gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

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Retinal detachment in brothers with X-linked retinoschisis

Meeting Abstract

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  • corresponding author E. Tsiroukis - Dept. of Ophthalmology, University of Leipzig
  • P. Meier - Dept. of Ophthalmology, University of Leipzig
  • P. Wiedemann - Dept. of Ophthalmology, University of Leipzig

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogP 173

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/dog2004/04dog664.shtml

Published: September 22, 2004

© 2004 Tsiroukis 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

X-linked juvenile retinoschisis is a bilateral X-chromosomal recessive hereditary disease which demonstrates splitting of the nerve fiber layer and stellate maculopathy.

Methods

In a family with 5 children which visited our clinic, the two brothers showed an X-linked retinoshisis, while the ophthalmologic findings of the three sisters and the parents were normal. The boys presented typical retinal findings, medium myopia, and ERG with B-wave-reduction. Both brothers suffered in the age of 20 years a schisis-retinal-detachment, so that they had to be operated on. A pars-plana-vitrectomy with instillation of silicon oil and scleral buckling was carried out. In our case we were able to achieve with that surgical management the reattachment of the retina and functionally the recovery of the visual acuity.

Conclusions

Concerning the very slow progression of the vitreoretinal changes of X-linked juvenile retinoschisis, observation instead of surgery is the primary course of management. However, for severe complications, such as retinal detachment or recurrent vitreous hemorrhage, pars-plana-vitrectomy is the first choice therapy.