Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome—Case Series and Literature Review

Figure 1

Portal vein CT venography- (CTV-) enhanced scan (a, b) and abdominal ultrasound (c, d) of P5 suggested dilation of the intrahepatic bile ducts with the maximum diameter of 30 mm in CTV. The liver was normal in size with smooth capsules and an unevenly distributed rough echo. There are multiple well-defined hypoechoic mass lesions in the liver and bilateral kidneys. The comet’s tail echoes diffused in the kidney ultrasound suggesting multiple calcifications. The spleen was not shown due to splenectomy. Three-dimensional reconstruction of the portal vein CTV-enhanced scan of P6 (e). The maximum diameter of the portal venous trunk was 19 mm (arrow). The spleen was markedly enlarged (asterisk), and intrahepatic portal vein branches were dilated, including the superior mesenteric vein (arrow), splenic vein (arrow), esophageal and gastric veins, and umbilical vein.

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