Macular edema in Cogan-Reese syndrome

Area of Science:

• Ophthalmology
• Retinal Diseases
• Uveitis

Background:

• Iridocorneo-endothelial (ICE) syndrome is a rare group of disorders characterized by glaucoma, corneal, and iris abnormalities.
• Retinal complications are infrequently reported in ICE syndrome, making them diagnostically challenging.

Observation:

• A middle-aged woman with Cogan-Reese Syndrome (CRS), a form of ICE syndrome, presented with intractable ocular hypertension (OHT).
• During follow-up, she developed cystoid macular edema (CME), a rare retinal complication in this context.

Findings:

• The CME was suspected to be inflammatory, potentially linked to the underlying pathophysiology of CRS.
• Treatment with topical nonsteroidal anti-inflammatory drugs (NSAIDs) led to successful resolution of the CME.
• The CME recurred upon cessation of NSAID treatment, but responded again to reintroduction of the medication.

Implications:

• This case highlights the possibility of inflammatory retinal complications, such as CME, in CRS.
• Topical NSAIDs represent a viable treatment option for CME associated with CRS.
• The findings suggest a potential role for long-term management of CME in CRS patients, given the risk of recurrence.