Cogan-Reese Syndrome with Secondary Angle-Closure Glaucoma: A Rare Case Report and Management Approach

Abstract

Cogan-Reese Syndrome is a rare and progressive ocular disorder classified under iridocorneal endothelial (ICE) syndromes, primarily affecting middle-aged females. It is characterized by abnormal corneal endothelial proliferation leading to distinct iris changes and secondary glaucoma. This case report details the clinical presentation, diagnostic approach, and management of a 42-year-old female diagnosed with CRS and secondary angle-closure glaucoma. The patient presented with progressive diminution of vision in her right eye over two months, with a history of prior glaucoma diagnosis and medical management. Ophthalmic evaluation revealed elevated intraocular pressure (IOP) in the right eye (26 mmHg) with characteristic Cogan-Reese Syndrome features, including iris nodules, ectropion uvea, peripheral anterior synechiae, and full optic disc cupping. Imaging and diagnostic tests, including optical coherence tomography, Humphrey Visual Field analysis, and pachymetry, confirmed glaucomatous changes and corneal endothelial dysfunction. Systemic laboratory investigations indicated elevated inflammatory markers, necessitating multidisciplinary assessment. Management focused on IOP control through a comprehensive medical regimen, including Brimonidine + Timolol, Brinzolamide, Travoprost, Ripasudil, and Pilocarpine eye drops, along with systemic Acetazolamide for additional pressure reduction. Neuroprotective therapy and lubricants were also prescribed. The patient was advised on regular IOP monitoring, with surgical intervention (trabeculectomy) as a potential next step if medical therapy proved insufficient. Given the systemic inflammatory markers, a rheumatology referral was recommended for further systemic evaluation. Cogan-Reese Syndrome remains a challenging condition due to its progressive nature and the potential for severe visual impairment. The case underscores the importance of early detection, detailed clinical evaluation, and individualized treatment strategies to mitigate disease progression. As Cogan-Reese Syndrome -related glaucoma often necessitates surgical intervention, long-term monitoring and multidisciplinary collaboration are crucial in optimizing patient outcomes and preserving visual function.

Keywords

Introduction

       
           
       
Fig. 1. Slit-Lamp Examination Of Right Eye Showing Iris Nodules

Image Courtesy: Captured By Author

       
           
       
Fig. 2. Right Eye AS-OCT showing Iridocorneal angle closure

       
           
       
Fig. 3. Left eye AS-OCT showing normal Iridocorneal Angle

Table 1. Ophthalmic Examination

       
           
       
 Fig. 4. Fundus Photo Showing Full Cupping In Right Eye And Normal Cup Disc Ratio In Left Eye

The patient was diagnosed with Cogan-Reese Syndrome (ICE Syndrome variant) with secondary angle-closure glaucoma in the right eye, along with nodules on the iris, peripheral anterior synechiae, and ectropion uvea. Additional findings included refractive error in both eyes and xanthelasma of the eyelids. Management involved medical therapy primarily aimed at intraocular pressure (IOP) control. The prescribed anti-glaucoma treatment included Brimonidine + Timolol eye drops two times a day, Brinzolamide eye drops two times a day, Travoprost eye drops four times a day, Ripasudil eye drops two times a day, and Pilocarpine eye drops four times a day for the right eye. Lubricants were advised drops four times a day for both eyes. Additionally, neuroprotective therapy with Cap. Alam-OD (Methylcobalamin, Alpha Lipoic Acid, Vitamins A, C, E, B6, Folic Acid, Selenium, and Zinc) was prescribed. Acetazolamide (Diamox) was included for intraocular pressure control with electrolyte monitoring, and NSAIDs (Flexon) were recommended for symptomatic relief of pain. Further recommendations included regular intraocular pressure monitoring and long-term follow-up, with surgical intervention (trabeculectomy) considered if medical therapy failed. Additionally, a rheumatology referral was suggested for systemic evaluation to assess any underlying conditions contributing to the patient’s ocular findings.

