遗传性出血性毛细血管扩张症肝脏病变的MDCT影像学特征

The MDCT findings of liver invoevement in hereditary hemorrhagic telangiectasia

目的探讨遗传性出血性毛细血管扩张累及肝脏病变的MDCT影像学特征及临床意义。方法回顾分析9例经临床明确诊断的遗传性出血性毛细血管扩张累及肝脏的影像学资料并总结其影像特征。结果 1肝动脉-肝静脉分流4例:动脉期见增粗迂曲的肝动脉及提前显影的肝静脉;肝实质弥漫性分布小斑片状边缘模糊强化灶,门脉期等密度。其中3例肝门区胆管轻度扩张;2肝动脉-门静脉分流1例:动脉期见扩张迂曲的肝动脉及提前显影的扩张门静脉;肝实质少量小斑片状强化灶,门脉期等密度;3肝动脉-肝静脉分流合并肝动脉-门静脉分流3例,动脉期肝动脉迂曲扩张,肝静脉和门静脉提前显影;肝实质弥漫性分布小斑片状强化灶,门脉期等密度;4门静脉-肝静脉分流1例:动脉期未见扩张肝动脉,门脉期见扩张门静脉及其远端分支旁小斑片状强化灶,延迟期呈稍高密度。结论遗传性出血性毛细血管扩张症累及肝脏的CT影像表现具有特征性,充分认识其影像表现并结合临床对其诊断及鉴别诊断具有重要意义。

Objective To investigate the MDCT findings and clinical significance of liver diease with hereditary hemor‐rhagic telangiectasia .Methods A retrospective analysis of the radiographic findings of 9 clinically confirmed cases of he‐reditary hemorrhagic telangiectasia was conducted .Results Four lesion types were seen ① Four cases involved the hepatic artery‐hepatic venous shunt .The imaging findings showed tortuous thickening of the hepatic artery and early enhance‐ment of the hepatic vein .The liver parenchyma showed diffuse or scattered small patchy lesions ,with blurred edges ,and appeared isodense during the portal phase .In three cases ,there was hilar bile duct dilation ;② One case involved the he‐patic artery‐portal venous shunt .The imaging findings showed tortuous dilation during the arterial phase and early en‐hancement and dilation of the portal vein .The liver parenchyma showed sparse patchy lesions ,with blurred edges ,and ap‐peared isodense during the portal phase ;③ Three cases involved the hepatic artery‐hepatic venous shunt and hepatic arter‐y‐portal venous shunt hybrid .The imaging results showed tortuous dilation during the arterial phase and early enhance‐ment of the hepatic and portal vein .The liver parenchyma showed diffuse small patchy lesions with blurred edges ,and ap‐peared isodense during the portal phase ;and ④ One case involved the portal vein‐hepatic venous shunt .There was no dila‐tion of the hepatic artery during the arterial phase .During the portal phase ,there was dilation of the portal vein and small patchy lesions at the distal branches ;the edges were blurred and appeared hyperdense during the delayed phase .Conclusion Liver diease with hereditary hemorrhagic telangiectasiat has specific radiographic findings .Integrating the radiographic findings with clinical manifestations is important for the diagnosis and differential diagnosis of this disease .