Macular Telangiectasia Type 2: Visual Acuity, Disease End Stage, and the MacTel Area

Background/AimMacular pigment optical density (MPOD) is centrally depleted early on in macular telangiectasia type 2 (MacTel). Contrast sensitivity (CS) might be related to MPOD, and thus impaired in early MacTel. The effect of low luminance was assessed on both CS and best corrected visual acuity (BCVA).MethodsThis is a cross-sectional study. Pelli-Robson charts were used for CS testing at 1 m in photopic (110 lux) and mesopic (1 lux) conditions. BCVA was tested with ETDRS charts and low luminance visual acuity (LLVA) with a 2.0 log unit neutral density filter. MPOD was obtained with dual-wavelength autofluorescence.ResultsOne hundred and three eyes of 52 patients with MacTel (mean±SD age 62.9±10.2, range 35–77) were compared with 34 healthy eyes of 17 controls (mean±SD age 65.2±7.4, range 53–78). CS was significantly lower in the eyes with MacTel. This impairment was higher in low light conditions (low light contrast sensitivity (LL-CS)). Eyes at the early stages of MacTel had significantly lower LL-CS than controls, but normal (photopic) CS. The results were similar but less pronounced for BCVA/LLVA. Decrease in CS was correlated with loss of MPOD.ConclusionsLow light conditions have a detrimental effect on visual performance in MacTel. Impaired CS might correlate with MPOD depletion as a pathognomonic finding in MacTel. Functional impairment might precede structural disintegration, indicating dysfunction at the cellular level. The applied tests might be useful as additional functional assessments in clinical routine and as outcome measures in future interventional clinical trials.

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Macular Telangiectasia Type 2 With Spontaneous Resolution of Intraretinal/Sub-Internal Limiting Membrane Hemorrhage in the Absence of Neovascularization

Journal of VitreoRetinal Diseases ◽

10.1177/2474126417731478 ◽

2017 ◽

Vol 1 (6) ◽

pp. 415-419 ◽

Cited By ~ 1

Author(s):

Varun Chandra ◽

Rohan Merani ◽

Alex P. Hunyor ◽

I-Van Ho ◽

Mark Gillies

Keyword(s):

Visual Acuity ◽

Multimodal Imaging ◽

Internal Limiting Membrane ◽

Macular Telangiectasia ◽

Corrected Visual Acuity ◽

Visual Improvement ◽

Macular Telangiectasia Type 2 ◽

The Right

Purpose: To describe a case of macular telangiectasia type 2 (MacTel) presenting with decreased vision due to intraretinal/sub-internal limiting membrane (ILM) hemorrhage in the absence of neovascularization. Method: Clinical examination and multimodal imaging were performed. Results: A 65-year-old female presented with blurred left vision, recording 20/160 in that eye. There was intraretinal hemorrhage at the left macula centrally, with sub-ILM hemorrhage superiorly and inferiorly. Optical coherence tomography (OCT) showed no evidence of subretinal neovascularization. Imaging of the right macula was consistent with MacTel. The blood spontaneously cleared and the left visual acuity gradually improved to 20/25 by 4 months. Fluorescein angiography confirmed MacTel, and once the hemorrhage resolved, both inner and outer retinal cavitation was identified on OCT of the left macula. The left best-corrected visual acuity remained at 20/25 at 2-year follow-up. Conclusion: Spontaneous resorption of hemorrhage was accompanied by visual improvement.

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Long-term Clinical Course of Korean Patients Diagnosed with Macular Telangiectasia Type 2

Journal of the Korean Ophthalmological Society ◽

10.3341/jkos.2021.62.8.1098 ◽

2021 ◽

Vol 62 (8) ◽

pp. 1098-1104

Author(s):

Kyung Hae Kang ◽

Ji Hyun Yoon ◽

Jong Woo Kim ◽

Chul Gu Kim ◽

Jae Hui Kim

Keyword(s):

