Combined central retinal artery and vein occlusion in Churg-Strauss syndrome: case report

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.