Case Presentation: A 78 year old female with history of previous cholecystitis s/p cholecystectomy in 1950s complicated by recurrent choledocholithiasis and cholangitis presented with a 1 day history of chills, epigastric abdominal pain, nausea and vomiting. Patient had recurrent episodes almost yearly, characterized by these symptoms. Similar to prior episodes, labs were indicative of biliary obstruction and active infection.The patient was diagnosed with acute cholangitis and treated with IV antibiotics. A CT scan of the abdomen and pelvis showed intrahepatic and extrahepatic biliary dilatation, a CBD dilatated to 2.7cm, but with extensive pneumobilia. MRCP showed similar findings in addition to narrowing of the CBD just prior to the duodenal insertion suggesting possibility of a stricture. Subsequent EUS showed CBD dilation to 20mm. An ERCP showed dilatation in the middle third of the main bile duct; consistent with a Type I choledochal cyst (CDC).There was no evidence of abnormal pancreaticobiliary junction (APBJ), however the patient was s/p sphincterotomy from past procedures. The patient elected to undergo extrahepatic bile duct excision and roux-en-y hepaticojejunostomy and did well.
Discussion: Our case highlights the need to suspect CDC as the etiology in patients who are s/p cholecystectomy. Biliary stasis within the cyst itself may be the cause of recurrent cholangitis, gallstone pancreatitis and choledocholithiasis in this patient population. Choledochal cysts are rather rare, with studies suggesting a rate of 1:100,000 in western populations. Most are diagnosed during childhood with only about 20% of cases detected in adult patients. ERCP has a 100% sensitivity for CDC although a number of other imaging modalities may be used in diagnosis including MRCP, ultrasound, CT scan. Cysts are classified based on location and fall into generally fall into one of five types.Type I CDCs are also highly associated with APBJ, however because the length of the common channel can be variable it may be obliterated or difficult to assess after a prior sphincterotomy.It is critical to recognize and characterize CDCs as type I and type IV as these are highly are associated with cholangiocarcinoma. Studies suggest that there is a 20-30 fold increase in risk when compared to the general population; this risk further increases with age. Recommended treatment for type I and type IV cysts is complete surgical resection to reduce rate of both infectious complications and malignancy.
Conclusions: Choledochal cyst may be the etiology of recurrent choledocholithiasis, cholangitis or gallstone pancreatitis. A high degree of suspicion is required to diagnose choledochal cysts as they are rare and generally more prevalent in the pediatric population. Diagnosis is critical, however, as it may be associated with increased risk for malignancy and warrant surgical treatment.