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Congenital retino-pigment epithelial malformation: a clinicopathological case report

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Abstract

An unusual unilateral fundus lesion in a 16-year-old boy is described consisting of hyperpigmentation and presumed hypertrophy of the retinal pigment epithelium combined with malformation and thickening of the overlying sensory retina. Based on its clinical and histological characteristics, this lesion is considered to be a congenital retinal pigment epithelial malformation.

Zusammenfassung

Die klinischen und histologischen Befunde einer ungewöhnlichen Fehlbildung der Netzhaut und des retinalen Pigmentepithels werden beschrieben. Das retinale Pigmentepithel ist im Gebiet der Fehlbildung hyperpigmentiert und vermutlich auch hypertrophiert. Die sensorische Netzhaut weist eine ungewöhnliche intraretinale Faltung hauptsächlich der gefäßführenden Schichten auf und erscheint histologisch verdickt. Sekundäre Veränderungen z. B. lokalisierte reaktive Pigmentepithelproliferationen, cystoide Degenerationen der sensorischen Netzhaut und präretinale Membranbildungen können auftreten.

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Authors and Affiliations

  1. University Eye Hospital, D-4300, Essen, Federal Republic of Germany

    H. Laqua, O. -E. Lund, F. H. Stefani & A. Wessing

  2. University Eye Hospital, D-8000, München, Federal Republic of Germany

    H. Laqua, O. -E. Lund, F. H. Stefani & A. Wessing

Authors
  1. H. Laqua
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  2. O. -E. Lund
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  3. F. H. Stefani
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  4. A. Wessing
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Laqua, H., Lund, O.E., Stefani, F.H. et al. Congenital retino-pigment epithelial malformation: a clinicopathological case report. Albrecht v Graefes Arch. klin. exp. Ophthal. 213, 23–31 (1980). https://doi.org/10.1007/BF02391208

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  • DOI: https://doi.org/10.1007/BF02391208

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