Case of the Month

Edited by Robert N. Johnson, MD

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Case #118 April, 2019

Presented by Michelle Peng, MD

A 64-year-old woman with blurred vision following cataract surgery

Case of the Month Listing

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Figure 1A: Color photo of right eye. Note the gray-whitish area at the temporal edge of the fovea and fine crystalline deposits.

Figure 1B: Color photo of left eye. Note the gray-whitish area at the temporal edge of the fovea, fine crystalline deposits and pigment hyperplasia.

Case History

A 64-year-old woman presented with persistently blurred vision in both eyes following uncomplicated cataract surgery.

On examination, visual acuity was 20/80 in the right eye and 20/50 in the left eye.  Pupils were equal round and reactive with no afferent pupillary defect.  The intraocular pressures and anterior segment were unremarkable. Fundus examination demonstrated blunted dilated venules, mildly ectatic capillaries, crystalline deposits, and retinal pigment epithelial hyperplasia in both eyes (Figure 1). Ocular coherence tomography (OCT) showed cystic cavitation, photoreceptor disruption, and outer retinal damage (Figure 2). OCT angiography demonstrated the telangiectatic capillaries particularly within the temporal macula (Figure 3). Early phase fluorescein angiography highlighted the irregularly dilated capillaries and revealed hyperfluorescence in late frames (Figure 4).

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Figure 2A: SD-OCT of the right macula. Note the atrophy and disruption of the retinal layers at the temporal edge of the fovea and small areas of cavitation.

Figure 2B: SD-OCT of the left macula. Note the atrophy and disruption of the retinal layers at the temporal edge of the fovea and areas of inner retinal hyperreflectivity due to RPE hyperplasia seen on the color photo (Figure 1B)

Figure 3A: OCT-angiogram of the right macula. Note the telangiectatic vessels.

Figure 3B: OCT-angiogram of the left macula. Note the telangiectatic vessels and some capillary nonperfusion.

Figure 4A: Early arteriovenous phase fluorescein angiogram of th right macula. Telangiectatic vessels can be seen on the nasal side of the fovea, but early dye leakage is already obscuring the view temporally.

Figure 4B: Late phase fluorescein angiogram of the right macula. Note the dye leakage in the fovea.

What is your Diagnosis?

Differential Diagnosis

Crystalline retinopathies – Toxicity (Methoxyflurane, Nitrofurantoin, Tamoxifen, Ritonavir, Triamcinolone, Talc), Metabolic disorders (Secondary hyperoxaluria) Inherited diseases (Bietti’s, Cystinosis), Vascular diseases (Talc, Macular Telangiectasia), Idiopathic (Chronic retinal detachment)

 

Additional History and Diagnosis

The patient has a history of well-controlled Type II diabetes mellitus. She had no other past ocular history. Based on her clinical history and exam findings the patient was diagnosed with macular telangiectasia type IIA.

 

Discussion

Macular telangiectasia type IIA is a clinical entity first described by Donald Gass as a bilateral, symmetric, paracentral capillary telangiectasia of unknown cause which develops in the fifth to sixth decade.1,2 More recent studies suggest that it is a primary neurodegenerative condition of blood vessels and Muller cells.3

The condition has five stages: Stage I has very minimal capillary dilation with little to no noted abnormalities. Stage 2 demonstrates slight graying of the parafoveolar retina but continues to have minimal telangiectatic changes. In stage 3 there is an increase in the number of blunted and dilated venules that extends into the parafoveolar retina at right angles. Stage 4 is characterized by the development of hyperplastic RPE which encircles the angular vessels. In stage 5 the parafoveal macula may develop subretinal neovascularization. Multiple small golden crystalline may be found in the inner retina in about half of patients.4,5

OCT typically demonstrates cystic cavities with variable disruption in retinal layers. These cavities tend to collapse leaving an area of atrophy. In later stages patients may present with a lamellar macular hole or reactive pigment hyperplasia.6,7 Early phase fluorescein angiography highlights the telangiectatic vessels. Diffuse hyperfluorescence is seen in late phases.8 Some patients may develop subretinal neovascularization which may lead to subretinal hemorrhage, disciform scar, or retinochoroidal hemorrhage. Loss of vision typically occurs slowly due to atrophy of the foveolar retina.2,4

Numerous treatment modalities have been tried but none appear to address non-neovascular macular telangiectasia.8,9 In cases of subretinal neovascularization anti-VEGF treatment may be used.10,11 A recent Phase II clinical trial of the ciliary neurotrophic factor implant showed decreased ellipsoid zone loss, the progression of disease.12

Take Home Points

  • Macular telangiectasia represents a primary neurodegenerative condition of blood vessels and Muller cells
  • Anti-VEGF treatments may be used for neovascular macular telangiectasia

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References

  1. Gass JDM. A fluorescein angiographic study of macular dysfunction secondary to retinal vascular disease v. retinal telangiectasias. Arch Ophthalmol 1968;80(5):592-605.
  2. Gass JDM, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982;100:769–80.
  3. Powner MB, Gillies MC, Tretiach M, et al. Perifoveal Muller cell depletion in a case of macular telangiectasia type 2. Ophthalmology. 2010;117:2407-2416.
  4. Agarwal A. Congenital and acquired idiopathic macular retinal telangiectasia. In: Agarwal, A. Gass’ Atlas of Macular Diseases. Fifth ed. Elsevier: 526-33.
  5. Gass JDM, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis; update of classification and follow-up study. Ophthalmology 1993;100:1536–46.
  6. Cohen SM, Cohen ML, El-Jabali F, et al. Optical coherence tomography findings in nonproliferative group 2a idiopathic juxtafoveal retinal telangiectasis. Retina 2007;27(1):59-66.
  7. Patel B, Duvall J, Tullo AB. Lamellar macular hole associated with idiopathic juxtafoveolar telangiectasia. Br J Ophthalmol 1988;72:550–1.
  8. Park DW, Schatz H, McDonald HR, Johnson RN. Grid laser photocoagulation for macular edema in bilateral juxtafoveal telangiectasis. Ophthalmol 1997;104(11):1838-46.
  9. De Lahitte GD, Cohen SY, Gaudric A. Lack of apparent short-term benefit of photodynamic therapy in bilateral, acquired, parafoveal telangiectasis without subretinal neovascularization. Am J Ophthalmol 2004;138(5):892-4.
  10. Kovach JL, Rosenfeld PJ. Bevacizumab (avastin) therapy for idiopathic macular telangiectasia type II. Retina 2009;29(1):27-32.
  11. Charbel Issa P, Finger RP, Kruse K, Baumuller S, Scholl HP, Holz FG. Monthly ranibizumab for nonproliferative macular telangiectasia type 2: a 12-month prospective study. Am J Ophthalmol 2011;151(5):876-886 e1.
  12. Chew EY, Clemons TE, Jaffe GJ, et al. Effect of ciliary neurotrophic factor on retinal neovascularization in patients with macular telangiectasia type 2. Ophthalmol 2019 Apr;126(4):540-549.

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