当前位置： SCI文献检索 > KLINISCHE MONATSBLATTER FUR AUGENHEILKUNDE期刊下所有文献 > [Congenital Retinal Dystrophies: Combining Ophthalmological Techniques to Improve the Read-out].

[Congenital Retinal Dystrophies: Combining Ophthalmological Techniques to Improve the Read-out].

Abstract：

:An early diagnosis, differential diagnosis and possible decision about therapeutic interventions has considerable consequences for the personal and social life of patients affected with inherited retinal dystrophies (IRD). For the ophthalmologist, the clinical heterogeneity interferes with a simple diagnostic approach. The present review suggests a structured clinical approach for the ophthalmological diagnosis of IRD and discusses the relevance of different methods for diagnosis, differential diagnosis and the evaluation of progression. A detailed history should be followed by non-invasive retinal imaging. An early diagnosis prior to visible fundus alterations is facilitated by combining optical coherence tomography, fundus and near-infrared autofluorescence. Spectral reflectance photography, OCT angiography and fluorescence lifetime imaging ophthalmoscopy are helpful in the early diagnosis of specific IRD. If retinal imaging is not sufficient for a diagnosis the multifocal electroretinogram is useful for early diagnosis and full-field electroretinogram for differential diagnosis of IRD. Patients should be referred to specialised IRD-centres for differential diagnosis and possible treatment. :Für Patienten mit hereditären Netzhautdystrophien (auf Englisch: inherited retinal dystrophies, IRD) ist die Früherkennung, Differenzialdiagnose und mögliche Therapieentscheidung von erheblicher persönlicher und sozialer Bedeutung. Für den Augenarzt kann dies aufgrund der Heterogenität der Erkrankungen und Verläufe sowie der Seltenheit der IRD eine Herausforderung sein. Die vorliegende Übersicht empfiehlt eine zielorientierte klinisch-ophthalmologische Diagnostik bei Verdacht auf IRD mit einer Bewertung der Relevanz der einzelnen Methoden und ihrer Kombination für Diagnose, Differenzialdiagnose und Beurteilung der Progression im Verlauf. Nach einer umfassenden Anamnese ist initial die Kombination von optischer Kohärenztomografie (OCT), Fundus- und Nahinfrarot-Autofluoreszenz zur Frühdiagnose einer IRD ggf. vor ophthalmoskopisch sichtbaren Läsionen sinnvoll. Spektrale Reflexionsfotografie, OCT-Angiografie und Fluorescence Lifetime Imaging Ophthalmoscopy sind bei einzelnen IRD hilfreich. Erlaubt die retinale Bildgebung keine sichere Diagnose, ist das multifokale Elektroretinogramm zur Frühdiagnose und das Ganzfeld-Elektroretinogramm zur Differenzialdiagnose von IRD geeignet. Eine Vorstellung in Schwerpunktzentren für IRD zur Differenzialdiagnostik und Therapie ist empfehlenswert.

journal_name

Klin Monbl Augenheilkd

journal_title

Klinische Monatsblatter fur Augenheilkunde

authors

Kellner U,Kellner S,Saleh M,Deutsch S,Weinitz S,Farmand G

doi

10.1055/a-1118-3705

subject

Has Abstract

pub_date

2020-03-01 00:00:00

pages

275-287

issue

3

eissn

0023-2165

issn

1439-3999

journal_volume

237

pub_type

杂志文章,评审

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