Pachychoroidale Erkrankungen

Zusammenfassung

Pachychoroidale Erkrankungen umfassen eine Gruppe von Erkrankungen, die charakteristische choroidale Merkmale zeigen. Diese können heutzutage durch multimodale Bildgebung immer differenzierter beschrieben werden und umfassen fokale oder diffuse Aderhautverdickungen mit erweiterten und hyperpermeablen choroidalen Gefäßen. Die richtige Diagnose und Abgrenzung von anderen exsudativen Erkrankungen ist für eine mögliche Therapie von großer Bedeutung.

Abstract

Pachychoroid spectrum disorders include uncomplicated pachychoroid, pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularisation, focal choroidal excavation and peripapillary pachychoroid syndrome. They are characterized by a thickened and hyperpermeable choroid and thinning of the choriocapillaris. The disorders are being diagnosed with increasing frequency and differentiation due to the advancement of multimodality imaging. Current understanding of the development, course, possible complications and treatment of these diseases is growing rapidly, but not all mechanisms have yet been elucidated. A correct diagnosis is important, especially the differentiation between the presence of active neovascularisation or a purely exudative stage, in order to initiate a therapy. It is also not yet clear why patients have a thickened choroid and why some of these patients develop pathological changes such as subretinal fluid, RPE changes or neovascularisation.

Kernaussagen

• Der Begriff „pachychoroid“ beschreibt zunächst lediglich das Vorliegen einer verdickten Choroidea und „Pachyvessel“ großlumige Gefäße der Haller-Schicht.

• Weitere Veränderungen sind großlumige Choroideagefäße mit gesteigerter Hyperpermeabilität sowie eine verdünnte Choriokapillaris.

• Bislang ließen sich die zugrunde liegenden Pathomechanismen für die Entstehung von pachychoroidalen Erkrankungen nicht abschließend klären.

• Für die Diagnosestellung sollte eine multimodale Bildgebung mittels FAG, ICGA und OCT (speziell SS-OCT oder SD-OCT mit EDI-Modus) durchgeführt werden. Die OCTA kann ergänzend weitere wichtige Befunde liefern.

• Es gibt bisher keine einheitliche Klassifizierung für die Erkrankungen des pachychoroidalen Spektrums.

• Für die Therapieentscheidung ist es sehr wichtig, zwischen einem rein exsudativen Befund oder dem Vorliegen einer exsudativen Neovaskularisation zu differenzieren.

Schlüsselwörter

Aderhaut - Anti-VEGF-Therapie - Chorioretinopathia centralis serosa

Key words

Pachychoroid - anti-VEGF therapy - central serous chorioretinopathy

Publication History

Article published online:
17 November 2022

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