Congenital dilatation of the intrahepatic ducts
(Caroli's disease)

m Aetiology and Pathology:
· This rare, congenital, nonfamilial condition is characterised by multiple irregular sacular dilatations of the intrahepatic ducts separated by segments of normal or stenotic ducts. Biliary stasis leads to stone formation and cholangitis.
m Clinical Picture:
· The patients present in childhood or in early adult life with jaundice. Associated conditions include congenital hepatic fibrosis, medullary sponge kidney and, rarely, cholangiocarcinoma.
m Treatment:
· Antibiotics for the cholangitis and the removal of calculi. As the condition can be limited to one lobe of the liver, lobectomy may be indicated.
Choledochal Cyst
m Aetiology and Pathology:
· Choledochal cyst is due to a specific weakness in a part of or the whole of the wall of the common bile duct. Anomalous junctions of the biliary pancreatic junction are frequently observed. Common pancreato­biliary channels may be associated with repeated attacks of pancreatitis.
m Clinical picture:
· Patients may present at any age, with attacks of jaundice of obstructive type and cholangitis. In some patients swelling may be detected in the upper abdomen. It is premalignant condition for carcinoma of the biliary tract and carry bad prognosis.
mInvestigations:
· Ultrasonography confirms the presence of the cyst.
· MRCP: can reveal the anatomy in relation to the pancreatic duct.

m Treatment:
· Radical excision of the cyst with reconstruction of the biliary tract using Roux-en-Y loop of jejunum.
SOURCE: DR. AYMAN SALEM'S BOOK
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