Korean J Ophthalmol.  2013 Oct;27(5):376-380. 10.3341/kjo.2013.27.5.376.

Acute Central Retinal Artery Occlusion Associated with Livedoid Vasculopathy: A Variant of Sneddon's Syndrome

• Song HB
• Woo SJ
• Jung CK
• Lee YJ
• Ahn J
• Park KH
• Kwon OK

• KMID: 1707293
• DOI: http://doi.org/10.3341/kjo.2013.27.5.376

Abstract

Livedoid vasculopathy (LV) is characterized by a long history of ulceration of the feet and legs and histopathology indicating a thrombotic process. We report a case of acute central retinal artery occlusion in a 32-year-old woman who had LV. She showed no discernible laboratory abnormalities such as antiphospholipid antibodies and no history of cerebrovascular accidents. Attempted intra-arterial thrombolysis showed no effect in restoring retinal arterial perfusion or vision. The central retinal artery occlusion accompanied by LV in this case could be regarded as a variant form of Sneddon's syndrome, which is characterized by livedo reticularis and cerebrovascular accidents.

Keyword

Antiphospholipid antibodies; Atrophic blanche; Livedoid vasculopathy; Retinal artery occlusion; Sneddon syndrome

MeSH Terms

Acute Disease
Adult
Diagnosis, Differential
Female
Fluorescein Angiography
Fundus Oculi
Humans
Retinal Artery Occlusion/diagnosis/*etiology/physiopathology
Sneddon Syndrome/*complications/diagnosis
*Visual Acuity

Figure

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  Fig. 1 (A,B) Physical examination of the patient showed itching and tender violaceous to dark erythematous non-elevated patches with central necrotic vesicles on the dorsum of both lower legs and feet. (C) The histopathology of the skin lesion showed perivascular lymphohistiocytic infiltration in the dermis. Fibrin material was observed in the vessel lumen (arrow) and extravasated red blood cells were present. No leukocytoclasis observed (H&E, ×100).

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  Fig. 2 Fundus photography and fundus fluorescein angiography (FFA) immediately after (A,B) and six weeks after (C,D) intra-arterial thrombolysis (IAT) therapy for acute central retinal artery occlusion. (A) The immediate post-IAT fundus photograph showed no change from the pre-IAT fundus photograph. (B) Early phase FFA still showed delayed retinal arterial filling and arteriovenous transit time. (C) Six weeks after thrombolysis, fundus photography showed severe atrophy of the macula and disc pallor. (D) The retinal arterial perfusion was restored except for the macular area on FFA.

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  Fig. 3 Right internal carotid angiography before the intra-arterial thrombolysis procedure. (A) Severe vasospasm (arrow) in the proximal cervical internal carotid artery was noted in response to catheter placement, which was relieved by intra-arterial nimodipine infusion (5 mg). (B) Selective angiography of the right ophthalmic artery showed no thrombus or steno-occlusive lesion in the ophthalmic artery.

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