Manuel Teixeira Gomes MD, Presenter: Nothing to Disclose

Raquel Marques Maia MD, Abstract Co-Author: Nothing to Disclose

Paulo Almeida Santos, Abstract Co-Author: Nothing to Disclose

Ana Beatriz Ramos, Abstract Co-Author: Nothing to Disclose

Joao Pires MD, Abstract Co-Author: Nothing to Disclose

Pedro Varzim, Abstract Co-Author: Nothing to Disclose

A. Manuel Ribeiro, Abstract Co-Author: Nothing to Disclose

00030490-DMT et al, Abstract Co-Author: Nothing to Disclose

1. Explain the pathophysiology of the two forms of Caroli´s disease. 2. Review the spectrum of radiologic findings on different imaging modalities, discussing their role on the diagnosis of this disease. 3. Discuss the differential diagnosis, main complications and treatment of this entity.  

A. Pathophysiology of Caroli´s disease: two forms of the disease (classic versus associated with congenital hepatic fibrosis). B. Clinical manifestations C. Review of imaging findings on different imaging modalities:      - Ultrasound      - CT      - MR / MR cholangiography      - Endoscopic retrograde / percutaneous transhepatic cholangiography D. Differential diagnosis E. Complications. Risk of malignancy (cholangiocarcinoma). F. Treatment: focal versus diffuse disease. 

The major teaching points of this exhibit are: 1. Major imaging features of Caroli disease include saccular dilatation of the intrahepatic bile ducts, intraductal calculi and, in the case of the periportal fibrosis type, the additional findings of cirrhosis, splenomegaly and varices. 2. ERCP, PTC and MR cholangiography are the best imaging modalities to demonstrate these findings, with MR offering the great advantage of being non-invasive.

Gomes, M, Maia, R, Santos, P, Ramos, A, Pires, J, Varzim, P, Ribeiro, A, et al, 0, Caroli Disease: Imaging Findings, Differential Diagnosis, and Main Complications.  Radiological Society of North America 2009 Scientific Assembly and Annual Meeting, November 29 - December 4, 2009 ,Chicago IL. http://archive.rsna.org/2009/8004408.html