Isolated Caroli's disease presenting as an exophytic mass in the liver

Abstract

A case of isolated Caroli's disease in a 2-year-old female presented as an exophytic cystic mass of the liver. A palpable mass below the right costal margin and displacement of the right colon were the main points of clinical presentation. Preoperatively, sonographic signs of intraluminal vascular tracts were identified. At laparotomy, the mass was found at the inferior border of the right lobe of the liver next to the gallbladder and was easily removed by wedge excision. Intraoperative liver biopsies and histologic findings of the excised lesion confirmed the diagnosis.

Clinical characteristics of Caroli’s syndrome

“…Caroli's disease is however characterized by multiple segmental cystic dilatations of the intrahepatic bile ducts [3] . Many authors believe that the two conditions are actually different stages of the same disease characterized by periportal fibrosis and ductal dilatation [4] .…”

Clinical characteristics of Caroli’s disease

“…Histologically, the main macroscopic and microscopic features of CD are: non-obstructive, localized dilatation of the bile ducts, intraluminal bulbar protrusions of the ductal wall and intra ductal vascular tracts containing patent portal venous and hepatic arterial channels that traverse the true lumen and terminate within the lumen [11] . The diagnosis of CD therefore rests on demonstrating that the cystic lesions are in continuity with the biliary tree.…”

An exceptional case of spontaneous fistulization of an intrahepatic biliary cyst in to the colon, operated Tikur Anbessa Specialized Hospital, Ethiopia - a case report

“…In our case, the presence of a single bile containing intrahepatic cyst in the absence of extrahepatic biliary tree involvement and in the absence features of hepatic fibrosis(even though not biopsy proven) suggests simple Caroli's disease.Although present from birth, Caroli's disease usually remains asymptomatic during the first 20 years, and may also remain so throughout life 18 . Unlike other biliary cysts, it rarely present in childhood and the diagnosis is usually made at an advanced age [19][20][21][22] . Our patient was 53 years old when diagnosed and he had single lobeinvolvement.…”

“…In patients like ours, the isolated monolobar form of disease, the absence of hepatic fibrosis and its complications, as well as the absence of cholelithiasis and absence of previous episodes of cholangitis could be possible explanations for the late presentation. Cholangitis, pancreatitis, hepatic fibrosis, and malignancy have all been reported at the time of presentation 2,5,[8][9][10][11] .…”

“…[5,6]If the defective remodeling involves the larger intrahepatic ducts Caroli's disease develops.But, when the entire intrahepatic biliarytree is involved the condition is designatedCaroli's syndrome;in which there is an associated congenital hepatic fibrosis [5][6][7] . Unlike other types of biliary cysts, Caroli's disease rarely present in childhood and the diagnosis is usually made at an advanced age [8][9][10] . Biliary cysts are associated with significant complications such as stone formation, cholangitis, secondary biliary cirrhosis and rupture 2,11 .…”