Macular Telangiectasia Type 2: Case Report and Clinical Review

Abstract

Background: Macular telangiectasia type 2 is a bilateral condition of unknown etiology, typically manifesting during the fourth to sixth decades of life. Its prevalence is likely underestimated as there is low disease awareness, both amongst clinicians and patients, and it is often misdiagnosed as age-related macular degeneration in later stages. It is important to understand the pathophysiology, natural history, prognosis, and potential complications to manage this condition properly and provide accurate patient education. Case Report: A 75-year-old white male presented with gradual worsening of vision at all distances for years. Fundus examination revealed bilateral foveal circular red spot with parafoveal pigment clumping in the left eye along with bilateral scattered refractile crystalline deposits. Optical coherence tomography of the macula revealed internal limiting membrane drape in both eyes with focal disruptions to the ellipsoid zone in the left eye. Optical coherence tomography angiography revealed telangiectatic vessels, evident in the superficial and deep plexus, increased intervascular spaces involving the superficial and deep plexus, as well as right-angled vessels and foveal avascular zone irregularities. There was no evidence of progression to proliferative disease and the patient’s vision had remained stable for six years. Conclusion: Macula telangiectasia type 2 is a neurodegenerative disease with the potential to cause vision loss. Newer imaging modalities, specifically optical coherence tomography and optical coherence tomography angiography, have become important diagnostic tools and useful in identifying this disease at earlier stages and differentiating it from other macular degenerative conditions. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/macular-telangiectasia-type-2-case-report-and-clinical-review) Please check COPE course expiry date prior to enrollment. The COPE course test must be taken before the course expiry date.