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Antenatally detected cystic biliary atresia: differential diagnoses of a double bubble.

Adewole VA, Wright NJ, Hallows R, Davenport M - Springerplus (2014)

Bottom Line: Postnatal ultrasound and MRCP confirmed a cystic lesion at the porta hepatis, mandating early laparotomy and a successful Kasai portoenterostomy.Although diagnosis of such lesions may be imprecise antenatally, awareness and detection does allow early postnatal investigation and management, which is vital to optimise outcome.This case highlights the need to be mindful of other important anomalies that can give this appearance and that may require early intervention.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatric Surgery, Royal Alexandra Children's Hospital, Eastern Road, Brighton, BN2 5BE England.

ABSTRACT
The 'double bubble' sign on antenatal ultrasound is often associated with duodenal atresia although there are numerous causes. We present a case of cystic biliary atresia presenting with a "double bubble" at 36-weeks gestation. Postnatal ultrasound and MRCP confirmed a cystic lesion at the porta hepatis, mandating early laparotomy and a successful Kasai portoenterostomy. Although diagnosis of such lesions may be imprecise antenatally, awareness and detection does allow early postnatal investigation and management, which is vital to optimise outcome. This case highlights the need to be mindful of other important anomalies that can give this appearance and that may require early intervention.

No MeSH data available.


Related in: MedlinePlus

Antenatal ultrasound at 36 weeks gestation. Two fluid filled structures in the fetal abdomen: a ‘double bubble’ sign.
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Fig1: Antenatal ultrasound at 36 weeks gestation. Two fluid filled structures in the fetal abdomen: a ‘double bubble’ sign.

Mentions: A male infant born at term was referred to our tertiary paediatric surgery centre at 2 days old with a history of bile-stained nasogastric aspirates. Although his 20-week fetal anomaly scan had been normal, a maternal US scan at 36 weeks for high fundal height and suspected polyhydramnios showed the appearance of a “double bubble” (Figure 1), but without polyhydramnios.The child was well on arrival, aspirates were clear and nothing abnormal could be palpated in his abdomen. An upper gastrointestinal contrast study showed free drainage into a normally-rotated, non-dilated duodenum and jejunum. An abdominal US identified a cystic structure measuring 38 mm in diameter in the region of the porta hepatis of uncertain aetiology (Figure 2).Figure 1


Antenatally detected cystic biliary atresia: differential diagnoses of a double bubble.

Adewole VA, Wright NJ, Hallows R, Davenport M - Springerplus (2014)

Antenatal ultrasound at 36 weeks gestation. Two fluid filled structures in the fetal abdomen: a ‘double bubble’ sign.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4117857&req=5

Fig1: Antenatal ultrasound at 36 weeks gestation. Two fluid filled structures in the fetal abdomen: a ‘double bubble’ sign.
Mentions: A male infant born at term was referred to our tertiary paediatric surgery centre at 2 days old with a history of bile-stained nasogastric aspirates. Although his 20-week fetal anomaly scan had been normal, a maternal US scan at 36 weeks for high fundal height and suspected polyhydramnios showed the appearance of a “double bubble” (Figure 1), but without polyhydramnios.The child was well on arrival, aspirates were clear and nothing abnormal could be palpated in his abdomen. An upper gastrointestinal contrast study showed free drainage into a normally-rotated, non-dilated duodenum and jejunum. An abdominal US identified a cystic structure measuring 38 mm in diameter in the region of the porta hepatis of uncertain aetiology (Figure 2).Figure 1

Bottom Line: Postnatal ultrasound and MRCP confirmed a cystic lesion at the porta hepatis, mandating early laparotomy and a successful Kasai portoenterostomy.Although diagnosis of such lesions may be imprecise antenatally, awareness and detection does allow early postnatal investigation and management, which is vital to optimise outcome.This case highlights the need to be mindful of other important anomalies that can give this appearance and that may require early intervention.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatric Surgery, Royal Alexandra Children's Hospital, Eastern Road, Brighton, BN2 5BE England.

ABSTRACT
The 'double bubble' sign on antenatal ultrasound is often associated with duodenal atresia although there are numerous causes. We present a case of cystic biliary atresia presenting with a "double bubble" at 36-weeks gestation. Postnatal ultrasound and MRCP confirmed a cystic lesion at the porta hepatis, mandating early laparotomy and a successful Kasai portoenterostomy. Although diagnosis of such lesions may be imprecise antenatally, awareness and detection does allow early postnatal investigation and management, which is vital to optimise outcome. This case highlights the need to be mindful of other important anomalies that can give this appearance and that may require early intervention.

No MeSH data available.


Related in: MedlinePlus