case_images: PMC10775735_01
case_images: Structural disorganization of the hepatic lobules with irregular hepatocellular nodule formation. Examination revealed mild hepatocellular enlargement, dilated hepatic sinusoids around the central vein, interstitial fibrous tissue proliferation, widened fibrous septa, significant dilatation of the bile ducts, bile duct hyperplasia, bile thrombus formation, and peripheral diffuse inflammatory cell infiltration.
case_images: 10.1177_03000605231221086-fig1.jpg
case_images: PMC10775735_01_10.1177_03000605231221086-fig1.jpg
case_images: fig1-03000605231221086
case_images: Pathologic examination showed dilated intrahepatic bile ducts with hepatic fibrosis consistent with Caroli disease, chronic fibrosclerotic splenomegaly, and vasodilatation and siltation in the fibrous connective tissue (Figures 1-4).
case_images: Special tests. Reticulostaining and Masson staining showed widened fibrous septa dividing the liver parenchyma, positive iron staining, positive periodic acid-Schiff staining, and negative diastase periodic acid-Schiff staining. Immunohistochemistry revealed negativity for hepatitis B surface antigen and hepatitis B core antigen and positivity for cytokeratin 7, cytokeratin 19, CD34, and pleural carcinoembryonic antigen.
case_images: 10.1177_03000605231221086-fig2.jpg
case_images: PMC10775735_01_10.1177_03000605231221086-fig2.jpg
case_images: fig2-03000605231221086
case_images: Pathologic examination showed dilated intrahepatic bile ducts with hepatic fibrosis consistent with Caroli disease, chronic fibrosclerotic splenomegaly, and vasodilatation and siltation in the fibrous connective tissue (Figures 1-4).
case_images: Pathologic examination findings. Examination revealed fibrotic thickening of the splenic tegument, atrophy and reduction of splenic microsomes, dilatation and bruising of the hepatic sinusoids, hyperplasia and swelling of endothelial cells, and fibrotic hemorrhage around the splenic artery.
case_images: 10.1177_03000605231221086-fig3.jpg
case_images: PMC10775735_01_10.1177_03000605231221086-fig3.jpg
case_images: fig3-03000605231221086
case_images: Pathologic examination showed dilated intrahepatic bile ducts with hepatic fibrosis consistent with Caroli disease, chronic fibrosclerotic splenomegaly, and vasodilatation and siltation in the fibrous connective tissue (Figures 1-4).
case_images: Abdominal computed tomography. The axial view without contrast showed an enlarged liver with no significant foci of abnormal intrahepatic enhancement. The spleen was enlarged and homogeneous and contained abnormal echoes within it.
case_images: 10.1177_03000605231221086-fig4.jpg
case_images: PMC10775735_01_10.1177_03000605231221086-fig4.jpg
case_images: fig4-03000605231221086
case_images: Pathologic examination showed dilated intrahepatic bile ducts with hepatic fibrosis consistent with Caroli disease, chronic fibrosclerotic splenomegaly, and vasodilatation and siltation in the fibrous connective tissue (Figures 1-4).