Medical Imaging Research Center, Shiraz University of Medical Sciences, Shiraz, Iran; Department of Radiology, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Radiology, Shiraz University of Medical Sciences, Shiraz, Iran.
J Med Imaging Radiat Sci. 2020 Mar;51(1):145-153. doi: 10.1016/j.jmir.2019.11.002. Epub 2019 Dec 26.
Owing to nonspecific clinical symptoms, Wilson disease (WD) diagnosis is often missed or delayed; hence, many patients reach end-stage liver disease. When cirrhosis takes place, it is difficult to distinguish between WD and other causes of cirrhosis by imaging alone. This study outlines abdominal computed tomography (CT) imaging findings that occur more frequently in patients with WD cirrhosis.
Fifty-seven patients with WD who had referred for liver transplantation took part in this study and underwent dynamic liver CT examination before transplantation. Qualitative and quantitative parameters including liver density, contour irregularity, dysmorphia, hypertrophy of caudate lobe, presence of focal parenchymal lesion, thickness of perihepatic fat layer, periportal thickness, lymphadenopathy, and other associated findings were recorded and evaluated.
Among these patients, 85.9% had contour irregularity, 28% had hepatic dysmorphia, and periportal thickening and cholelithiasis were found in 25.5% and 12.3% of patients, respectively. Splenomegaly, lymphadenopathy, and portosystemic shunting were observed in all patients. Also, hyperdense nodules (>20 mm) and honeycomb pattern were detected in 65.2% and 15.2% of patients, respectively, in the arterial phase. In the portal phase, these findings were detected only in 13% and 4.3% of patients. Hypertrophy of caudate lobe was seen only in 12.2% of patients.
WD-associated cirrhosis has many CT imaging findings, although most of them are nonspecific. Some findings, such as hyperdense nodules and honeycomb pattern in non-contrast-enhanced CT scan and arterial phase of triphasic CT scan with lack of hypertrophy of caudate lobes, are hallmarks of WD.
由于临床症状不特异，Wilson 病（WD）的诊断常被延误或漏诊；因此，许多患者发展至终末期肝病。当出现肝硬化时，单凭影像学很难将 WD 与其他原因导致的肝硬化相鉴别。本研究总结了 WD 肝硬化患者更常见的腹部 CT 影像学表现。
本研究纳入了 57 例因肝硬化拟行肝移植的 WD 患者，这些患者在移植前均接受了动态肝脏 CT 检查。记录并评估了包括肝实质密度、轮廓不规则、肝变形、尾状叶肥大、局灶性实质病变、肝周脂肪层厚度、门脉周围增宽、淋巴结肿大等定性和定量参数。
在这些患者中，85.9%存在轮廓不规则，28%存在肝变形，25.5%存在门静脉周围增宽，12.3%存在胆石症。所有患者均存在脾肿大、淋巴结肿大和门体分流。此外，动脉期 65.2%和 15.2%的患者分别可见高密度结节（＞20mm）和蜂巢样改变，门静脉期仅分别有 13%和 4.3%的患者可见上述表现。尾状叶肥大仅见于 12.2%的患者。
WD 相关性肝硬化的 CT 影像学表现较多，但多数缺乏特异性。一些表现，如平扫 CT 增强扫描时的高密度结节和蜂巢样改变，以及三期增强 CT 扫描时缺乏尾状叶肥大且仅在动脉期出现，这些均为 WD 的特征性表现。
