Background: The diagnosis of ocular sarcoidosis is particularly challenging in patients who present with atypical clinical findings and no evidence of systemic disease. We describe a patient whose initial clinical manifestation of sarcoidosis was a unilateral hemorrhagic retinopathy.
Method: Clinicopathologic case study.
Results: The diagnosis of sarcoidosis in this patient was delayed for over a year principally because of the atypical retinal findings and because repeated chest X-rays and serum angiotensin covering enzyme (ACE) levels were normal. Surgical enucleation of the eye once it was blind revealed a large noncaseating granuloma of the ciliary body. Inflammation and thrombosis of several large caliber retinal veins and a single branch of the retrolaminar hemicentral retinal vein were found.
Conclusions: Major retinal branch vein occlusion and hemi-central retinal branch occlusion are rare complications of ocular sarcoidosis. Normal serum ACE levels do not exclude the diagnosis of ocular sarcoidosis even in patients with considerable amounts of intraocular granulomatous inflammation.