Choledochal cyst

Orpha code: 480501OMIM code:

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.

Disease data
Classification

Morphological anomaly

Synonyms
Congenital cystic dilatation of the biliary tract
Wrodzone torbielowate poszerzenie przewodów żółciowych
ORPHA code
480501
OMIM code
-
ICD10 code
Q44.4
ICD11 code
LB20.20

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl