Hepatic multiple hyperintense cystic lesions: a rare caroli disease

Abstract

Caroli’s disease is a rare malformation of the biliary tract as its incidence rate is one per million of people. Most cases of hepatic cystic lesions are asymptomatic and are discovered incidentally during abdominal ultrasonography. It is reported that Caroli’s disease is an autosomal-recessive disorder with multifocal segmental non-obstructive dilation features of intrahepatic bile ducts that may involve a segment, a lobe, or the whole liver. Several complications might be developed in some cases, such as cholangiocarcinoma. Therefore, early diagnosis is vital for proper management. There is a lack of literature discussing Caroli’s disease among Africans compared to Asians. Females are more affected than males. Moreover, most of Caroli’s disease cases were about 22 years old of age. This study describes the existing case’s novelty in that we discuss a rare Caroli’s disease case of an African male patient; moreover, the patient age in mid-adulthood. In addition, the severity of the current case is that both hepatic lobes showed severe multiple cystic lesions. The embryonic justification of the case is the large cranial portion of the hepatic diverticulum gives rise to the cords of hepatocytes and intrahepatic biliary networks. On the other hand, the small caudal portion forms the extrahepatic biliary tract. The hepatocytes as well as cholangiocytes, are differentiated from the hepatoblasts under the influence of typical hepatic genes, α-fetoprotein, liver-specific transcription factors, and albumin. Based on the above, Caroli disease is defined by biliary tract malformation, whereas Caroli syndrome is determined by the presence of associated congenital hepatic fibrosis.