Choledochal cysts

GI Joe Gastroenterology Rounds

Choledochal Cysts

1. Classification and Description of Choledochal Cysts

Choledochal cysts are classified into five main types, with variations within Type I and Type IV. The classification dictates the typical appearance and influences management strategies.

• Type IA is the most common type, accounting for 50-85% of cases. It involves "cystic dilation of the entire extrahepatic biliary tree, sparing the intrahepatic bile ducts."
• Type II are "true diverticulum of the extrahepatic bile duct" and are relatively rare (2%).
• Type III, known as "choledochocele," involves "dilation of the distal CBD limited to the intraduodenal part of the CBD" (1-5%).
• Type IVa involves "multiple intra- and extrahepatic bile duct dilations (cystic or fusiform)" (15-35%).
• Type IVb involves "multiple dilations of the extrahepatic biliary tree."
• Type V, or "Caroli disease," refers to "multiple dilations of the intrahepatic bile ducts" (20%). Caroli syndrome combines Caroli disease with congenital hepatic fibrosis and is often associated with polycystic kidney disease.

2. Clinical Manifestations

Patients with choledochal cysts typically present with a triad of symptoms:

• Abdominal pain
• Recurrent cholangitis
• Obstructive jaundice

The presence of "CBD dilation without evidence of an obstructing stone or mass" should prompt consideration of choledochal cysts.

3. Management Strategies

Management varies significantly based on the type of cyst, with surgical excision being a common approach due to malignancy risk.

• For Type IA, IB, IC, IVA, and IVB, the primary management is complete excision and hepaticojejunostomy. Segmental hepatectomy may be considered for localized intrahepatic involvement in Type IVA.
• For Type II, "Simple cyst excision" is typically sufficient.
• For Type III, "Sphincterotomy" is the recommended treatment.
• For Type V (Caroli disease), "segmental hepatectomy if localized" is an option, with "Liver transplantation" considered for diffuse disease.

4. High Risk of Malignancy

A critical aspect of choledochal cysts is their significant association with malignancy, particularly cholangiocarcinoma.

• There is an increased risk of malignancy in choledochal cysts type 1, 4, and 5.
• The most common cancer is cholangiocarcinoma. Other associated malignancies include anaplastic, undifferentiated, and squamous cell carcinoma.
• The overall lifetime risk of malignancy is 10-15%, which represents a "20-30-fold increase compared to the general population."
• The risk of malignancy increases with increasing age.
• Crucially, Type II and Type III cysts are associated with a low risk of malignancy.

5. Association with Abnormal Pancreaticobiliary Junction (APBJ)

Many choledochal cysts are linked to an abnormal pancreaticobiliary junction, which is a key contributor to the increased malignancy risk.

• In this anomaly, "The pancreatic duct drains into the bile duct, leading to pancreatic reflux and chronic inflammation."
• This reflux and inflammation "increases the risk of malignancy of the bile duct and gallbladder."