[ XXXX : congenital cystic and segmentary dilatation of the intrahepatic bile ducts].

@entity1 @entity82 @entity101 @entity2695 @entity600 @entity281 @entity7872 @entity8767 @entity13550 @entity9279
Congenital cystic segmentary dilatation of the intrahepatic biliary ducts is a rare disease. Although several cases has been described in medical literature, its occurrence such as it was originally referred is exceptional. Some cases coexist with @entity7872 and portal @entity101 . A characteristic phenomenon is the formation of stones and canalicular @entity281 . In this article, three cases of @entity13550 (females, 57, 53 and 58 years old) are described. Case n 1 was a complex @entity600 including: a) cystic and segmentary dilatations of the intrahepatic biliar ducts; b) intra and @entity9279 and recurrent infections; c) fusion of the choledocus and gallbladder in a common sac; d) hepatic cavernosus @entity2695 and a supernumerarious lobe. Case n 2 showed: a) cystic and segmentary dilatation of the intrahepatic biliar ducts; b) portal @entity82 ; c) @entity8767 ; d) portal @entity101 . Case n 3 showed: a) cystic and segmentary dilatation of the intrahepatic biliar ducts; b) portal @entity82 ; c) @entity8767 and spleen; d) portal @entity101 . Prognostic of this @entity1 is variable and depends on the recurrent infections and the coexistence of other @entity600 which are frequently severe. Surgery is the only treatment for the disease although there are no definite conclusions concerning the long-term results.
