From the Guidelines
The recommended approach for this 22-year-old female with elevated alkaline phosphatase and imaging findings consistent with a choledochal cyst (fusiform dilatation of the distal biliary duct) would be complete surgical excision of the biliary system. This is the definitive treatment for choledochal cysts due to the significant risk of malignant transformation if left untreated 1. The patient's presentation with asymptomatic elevation of alkaline phosphatase, normal bilirubin, and minimal elevation of AST, along with the characteristic imaging findings on ultrasound and MRCP, strongly suggests a type I choledochal cyst. While ERCP with sphincterotomy with or without stent placement might temporarily relieve any obstruction, it does not address the underlying abnormality and associated cancer risk 1. Surgical management typically involves complete excision of the cyst with reconstruction via hepaticojejunostomy to restore biliary drainage. This approach eliminates the risk of malignancy developing in the abnormal biliary epithelium and provides definitive treatment. Some key points to consider in the management of choledochal cysts include:
- The risk of malignant transformation is significant, and surgical excision is the only way to eliminate this risk 1
- ERCP with sphincterotomy and stent placement may be used as a temporary measure to relieve obstruction, but it is not a definitive treatment 1
- Surgical excision of the cyst with reconstruction via hepaticojejunostomy is the recommended treatment for choledochal cysts 1 Observation with repeat imaging in 6 months would be inappropriate given the established diagnosis and known malignancy risk of these congenital anomalies. The most recent and highest quality study recommends surgical excision of the cyst in patients with choledochal cysts, especially those with an anomalous pancreaticobiliary junction 1. Therefore, complete surgical excision of the biliary system is the recommended approach for this patient.
From the Research
Diagnosis and Management of Choledochal Cysts
The patient's symptoms and laboratory results suggest the presence of a choledochal cyst, which is a rare cystic dilatation of the intrahepatic and/or extrahepatic bile ducts 2. The diagnosis is supported by the MRCP showing fusiform dilatation of the distal biliary duct.
Treatment Options
The treatment options for choledochal cysts depend on the type and classification of the cyst. Complete cyst excision is necessary for choledochal cysts with high potential of malignant transformation, such as types I and IV 2.
Role of ERCP
ERCP with endoscopic sphincterotomy (ES) and stone removal is a valuable therapeutic modality in choledocholithiasis with jaundice, dilated common bile duct, acute pancreatitis, or cholangitis 3. However, in patients with choledochal cysts, ERCP may not be the first line of treatment, and surgical intervention may be necessary to mitigate the risk of cyst-related complications such as cholangitis and malignant transformation 2.
Recommended Follow-up
Based on the evidence, the recommended follow-up for the patient would be:
- Complete cyst excision, as the patient has a type of choledochal cyst that requires surgical intervention 2
- Avoidance of unnecessary ERCP, as the patient does not have a low likelihood of biliary stone or stricture 3
- Surveillance for dysplasia should be considered for lesions that are not resected 4
Key Points to Consider
- Choledochal cysts are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts 2
- Complete cyst excision is necessary for choledochal cysts with high potential of malignant transformation 2
- ERCP with ES and stone removal is a valuable therapeutic modality in choledocholithiasis with jaundice, dilated common bile duct, acute pancreatitis, or cholangitis 3
- MRCP provides information equivalent to that provided with ERCP, without potential complications, for the preoperative assessment of choledochal cysts 5