Preferred Name

Choledochal cyst

ID

http://www.orpha.net/ORDO/Orphanet_480501

alternative_term

Congenital cystic dilatation of the biliary tract

definition

A rare biliary tract disease characterized by congenital fusiform or cystic dilatation of intra- and/or extrahepatic bile ducts. Females are much more often affected than males. Clinical signs and symptoms include abdominal pain, jaundice, presence of a palpable abdominal mass, nausea, vomiting, or fever. Depending on the age of the patient, the condition may be complicated by stone formation, hepatomegaly, rupture with subsequent bile peritonitis, cholangitis, cholecystitis, biliary strictures, pancreatitis, or secondary biliary cirrhosis. The risk of malignancy, particularly cholangiocarcinoma, is significantly increased.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=480501

hasDbXref

ICD-10:Q44.4

ICD-11:LB20.20

label

Choledochal cyst

notation

ORPHA:480501

part_of

http://www.orpha.net/ORDO/Orphanet_506210

http://www.orpha.net/ORDO/Orphanet_101941

prefixIRI

ORDO:Orphanet_480501

prefLabel

Choledochal cyst

treeView

http://www.orpha.net/ORDO/Orphanet_506210

http://www.orpha.net/ORDO/Orphanet_101941

subClassOf

http://www.orpha.net/ORDO/Orphanet_377791

http://www.orpha.net/ORDO/Orphanet_557493

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