Intrahepatic cystic biliary dilatation constitutes a significant prognostic factor in patients with primary sclerosing cholangitis

Abstract

• Cystic dilatation of the intrahepatic biliary ducts affects young patients with primary sclerosing cholangitis and is characterized by a markedly variable radiological evolution. • Biliary wall inflammation, found in explanted livers, could be a key feature in the pathogenesis of cystic dilatation. • Cystic dilatation of the intrahepatic biliary ducts is characterized by an unfavorable course and constitutes a significant prognostic factor of primary sclerosing cholangitis.

Primary Sclerosing Cholangitis: Diagnostic Criteria

“…Lastly, the occurrence of biliary cystic dilatation (illustrated in Figure 3 ) within the intrahepatic bile ducts was recorded in 21 patients diagnosed with PSC [ 41 , 42 , 43 , 44 , 45 , 46 , 47 ] as well as in five livers that underwent explantation [ 48 ].…”

“…However, these findings have yet to be validated in external cohorts [39,40]. Lastly, the occurrence of biliary cystic dilatation (illustrated in Figure 3) within the intrahepatic bile ducts was recorded in 21 patients diagnosed with PSC [41][42][43][44][45][46][47] as well as in five livers that underwent explantation [48]. Lastly, the occurrence of biliary cystic dilatation (illustrated in Figure 3) within the intrahepatic bile ducts was recorded in 21 patients diagnosed with PSC [41][42][43][44][45][46][47] as well as in five livers that underwent explantation [48].…”

Imaging of the biliary tract

“…PSC affects only the intrahepatic bile ducts in 20-30% of cases and only the extra-hepatic bile ducts in 10% of cases [45,46]. Several phenotypes of PSC can be distinguished on imaging, such as, for example, slightly progressive forms versus rapidly progressive forms, stenosing forms versus destructive inflammatory forms producing cystic dilatations of the bile ducts [47].…”