Clinical and morphologic features of macular telangiectasia type 2: natural course of the disease

Year 2024, Volume: 49 Issue: 3, 760 - 768, 30.09.2024

Püren Işık , İlgin Kaya , Ebru Esen , Nihal Demircan , Hülya Binokay

https://doi.org/10.17826/cumj.1510175

Abstract

Purpose: The purpose of this study is to report the clinical characteristics of macular telangiectasia type 2 (MacTel 2) in a natural disease course.
Materials and Methods: A retrospective analysis of patients diagnosed with MacTel 2 over a 4-year period. Best-corrected visual acuity (BCVA), fundus photography, and optical coherence tomography (OCT) images were reviewed. Differences in BCVA, fundoscopic findings, and OCT parameters were compared between the initial and final visits.
Results: The study included 28 eyes from 14 patients (11 women, 3 males), with a mean age of 65.5 ± 9.8 years. The mean follow-up period was 55.6 ± 32.9 months. The mean BCVA at baseline and final follow-up were 0.51 ± 0.6 and 0.7 ± 0.62 logMAR, respectively. At the first and last visits, the right-angle venules were the most common fundoscopic finding (78.6% for both). Subretinal neovascularization (NV) was initially present in two eyes and developed in one eye during follow-up. The mean temporal macular thickness decreased significantly during the follow-up period, while the central and nasal thickness did not show a significant change. At the last visit, focal ellipsoid zone (EZ) loss was found in 27 eyes (96.4%), compared to 24 eyes (85.7%) at baseline. External limiting membrane (ELM) loss was found in 23 eyes (82.1%) at the first visit and in 25 eyes (89.3%) at the final visit. The increase in mean length of the EZ and ELM loss during the follow-up was not statistically significant.
Conclusion: Despite the progressive effect of the disease on central visual acuity, it is very important to closely monitor these eyes for the development of secondary NV, which may develop due to degenerative and atrophic changes in the macula.

Keywords

ellipsoid zone, external limiting membrane, hyporeflective cavitation, macular telangiectasia type 2

Maküler telenjiektazi tip 2'nin klinik ve morfolojik özellikleri: hastalığın doğal seyri

Abstract

Amaç: Bu çalışmanın amacı maküler telenjiektazi tip 2'nin (MacTel 2) doğal hastalık seyrindeki klinik özelliklerini bildirmektir.
Gereç ve Yöntem: MacTel 2 tanılı hastaların 4 yıllık bir süre boyunca en iyi düzeltilmiş görme keskinliği (EİDGK), fundus fotoğrafı ve optik koherens tomografi (OKT) görüntüleri incelenerek retrospektif analizleri yapıldı. EİDGK, fundoskopik bulgular ve OKT parametreleri ilk ve son vizitler arasında karşılaştırıldı.
Bulgular: Çalışmaya ortalama yaşı 65,5 ± 9,8 yıl olan 14 hastanın (11 kadın, 3 erkek) 28 gözü dahil edildi. Ortalama takip süresi 55,6 ± 32,9 ay idi. Başlangıç ve son vizit ortalama EİDGK sırasıyla 0,51 ± 0,6 ve 0,7 ± 0,62 logMAR idi. İlk ve son vizitlerde, dik açılı venüller en yaygın fundoskopik bulguydu (%78,6). Subretinal neovaskülarizasyon (NV) iki gözde başlangıçta mevcuttu ve takip sırasında bir gözde gelişti. Ortalama temporal makula kalınlığı takip süresi boyunca anlamlı olarak azalırken, santral ve nazal kalınlık da anlamlı bir değişiklik bulunmadı. Başlangıçta 24 gözde (%85,7) fokal ellipsoid zon (EZ) kaybı mevcutken, son vizitte 27 gözde (%96,4) saptandı. Dış limitan membran kaybı ilk vizitte 23 gözde (%82,1) saptanırken, son vizitte 25 gözde (%89,3) mevcuttu. Takip süresince ortalama EZ ve dış limitan membran uzunluğu kaybındaki artış istatistiksel olarak anlamlı bulunmadı.
Sonuç: Hastalığın merkezi görme keskinliği üzerindeki ilerleyici etkisine rağmen, bu gözlerin maküladaki dejeneratif ve atrofik değişikliklere bağlı olarak gelişebilecek ikincil NV gelişimi açısından yakından izlenmesi önemlidir.

Keywords

Dış limitan membran, ellipsoid zon, hiporeflektif kavitasyon, makular telanjiektazi tip-2

Supporting Institution

Çukurova Üniversitesi

Thanks

Optik koherens tomografi çekimlerinde büyük emeği olan teknisyenimiz Pınar Çakır' a teşekkür ederiz.

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