14 May, 2021

CONGENITAL CYSTIC DILATATIONS OF THE BILE DUCTS: ATTEMPT FOR MODIFICATION IN CLASSIFICATION

Gustavo Adrian NARI
Alesio LOPEZ
Angel JOSEPH
Flavia LOPEZ
Maria Eugenia DE-ELIAS
Lorna ROMERO
DOI: 10.1590/0102-672020190004e1573
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INTRODUCTION

Congenital cystic dilatations of the bile duct are infrequent affecting the intrahepatic bile duct as much as the extrahepatic or both at the same time3-4. Vater was who first described a congenital dilatation of the extrahepatic bile duct, but was Douglas’ description in 1852 that gave full knowledge about this rare affection17-19. On the other hand, Jacques Caroli described in 1958 the congenital cystic dilatation of the intrahepatic bile duct as an infrequent cause of intrahepatic cholestasis9,10,12. These dilatations are characterized by their polymorphism as the dilatations can affect any part of the bile duct. The rareness of this pathology has caused the majority of the studies to be clinical case reports and the importance of its knowledge lies in the fact that the treatment can be wrong.

The aim of the present communication is to present eight cases and revise the literature with the intention to evaluate if there have been changes in the therapeutic suggestions in recent years and suggest the aggregation of two subtypes in the classic classification.


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ABCD – BRAZILIAN ARCHIVES OF DIGESTIVE SURGERY is a periodic with a single annual volume in continuous publication, official organ of the Brazilian College of Digestive Surgery - CBCD.

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