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A familial case of pigmented paravenous retinochoroidal atrophy with asymmetrical fundus manifestations

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Abstract

Purpose

To report two Japanese sibling cases with pigmented paravenous retinochoroidal atrophy (PPRCA) with asymmetrical manifestation.

Methods

A 33-year-old female and her 31-year-old brother were referred to our hospital. Funduscopic examination, angiography, and electroretinogram were performed.

Results

Both had retinal pigment epithelium atrophy with pigmentation along the retinal veins, which was consistent with PPRCA. The younger brother was more severely affected than his sister. Surprisingly, the extent and degree of the affected region was clearly different between both eyes of each patient.

Conclusions

These findings have rarely been reported previously, and these cases stress the importance of considering gender, familial involvement, and variable manifestations of the disease in clinical care of PPRCA.

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Author information

Authors and Affiliations

  1. Department of Ophthalmology, University of Tokyo School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Ryo Obata, Yasuo Yanagi, Aya Iriyama & Yasuhiro Tamaki

  2. Department of Ophthalmology, Saitama Red-Cross Hospital, 8-3-33 Kamiochiai, Saitama-shi, Saitama, 338-8553, Japan

    Ryo Obata

Authors
  1. Ryo Obata
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  2. Yasuo Yanagi
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  3. Aya Iriyama
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  4. Yasuhiro Tamaki
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Corresponding author

Correspondence to Ryo Obata.

Additional information

The authors received no specific financial support for this report and have no financial interest in any company or product discussed in it.

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Obata, R., Yanagi, Y., Iriyama, A. et al. A familial case of pigmented paravenous retinochoroidal atrophy with asymmetrical fundus manifestations. Graefe's Arch Clin Exp Ophthalmo 244, 874–877 (2006). https://doi.org/10.1007/s00417-005-0179-0

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  • DOI: https://doi.org/10.1007/s00417-005-0179-0

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