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Combined central retinal artery and vein occlusion in
Churg-Strauss syndrome: case report.Acta
Ophthalmol Scand. 2006 Oct;84(5):703-6.
PURPOSE: To
describe a rare case of Churg-Strauss syndrome presenting with
severe visual loss due to a combined central retinal vein and artery
occlusion. METHODS: A 42-year old man with a medical history of
asthma and blood hypereosinophilia developed a sudden loss of vision
in his right eye. We describe the clinical features and evolution of
the case after treatment. RESULTS: A combined occlusion of the
central retinal artery and central retinal vein was diagnosed by the
funduscopic appearance of retinal whitening, macular cherry-red
spot, papilloedema, retinal haemorrhages in all four quadrants and
dilated and tortuous veins. The diagnosis was confirmed by a
fluorescein angiogram showing absence of retinal filling and normal
choroidal filling. Churg-Strauss syndrome was diagnosed based on the
necessary presence of four of six criteria for the disease proposed
by the American College of Rheumatology. Corticosteroid therapy was
initiated. However, during the following year when tapering off the
daily dosage, the patient experienced two relapses, with pulmonary
symptoms and hypereosinophilia, and the corticosteroid dosage had to
be augmented. The patient presented with neovascular glaucoma 7
weeks after the vascular occlusion and experienced no visual
improvement. CONCLUSION: Combined central retinal artery and vein
occlusion can occur in Churg-Strauss syndrome. We suggest that
regional vasculitis may be the pathological mechanism underlying the
vascular occlusions observed in our case. The condition carries a
very poor prognosis for vision, due to the resulting retinal
ischaemia, and a poor general prognosis due to the late stage of the
systemic disease. Corticosteroids should be instigated promptly in
order to prevent further systemic or ocular vasculitis.
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