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Functional and Morphologic Investigations of Grouped Congenital Hyperplasia of the Retinal Pigment Epithelium (CHRPE) "bear tracks"

1Meyer C. H., 1Kunze S., 2Becker R.,
1Philipps-Universität Marburg, Medizinisches Zentrum für Augenheilkunde (Marburg)
2Justus-Liebig-Universität Gießen, Zentrum für Augenheilkunde, Abt. Schielbehandlung und Neuroophthalmologie (Marburg)

Purpose: In grouped congenital hypertrophy of the retinal pigment epithelium (CHRPE) previous reports described corresponding scotoma. Although these lesions are the consequence of damaged phoptoreceptors, they remain locally limited and undetectable for the conventional ganzfeld electroretinogram (ERG) and elektrooculogram (EOG).
Method: We investigated two patients with a unilateral grouped CHRPE (bear tracks) with modern methods such as optical coherence tomography (OCT), microperimetry (SLO), und multifocal eletroretinogram (mfERG).
Results: Both patients demonstrated in the area of the CHRPE: 1. On OCT a thickened hyperreflective signal, which corresponded to a histological melanin enriched hypertrophy of the RPE. 2. Microperimetry revealed a relative scotoma. 3. Multifocal ERG determined in the sector of the CHRPE a reduced sum-potencial, compared to the other quadrants.
Conclusions: Grouped CHRPE lead to a morphological thickening and functional alteration of the RPE, which can be detected with new sensitive methods.

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