Choledochal Cyst

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

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.