Multiple retinal vascular occlusions in Leriche syndrome.

A 45-year old man, nondiabetic, nonhypertensive, chronic smoker with a recent history (2 months) of stroke presented with complaints of sudden, painless decrease in vision in the right eye (RE) of 3 days duration. His visual acuity in the RE and left eye (LE) was PL negative and 6/9, N6, respectively. Anterior segment of both eyes was normal. Retinal examination findings of both eyes are described in Fig. 1. A working diagnosis of bilateral nonischemic central retinal vein occlusion with cilio-retinal artery occlusion in the RE was made. Evaluation of the previous records revealed a past diagnosis of Leriche syndrome (LS) with prior CT-angiography revealing the presence of thrombotic atherosclerotic plaques involving distal abdominal aorta at the aortic bifurcation and extending into the common iliac arteries [Fig. 2]. The patient was advised to undergo fundus fluorescein angiography and optical coherence tomography [Figs. 3 and 4]. Carotid Doppler showed soft plaques in the left carotid bulb region causing 70%–80% stenosis. A final diagnosis of multiple level retinal vascular occlusions in both eyes following blockage with emboli secondary to LS was made and the patient referred to a vascular surgeon.

Figure 1

Montage color fundus photography images of both eyes. Clinical examination of the right eye fundus showed a clear media with dilated retinal veins (white arrow), scattered retinal hemorrhages (yellow arrow) involving the posterior pole and retinal periphery and an elevated, pale-looking optic nerve head with blurred disc margins (black arrow). An area of dense white retinal opacification was noted at the posterior pole with the presence of surrounding retinal hemorrhages (red arrow). No cherry-red spot was seen. The left eye fundus examination revealed the presence of a hyperemic optic nerve head (pink arrow) with dilated retinal veins (blue arrow) and scattered retinal hemorrhages (green arrow) in the periphery. The macula looked normal on examination with a normal foveal reflex

Figure 2

CT angiography images of patient's abdomen. (a and b) Coronal and sagittal maximum intensity projection CT angiography images demonstrating the thrombotic atherosclerotic plaques involving the distal abdominal aorta at the aortic bifurcation and extending into the common iliac arteries (white arrow)

Figure 3

Fluorescein angiography features of both eyes. (a-d) Fluorescein angiography of the RE showed delayed arm-retina time (21 s) and artery-vein transit time. Multiple pin-head hyperfluorescent spots were noted, spread all throughout the fundus and within the retinal vessels suggestive of multiple emboli. A patch of blocked retinal and choroidal fluorescence was noted at the posterior pole and extending to the nasal macula corresponding to the area of retinal whitening noted on clinical examination. A fern-like perivascular leakage pattern with optic disc leakage was noted as well in the right eye in the late stages of the angiogram. (e-h) Fluorescein angiography of the left eye showed a delay in the arm-retina and artery-vein transit time with multiple pin-head hyperfluorescent spots along the retinal vessels scattered all throughout the retinal periphery. Optic disc leakage with perivascular leakage at the posterior pole was noted in the left eye in the late phase of the angiogram

Figure 4

Optical coherence tomography findings in both eyes. (a) Optical coherence tomography (OCT) of the right eye showed inner retinal layer hyperreflectivity with underlying shadowing of the outer retinal layers with hyperreflectivity of the RPE at the fovea suggestive of resolving central retinal artery occlusion along with cilioretinal artery and central retinal vein occlusion. (b) Left eye OCT showed minimal hyperreflectivity of the inner retinal layers at the fovea with normal outer retinal layer reflectivity suggestive of perfused central retinal artery occlusion as well. There was no presence of macular edema in both eyes

Leriche syndrome, also known as aortoiliac occlusive disease, is caused by atherosclerosis affecting the distal abdominal aorta, iliac and femoropopliteal vessels.[1] Eventually, aortoiliac segments become stenotic by more than 50%, resulting in exercise-induced claudication. Further disease progression leads to impotence in men, secondary to reduced penile arterial flow.[2] The primary complication in LS is limbal ischemia; though myocardial infarction, gangrene and even death have been reported.[345]

The ocular complications in LS have not been reported earlier. In our case, we identified multiple emboli within the retinal vessels suggestive of embolization from the atheromatous plaque leading to bilateral retinal artery and vein occlusions.

To conclude, the presence of an ophthalmic or retinal artery occlusion in individuals over 45 years of age should immediately raise clinical suspicion for giant cell arteritis or other systemic life-threatening conditions. LS can lead to sudden, painless loss of vision due to multiple retinal vascular occlusions.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

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