Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review
Abstract
:1. Introduction
2. Materials and Methods
3. Results
- Category 1: IPT without peripheral exudates and without macular involvement (patient 1)
- Category 2: IPT with peripheral exudates and without macular involvement (patient 2)
- Category 3: IPT with peripheral exudates and cystoid macular edema without exudates (patients 3,4,5)
- Category 4: IPT with peripheral exudates and macular hard exudates and edema (patient 6)
3.1. Categories 1 and 2
3.2. Category 3
3.3. Category 4
4. Literature Review
5. Discussion
5.1. Definitions, Classification, and Presentation
5.2. Diagnostics
5.3. Treatment
6. Conclusions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Term | Characteristics |
---|---|
Coats disease | Idiopathic, nonhereditary aneurysmal retinal telangiectasia associated with intraretinal exudation and frequent exudative retinal detachment, occurring in patients aged a few months to seven decades with the peak in the first decade of life [2] |
Leber miliary aneurysms | A milder variant of Coats disease, usually occurring in young adults and located at the retinal periphery [7,8,9] |
Idiopathic retinal telangiectasia | A descriptive name for a Coats disease |
MACTEL type 1 | Macular telangiectasia type 1—aneurysmal type of macular telangiectasia, considered a central variant of Coats disease [13] |
MACTEL type 2 | Macular telangiectasia type 2—non-aneurysmal perifoveal capillary telangiectasia, associated with atrophy of neurosensory retina, presenting in the non-proliferative or proliferative form [13,14] |
Case No/Gender | Proposed Category | Age (Years) | Eye, BCVA, Presentation of the Macula | Treatment | Follow-Up | Final BCVA and Disease State |
---|---|---|---|---|---|---|
1. M | 1 | 20 | RE, 20/20; no exudates, macular area normal | Observation | 12 months | 20/20; no progression |
2. F | 2 | 60 | LE, 20/25; peripheral hard exudates, macular area normal | LPC in the periphery | 12 months | 20/25; no progression |
3. M | 3 | 20 | LE, 20/60; peripheral hard exudates, CME without exudates | LPC in the periphery, intravitreal anti-VEGF | 12 months | 20/30; remission of CME |
4. M | 3 | 44 | RE, 20/25; demarcated peripheral hard exudates, mild CME without exudates | LPC in the periphery, intravitreal anti-VEGF | 20 months | 20/80; progression of CME despite treatment |
5. M | 3 | 55 | RE, 20/100; asteroid hyalosis, localized hard exudates outside the fovea, CME without exudates; risk of macular hole formation | LPC in the periphery | 6 months | 20/100; no improvement, intravitreal anti-VEGF scheduled, possible surgical treatment |
6. M | 4 | 21 | LE, 6/200; peripheral and central hard exudates, CME | LPC in the periphery and GRID in the macula | 12 months | 20/20; remission of ME |
Study | Population | Treatment | Mean Follow-Up | Main Outcome |
---|---|---|---|---|
Smithen et al., 2005 [16] | 13 adults >35 years | LPC in 11 cases; 2 cases observed (short follow-up) | 5.8 years (range: 0–17) | Average loss of 2.1 lines. BCVA improvement in 2 cases, stability in 3 cases, and decline in 6 cases. At the final follow-up, BCVA ≥ 20/40 in 5 cases and BCVA < 20/200 in 3 cases. |
Goel et al., 2011 [17] | 3 adults | Single intravitreal bevacizumab followed by LPC | 9 months | Significant improvement of BCVA in all cases of from counting fingers to 20/300, counting fingers to 20/240, and 20/240 to 20/120; regression of hard exudates from the macula in all cases |
Wang et al., 2011 [18] | 3 adults | 2 injections of bevacizumab followed by LPC | 0.5–2 years (2, 0.5, 1 years, respectively) | Significant improvement in BCVA, reduction of CRT, and regression of telangiectasias.
|
Zheng et al., 2014 [19] | 5 adults | Intravitreal bevacizumab followed by LPC (3 cases) or intravitreal triamcinolone (1 case) or subsequent intravitreal bevacizumab (average of 2 injections during follow-up) | 10.6 months | Resolution of subretinal fluid and telangiectasias without significant improvement in BCVA (range: 1.42–1.25 logMAR). Vitreoretinal fibrosis in two cases. |
Park et al., 2016 [20] | 13 adults | LPC combined with intravitreal bevacizumab (mean no. of injections: 2.69 and mean no of laser sessions: 1.68) | 24.8 months | Mean BCVA change from 0.72 logMAR to 0.68 logMAR (statistically insignificant). BCVA improvement of more than 3 lines in 3 patients (23%) and stability in 7 patients (54%). Mean CRT was significantly decreased from 473 to 288 μm. Poor baseline BCVA and subfoveal hard exudates correlated with poor final BCVA result. |
Rishi et al., 2016 [21] | 48 adults ≥ 35 years 32 cases observed > 6 months | LPC (60.4%), observation (27.08%), surgery (6.2%), cryotherapy (4%), LPC plus cryotherapy (2%) | 40 months (range: 1–122 months) | Patients with follow-up longer than 6 months (32 cases):
|
Zhang et al., 2018 [22] | 12 adults | Intravitreal ranibizumab or conbercept followed by LPC | 23.10 ± 7.8 | Mean BCVA improvement significant from 1.27 ± 0.69 to 1.05 ± 0.73 logMAR; mean injection no. 2.33 ± 0.65, mean no. of laser treatments 2.5 ± 0.8 |
Reference studies: children and adults reported in one cohort | ||||
Shields et al., 2001 [10] | 124 eyes observed > 6 months Age 1 month to 63 years (average: 5 years) | Cryotherapy (42%), LPC (13%), observation (18%), surgery 17% and enucleation 11% | 55 months (range: 6–300 months) | Anatomic improvement and stability in 76%. BCVA ≥ 20/50 in 14%, 20/60 to 20/100 in 6%, 20/200 to finger counting in 24%, and hand motion to light perception in 40% |
Shields et al., 2019 [15] | 351 cases, data from 45 years Age 0–79 years, median: 6 years | Overall (1973–2018): observation (21%), LPC (42%), cryotherapy (55%), sub-Tenon corticosteroids (12%), intravitreal corticosteroids (4%), anti-VEGF (10%), and primary enucleation (5%) Years 2010–2018: observation (11%), LPC (72%), cryotherapy (68%), sub-Tenon corticosteroids (29%), intravitreal corticosteroids (9%), anti-VEGF (18%), primary enucleation (1%) | 58 months (range: 0–466 months) | BCVA overall Verbal
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Gawęcki, M. Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review. J. Clin. Med. 2021, 10, 1767. https://doi.org/10.3390/jcm10081767
Gawęcki M. Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review. Journal of Clinical Medicine. 2021; 10(8):1767. https://doi.org/10.3390/jcm10081767
Chicago/Turabian StyleGawęcki, Maciej. 2021. "Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review" Journal of Clinical Medicine 10, no. 8: 1767. https://doi.org/10.3390/jcm10081767
APA StyleGawęcki, M. (2021). Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review. Journal of Clinical Medicine, 10(8), 1767. https://doi.org/10.3390/jcm10081767