肝脏遗传性出血性毛细血管扩张症的CT表现

CT manifestations of hepatic hereditary hemorrhagic telangiectasia

目的:探讨肝脏遗传性出血性毛细血管扩张症(HHTT)的临床及CT表现特点。方法:回顾性分析2005年9月12日-2017年3月27日经临床证实的19例HHTT患者(男8例,女11例,平均年龄52岁)的临床与CT影像学资料,2位高级职称放射诊断医师独立分析读片。结果:CT平扫所有患者的肝实质密度未见异常。CT多期增强特征征象为动脉期肝内弥漫性血管异常,包括:(1)肝动静脉畸形15例(78.95%),表现为动脉期图像上可见肝实质染色明显不均,内弥漫分布点状、结节及小斑片状明显强化灶,肝动脉增粗、纡曲,肝静脉提前显影;(2)肝动脉-肝静脉瘘8例(42.11%),动脉期图像上可见肝动脉增粗、纡曲,肝静脉增粗并提前显影,肝实质内弥漫分布点状、结节状强化灶;(3)肝动脉-门静脉瘘13例(68.42%),表现为肝动脉增粗纡曲,门静脉提前显影,12例(92.3%)伴有门静脉扩张(平均直径约14.92mm);(4)混合型动静脉瘘6例(31.58%),兼有肝动脉-肝静脉瘘和肝动脉-门静脉瘘的特点;(5)门静脉-肝静脉瘘2例(10.53%),门静脉期图像上可见门静脉及其分支增粗,肝静脉汇合支早显;(6)毛细血管扩张14例(73.68%),表现为动脉期肝实质内有少量或弥漫分布的小结节状明显强化灶,在门静脉期和延迟期图像上呈等密度。肝总动脉直径5.1~13.5mm,平均直径约7.37mm,>5mm者16例(84.21%);门静脉扩张17例(89.47%);肝局灶性病变13例(68.42%),表现为动脉期图像上肝实质内有少量或弥漫分布的斑片状明显强化灶、边界模糊,静脉期4例呈稍高密度、9例呈等密度,延迟期均呈等密度。结论:肝脏遗传性出血性毛细血管扩张症CT动态增强扫描表现具有特征性,提高认识并熟知其影像特点具有重要的临床意义。

Objective:To explore the clinical features and CT findings of hepatic hereditary he-morrhagic telangiectasia(HHHT)in order to improve the understanding and efficacy of early diagnosis for this disease.Methods:Clinical data and imaging findings of 19 patients(8 males and 11 females with an average age of 52)with HHHT confirmed by the clinic from September 2005 to March 2017 were analyzed retrospectively and related literatures were reviewed.All cases underwent MDCT scan,and MRI examination in 3 cases.The images were reviewed independently by two expert radiologists.Results:CT plain scan showed negative findings in all patients.Dynamic contrast-enhanced CT manifestations were characterized by diffuse vascular abnormalities:①hepatic arteriovenous malformations(HAVMs)in 15 cases(78.95%),which showed dilated and tortuous hepatic artery and early enhancement of hepatic vein,liver parenchyma was obviously and unevenly enhanced with lots of significantly enhanced nodule and patchy region in hepatic arterial phase,and there was no obvious abnorma-lity in portal venous phase and delayed phase.②Arteriovenous shunts in 8 cases(42.11%),which showed dilated and tortuous hepatic artery and early enhancement of hepatic vein.③arterioportal shunts in 13/19(68.42%),which showed dilated and tortuous hepatic artery and early enhancement,blood flow increase and dilation(n=12)of the portal vein in arterial phase.④mixed shunts in 6 cases(31.58%),which showed dilated and tortuous hepatic artery and early enhancement of hepatic vein and portal vein in arterial phase.⑤portal venous shunts in 2 cases(10.53%),which showed dilated portal vein and its branches,significantly enhanced region between the hepatic vein and portal vein in portal venous phase and delayed phase,and without obvious abnormality in artery phase.⑥telangiectases in 14 cases(73.68%),which showed dilated and tortuous hepatic artery and significantly enhanced nodule with little or diffuse distribution in arterial phase and without obvious abnormality in portal venous and delayed phase.Dilated and tortuous hepatic artery(mean diameter of 7.37mm,ranged from 5.1 to 13.5mm)was found in 16 cases(84.21%).Besides,13 cases(68.42%)displayed focal liver lesions or transient hepatic attenuation differences(THADs)with the similar typical image findings of significantly enhanced and uneven distribution in hepatic arterial phase and without obvious abnormity in portal venous phase and delayed phase.Conclusion:HHHT can be clearly diagnosed by dynamic contrast-enhanced CT scan with certain characteristic findings,to increase awareness and familiarness with its CT features have leading clinical significance.