PMC6057302_01
(a) The splenic vein and superior mesenteric vein drained directly into the inferior vena cava after confluence (arrow). (b) Note that the branch overlapping with the portal vein in (a) originated from the hepatic artery, not from the portal vein (arrow head). (c) A barely perceptible small branch of the portal vein measuring only 3 mm supplied the left lobe of the liver (arrow). (d) A faintly visible mass in segment 5 approximately 55 mm in size and poorly demarcated with mildly heterogeneous enhancement on arterial phase (arrow), which conferred a diagnosis of tumour initially.
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Abdominal enhanced CT with multiplanar reconstruction revealed that the splenic vein and superior mesenteric vein drained directly into the inferior vena cava after confluence (Figures 1(a) and 1(b)), and only two branches of hepatic veins drained into the inferior vena cava. A barely perceptible small branch of the portal vein measuring 3 mm supplied the left lobe of the liver (Figure 1(c)). Arterial phase of enhancement showed mildly heterogeneous enhancement (Figure 1(d)), while portal venous and delayed phases demonstrated iso- to hypoattenuating.
Piggyback liver transplantation was conducted. (a) CT imaging illustrated the reconstructed portal vein was in a satisfactory condition. (b) MR showed the transplanted liver was almost normal in signal 2 years following transplantation.
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Two years after the transplantation, physical examination as well as CT and MR imaging showed the patient was in a satisfactory condition (Figures 2(a) and 2(b)); the transplanted liver was practically normal in contour and signal intensity.
(a, b) Pathologically, the lesion was nodular in appearance and lacked a normal hepatic lobule texture, with absence of normal hepatic veins and bile ducts, while multiple fibrous connective tissues and chronic inflammatory cells scattered over.
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Some hepatocytes were characterized by hydropic degeneration, cholestasis, and regeneration (Figures 3(a) and 3(b)).