To explore the definition of biliar atresia disease. To describe the radiological patterns of the disease. To present structural malformations associated to biliary atresia. To provide key points to the differential diagnosis. To propose a standard report with the components of interest for the Pediatric and Hepatology group.
Biliary atresia is a rare disorder in children that affects the biliary tract with progressive fibrosing obstruction and liver failure.
Early detection in children with persisting jaundice can improve on the quality of life and prevent a poor outcome.
The diagnosis is based on laboratory test,
imaging findings and liver biopsy. Current management includes multidisciplinary care teams,
Kasai portoenterostomy and prevention of cholangitis,
but in cases of failure and end-stage cirrhosis a liver...
Findings and procedure details
The pathogenesis of Biliary Atresia appears to be multifactorial,
but the cause is not understood yet. Congenital malformation such heterotaxia,
spleen anomalies (polysplenia,
and preduodenal portal vein,
has been reported in 20% of the cases with biliary atresia. It has also been associated with prenatal exposure to viruses (Cytomegalovirus and rotavirus). We present a review of the disease,
the imaging findings and the current management.
It is important for the radiologist to understand the pathophysiological events present in the development of the biliary atresia and the imaging findings in order to communicate effectively the findings to the pediatric care team.
An early diagnosis can improve the quality of live and the survival rate of this patients.
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doi: 10.1097/MPG.0000000000000755. Nakamura H , Murase N , Koga H 3, Cazares J Lane GJ 3, Uchida H , Yamataka A .Classification ofbiliary atresiain the laparoscopic era. Pediatr Surg Int. 2016 Dec;32(12):1209-1212.
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