Caroli Disease, also known as Caroli Syndrome or Caroli’s disease, is a rare congenital disorder characterized by the presence of dilated intrahepatic bile ducts within the liver. This condition is named after the French physician Jacques Caroli, who first described it in the mid-20th century.
In individuals affected by Caroli Disease, the bile ducts within the liver become abnormally widened and filled with stagnant bile, leading to the formation of cystic structures. These cysts can vary in size and number, and their presence can impair the normal flow of bile, resulting in recurrent episodes of cholangitis (inflammation of the bile ducts) and liver abscesses. Furthermore, over time, chronic inflammation and scarring of the liver can occur, leading to the development of complications such as liver fibrosis, cirrhosis, or even liver failure.
Caroli Disease is usually present at birth, but symptoms may not manifest until later in life, often during adolescence or early adulthood. The most common symptoms include recurrent abdominal pain, fever, jaundice, and findings of an enlarged liver upon physical examination. Imaging studies, such as ultrasound, CT scan, or MRI, are crucial in confirming the diagnosis by visualizing the characteristic cystic dilatation of the bile ducts.
Treatment for Caroli Disease aims to manage the associated complications, such as infections and liver dysfunction. Medication may be administered to relieve symptoms and prevent infections, while surgical interventions may be required in more severe cases, including liver resection or transplantation. Regular monitoring and follow-up are essential to assess liver function and detect any potential progression of the disease.
