Background: The diagnosis of ocular sarcoidosis is particularly challe
nging in patients who present with atypical clinical findings and no e
vidence of systemic disease. We describe a patient whose initial clini
cal manifestation of sarcoidosis was a unilateral hemorrhagic retinopa
thy. Method: Clinicopathologic case study. Results: The diagnosis of s
arcoidosis in this patient was delayed for over a year principally bec
ause of the atypical retinal findings and because repeated chest X-ray
s and serum angiotensin converting enzyme (ACE) levels were normal. Su
rgical enucleation of the eye once it was blind revealed a large nonca
seating granuloma of the ciliary body. Inflammation and thrombosis of
several large caliber retinal veins and a single branch of the retrola
minar hemicentral retinal vein were found. Conclusions: Major retinal
branch vein occlusion and hemi-central retinal branch occlusion are ra
re complications of ocular sarcoidosis. Normal serum ACE levels do not
exclude the diagnosis of ocular sarcoidosis even in patients with con
siderable amounts of intraocular granulomatous inflammation.