Choledochal Cyst

  • A choledochal cyst is an isolated or combined congenital dilatation of the extrahepatic or intrahepatic biliary tree.
  • Classification of Choledochal cyst
  • Type I - a type I cyst is characterized by fusifonn dilatation of the bile duct.- this is the most common type
  • Type II - a type II cyst appears as a diverticulum protruding from the wall of the CBD.
  • Type III - a type III cyst is dilatation of the biliary tract within the duodenum; also k/a choledochocele Type IV
  • Type IV A - multiple dilatation of the intrahepatic and extrahepatic bile ducts.
  • Type IV B - multiple dilatation involving only the extrahepatic bile ducts.
  • Type V - consists of multiple dilatation of the intrahepatic bile ducts; also known as Carolis disease.
Clinical Features:
  • more common in females than in males (4:1)
  • typically choledochal cysts present in children beyond the toddler age group.
  • the classical triad presents only in a minority of patients; it consists of
  • abdominal pain
  • jaundice
  • abdominal mass
  • If the disorder is left undiagnosed, patients may develop cholangitis or pancreatitis. Cholangitis may lead to the development of cirrhosis and portal hypertension.
  • Choledochal cysts are seen to be frequently associated with an anomalous junction of the pancreatic and biliary duct.
Complications caused by choledochal cyst
  • recurrent cholangitis
  • pancreatitis
  • gall stones
  • cirrhosis with portal hypertension
  • portal vein thrombosis with resultant portal hypertension
  • cyst rupture with biliary peritonitis (very rarely)
  • malignancy:
  • Choledochal cyst is a risk factor for the following cancers :biliary tree (most common) ,liver ,gall bladder ,duodenum pancreas
  • ERCP is the most reliable investigation but is invasive
  • MRCP is the best noninvasive investigation
  • Cholecystectomy + Cyst excision with reconstruction via a biliary-enteric Roux-en-Y anastomosis is the treatment of choice for all choledochal cysts except type III (choledochocele) and intrahepatic cysts of type IV & type V
  • (Caroli's disease)
  • Treatment of type III (Choledochocele)
  • transduodenal sphincteroplasty or sphincterotomy - cyst excision
  • Treatment of intrahepatic cysts of type IV & V
  • if the intrahepatic cysts are localized, hepatic lobe is resected
  • if the intrahepatic cysts are diffuse only effective treatment is liver transplantation.
Exam Question
  • Choledochal Cyst is associated with anomalous junction of the pancreatic and biliary duct.
  • Surgical removal is the treatment of choice in a case of Choledochal Cyst.
  • Choledochal cyst if ruptures ,can cause biliary peritonitis.
  • Type I is the most common.
  • In case of choledochal cyst, side-to-side choledochoduodenostomy is NOT recommended because of the risk of recurrent cholangitis.
  • Conjugated hyperbilirubinemia in infancy may be seen in Choledochal Cyst.
  • Choledochal Cyst presents with Pain, lump and progressive jaundice.
  • Saccular diverticulum of extrahepatic bile duct in choledochal cyst is classified as Type-II.
  • Choledochal Cyst may predispose to Cholangiocarcinoma.
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