PMC5466357_01
(A) Visual aspect of the patient's abdomen after a 10-L paracentesis; (B) computed tomographic (CT) scan of the abdomen evidencing kidneys of increased size with numerous bilateral cysts and enlarged liver also with multiple cysts; (C) CT scan showing significantly distended abdomen due to large-volume ascites, hepatomegaly, and nephromegaly; and (D) visual aspect of the abdomen after liver-kidney transplantation.
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The visual aspect of her abdomen following this procedure is presented in Figure 1A. A computed tomography (CT) angiography scan of her abdomen showed kidneys of increased size and massive hepatomegaly, translated into an estimated liver volume of 8 L (Figure 1B and C). She shows significant improvement in quality of life following the reduction in abdominal volume (Figure 1D).
Angio-computed tomography of the patient's abdomen showing multiple liver cysts and, as a result, severe compression of the (A) right hepatic vein (arrow), (B) left hepatic vein (arrow), (C) portal vein (arrow), and (D) inferior vena cava (arrow).
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C). Contrast enhancement disclosed a compressive effect of such cysts on hepatic veins, leading to the more defined diagnosis of functional BCS associated with portal hypertension caused by extrinsic portal compression (Figure 2).
Liver explant histopathology. (A) Evidence of chronic venous flow obstruction, with signs of recent hemorrhage in the centrilobular area (hematoxylineosin, 50x) and (B) reduction in centrilobular vein lumen (black arrow) (hematoxylineosin, 100x).
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Histopathologic analysis of the liver explant showed blocking alterations of venous flow secondary to perisinusoidal fibrosis, congestion, with no evidence of thrombosis, consistent with the diagnosis of BCS (Figure 3).