Visual Acuity ◽

Final Visit ◽

Ellipsoid Zone ◽

Visual Deterioration ◽

Macular Telangiectasia ◽

Macular Telangiectasia Type 2 ◽

The Mean

Purpose: To evaluate long-term visual prognosis and changes in the retinal microstructure in patients with macular telangiectasia type 2.Methods: Retrospective analysis of medical records were performed for 61 eyes (32 patients) diagnosed with macular telangiectasia type 2. The visual acuity at diagnosis was compared with that at the final visit. In addition, cases were classified into three grades based on optical coherence tomography (OCT) findings: grade 1, inner retinal cavities only; grade 2, outer retinal cavities with ellipsoid zone disruption; and grade 3, ellipsoid zone disruption with proliferation of retinal pigment epithelium. Change in the grade during the follow-up was verified. In addition, visual acuities were compared between eyes with and without a change in grade.Results: The mean follow-up period was 41.5 ± 33.2 months. Visual acuity had deteriorated significantly from the mean logarithm of the minimal angle of resolution 0.23 ± 0.26 at diagnosis to a mean of 0.31 ± 0.31 at the final visit (p < 0.001). When classified using OCT, 65.6%, 22.9%, and 11.5% were grades 1, 2, and 3, respectively, at diagnosis and 47.5%, 36.1%, and 16.4% at the final visit. Progression of grade was noted in 22.9%. A higher degree of visual deterioration was noted in eyes showing progression than in eyes without (p = 0.002).Conclusions: During the long-term follow-up period, visual deterioration was noted in patients with macular telangiectasia type 2. However, the degree of deterioration was not high. Progression in the disruption of the retinal microstructure was the likely cause of visual deterioration.

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Autofluorescence patterns and visual acuity in macular telangiectasia type 2

Acta Ophthalmologica ◽

10.1111/j.1755-3768.2013.3466.x ◽

2013 ◽

Vol 91 ◽

pp. 0-0

Author(s):

K BALASKAS ◽

I LEUNG ◽

FB SALLO ◽

TE CLEMONS ◽

AC BIRD ◽

...

Keyword(s):

Visual Acuity ◽

Macular Telangiectasia ◽

Macular Telangiectasia Type 2

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Retinal cavitations in macular telangiectasia type 2 (MacTel): longitudinal structure–function correlations

British Journal of Ophthalmology ◽

10.1136/bjophthalmol-2019-315416 ◽

2020 ◽

Vol 105 (1) ◽

pp. 109-112 ◽

Cited By ~ 1

Author(s):

Cindy X Cai ◽

John Choong ◽

Sina Farsiu ◽

Stephanie J Chiu ◽

Emily Y Chew ◽

...

Keyword(s):

Visual Acuity ◽

Average Rate ◽

Intraclass Correlation ◽

Rate Of Change ◽

Ellipsoid Zone ◽

Macular Telangiectasia ◽

Longitudinal Structure Function ◽

Prospective Phase ◽

Macular Telangiectasia Type 2

Background/aimsTo quantify retinal cavitation size over time in macular telangiectasia type 2 (MacTel) and to correlate changes with visual acuity and area of ellipsoid zone loss.MethodsOptical coherence tomography (OCT) macula volume scans from sham eyes included in a prospective, phase II clinical trial of human ciliary neutrophic factor for MacTel at baseline, 1 year and 2 years of follow-up were analysed. Cavitations were segmented by two independent readers. Total cavitation volume was compared with area of ellipsoid zone loss and best-corrected visual acuity (BCVA).ResultsFifty-one eyes from 51 unique patients (mean age 62 years, range 45–79 years) were included. Intraclass correlation between readers for cavitation volume was excellent (>0.99). Average cavitation volume was 0.0109 mm3, 0.0113 mm3 and 0.0124 mm3 at baseline, 1 year and 2 years, respectively. The average rate of cavitation volume change was +0.0039 mm3/year. 10 eyes (20%) had a significant change in cavitation volume during the study (3 decreased, 7 increased). Eyes with increased cavitation volume had worse BCVA compared with eyes with no change/decreased cavitation volume (71.5 vs 76.1 ETDRS letters, respectively). Cavitation volume was negatively correlated to BCVA (r=−0.37) but not to area of ellipsoid zone loss. Cavitation volume was negatively predictive of BCVA in both univariate and multivariate mixed-effects modelling with ellipsoid zone loss.ConclusionsRetinal cavitations and their rate of change in MacTel can be reliably quantified using OCT. Cavitations are negatively correlated with visual acuity and may be a useful OCT-based biomarker for disease progression and visual function in MacTel.

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Long-Term Outcomes in Macular Telangiectasia Type 2 With Subretinal Neovascularization

Journal of VitreoRetinal Diseases ◽

10.1177/2474126420927149 ◽

2020 ◽

Vol 4 (5) ◽

pp. 386-392

Author(s):

Alexander R. Bottini ◽

Barton L. Blackorby ◽

Madelyn Michaels ◽

Katherine Burkett ◽

Sabin Dang ◽

...

