What is the management of choledochal cysts?

Management of Choledochal Cysts

Definitive Treatment

Complete surgical excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice for all resectable choledochal cysts to prevent malignant transformation and recurrent complications. 1

Treatment Rationale

• Incomplete excision or internal drainage procedures (cyst enterostomy) are inadequate because they leave residual cyst tissue that can undergo malignant transformation and cause recurrent symptoms 2, 3
• The risk of cholangiocarcinoma in choledochal cysts is 7.0%, with Type I (solitary extrahepatic) and Type IV (intra- plus extrahepatic) cysts having the greatest malignancy risk 1
• Over 90% of patients have an anomalous pancreaticobiliary duct junction (>1 cm proximal to the ampulla), allowing pancreatic enzyme reflux into the biliary system, contributing to inflammation and malignancy risk 1

Management Algorithm by Clinical Presentation

Uncomplicated Choledochal Cysts

• Proceed directly to complete cyst excision with Roux-en-Y hepaticojejunostomy 1, 2
• This can be performed via open or minimally invasive (laparoscopic) approach with acceptable morbidity and mortality 2

Complicated Choledochal Cysts (71% of adults)

Complications include cystolithiasis (49%), cholangitis (32%), acute pancreatitis (10%), hepatolithiasis (7%), malignancy (3%), portal hypertension (2%), and chronic pancreatitis (2%) 4

Staged management approach: 4, 5

1. Acute cholangitis with sepsis:

   • Perform endoscopic biliary drainage (ERCP with stent placement) or percutaneous transhepatic biliary drainage as temporizing measures 5
   • Stabilize with antibiotics (third-generation cephalosporins, ureidopenicillins, carbapenems, or fluoroquinolones) 6
   • Proceed to definitive cyst excision once infection resolves 4

2. Acute pancreatitis:

   • Manage conservatively with supportive care 4
   • Perform definitive surgery after acute episode resolves 4

3. Portal hypertension:

   • Consider endoscopic stenting as bridge to surgery 4
   • May require staged treatment approach 5

4. Spontaneous perforation:

   • Requires urgent surgical intervention with external drainage 5
   • Definitive cyst excision performed at same operation if patient stable, or staged if unstable 5

Diagnostic Workup

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

Surgical Technique Considerations

• Complete cyst excision is mandatory - partial excision leaves residual tissue at risk for malignancy 2, 3
• Roux-en-Y hepaticojejunostomy provides biliary-enteric continuity after cyst excision 1, 4
• Minimally invasive approaches are increasingly utilized with acceptable outcomes 2
• Patients should be referred to high-volume hepatopancreaticobiliary centers given the complex nature and limited experience at most institutions 2

Postoperative Complications

• Overall morbidity is 17.5% (wound infection 13%, bilioenteric anastomotic leak 7%) 4
• Anastomotic stricture occurs in approximately 2-3% and may require redo hepaticojejunostomy 4, 5
• Mortality is low (<1%) in contemporary series 4

Long-Term Surveillance Protocol

Despite complete excision, patients remain at slightly increased risk for biliary malignancy and require lifelong surveillance: 1

• Years 0-20 post-resection:

  • Liver function tests and CA19-9 annually 1
  • Ultrasound biannually 1

• After 20 years post-resection:

  • Liver function tests and CA19-9 every 2 years 1
  • Ultrasound every 3 years 1

• The malignancy risk is primarily concentrated in the first 20 years after resection 1

Critical Pitfalls to Avoid

• Never perform simple internal drainage (cyst enterostomy) as definitive treatment - this leaves cyst remnant at high risk for malignancy 2, 3
• Do not delay definitive surgery in complicated cases - temporize with drainage procedures but plan for complete excision 4, 5
• Complicated cysts (especially Type IV-A) require careful preoperative planning and may necessitate staged procedures 5
• Ensure complete anatomic delineation with MRCP before surgery to plan appropriate reconstruction 1, 7