Unusual retinopathies

Jade S Schiffman MD (Dr. Schiffman, Director of the Eye Wellness Center in Houston, Texas, has no relevant financial relationships to disclose.)
James W Walters PhD OD (Dr. Walters of the University of Houston has no relevant financial relationships to disclose.)
Rosa Ana Tang MD MPH MBA (Dr. Tang of the University Eye Institute at the University of Houston and Neuro-Ophthalmology of Texas, PLLC has no relevant financial relationships to disclose.)
James Goodwin MD, editor. (Dr. Goodwin of the University of Illinois at Chicago has no relevant financial relationships to disclose.)
Originally released December 21, 2009; last updated June 2, 2019; expires June 2, 2022


Primary eye care providers are usually the first ones to encounter unusual retinopathies, but confuse them with other entities such as optic neuritis. Neuro-ophthalmologists are the primary referral points, although referral to general neurology usually occurs if such conditions are accompanied by headaches or dizziness. Patients with retinopathies and choroidopathies, if unrecognized, are usually referred by their primary eye care providers to neuro-ophthalmologists, general neurologists, or uveitis subspecialists (for choroidal conditions), although all groups of subspecialists see all these types of patients. Occasionally, a patient may be referred to a multiple sclerosis specialist if the primary eye care provider misdiagnoses a retinal condition as an optic neuritis. This article addresses the more unusual forms of retinopathies and choroidopathies that might prove challenging to diagnose. Such uncommon pathologies include Usher syndrome, systemic lupus erythematosus, polypoidal choroidal vasculopathy, and punctate inner choroidopathy, among others.

Key points


• Ocult retinopathies and choroidopathies can be misdiagnosed as other conditions, such as optic neuropathies.


• Clinical examination, including a thorough medical history, is essential for diagnosis.


• Using ancillary tests such as ocular coherence tomography (OCT) has helped in the early diagnosis of conditions that are not readily apparent with routine ophthalmoscopy, eliminating the need for advanced neuroimaging.


• Prompt diagnosis and treatment of retinopathies and choroidopathies is essential to avoiding further complications and permanent blindness.


• Recognizing the underlying systemic disease may prove critical to treat the eye disorder (eg, systemic lupus erythematosus or HIV).

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