From the Guidelines
Regular surveillance imaging is strongly recommended for a 65-year-old female with Caroli disease and multiple right lobe liver cysts to prevent significant morbidity and mortality associated with cholangiocarcinoma and other complications. The risk of developing cholangiocarcinoma, which occurs in patients with Caroli disease, highlights the importance of monitoring, as pre-operative detection of this cancer is difficult in patients with abnormal anatomy associated with biliary dilatation 1. According to the EASL clinical practice guidelines, patients with Caroli disease or syndrome may undergo surveillance for cholangiocarcinoma using cholangiopancreaticography every 12 months after diagnosis 1.
Risks of Not Undergoing Surveillance Imaging
- Development of cholangiocarcinoma, with recurrence rates of up to 75% reported for patients with Caroli disease or syndrome who underwent liver resection or transplantation 1
- Recurrent cholangitis (bile duct infections)
- Sepsis
- Liver abscess formation
- Progressive liver dysfunction leading to cirrhosis and liver failure
- Cyst complications such as hemorrhage, rupture, or significant growth could occur undetected
Recommended Surveillance Protocol
- MRI or ultrasound imaging every 6-12 months, depending on the patient's specific risk factors and disease characteristics
- Regular liver function tests
- Monitoring for signs of infection or biliary obstruction
The disease's progressive nature means that without surveillance, serious complications may develop silently until they become advanced and more difficult to treat. Early detection of complications or malignant transformation through regular surveillance imaging allows for timely intervention, which can be life-saving 1.
From the Research
Risks of Not Undergoing Surveillance Imaging
The risks of not undergoing surveillance imaging in a 65-year-old female with a history of numerous liver cysts in the right lobe diagnosed with Caroli's disease include:
- Development of cholangitis, which can lead to fibrosis of the liver, hepatolithiasis, portal hypertension, and cholangiocarcinoma 2
- Increased risk of hepatolithiasis, which can cause recurrent attacks of cholangitis and potentially lead to more severe complications 2, 3
- Potential development of cholangiocarcinoma, which is associated with Caroli's disease in 7% of cases 4
- Progression of the disease, which can lead to diffuse forms of Caroli's disease, making treatment more challenging 3
- Increased risk of portal hypertension and esophageal varices, which can be life-threatening if left untreated 2
Importance of Surveillance Imaging
Surveillance imaging is crucial in patients with Caroli's disease to: