The medical records of a patient with unilateral visual impairment affected by sarcoidosis were retrospectively reviewed. The present study protocol was conducted in accordance with the Declaration of Helsinki. After a full explanation of the purpose and protocol for this study was provided to the patient, informed consent was obtained.
A 55-year-old woman developed blurred vision in her both eyes 5 months prior to presentation. She initially visited a separate eye clinic and was diagnosed as having bilateral uveitis. She received topical betamethasone, and her blurred vision reduced in severity. After consultation, she commenced with pulmonary medicine. A chest X-ray demonstrated bilateral hilar lymphadenopathy. Laboratory tests showed an elevated angiotensin-converting enzyme (ACE) in the serum. The specimens from a skin biopsy showed noncaseating granulomas confirming the diagnosis of systemic sarcoidosis. Moreover, for a month prior to the visit, she had suffering from severe vision loss in her left eye.
The initial ophthalmic examination disclosed a best-corrected visual acuity (BCVA) of 20/20 in the right eye and hand motion in the left eye. The intraocular pressure (IOP) was within normal range in both eyes. The relative afferent pupil defect (RAPD) was positive in her left eye. Although a slit-lamp examination showed no cell infiltration in the anterior chamber, gonioscopy indicated tent-shaped peripheral anterior synechia in both eyes. A fundus examination of her left eye demonstrated light vitreous opacity, severe disc edema, and hyperemia and a central retinal vein occlusion phenotype including engorgement of all branches of the central retinal vein, dot, and flame-shaped hemorrhages (Fig. 1b). Her right eye demonstrated retinal perivasculitis in the peripheral area. Fluorescein fundus angiography showed hyperfluorescein in the optic disc, but there was no ischemic area in the retina (Fig. 1d). Brain magnetic resonance imaging (MRI) revealed irregular hypertrophy of the left retrobulbar optic nerve (Fig. 2a). Moreover, the post-contrast T1-weighted image showed enhancement of the retrobulbar optic nerve (Fig. 2b). From these findings, it was presumed that retrobulbar lesion related sarcoidosis caused optic disc edema and central retinal vein occlusion. Against our suggestion, she declined to be hospitalized on that day.
When she was hospitalized a week later, the BCVA of her left eye was reduced to no light perception. Moreover, her left fundus showed increased subretinal hemorrhages. Three sets of pulse therapy with intravenous methylprednisolone (1 g/day for 3 days) following oral prednisolone (25 mg/day) were applied, yielding a positive response. Left eye improved according to the counting fingers scale and optic disc edema, and retinal hemorrhages were reduced. Over the 2 months of oral prednisolone tapering, she complained of ocular pain in her left eye. There was a subsequent relapse of recurrence of optic disc edema and engorgement of retinal veins. One set of pulse therapy with intravenous methylprednisolone (1 g/day for 3 days) following oral prednisolone (25 mg/day) was applied, resulting in an improvement concerning the ocular pain and fundus phenotype. Since then, no relapse has occurred even after tapering the prednisolone. Seven months after the initial visit, optic disc edema, retinal hemorrhages, and engorgement of retinal veins had disappeared, and the optic disc became pale in appearance (Fig. 1e). The BCVA of her left eye was stable at light perception. The hypertrophy at the left retrobulbar optic nerve had resolved when observed under a T2-weighted image scan (Fig. 2c). During the clinical course, there was temporal intraocular pressure elevation and bilateral uveitis that could be maintained by topical betamethasone and antiglaucoma medication.