Definition:
Caroli disease is categorized as an uncommon congenital disease that involves the cystic dilatation of the liver's intrahepatic bile ducts. It has two types, known as Simple Caroli disease and Complex Caroli disease. The first type is characterized by ectasia or dilatation of bile ducts, while the latter includes the presence of portal hypertension and hepatic fibrosis besides ectasia.
Diagnosis:
There are preferred examinations that play major roles in the disease's diagnosis. These include ultrasonography, nuclear scintigraphy, CT imaging, MRI, ERCP, and PTC. Laboratory exams are also vital, including liver function tests and bilirubin level analysis.
Treatment:
Therapy will depend on clinical manifestations, as well as the biliary abnormality location. It is very important to treat the disease with antibiotics that have broad-spectrum agents. Preventing complications is advisable but there has been no proven method that's effective. Other treatments include procedures such as ERCP, PTC, sphincterotomy, lobectomy, and internal surgical bypass.
Symptoms and Signs:
Patients suffering from Caroli disease are generally feverish, and may have abdominal pain and bilirubinemia. Test results on liver function may show abnormality, including high levels of alkaline phosphatase. Variceal bleeding can result from portal hypertension related to the disease. Other symptoms are nonspecific, such s vomiting, nausea, and enlarged liver. Patients may not experience symptoms for long periods.
Causes:
The disease is likely caused by an in-utero occurrence that arrests the remodeling of the ductal plate found at the bigger intrahepatic bile ducts level. Inadequate ductal plates resorption leads to formation of several primal bile ducts that surrounds the middle portal vein. These dilate, enlarge, and turn into ectatic.
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