What is the recommended treatment for children diagnosed with a Choledochal cyst?

Management of Choledochal Cysts in Children

Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy is the definitive treatment for children with choledochal cysts to prevent malignant transformation and other complications. 1

Diagnosis and Classification

• Choledochal cysts are congenital dilations of the biliary tree classified according to the Todani classification
• Higher prevalence in Asian populations (1:13,000 in Japan vs 1:100,000 in Western countries) 1
• Diagnostic approach:
  • Ultrasound as first-line imaging modality
  • MRCP (Magnetic Resonance Cholangiopancreatography) is the gold standard when available 1
  • CT scan may be used as an alternative imaging modality 2

Surgical Management

Recommended Procedure

• Complete excision of the extrahepatic portion of the cyst with Roux-en-Y hepaticojejunostomy is the standard of care 1, 3
• This approach:
  • Removes the risk of malignant transformation
  • Prevents recurrent cholangitis and other complications
  • Provides long-term resolution of symptoms 3

Surgical Approaches

1. Open surgical approach:

   • Traditional method with excellent outcomes
   • Complete cyst excision with biliary reconstruction

2. Laparoscopic approach:

   • Increasingly used with good outcomes
   • Feasible even in symptomatic neonates 4
   • Benefits include:
     • Minimized surgical trauma
     • Faster recovery
     • Shorter hospital stay (median 6 days) 4

3. Robotic-assisted approach:

   • Emerging technology showing promise
   • Can facilitate precise dissection and anastomosis 5

Technical Considerations

• Anastomosis must be performed on healthy, non-ischemic, non-inflamed bile duct tissue 1
• Proper exposure of proximal and distal healthy bile duct is critical for successful repair 1
• For Roux-en-Y hepaticojejunostomy:
  • The jejunum can be exteriorized through an extended umbilical port site for extracorporeal creation of the Roux limb 4
  • End-to-side anastomosis between the common hepatic duct stump and Roux loop is performed 4

Postoperative Care and Complications

Early Complications (5.3% rate) 3

• Anastomotic leakage
• Wound infection
• Bleeding 1

Late Complications

• Cholangitis
• Anastomotic stricture
• Intrahepatic stone formation
• Malignancy risk (primarily in the first 20 years after resection) 1

Long-term Surveillance

• Essential due to ongoing malignancy risk (approximately 5.6% even after resection) 1
• Recommended monitoring:
  • Liver function tests annually for 20 years
  • CA19-9 annually
  • Ultrasound biannually for 20 years 1

Special Considerations

• For Type IV choledochal cysts (multiple cystic dilations):

  • Complete excision of extrahepatic portion with Roux-en-Y hepaticojejunostomy remains the standard approach 1
  • For Caroli disease (Type V), liver transplantation or hepatectomy may be required 3

• For neonates with symptomatic choledochal cysts:

  • Early intervention is recommended to prevent complications
  • Laparoscopic approach is feasible and safe
  • Recovery is typically uneventful with normalization of liver function tests within 3-16 weeks postoperatively 4

Alternative Approaches

While some studies have explored alternative biliary reconstructions such as hepaticojejunostomy without Roux-en-Y 2, the standard Roux-en-Y hepaticojejunostomy remains the preferred and most widely validated approach for children with choledochal cysts 1, 3, 6.

Outcomes

• No mortality reported in large case series 3
• Good long-term results with complete excision and Roux-en-Y hepaticojejunostomy 3
• Long-term follow-up is necessary to monitor for potential complications 3