Choledochal cysts

Choledochal cysts are congenital conditions associated with benign cystic dilatations of the bile ducts. They are uncommon in the Western countries but are relatively common in East Asian countries. They can occur at any age but are more common in children and females.

Etiology

The exact cause of choledochal cysts is unknown. However, it is believed that an abnormal connection between the pancreatic duct and the common bile duct at an early stage of embryonic development may be responsible. This abnormal connection may allow pancreatic juice to reflux into the bile duct, leading to inflammation and dilation of the bile duct.

Classification

Choledochal cysts are classified into five types according to the Todani classification:

• Type I: Cystic dilatation of the common bile duct
• Type II: Diverticulum of the common bile duct
• Type III: Choledochocele (cystic dilatation of the intraduodenal portion of the common bile duct)
• Type IV: Multiple cysts involving both intrahepatic and extrahepatic bile ducts
• Type V: Multiple cysts involving only intrahepatic bile ducts (Caroli's disease)

Clinical Presentation

The classic triad of symptoms for choledochal cysts includes abdominal pain, jaundice, and an abdominal mass. However, this triad is only present in a minority of patients. Other symptoms may include nausea, vomiting, and pancreatitis.

Diagnosis

The diagnosis of choledochal cysts is usually made by imaging studies such as ultrasound, computed tomography (CT) scan, magnetic resonance imaging (MRI), and magnetic resonance cholangiopancreatography (MRCP). Endoscopic retrograde cholangiopancreatography (ERCP) can also be used for diagnosis and treatment.

Treatment

The treatment of choice for choledochal cysts is surgical removal of the cyst (cystectomy) with reconstruction of the bile ducts. This is usually done by a Roux-en-Y hepaticojejunostomy. The prognosis after surgery is generally good, but there is a risk of complications such as cholangitis, liver cirrhosis, and cholangiocarcinoma.

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