@hemo_shk @IhabFathiSulima Caroli disease is a rare congenital disorder characterized by segmental,multifocal dilatation of the intrahepatic bile ducts. It is caused by a developmental abnormality in the ductal plate of the liver,leading to saccular or fusiform dilatations of the intrahepatic biliary tree
@hemo_shk @IhabFathiSulima Types:
1.Simple Caroli disease: Involves bile duct dilation without other abnormalities.
2.Caroli syndrome: Associated with congenital hepatic fibrosis, leading to portal hypertension and complications such as esophageal varices.
@hemo_shk @IhabFathiSulima Clinical Presentation:
•Recurrent cholangitis (infection of the bile ducts)
•Right upper quadrant abdominal pain
•Jaundice
•Hepatomegaly may be present in advanced cases
•Portal hypertension and splenomegaly are more common in Caroli syndrome
@hemo_shk @IhabFathiSulima Complications:
•Recurrent biliary infections
•Intrahepatic stone formation
•Cholangiocarcinoma (increased risk)
•Liver abscesses and sepsis
@hemo_shk @IhabFathiSulima Diagnosis:
•Imaging studies: Ultrasound, CT, or MRI can reveal dilated intrahepatic bile ducts. MRI with MR cholangiopancreatography (MRCP) is particularly useful in visualizing the extent of bile duct involvement.
•ERCP : May be used for both diagnosis and treatment.
@hemo_shk @IhabFathiSulima Treatment:
•Medical management: Antibiotics for cholangitis,ursodeoxycholic acid to improve bile flow.
•Interventional procedures: Endoscopic stone removal,biliary drainage.
•Surgical options:Liver resection for localized disease,liver transplantation
@hemo_shk @IhabFathiSulima Prognosis:
Prognosis varies depending on the presence and severity of complications such as recurrent infections, portal hypertension, and the development of cholangiocarcinoma. Regular monitoring is essential.
#يحيى_السنوار
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