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Am J Ophthalmol Case Rep. 2017 Sep 01;8:14-17. doi: 10.1016/j.ajoc.2017.08.003. eCollection 2017 Dec.

Unilateral pigmented paravenous retinochoroidal atrophy with retinitis pigmentosa in the contralateral eye: A case report.

American journal of ophthalmology case reports

Shuichiro Aoki, Tatsuya Inoue, Mari Kusakabe, Masaya Fukushima, Kohdai Kitamoto, Asako Ogawa, Motoshi Yamamoto, Ryo Obata

Affiliations

  1. Department of Ophthalmology, The University of Tokyo, Tokyo, Japan.

PMID: 29260108 PMCID: PMC5731675 DOI: 10.1016/j.ajoc.2017.08.003

Abstract

PURPOSE: We describe a sporadic case of unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) with retinitis pigmentosa (RP) in the contralateral eye.

OBSERVATIONS: a 24-year-old female aware of the narrowing of visual field was examined at our hospital. Funduscopic examination revealed left eye showing retinochroidal atrophy along the retinal veins with pigment accumulation while right eye showing peripheral diffuse retinal pigmented epithelium atrophy with bone spicule pigmentation. Fundus autofluorescence, electroretinogram, visual field test and optic coherent tomography were also performed and obtained results were compatible with funduscopic observation.

CONCLUSIONS AND IMPORTANCE: Simultaneous manifestation of PPRCA and RP observed in this case is rare and supports a shared genetic basis between the two diseases. Further genetic investigations are needed to elucidate the etiology and to properly manage PPRCA.

Keywords: Pigmented paravenous retinochoroidal atrophy (PPRCA); Retinitis pigmentosa (RP)

References

  1. Eye (Lond). 2000 Jun;14 ( Pt 3A):395-7 - PubMed
  2. Hum Mutat. 2004 Nov;24(5):355-69 - PubMed
  3. Am J Ophthalmol. 1948 Nov;31(11):1485-7 - PubMed
  4. Arch Ophthalmol. 1986 Nov;104(11):1636-40 - PubMed
  5. Indian J Ophthalmol. 2016 Oct;64(10 ):775-777 - PubMed
  6. Am J Ophthalmol. 1979 Mar;87(3):286-91 - PubMed
  7. Am J Hum Genet. 2001 Jul;69(1):198-203 - PubMed
  8. Exp Ther Med. 2014 Jun;7(6):1439-1445 - PubMed
  9. Invest Ophthalmol Vis Sci. 2005 Jan;46(1):322-8 - PubMed
  10. Am J Ophthalmol. 2006 Apr;141(4):763-5 - PubMed
  11. Doc Ophthalmol. 1986 Sep 30;63(3):209-37 - PubMed
  12. Int Ophthalmol. 1984 Jan;7(1):11-4 - PubMed
  13. Am J Ophthalmol. 1985 May 15;99(5):605-6 - PubMed
  14. Am J Ophthalmol. 1978 Feb;85(2):215-7 - PubMed
  15. Am J Ophthalmol. 1989 Oct 15;108(4):365-9 - PubMed
  16. Br J Ophthalmol. 2011 Jun;95(6):811-7 - PubMed
  17. Nat Genet. 1999 Oct;23(2):217-21 - PubMed

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