gms | German Medical Science

102. Jahrestagung der DOG

Deutsche Ophthalmologische Gesellschaft e. V.

23. bis 26.09.2004, Berlin

Pigmented paravenous retinochoroidal atrophy associated with a macular hole: a case report

Meeting Abstract

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  • corresponding author M. Huemmeke - Department of Ophthalmology, University Hospital Münster, Münster
  • H. Busse - Department of Ophthalmology, University Hospital Münster, Münster
  • C. E. Uhlig - Department of Ophthalmology, University Hospital Münster, Münster

Evidenzbasierte Medizin - Anspruch und Wirklichkeit. 102. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft. Berlin, 23.-26.09.2004. Düsseldorf, Köln: German Medical Science; 2004. Doc04dogSO.05.13

The electronic version of this article is the complete one and can be found online at:

Published: September 22, 2004

© 2004 Huemmeke et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.




The aim of this study is to discuss macular involvement in pigmented paravenous retinochoroidal atrophy (PPRCA). PPRCA is a rare disease characterized by bilateral retinochoroidal atrophy and pigmentation along the retinal veins. The macula has been reported to be affected in only a small minority of cases.


A 60-year-old Turkish patient presented with decreased visual acuity of his right eye. The following investigations were performed: anamnesis, visual acuity testing, tonometry, perimetry, amsler-chart-testing, funduscopy, Ishihara- and Farnsworth 28 Hue de Roth color vision tests, ultrasound scan, electroretinogram (ERG), electrooculogram (EOG), fluorescein angiography (FAG), indocyanine green angiography (ICG).


Anamnesis: no parental consanguinity, first-grade relatives (P, F1) without visual acuity reducing diseases. Patient's best visual acuity: 0.1 (right eye = OD) and 0.9 (left eye = OS). Colour vision tests: dyschromatopsia of both eyes. Funduscopy: large areas of chorioretinal atrophy associated with multiple accumulations of bone corpuscle pigmentation. Macular hole stage IV in the right eye. EOG: Arden index 142% (OD) and 182% (OS), indicating a dysfunction of the retinal pigment-epithelium-photoreceptor complex. FAG: window defects in the peripapillary area extending to the superior and inferior vascular arcades (both eyes), hyperfluorescence in the central macula of the right eye. ICG: large areas of chorioretinal atrophy extending into the foveal region (OD > OS).


Diagnosis was bilateral PPRCA with a macula hole in the right eye. Our angiographic and electrophysiologic data are supported by other studies and confirm an involvement of the RPE in PPRCA. This affection is pronounced near to the superior and inferior vascular arcades but also extends into the macula and might be a reason for the hole.