What is the management of choledochal cysts?

Management of Choledochal Cysts

Definitive Treatment

Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice and should be performed in all patients to prevent malignant transformation and recurrent complications. 1

This recommendation is based on American Association for the Study of Liver Diseases guidelines and represents the standard of care, as incomplete excision or simple drainage procedures result in unacceptable rates of recurrent symptoms and malignant transformation in the cyst remnant. 1, 2

Initial Diagnostic Workup

• Contrast-enhanced MRI with MRCP is the superior imaging modality for accurate assessment of biliary anatomy and cyst characterization 1, 3
• Contrast-enhanced CT is an acceptable alternative but less accurate than MRI/MRCP 1
• Ultrasound is often the initial imaging but has significant limitations in fully characterizing the cyst 1

Management of Acute Complications

Before proceeding to definitive surgery, certain complications require stabilization:

• Acute cholangitis with sepsis: Stabilize with antibiotics (third-generation cephalosporins, ureidopenicillins, carbapenems, or fluoroquinolones) before cyst excision 1
• Severe cholangitis: Perform endoscopic, percutaneous, or external surgical biliary drainage prior to definitive surgery 4, 5
• Portal hypertension: Manage conservatively with staged treatment using endoscopic stenting before definitive surgery 4
• Acute pancreatitis: Manage conservatively before proceeding to surgery 4

Adults present with complications in 71% of cases (versus 33% in children), with cystolithiasis (49%), cholangitis (32%), and acute pancreatitis (10%) being most common. 4

Type-Specific Surgical Approach

Type I and Type II Cysts (Extrahepatic Only)

• Complete excision of the extrahepatic cyst and gallbladder with Roux-en-Y hepaticojejunostomy 1, 6

Type IV Cysts (Intrahepatic and Extrahepatic)

• Complete excision of the extrahepatic component with Roux-en-Y hepaticojejunostomy PLUS extended right or left hepatectomy is recommended 3
• En bloc resection of extrahepatic bile ducts and gallbladder 3
• Regional lymphadenectomy 3
• Segmental or lobar liver resection based on extent of intrahepatic involvement 3
• Consider removal of segment 1 of the liver as it may preferentially harbor metastatic disease 3

Type IV cysts are significantly more associated with complications and require more extensive resection. 5

Preoperative Staging (Especially for Type IV)

• Chest radiography 3
• CT abdomen (unless abdominal MRI/MRCP already performed) 3
• Laparoscopic exploration may be needed to determine presence of peritoneal or superficial liver metastases 3

Surgical Approach Considerations

• Minimally invasive (laparoscopic) approaches are acceptable with comparable morbidity and mortality to open surgery 2, 7
• Laparoscopic excision with four-port technique has been successfully performed with mean operative time of 228 minutes and hospital stay of 5.8 days 7
• Referral to high-volume hepatopancreaticobiliary centers is strongly recommended given the complex nature and limited experience at most centers 2

Expected Surgical Outcomes

• Overall morbidity: 17.5% (wound infection 13%, bilioenteric anastomotic leak 7%) 4
• Anastomotic stricture requiring redo hepaticojejunostomy: approximately 2-3% 4
• Mortality: <1% in contemporary series 4

Post-Resection Surveillance Protocol

Long-term surveillance is mandatory due to persistent risk of metachronous cancer:

• Liver function tests and CA19-9 annually for 20 years, then biannually 1, 3
• Ultrasound biannually for 20 years, then every 3 years 1, 3
• The malignancy risk is primarily limited to the first 20 years after resection 1, 3

This intensive surveillance is justified because the incidence of cholangiocarcinoma in choledochal cysts is 7.0%, and patients remain at slightly increased risk even after complete excision. 1, 2

Critical Pitfalls to Avoid

• Never perform simple cyst enterostomy or internal drainage alone - this results in recurrent symptoms and malignant transformation in the cyst remnant 2, 6
• Inadequate biliary drainage increases risk of sepsis and compromises surgical outcomes 3
• Failure to excise the gallbladder increases cancer risk 7
• Type IV cysts require hepatectomy in addition to extrahepatic excision - extrahepatic excision alone is inadequate 3