DISCUSSION

Iridocorneal endothelial (ICE) syndromes, including Cogan-Reese Syndrome, represent a spectrum of rare and progressive ocular disorders that predominantly affect middle-aged women. These conditions are characterized by the proliferation of abnormal corneal endothelial cells, which migrate across the anterior segment, leading to profound structural alterations in the iris and iridocorneal angle. Cogan-Reese Syndrome is distinguished by the presence of iris nodules, ectropion uvea, and peripheral anterior synechiae, all of which contribute to the progressive elevation of intraocular pressure and subsequent optic nerve damage. The underlying pathophysiology of Cogan-Reese Syndrome involves aberrant endothelial cell proliferation, which forms a membrane over the trabecular meshwork and the iris, leading to progressive angle closure. This process disrupts aqueous humor outflow, resulting in intraocular pressure (IOP) elevation and glaucomatous optic neuropathy. The clinical course of Cogan-Reese Syndrome is often insidious, with progressive visual field loss secondary to uncontrolled intraocular pressure (IOP). Unlike other forms of glaucoma, ICE syndromes do not have a genetic or hereditary basis, and their etiology remains unclear, though viral infections and inflammatory triggers have been proposed as potential contributing factors. Diagnosis of Cogan-Reese Syndrome relies on a combination of clinical assessment and advanced imaging techniques. Slit-lamp biomicroscopy reveals characteristic iris abnormalities, while gonioscopy is crucial in evaluating the extent of angle closure and peripheral anterior synechiae formation. Optical coherence tomography provides detailed imaging of the anterior segment and optic nerve head, aiding in both diagnosis and disease monitoring. Humphrey Visual Field testing is essential for assessing functional visual impairment, while corneal pachymetry can help detect endothelial dysfunction, a hallmark of ICE syndromes. In our patient, the presence of classical Cogan-Reese Syndrome features, including iris nodules, ectropion uvea, peripheral anterior synechiae, and glaucomatous optic neuropathy, confirmed the diagnosis. Elevated inflammatory markers raised the possibility of systemic involvement, underscoring the need for a multidisciplinary approach to management. Although medical therapy remains the first-line treatment for Cogan-Reese Syndrome -related glaucoma, its progressive nature often necessitates surgical intervention when pharmacological measures fail to control IOP adequately. Trabeculectomy, glaucoma drainage devices, and cyclodestructive procedures are potential surgical options for advanced cases. This case highlights the complexity of Cogan-Reese Syndrome management and emphasizes the importance of regular monitoring to prevent irreversible vision loss. Given the progressive nature of ICE syndromes, early detection, comprehensive ophthalmic evaluation, and timely intervention are paramount in preserving visual function and optimizing patient outcomes.

CONCLUSION

Cogan-Reese Syndrome is a rare but potentially vision-threatening disorder that necessitates prompt diagnosis and individualized treatment strategies. As a subset of iridocorneal endothelial (ICE) syndromes, Cogan-Reese Syndrome is marked by progressive endothelial proliferation, leading to significant anterior segment abnormalities and secondary glaucoma. Given the risk of irreversible optic nerve damage, timely intervention to control intraocular pressure (IOP) is crucial in preventing permanent vision loss. The management of Cogan-Reese Syndrome is complex and requires a multidisciplinary approach involving ophthalmologists, glaucoma specialists, and potentially rheumatologists for systemic evaluation when inflammatory markers are elevated. While medical therapy remains the cornerstone for initial intraocular pressure control, many cases eventually necessitate surgical intervention, such as trabeculectomy or glaucoma drainage devices, to maintain long-term ocular health. Regular follow-up and comprehensive ophthalmic evaluations, including gonioscopy, optical coherence tomography (OCT), and visual field testing, are essential to monitor disease progression and assess treatment efficacy. Additionally, patient education regarding the chronic nature of Cogan-Reese Syndrome and the importance of adherence to prescribed therapies is vital in optimizing outcomes. This case underscores the significance of early recognition, meticulous clinical assessment, and long-term management to mitigate disease progression. As Cogan-Reese Syndrome continues to pose challenges due to its progressive nature, an individualized and proactive approach is paramount in preserving visual function and enhancing the patient's quality of life.

Declaration:

Consent

No identifiable patient information or images that reveal the patient’s identity have been included in the manuscript. The images used are strictly clinical in nature and anonymized to ensure privacy. Verbal consent was obtained from the patient for using de-identified clinical data for academic and publication purposes.

IRB Approval

This case report describes a rare clinical presentation and management approach of Cogan-Reese Syndrome with Secondary Angle-Closure Glaucoma. The report is based on routine clinical diagnosis and standard medical treatment without any experimental interventions, modifications in standard care, or research involving human subjects. As per our institution’s guidelines, retrospective case reports that do not involve experimental procedures, new drug trials, or patient-identifiable data are exempt from mandatory Institutional Research Board (IRB) approval.

Conflicts of Interest

The authors declare no conflicts of interest

Funding

This research study was conducted without any financial support.

Data Availability Declaration:

All relevant data supporting the findings of this case study are included within the article and in uploaded related files section. Further details can be made available from the corresponding author upon reasonable request.

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