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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

Cholangiogram showing large cystic dilatation and abnormal etiolated, hypoplastic intrahepatic biliary ducts consistent with cystic biliary atresia.
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Fig4: Cholangiogram showing large cystic dilatation and abnormal etiolated, hypoplastic intrahepatic biliary ducts consistent with cystic biliary atresia.

Mentions: Liver biochemistry at day 9 showed total bilirubin of 113 μmol/L with a conjugated bilirubin of 83.9 μmol/L (74% of total); alkaline phosphatase (ALP) 280 iu/L (normal <449 iu/L) and alanine transferase 71 iu/L (reference range <41 iu/L).Magnetic Resonance Cholangiopancreatography (MRCP) was requested to evaluate the biliary system (Figure 3a and b) and showed a 32.8 mm × 40 mm cyst in the region of the porta hepatis. He was then transferred to a specialist paediatric liver centre where a laparotomy was carried out on day 20. This showed a mucus-containing cyst which on intra-operative cholangiography (Figure 4) had a tenuous connection to a non-dilated irregular biliary tree. The transected porta hepatis did not show any visible bile duct consistent with Type III BA (Japanese Association of Pediatric Surgeons (JAPS) classification). A Kasai-type portoenterostomy was performed following which bile flow was restored and his jaundice cleared. He is now 4 months old, thriving and jaundice free.Figure 3


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

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

Cholangiogram showing large cystic dilatation and abnormal etiolated, hypoplastic intrahepatic biliary ducts consistent with cystic biliary atresia.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig4: Cholangiogram showing large cystic dilatation and abnormal etiolated, hypoplastic intrahepatic biliary ducts consistent with cystic biliary atresia.
Mentions: Liver biochemistry at day 9 showed total bilirubin of 113 μmol/L with a conjugated bilirubin of 83.9 μmol/L (74% of total); alkaline phosphatase (ALP) 280 iu/L (normal <449 iu/L) and alanine transferase 71 iu/L (reference range <41 iu/L).Magnetic Resonance Cholangiopancreatography (MRCP) was requested to evaluate the biliary system (Figure 3a and b) and showed a 32.8 mm × 40 mm cyst in the region of the porta hepatis. He was then transferred to a specialist paediatric liver centre where a laparotomy was carried out on day 20. This showed a mucus-containing cyst which on intra-operative cholangiography (Figure 4) had a tenuous connection to a non-dilated irregular biliary tree. The transected porta hepatis did not show any visible bile duct consistent with Type III BA (Japanese Association of Pediatric Surgeons (JAPS) classification). A Kasai-type portoenterostomy was performed following which bile flow was restored and his jaundice cleared. He is now 4 months old, thriving and jaundice free.Figure 3

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