Keyword(s):

Visual Acuity ◽

Final Visit ◽

Long Term Outcomes ◽

Subretinal Neovascularization ◽

Macular Telangiectasia ◽

Anti Vegf ◽

Macular Telangiectasia Type 2

Purpose: This work reports long-term outcomes in macular telangiectasia type 2 (MacTel) with subretinal neovascularization (SRNV). Methods: A retrospective, single-center review of medical records was performed on all patients with a diagnosis of MacTel presenting between May 2004 and October 2019. Medical and ocular history, best-corrected visual acuity (BCVA) at baseline and final visit, optical coherence tomography data, and treatment history of SRNV secondary to MacTel were recorded. Results: A total of 471 eyes were diagnosed with MacTel. SRNV was present in 44 eyes (9.3%), of which 38 eyes met inclusion criteria for SRNV. Average follow-up duration in the SRNV group was 78.4 months. All SRNV patients underwent antivascular endothelial growth factor (anti-VEGF) therapy. There was no significant change from mean baseline (0.59 ± 0.45) to final (0.70 ± 0.49) BCVA in the SRNV group as a whole ( P = .13). Subgroup analysis revealed 17 of 38 eyes had SRNV at diagnosis and received immediate anti-VEGF treatment. In this subgroup mean pretreatment BCVA was 0.89 ± 0.43 and the mean final BCVA was 0.87 ± 0.61 ( P = .84). The remainder (21 of 38 eyes) developed SRNV during follow-up. In this subgroup, final BCVA after initiation of treatment was 0.56 ± 0.32, an improvement in BCVA from SRNV onset ( P = .04) and a decrease from pre-SRNV onset baseline BCVA ( P = .008). Conclusions: Visual acuity is maintained, not improved, in long-term follow-up of MacTel with SRNV treated with anti-VEGF. Patients presenting with SRNV have a worse prognosis than those who develop SRNV during follow-up.

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Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study—Report No. 1

Scientific Reports ◽

10.1038/s41598-020-73803-9 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Young Ho Kim ◽

Yoo-Ri Chung ◽

Jaeryung Oh ◽

Seong-Woo Kim ◽

Christopher Seungkyu Lee ◽

...

Keyword(s):

Visual Acuity ◽

Macular Hole ◽

Early Stage ◽

Disease Process ◽

Disease Stage ◽

Optical Coherence ◽

Macular Telangiectasia ◽

Macular Telangiectasia Type 2 ◽

Sd Oct

Abstract We analysed the imaging findings of macular telangiectasia (MacTel) type 2 in Korea using spectral domain optical coherence tomography (SD-OCT) and investigated their relationship with visual acuity and clinical stages. A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and included 129 patients. We analysed all the SD-OCT images encompassing the macular area. Hyporeflective cavities (77.7%) were the most frequently detected abnormalities in SD-OCT. Disruption of the external limiting membrane, ellipsoid zone, and interdigitation zone were found in 67 (40.4%), 87 (52.4%), and 94 eyes (56.6%), respectively. Four eyes (2.4%) had lamellar macular hole, and five eyes (3.0%) full-thickness macular hole. Neovascularisation, either subretinal or intraretinal, was found in 14 eyes (8.4%). Eyes with outer retinal hyperreflective band disruption had lower visual acuity than those without them. The presented characteristic clinical features of OCT in MacTel type 2 can not only aid in differentiating this disease from others but are also helpful for better judgement of the disease stage in daily clinical practice. Inner retinal hyporeflective cavities without outer retinal abnormalities on SD-OCT, although classified as severity scale 3, could be considered a relatively early stage in the disease process in terms of vision.

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Improvements in visual acuity and macular morphology following cessation of anti-estrogen drugs in a patient with anti-estrogen maculopathy resembling macular telangiectasia type 2: a pathogenic hypothesis

BMC Ophthalmology ◽

10.1186/s12886-019-1280-1 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 2

Author(s):

Akihiro Shinkai ◽

Wataru Saito ◽

Yuki Hashimoto ◽

Susumu Ishida

Keyword(s):

Visual Acuity ◽

Vision Loss ◽

Pigment Epithelium ◽

Fundus Autofluorescence ◽

Retinal Pigment ◽

Macular Telangiectasia ◽

History Of ◽

Macular Telangiectasia Type 2 ◽

The Relationship

Abstract Background The relationship between anti-estrogen drugs and macular telangiectasia type 2 (MacTel-2) remains unknown. Here we report a case with anti-estrogen maculopathy resembling MacTel-2 with improved visual function and macular morphology following cessation of anti-estrogen drugs. Case presentation A 53-year-old woman presented with a 5-month history of central vision loss and anorthopia in both eyes. She had received oral tamoxifen followed by toremifene for 69 months. Funduscopy, fluorescein angiography, and optical coherence tomography (OCT) revealed MacTel-2-like findings OU. Fundus autofluorescence (FAF) showed hyper-autofluorescence at the fovea OU. Visual acuity, macular morphology on OCT, and FAF findings gradually improved after cessation of anti-estrogen drugs. Conclusions In the present case, visual acuity, macular morphology, and impairment of the retinal pigment epithelium (RPE) improved following cessation of anti-estrogen drugs, suggesting the relationship between retinal toxicity of anti-estrogen drugs and the development of MacTel-2-like findings. From these results and the previous observations, toxicity of both photoreceptor and RPE cells caused by anti-estrogen drugs may contribute to the development of anti-estrogen maculopathy similar to MacTel-2.

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