gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

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Fundus autofluorescence in children and teenagers with hereditary retinal diseases

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  • corresponding author B. K. Wabbels - University of Regensburg, Dept. of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, Regensburg
  • K. Paunescu - University of Regensburg, Dept. of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, Regensburg
  • A. Demmler - University of Regensburg, Dept. of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, Regensburg
  • B. Lorenz - University of Regensburg, Dept. of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, Regensburg

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogDO.01.02

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/dog2004/04dog002.shtml

Published: September 22, 2004

© 2004 Wabbels et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

Text

Objective

In adults, evaluation of fundus autofluorescence (AF) plays an important role in the differential diagnosis of retinal diseases. The aim of this study was to evaluate the feasibility of recording AF in children and teenagers and to characterise typical AF findings of various hereditary retinal diseases.

Methods

Fifty patients aged 2 to 18 years with hereditary retinal diseases were evaluated using the HRA (Heidelberg Retina Angiograph). To enhance the AF signal, a mean of up to 16 single images was calculated.

Results

In many children as young as 5 years of age and some 2-year-old children good AF images could be obtained. To achieve high quality images, larger image series (about 50 single images) were taken and appropriate single images to calculate the mean were chosen manually. Characteristically, M. Stargardt shows a central oval area of reduced AF, often surrounded by a more irregular AF. In patients with M. Best, a central round structure with regular or irregular intense AF is visualised, carriers have normal AF. In early-onset severe retinal dystrophy (EOSRD) with RPE65-mutations AF is completely absent (Lorenz et al., Ophthalmology 2004, in press), whereas in other forms of Leber congenital amaurosis, AF is about normal. In many patients with rod-cone-dystrophies, a central "rod ring" of increased AF is present. Some patients with X-linked retinoschisis show central radial structures of increased AF.

Conclusions

Fundus autofluorescence may visualise a disease specific distribution of lipofuscin in the retinal pigment epithelium, often not (yet) visible on ophthalmoscopy. AF images can be used in children to differentiate hereditary retinal diseases and to facilitate follow-up controls. In many cases, four single images are sufficient to analyse the AF pattern.

DFG Lo 457/3- 1-3, DFG Lo 457/5-1,2