case_images: PMC10632057_01
case_images: Anteroposterior supine abdominal radiograph shows abnormal soft tissue density in the left upper quadrant (white arrow) with inferior displacement of the stomach bubble (black arrow).
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case_images: fig1
case_images: The lung bases were clear, and there were no osseous abnormalities (Figure 1).
case_images: (a) Transverse grayscale image of the right hepatic lobe shows diffuse, heterogeneous, and mildly coarsened echotexture with periportal hyperechogenicity and thickening (white arrows) suggesting fibrosis. (b) Transverse ultrasound image with color Doppler shows the left portal vein (white arrow) with recanalized umbilical vein extending anteriorly to just beneath the skin (black arrow).
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case_images: fig2
case_images: An abdominal ultrasound was then performed and revealed hepatosplenomegaly with heterogeneous echogenic liver parenchyma and a recanalized umbilical vein (Figure 2).
case_images: Coronal and sagittal maximum intensity projection CT with IV contrast images shows hepatomegaly with enlarged left lobe (asterisk) and recanalized umbilical vein extending from the left portal vein anteriorly near the umbilicus (black arrow) and joining the bilateral inferior epigastric veins (white arrows) to reach the iliac veins.
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case_images: fig3
case_images: The scan also demonstrated signs of portal hypertension, with a diminutive right portal vein, enlarged left portal vein with recanalization of the umbilical vein, and upper abdominal varices (Figure 3).
case_images: (a) Axial MR elastography image shows increased liver stiffness (white arrows) corresponding to fibrosis. (b) Coronal magnetic resonance cholangiopancreatography (MRCP) maximum intensity projection image shows scattered mixed fusiform and saccular dilatations of the intrahepatic bile ducts (arrowheads). (c) Axial T2 weighted MR image shows hepatomegaly with enlarged left hepatic lobe (asterisk) and periportal thickening and T2 hyperintensity (white arrows).
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case_images: fig4
case_images: A magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP) and MRI elastography exam were then performed, revealing stage 3-4 hepatic fibrosis, as well as multiple ectatic intrahepatic bile ducts (Figure 4).
case_images: Axial T2 weighted MRI image without IV contrast shows borderline enlarged bilateral kidneys with lack of corticomedullary differentiation (arrowheads), suggesting some mild degree of autosomal recessive polycystic kidney disease.
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case_images: PMC10632057_01_CRIPE2023-4497784.005.jpg
case_images: fig5
case_images: The abdominal MRI also demonstrated borderline enlarged kidneys bilaterally, with relative lack of corticomedullary differentiation (Figure 5).