Ischaemic cholangiopathy and sickle cell disease.
We report a case of a 6-year-old girl of Afro-Caribbean origin, known to have sickle cell disease (SCD), with recurrent history of jaundice and abdominal pain. She was extensively investigated, including endoscopic retrograde cholangiopancreatography (ERCP), which revealed diffuse cholangiopathy of both extrahepatic and intrahepatic bile ducts. A pigtail stent was placed and balloon dilatation was performed for stricture of the extrahepatic duct. Since then, she remains well and asymptomatic. We suggest that cholangiopathy is the consequence of sickling in the end arteries of the biliary arterial tree.
Ahmed
Mansoor
M
Institute of Liver Studies, King' College Hospital, Denmark Hill, London, UK.
Dick
Moira
M
Mieli-Vergani
Giorgina
G
Harrison
Phil
P
Karani
John
J
Dhawan
Anil
A
D000755:Anemia, Sickle Cell / Q000150:complications*; D017734:Bile Ducts, Extrahepatic / Q000098:blood supply*; D002404:Catheterization; D002648:Child; D002779:Cholestasis / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D007511:Ischemia / Q000175:diagnosis* / Q000628:therapy; D015607:Stents