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

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.

Cystic Biliary Atresia: A Delayed Diagnosis of Obstructive Jaundice

“…The CBA is classified according to Japanese Association of Paediatric Surgeon (JAPS) which based on the level of the most proximal obstruction; type I is at the level of the common bile duct, type II at the level of the common hepatic duct and type III at the level of the porta hepatis. 5 USG plays a crucial role for investigating infant with persistent jaundice; to establish the choledochal structural anomalies, inspissated bile syndrome, perforated biliary duct, and to confirm the diagnosis of BA. 5 For non-cystic BA, the 'triangular cord' is the pathognomonic sign for establishing the diagnosis.…”

“…5 USG plays a crucial role for investigating infant with persistent jaundice; to establish the choledochal structural anomalies, inspissated bile syndrome, perforated biliary duct, and to confirm the diagnosis of BA. 5 For non-cystic BA, the 'triangular cord' is the pathognomonic sign for establishing the diagnosis. 6 Triangular cord is characterized by echogenic appearance anterior to the wall of the right portal vein of >4 mm on longitudinal scan and corresponds to the obliterated proximal remnant in the porta hepatis.…”

Atypical Double-Bubble in MRI of a Fetus with Double Atresia Involving Esophagus and Jejunum

“…The double-bubble sign, originally described on plain radiography, but now also appreciable on ultrasound and MRI, is a result of excessive fluidfilled structures in the abdomen. Therefore, double-bubble sign is seen prenatally in fetuses with various alimentary tract pathologies [1][2][3][4][5]. Although the most frequent pathology for the double-bubble sign is duodenal stenosis/atresia [1], it is not exclusively pathognomonic for duodenal atresia.…”

“…Although the most frequent pathology for the double-bubble sign is duodenal stenosis/atresia [1], it is not exclusively pathognomonic for duodenal atresia. Pathologies other than duodenal stenosis/atresia presenting with a double-bubble sign include cystic biliary atresia [2], colonic duplication [3], malrotation with midgut volvulus [4], and triple gut atresia [5].…”