Choledochal cysts

Choledochal cysts are congenital conditions involving cystic dilatation of bile ducts.^[1] They are uncommon in western countries^[2] but not as rare in East Asian nations like Japan and China.

Signs and symtoms

Most of them present in 1st year of life; adult presentation is rare and usually at this stage is associated with complication . Classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass is found only in minority of patients.

Diagnosis

Direct Hyperbilirubinemia

Types

They were classified into 5 types by Todani in 1977.^[3]

Classification was based on site of the cyst or dilatation. Type I to IV has been subtyped.

• Type I: Most common variety (80-90%) involving saccular or fusiform dilatation of a portion or entire common bile duct (CBD) with normal intrahepatic duct.
• Type II: Isolated diverticulum protruding from the CBD.
• Type III or Choledochocele: Arise from dilatation of duodenal portion of CBD or where pancreatic duct meets.
• Type IVa: Characterized by multiple dilatations of the intrahepatic and extrahepatic biliary tree.
• Type IVb: Multiple dilatations involving only the extrahepatic bile ducts.
• Type V or Caroli's disease: Cystic dilatation of intra hepatic biliary ducts.

Treatment

Choledochal cysts are treated by surgical excision of the cyst with the formation of a roux-en-Y anastomosis to the biliary duct. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree.

References