 |
Journal of IMAB - Annual Proceeding (Scientific Papers)
Publisher: Peytchinski, Gospodin Iliev
ISSN: 1312-773X (Online)
Issue: Volume 15, book 1, 2009
Subject Collection: Medicine
Page: 66 - 68
DOI: 10.5272/jimab.1512009_66
Online date: Aug. 11, 2009,
BILE DUCT SYSTEM MALFORMATION - EMBRYOLOGICAL
AND PATHOLOGICAL ASSOCIATION. TREATMENT /REVIEW ARTICLE/
Ludmil
M. Veltchev1, Manol A. Kalniev2, Todor A. Todorov3
1) Fellow, Master’s Program in Hepatobiliary Pancreatic Surgery, Henri
Bismuth Hepatobiliary Institute, 12-14, avenue Paul Vaillant-Couturier,
94804 Villejuif Cedex; 2) Department of Anatomy, Cytology and
Histology, University of Medicine, Sofia, Bulgaria; 3) Department
of Pathology, University of Medicine, Sofia, Bulgaria.
ABSTRACT: Cystic diseases of the liver which are
in most cases hereditary, are related to an embryonic disorder know as
ductal plate malformation. These diseases correspond to partial or total
arrest of remodeling of the ductal plate, leading to more or less complete
persistence of the excess of embryonic biliary structures. The ductal
plate malformation may concern different segments of the intrahepatic
biliary tree (segmental bile ducts, interlobular bile ducts and the smallest
bile duct ramifications) leading to various pathoclinical entities Congenital
cystic lesions of bile ducts may affect intra or extrahepatic bile ducts.
Intrahepatic lesions include five entities: congenital hepatic fibrosis,
Caroli’s syndrome, von Meyenburg complexes, simple cyst of the liver and
polycystic liver disease. Congenital hepatic fibrosis and von Meyenburg
complexes are secondary to ductal plate malformation affecting the smallest
intrahepatic bile ducts.
Choledocal cysts, Caroli’s disease and Caroli’s syndrome belong to the
some family of congenital malformations of the large bile ducts (1). The
former affects the extrahepatic bile duct (including occasionally the
left and right branch of the hepatic duct) while the latter affects segmental
intrahepatic bile ducts. Both are extremely rare (in the order of 1:10.000
or 100.000 and 1:1.000.000 births respectively.
Key words: Caroli’s disease, biliary dilation, complications.
Page: 66-68; FULL TEXT PDF (282 KB)