Biliary atresia in neonates and infants: triangular area of high signal intensity in the porta hepatis at T2-weighted MR cholangiography with US and histopathologic correlation

PURPOSE: To correlate a triangular area of high signal intensity in the porta hepatis on T2-weighted magnetic resonance (MR) cholangiograms of biliary atresia with ultrasonographic (US) and histopathologic findings in a portal mass observed during a Kasai procedure. MATERIALS AND METHODS: Twenty-one consecutive neonates and infants (age range, 13-88 days; mean age, 59 days) with cholestasis underwent US and single-shot MR cholangiography. In 12 patients with biliary atresia diagnosed at histopathologic examination, MR cholangiographic findings in the porta hepatis were correlated with US and histopathologic findings in the portal mass. RESULTS: At US, eight of the 12 patients had round, linear, or tubular hypoechoic portions within a triangular cord; MR cholangiography revealed a triangular area of high signal intensity confined to the porta hepatis. Histopathologic examination of the portal mass revealed a cystic or cleftlike lesion surrounded by loose myxoid mesenchyme and platelike fetal bile ducts. Neither the large cystic lesion without ductal epithelium nor the small cleftlike lesion with scanty epithelium demonstrated bile staining. Similar areas of high signal intensity were not seen on T2-weighted images in the remaining patients (four with biliary atresia and nine with neonatal hepatitis). CONCLUSION: In biliary atresia, T2-weighted single-shot MR cholangiography can show a triangular area of high signal intensity in the porta hepatis that may represent cystic dilatation of the fetal bile duct.