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Vascular Ehlers-Danlos Syndrome: Imaging Findings

Massimo Zilocchi1,2, Thanila A. Macedo1, Gustavo S. Oderich3, Terri J. Vrtiska1, Pietro R. Biondetti2 and Anthony W. Stanson1

1 Department of Radiology, Mayo Clinic, 200 First St., SW, Rochester, MN 55905.
2 Present address: Department of Radiology, Fondazione IRCCS, Ospedale Maggiore Policlinico, Mangiagalli Regina Elena, Milan, Italy.
3 Division of Vascular Surgery, Mayo Clinic, Rochester, MN.


Figure 1
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  		Fig. 1 —Spectrum and distribution of vascular findings in Ehlers-Danlos syndrome: total of 83 abnormal vascular findings including ectasia, aneurysm, dissection, and occlusion. DTA = descending thoracic aorta, IMA = inferior mesenteric artery, SFA = superior femoral artery, SMA = superior mesenteric artery.

 

Figure 2
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  		Fig. 2 —22-year-old woman with common iliac and hypogastric artery aneurysms. CT angiogram with 3D reconstruction reveals dissecting aneurysm of right common iliac artery (thin arrow) as well as aneurysms of right hypogastric artery and left common iliac artery (thick arrows).

 

Figure 3
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  		Fig. 3A —51-year-old woman with vascular Ehlers-Danlos syndrome. CT angiography of abdomen shows ruptured splenic vein aneurysm (thin arrow) and thrombus (thick arrow) in portal vein.

 

Figure 4
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  		Fig. 3B —51-year-old woman with vascular Ehlers-Danlos syndrome. Three days later, patient developed spontaneous rupture of left inferior epigastric artery with active bleeding and a rectus sheath hematoma shown in this CT image.

 

Figure 5
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  		Fig. 3C —51-year-old woman with vascular Ehlers-Danlos syndrome. Patient underwent coil embolization complicated by perforation of left internal mammary artery, extravasation of contrast material, and development of hemomediastinum (arrow), shown in this radiograph obtained during conventional angiography.

 

Figure 6
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  		Fig. 4A —47-year-old man with multiple vascular abnormalities. Selective left renal artery angiogram shows intimal flap consistent with dissection (arrow) associated with cortical infarcts.

 

Figure 7
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  		Fig. 4B —47-year-old man with multiple vascular abnormalities. After 5 days, repeat angiogram reveals new dissection of gastroduodenal artery (arrow).

 

Figure 8
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  		Fig. 4C —47-year-old man with multiple vascular abnormalities. Three days later, 3D MR angiography with gadolinium bolus shows new dissection of celiac artery (arrow).

 

Figure 9
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  		Fig. 4D —47-year-old man with multiple vascular abnormalities. Enhanced CT scan of pelvis 3 years later shows patient has developed new dissection and ectasia of left common and external iliac arteries (arrow).

 

Figure 10
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  		Fig. 5A —34-year-old woman with vascular Ehlers-Danlos syndrome. CT angiography of abdomen with volume-rendered 3D reconstruction shows 1.2-cm saccular aneurysm (arrow) arising from inferior wall of celiac axis.

 

Figure 11
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  		Fig. 5B —34-year-old woman with vascular Ehlers-Danlos syndrome. CT angiography of pelvis with volume-rendered reconstruction shows bilateral common iliac arteries, left internal iliac aneurysm, and right internal iliac ectasia.

 

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