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 ofIf 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.
  • abdominal pain
  • jaundice
  • abdominal mass

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

Diagnosis

• ERCP is the most reliable investigation but is invasive
• MRCP is the best noninvasive investigation

Treatment

• 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.

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