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Biliary Atresia and Choledochal Cyst

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Imaging Non-traumatic Abdominal Emergencies in Pediatric Patients

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Abstract

Biliary atresia (BA) and choledochal cyst (CC) are two well-known causes of obstructive jaundice in neonates and young infants and have similar clinical presentation.

BA is a progressive obstructive cholangiopathy diagnosed in the newborn period and characterized by partial or complete lack of the extrahepatic bile ducts.

CC is a dilatation of the biliary tract due to congenital anomaly of the bile duct; it is usually present prior to the age of 2 years and can be antenatally diagnosed.

Patients with BA or CC usually have jaundice and acholic stools in the neonatal or young infantile period.

However, BA and CC are two entities with dramatically different approaches and prognosis. While BA requires portoenterostomy, CC is usually treated by cyst excision with hepaticojejunostomy or choledochojejunostomy. Children with BA usually have poor long-term prognoses even after Kasai portoenterostomy.

Although most patients who undergo surgery before 60 days of age show good bile excretion, ultimately many of them develop cirrhosis and liver failure.

Conversely, patients with CC have good outcomes with early surgical management. Because of the difference in management and prognosis between BA and CC, radiologic, particularly ultrasonographic (US), differentiation of the two entities is important.

In almost 10 % of BA cases, there is a cyst at the porta hepatis. For this reason BA with cystic component is likely to be mistaken for CC.

This short review attempts to summarize the imaging modalities of the two entities, in order to differentiate them. This is of paramount relevance in the differential diagnosis between cystic BA and CC.

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Authors and Affiliations

  1. Department of Diagnostic Imaging, Children’s Hospital Bambino Gesù, Rome, Italy

    Lidia Monti

  2. Department of Radiological Sciences, Institute of Radiology, Catholic University of Sacred Heart, Rome, Italy

    Amato Infante & Marco Salsano

Authors
  1. Lidia Monti
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  2. Amato Infante
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  3. Marco Salsano
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Correspondence to Lidia Monti .

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Editors and Affiliations

  1. Department of Emergency Radiology, S. Camillo Hospital, Rome, Italy

    Vittorio Miele

  2. Department of Emergency Radiology, S. Camillo Hospital, Rome, Italy

    Margherita Trinci

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Monti, L., Infante, A., Salsano, M. (2016). Biliary Atresia and Choledochal Cyst. In: Miele, V., Trinci, M. (eds) Imaging Non-traumatic Abdominal Emergencies in Pediatric Patients. Springer, Cham. https://doi.org/10.1007/978-3-319-41866-7_7

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  • DOI: https://doi.org/10.1007/978-3-319-41866-7_7

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