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《Radiologic Practice》 2018-04
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CT manifestations of hepatic hereditary hemorrhagic telangiectasia

RUAN Zhi-bing;JIAO Jun;FAN Guang-ming;Department of Imaging,the Affiliated Hospital of Guizhou Medical University;  
Objective:To explore the clinical features and CT findings of hepatic hereditary hemorrhagic 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:(1)hepatic 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 abnormality in portal venous phase and delayed phase.(2)Arteriovenous shunts in 8 cases(42.11%),which showed dilated and tortuous hepatic artery and early enhancement of hepatic vein.(3)arterioportal 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.(4)mixed 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.(5) portal 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.(6)telangiectases 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 7.37 mm,ranged from5.1 to 13.5 mm)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.
【Fund】: 贵阳市科技类计划子课题(GY2015-32)
【CateGory Index】: R575;R816.5
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