst
Acknowledgements
Figure courtesy of Dr. S Sairam, Fetal Medicine Unit, St George's Hospital, London.
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