Antenatal presentation of biliary atresia

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Abstract

Objectives

To investigate the incidence, presence of associated anomalies, type of malformation, and natural history of children with biliary atresia (BA) who presented antenatally.

Study design

The database of 194 infants diagnosed with BA at our tertiary referral unit between 1991 and 2002 was reviewed for infants in whom routine antenatal ultrasound had revealed biliary cystic malformations (BCMs). A retrospective analysis of their medical records was undertaken.

Results

Nine infants (six girls) with BCM were identified (4.6% of total referred patients with BA); all were born after 37 weeks' gestational age and presented to us at a median age of 4 weeks (range, 1-14 weeks). The presence of the BCM was confirmed on postnatal ultrasonography. Median age at corrective surgery was 5 weeks (range, 2-16 weeks). At surgery, six patients had type III BA, including one with the biliary atresia-splenic malformation syndrome; two had type II BA; and one had type I BA. Postoperatively, the infants were followed for a median of 2 years (range, 6 months to 11 years). All infants successfully cleared their jaundice.

Conclusions

Improved techniques in routine antenatal ultrasonography can allow early recognition of BCM. This study suggests that BCM noted antenatally may represent early presentation of both biliary atresia-splenic malformation and nonsyndromic BA.

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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