Management of Choledochal Cysts
Complete surgical excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy is the recommended treatment for patients with choledochal cysts to prevent malignant transformation and recurrent complications. 1, 2
Understanding Choledochal Cysts
Choledochal cysts are congenital malformations of the biliary system with prevalence varying from 1:100,000 in Western countries to 1:13,000 in Japan 1. They are categorized according to the Todani classification based on location, shape, and multiplicity 1.
Clinical Presentation
- Patients may present with jaundice (84-90%), weight loss (35%), abdominal pain (30%), nausea and vomiting (12-25%), and fever (10%) 1
- Many patients are asymptomatic and diagnosed incidentally 1
- Cholangitis is a common presentation in patients with biliary tract malignancy complicating choledochal cysts 3
Diagnostic Approach
Imaging Studies
- Contrast-enhanced MRI with magnetic resonance cholangiopancreatography (MRCP) is superior for accurate assessment of biliary obstruction 1
- Contrast-enhanced CT is an alternative but less accurate than MRI/MRCP 1
- Ultrasound is often the first imaging modality but has limitations in fully characterizing the cyst 1
Treatment Algorithm
Primary Treatment
- Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice 1, 2, 4
- This approach prevents potential malignant transformation and recurrent complications 3, 2
- The procedure can be performed with low operative morbidity and absence of long-term complications 3
Surgical Considerations
- Resection should include the entire cyst to prevent malignant transformation in any remnant tissue 2
- In complex cases, the resection can be done from inside the cyst to minimize risk of injury to neighboring vascular structures 4
- Minimally invasive approaches are increasingly being used with acceptable morbidity and mortality 2
Alternative Approaches
- Simple internal drainage procedures (cystoenterostomy) should be reserved only for highly specific indications such as severe liver disease or patients unfit for major surgery 4
- These procedures have a high incidence of late complications and should not be considered first-line treatment 3, 4
Post-Treatment Management and Surveillance
Follow-up Protocol
- Patients with choledochal cysts should undergo surveillance after resection due to the risk of metachronous cancer 1
- Recommended follow-up includes liver function tests and CA19-9 annually for 20 years (then biannually) 1
- Ultrasound should be performed biannually for 20 years (then every 3 years) 1
Special Considerations
Malignancy Risk
- Patients with a history of internal drainage of choledochal cyst are at higher risk for developing biliary tract malignancy 3
- Even after complete excision, patients may remain at a slightly increased risk of biliary malignancy 2
- The risk of malignancy appears primarily limited to the first 20 years after resection 1
Complex Cases
- Patients with associated biliary malformations or hepatic involvement may require more extensive procedures 4
- In some cases, hemihepatectomy may be necessary in addition to cyst excision 3
- Due to the complex nature of choledochal cysts, treatment should be performed at high-volume hepatopancreaticobiliary centers 2