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目的总结遗传性出血性毛细血管扩张症(hereditary hemorrhagic telangieetasia,HHT)患者多排螺旋CT(multi-detector helical computed tomography,MDCT)的影像学特征。方法回顾性分析14例HHT患者的MDCT影像特征,所有病例均行容积再现(VR)、最大密度投影(MIP)及多平面重建(MPR)。结果 14例患者中,肺部受累5例,CT轴位显示结节状或团块状强化灶,VR及MIP显示瘤体及供血动脉和引流静脉。肝脏受累12例,包括肝动脉-肝静脉分流6例,肝动脉-门静脉分流2例,门静脉-肝静脉分流4例。肝动脉-肝静脉分流:轴位动脉期可见肝门增粗、迂曲的血管影,肝静脉提前显影,MIP及VR显示肝动脉及其分支迂曲扩张;肝动脉-门静脉分流:MIP及MPR肝动脉和门静脉同时显影,MPR同时显示肝内多发斑片状异常强化区;门静脉-肝静脉分流:门静脉期MPR及MIP可见门静脉与肝静脉分支间迂曲扩张的交通血管及斑片状强化区。胰腺受累5例,动脉期MPR及MIP显示胰头周围扩张的血管团2例及瘤样扩张1例,胰头或胰尾部结节状异常强化灶2例。脾脏受累1例,动脉期MPR表现为脾脏内多发斑片状异常强化灶及脾动脉瘤形成。小肠受累1例,VR及MIP显示为肠系膜上静脉分支远端迂曲的血管团。结论 HHT累及多脏器的影像表现具有特异性,CT血管重建能够清晰显示HHT的血管变异,MDCT有助于临床诊断。
Objective To summarize the imaging features of multi-detector helical computed tomography (MDCT) in patients with hereditary hemorrhagic telangieetasia (HHT). Methods The features of MDCT in 14 patients with HHT were retrospectively analyzed. All patients underwent VR, MIP and MPR. Results Of the 14 patients, 5 had pulmonary involvement, and the axial CT showed nodular or massive lesions. VR and MIP showed the tumor, the feeding artery and the drainage veins. Liver involvement in 12 cases, including hepatic artery - hepatic vein shunt in 6 cases, hepatic artery - portal vein shunt in 2 cases, portal vein - hepatic vein shunt in 4 cases. Hepatic artery - hepatic vein shunt: hepatic portal thickening, tortuous vessel shadow, prerenal hepatic vein development, MIP and VR showed tortuous dilation of hepatic artery and its branches; hepatic artery - portal vein shunt: MIP and MPR hepatic artery And portal vein at the same time, MPR also showed multiple intrahepatic patchy anomalous area; portal vein - hepatic venous shunts: Portal venous phase MPR and MIP can be seen between the portal vein and hepatic vein tortuous dilatation traffic vessels and patchy enhancement zone. Pancreatic involvement in 5 cases, arterial MPR and MIP showed the expansion of pancreatic head around the vascular groups in 2 cases and tumor-like dilatation in 1 case, pancreatic or pancreatic tail nodular anomaly in 2 cases. 1 case of spleen involvement, MPR in the arterial phase showed multiple patchy abnormalities in spleen and splenic aneurysm. 1 case of intestinal involvement, VR and MIP showed distal tortuous superior mesenteric vein branch. Conclusions The imaging manifestations of HHT involving multiple organs are specific. CT angiography can clearly show the vascular variation of HHT, and MDCT is helpful for clinical diagnosis.