Caroli disease also is known as communicating cavernous ectasia. Caroli’s disease is a rare inherited disorder of the liver. It is characterized by saccular and cystic dilatation of intra-hepatic biliary ducts. Caroli disease is associated with recurrent bacterial cholangitis and stone formation. It is distinct from other diseases that cause ductal dilatation caused by obstruction. It is not one of the many choledochal cyst derivatives. It may be associated with autosomal-recessive polycystic kidney disease. The likely mechanism involves an in utero event that arrests ductal plate remodeling at the level of the larger intrahepatic bile ducts.
Insufficient resorption of the ductal plates leads to the formation of multiple primitive bile ducts surrounding the central portal vein. These enlarge, dilate, and become ectatic. There are two types of Caroli disease. Simple caroli disease simply entails the bile duct dilatation or ectasia that by definition is part of Caroli disease and second is complex Caroli disease, hepatic fibrosis and portal hypertension are present as well. The complex form or syndrome is associated with the presence of bands of fibrous tissue in the liver (congenital hepatic fibrosis) and high blood pressure in the portal artery (portal hypertension).
Caroli disease predominantly affects females. The treatment for a caroli disease depends on a number of factors including your health and the size and position of the this inherited disorder. Liver transplantation may be required. This is the best alternative when recurrent cholangitis is not present. Antibiotic treatment with broad-spectrum agents is extremely important.Ultrasonography is very helpful. It is the examination of choice. They are largest in the superior part of the liver. The intraluminal portal vein sign is related to the protrusion of the portal vein branches into the cyst wall.
Color flowing Doppler ultrasonography is useful in showing blood flowing in these branches but no flowing is existing in the bile-containing spaces. Portal branches bridge the cyst walls. Ultrasonography can too assist in the diagnosis of complications and in the follow-up of patients with Caroli disease. Ultrasonography-guided needle ambition of bile from the cystlike lesions may be advantageous in the diagnosis of cholangitis and in confirming that the cysts transmit with the biliary tree. Ultrasonography-guided heart biopsy may be performed, if needed, to receive liver samples for histologic evaluation to affirm this circumstance.