Biliary Atresia and Choledochal Cyst
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.
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Biliary Atresia and Choledochal Cyst Lidia Monti, Amato Infante, and Marco Salsano
7.1
Introduction
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. L. Monti (*) Department of Diagnostic Imaging, Children’s Hospital Bambino Gesù, Rome, Italy e-mail: [email protected] A. Infante • M. Salsano Department of Radiological Sciences, Institute of Radiology, Catholic University of Sacred Heart, Rome, Italy
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.
7.2
Biliary Atresia
BA is an inflammatory cholangiopathy that results in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts and is the most frequent and severe cause of neonatal cholestasis affecting approximately 1 on 10,000– 20,000 births. The inflammatory process of the biliary tree leads to progressive obliterative scarring of the bile ducts. In BA the extrahepatic biliary system is disrupted. Progressive damage of extrahepatic
© Springer International Publishing Switzerland 2016 V. Miele, M. Trinci (eds.), Imaging Non-traumatic Abdominal Emergencies in Pediatric Patients, DOI 10.1007/978-3-319-41866-7_7
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and intrahepatic bile ducts secondary to inflammation occurs, leading to fibrosis, biliary cirrhosis, and liver failure. Although the exact etiology remains unknown, the primary therapy is surgical. Only early surgical treatment can stall biliary cirrhosis, which is why rapid identification of BA is crucial. Previously, the disease was described as occurring in two distinct clin
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