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Retina

Case 77

Patient presentation: A 58-year-old woman was referred to a tertiary retina clinic by her optometrist due to an atypical retinal examination. She did note her visual acuity had progressively declined over the last 2 years, but she was informed by another eye care provider that it was due to refractive error. Her past medical history was significant for type 2 diabetes mellitus and hypertension, both of which were controlled with oral medications. She had no past ocular history. BCVA was 20/40 OD and 20/50 OS. IOP was normal and there was no RAPD. Anterior segment examination demonstrated 1+ nuclear sclerotic cataracts OU. Fundus photographs and fundus autofluorescence images were taken and are shown below:

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Question 1: What are the most striking features of the fundus photographs and FAF images above?

OCT macula images were also taken and are shown below:

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Question 2: What are the most prominent findings in the OCT macula images above?

Question 3: Based on the patient presentation, fundus examination, FAF and OCT imaging, what is the most likely diagnosis?

Question 4: What is the treatment for this patient?

References:

  1. Heeren TFC, Chew EY, Clemons T, Fruttiger M, Balaskas K, Schwartz R, Egan CA, Charbel Issa P; MacTel Study Group. Macular Telangiectasia Type 2: Visual Acuity, Disease End Stage, and the MacTel Area: MacTel Project Report Number 8. Ophthalmology. 2020 Nov;127(11):1539-1548.

  2. Wu L, Evans T, Arevalo JF. Idiopathic macular telangiectasia type 2 (idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2). Surv Ophthalmol. 2013 Nov-Dec;58(6):536-59

  3. Chew EY, Clemons TE, Jaffe GJ, Johnson CA, Farsiu S, Lad EM, Guymer R, Rosenfeld P, Hubschman JP, Constable I, Wiley H, Singerman LJ, Gillies M, Comer G, Blodi B, Eliott D, Yan J, Bird A, Friedlander M; Macular Telangiectasia Type 2-Phase 2 CNTF Research Group. Effect of Ciliary Neurotrophic Factor on Retinal Neurodegeneration in Patients with Macular Telangiectasia Type 2: A Randomized Clinical Trial. Ophthalmology. 2019 Apr;126(4):540-549.

Learning Objectives:

  1. Describe the typical OCT features of macular telangiectasia type 2

  2. Understand the risk factors and management of mac tel

  3. Learn the classification of macular telangiectasia

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