0 There are 5 types according to their cholangiographic images:
Type 1: Fusiform or saccular dilatation of the extrahepatic biliary tract
Type 2: Diverticular dilatation of extrahepatic bile ducts
Type 3: Choledochocele (dilatation of the common bile duct within the duodenum)
Type 4a: Multiple cysts in the extrahepatic and intrahepatic bile ducts
Type 4b Multiple cysts in extrahepatic bile ducts
Type 5: Single or multiple intrahepatic cysts
0 If there is hepatic fibrosis together with intrahepatic bile duct cysts in type 5, it is called "Caroli's disease".
Type 1 Caroli's Disease: Localized dilatations of the intrahepatic ducts are in contact with the intrahepatic bile ducts, although they are adequately drained; The incidence of cholangitis, abscess and stone is high.
Type 2 Caroli's Disease, Caroli's syndrome: There are hepatic fibrosis, portal hypertension and kidney disease with cholangitis.
0 Intrahepatic bile cysts that are not associated with the bile ducts are called Meyenberg's disease.
0 75% of cysts are Type I.
Clinic
• It can be recognized by antenatal ultrasound. Type lC cysts are the earliest detectable cysts by fetal ultrasound.
• There are two types as adult and infant.
• Infant type; obstructive jaundice, acholic stool, and hepatomegaly.
• Adult type; It stays quiet until 2 years old. There is recurrent jaundice, right upper quadrant pain, and a right upper quadrant mass. There are hepatomegaly, splenomegaly, hypersplenism and portal hypertension. If left untreated, he may die from complications such as biliary cirrhosis, bleeding from portal hypertension, cyst rupture, and pancreatitis.
• Stones can be seen with cysts and almost all are pigment stones.
• There is a close relationship between choledochal cysts and biliary tract carcinomas.
Diagnosis
• The first imaging method to be performed is ultrasonography of the biliary tract.
• Hepatobiliary scintigraphy also has an important place in the diagnosis.
• PTC, ERCP, CT, MRI
• Oral cholecystography and cholangiography are not used much anymore.
Treatment
• Cystectomy and hepaticoenterostomy are performed.
• It is operated under elective conditions, except for cyst perforation.
• Early complications; anastomotic leakage, bleeding, acute pancreatitis, pancreatic fistula, adhesive intestinal obstruction.
• late complications; recurrent cholangitis and internal drainage, malignancies.
