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Morphologische Charakteristika bei makulären Teleangiektasien Typ 2

Morphological characteristics in macular telangiectasia type 2

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Zusammenfassung

Makuläre Teleangiektasien Typ 2 sind charakterisiert durch atrophische Veränderungen der zentralen Netzhaut, die mit einem definierten vaskulären Phänotyp einhergehen. Die Erkrankung manifestiert sich in einem ovalen zentralen Netzhautbereich einer Größe von etwa 2 Papillendurchmessern mit einer topographischen Prädisposition temporal des fovealen Zentrums. Funduskopisch sind ein leichter Transparenzverlust der parazentralen Netzhaut, kristalline Ablagerungen, ektatische Kapillaren, verdickte zentrale Venolen und plaqueartige Hyperpigmentierungen charakteristisch. Als Komplikationen können im Verlauf der Erkrankung Neovaskularisationen oder auch Makulaforamina auftreten. Angiographisch lassen sich eine parazentrale Leckage und meist auch ektatische Kapillaren darstellen. In der OCT (optische Kohärenztomographie)-Untersuchung zeigen sich häufig hyporeflexive Kavitäten und Substanzverluste in der Photorezeptorschicht. Besonders charakteristisch ist ein erhöhtes (para)zentrales Fundusautofluoreszenzsignal aufgrund einer Verminderung der Makulapigmentdichte.

Abstract

Macular telangiectasia type 2 is characterized by atrophic alterations of the central retina which is accompanied by a defined vascular phenotype. The disease manifests within an oval central retinal area the size of approximately two disc diameters, with a topographic predisposition temporal to the foveal center. Funduscopy reveals reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, thickened venules and retinal pigment plaques. Secondary neovascularization and macular holes may occur during the disease course. Fluorescein angiography usually shows a diffuse leakage and often ectatic capillaries. On optical coherence tomography (OCT) examination, hyporeflective cavities and focal atrophy of the photoreceptor layer represent a frequent finding. A characteristic sign is an increased (para) central signal on fundus autofluorescence imaging due to a reduced density of macular pigment.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. P. Charbel Issa, T.F.C. Heeren, E. Krüger, M. Zeimer, D. Pauleikhoff und F.G. Holz geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Universitäts-Augenklinik Bonn, Ernst-Abbe-Str. 2, 53127, Bonn, Deutschland

    P. Charbel Issa, T.F.C. Heeren, E. Krüger & F.G. Holz

  2. Augenklinik, St. Franziskus Hospital Münster, Münster, Deutschland

    M. Zeimer & D. Pauleikhoff

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  1. P. Charbel Issa
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  2. T.F.C. Heeren
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  3. E. Krüger
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  4. M. Zeimer
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  5. D. Pauleikhoff
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  6. F.G. Holz
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Correspondence to P. Charbel Issa.

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Charbel Issa, P., Heeren, T., Krüger, E. et al. Morphologische Charakteristika bei makulären Teleangiektasien Typ 2. Ophthalmologe 111, 819–828 (2014). https://doi.org/10.1007/s00347-014-3082-4

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