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Klin Monbl Augenheilkd 2013; 230(9): 914-919
DOI: 10.1055/s-0033-1350759
Übersicht
Georg Thieme Verlag KG Stuttgart · New York

Hereditäre Vitreoretinopathien und Netzhautablösung im Kindesalter

Paediatric Retinal Detachment and Hereditary Vitreoretinal Disorders
P. Meier
Universität Leipzig, Universitätsklinikum AöR, Klinik und Polilinik für Augenheilkunde, Leipzig
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Further Information

Publication History

eingereicht 11 July 2013

akzeptiert 15 July 2013

Publication Date:
28 August 2013 (online)

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Zusammenfassung

Die Zahl der Netzhautablösungen bei Kindern ist im Vergleich zum Erwachsenenalter relativ gering, hereditäre vitreoretinale Erkrankungen (z. B. Stickler-Syndrom, Wagner-Syndrom, Kniest-Syndrom, familiär exsudative Vitreoretinopathie, kongenitale X-chromosomale Retinoschisis, Knobloch-Syndrom, Incontinentia pigmenti, Norrie Disease) gelten als prädisponierender Faktor. Hereditäre vitreoretinale Erkrankungen sind durch eine abnorme Glaskörperarchitektur und assoziierte Netzhautveränderungen gekennzeichnet. Zusätzlich werden weitere okuläre Veränderungen diagnostiziert. Ein Teil der hereditären Erkrankungen weist auch charakteristische systemische Anomalien auf, deren Kenntnis hilft bei der Sicherung der Diagnose. Vitreoretinopathien sind die häufigste Ursache für hereditäre Netzhautablösungen. Bei der Mehrzahl der betroffenen Augen ist eine primäre Vitrektomie erforderlich, operationstechnische Spezifika, die sich für die einzelnen Erkrankungen ergeben, werden diskutiert.

Abstract

The number of retinal detachments in children is very low in comparison to the number in adults. One predisposing factor for development of paediatric retinal detachment is suffering from hereditary vitreoretinal degeneration (e.g., Stickler syndrome, Wagner syndrome, Kniest dysplasia, familial exudative vitreoretinopathy, congenital X-linked retinoschisis, Knobloch syndrome, incontinentia pigmenti, Norrie disease). Hereditary vitreoretinopathies are characterised by an abnormal-appearing vitreous gel with associated retinal changes. In most of these eyes further ocular abnormalities can be diagnosed. A group of hereditary disorders is associated with characteristic systemic abnormalities. Allied conditions should be considered in the clinical diagnosis. Vitreoretinopathies are the most common cause of inherited retinal detachment. In most eyes primary vitrectomy is necessary, and disease-specific surgical treatment is discussed.

Schlüsselwörter

Netzhautablösung - Kinder - hereditäre Vitreoretinopathie

Key words

retinal detachment - children - hereditary vitreoretinopathy
 

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