CT Abdoment With Contrast for Caroli’s Disease

Congenital Hepatic Fibrosis (Caroli’s Disease) The current definition of Caroli’s disease is somewhat muddled. Some authors limit this term to the rare communicating biliary saccular ectasia as originally describe by Caroli;
whereas others apply it to a broader spectrum of congenital hepatic fibrosis. Some employ Caroli’s disease as a descriptive term and then associate it either with congenital hepatic fibrosis or infantile polycystic kidney disease.
Still others use the term Caroli’s disease to describe the rarer isolated biliary ectasia and Caroli’s syndrome if both ectasia and hepatic fibrosis are evident.
Embryologically, intrahepatic bile ducts develop from liver progenitor cells adjacent to portal vein mesenchyme and form ductal plates, which eventually evolve into bile ducts. Lack of or disordered ductal plate remodeling leads to a number of congenital intrahepatic bile duct disorders, including congenital hepatic fibrosis.
The latter represents a cholangiopathy with surrounding fibrosis. Kidney involvement is variable in these patients and ranges from renal tubular ectasia to various forms of polycystic disease, most often autosomal recessive and only rarely autosomal dominant. An interesting association is Caroli’s disease and occasional
acute pancreatitis.
Histologically, bile ductules become ectatic but still maintain their communication with bile ducts. As an isolated finding, ectasia is found only in a minority; over time, progressive fibrosis ensues and accounts for the typical appearance of extensive periportal fibrosis and biliary dilation.
Two manifestations predominate: (1) Those patients developing extensive fibrosis early in life tend not to have prominent intrahepatic bile duct dilation. (2) Dilation is more common in those individuals who are asymptomatic until adulthood, when signs and symptoms of chronic bile stasis develop. In some of the latter
patients dilated ducts predominate in one lobe.
Also, in some patients Caroli’s disease is associated with extrahepatic bile duct dilation, and in an occasional patient an extrahepatic choledochal cyst is the initial presentation. Calculi tend to develop either in intrahepatic cysts, in bile ducts, or both.
With severe involvement death occurs during the neonatal period from renal causes, before liver damage is evident. Some develop hepatomegaly early in life, others remain asymptomatic, and still others progress to
fibrosis, liver failure, and portal hypertension; varices and hematemesis is their initial presentation. Bile stasis and infection lead to acute cholangitis, at times at an early age. Intrahepatic calculi develop in some, but the involved ducts are not obstructed. Hepatic function tends to be preserved until relatively late. Generally most of the liver is involved, although occasionally cysts are limited to one lobe; some patients have a preponderance of left lobe disease.
eponderance of left lobe disease. These patients are at increased risk of a developing hepatocellular carcinomas or cholangiocarcinomas. A not uncommon progression consists of calculi formation, pyogenic cholangitis, intrahepatic abscesses, and eventual cholangiocarcinoma. In adults, Caroli’s disease can be suspected with most imaging CT ABDOMEN WITH CONTRAST in below :
CT Abdomen with contrast indications Caroli’s Disease A,B: Transverse postcontrast CT images identify round, hypodense structures (arrows) adjacent to bileducts. C: A contrast-enhanced image shows hypodense regions in the renal medulla bilaterally. D: Endoscopic retrograde cholangiopancreatography (ERCP) identifies saccular intrahepatic cavities (arrows) communicating with bile ducts. The extrahepatic bileducts (curved arrow) are normal. (Source: Fulcher AS, Turner MA, Sanyal AJ. Caroli disease and renal tubular ectasia. Radiology2001;220:720–723, with permission from the Radiological Society of North America.)
Findings include hepatomegaly, parenchymal fibrosis, numerous cysts scattered throughout the liver, or multiple cyst-like dilated bile ducts. It is necessary to show that the cysts communicate with bile ducts to distinguish this condition from autosomaldominant polycystic disease and multiple intrahepatic abscesses. Although some intrahepatic abscesses do communicate with bile ducts, differentiation of abscesses from dilated bile ducts is generally straightforward with cholangiography.
Ultrasonography reveals extensive collaterals and a prominent periportal hyperechoic pattern, a finding also seen in other conditions. Liver parenchymal texture tends toward a heterogeneous appearance containing multiple high echoes.
With progressive fibrosis adults tend to develop portal hypertension, generally attributable to intrahepatic compression of portal vein branches by fibrosis. Doppler US findings vary depending on underlying hemodynamics.
Intrinsically, the portal vein is patent, although some patients have portal vein thrombosis and cavernous transformation. Magnetic resonance cholangiography also detects cystic intrahepatic biliary dilation. Technetium-99m-IDA scintigraphy reveals bile stasis in cystic structures. Scintigraphy confirms that these cysts communicate with the bile ducts.
Regardless of how it is performed, cholangiography confirms the diagnosis by identifying numerous segmental dilated intrahepatic bile ducts, either saccular or fusiform in appearance. 
CT Abdoment With Contrast for Caroli’s

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