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Bull's-eye macular dystrophy associated with peripheral involvement

R Suzuki1, T Hirose

  • 1Department of Ophthalmology, Yamaguchi University School of Medicine, Ube City, Japan. suzuki@po.cc.yamaguchi-u.ac.jp

Ophthalmologica. Journal International D'Ophtalmologie. International Journal of Ophthalmology. Zeitschrift Fur Augenheilkunde
|July 22, 1998
PubMed
Summary

This study identifies rare bull's-eye macular dystrophy cases with severe peripheral vision loss, unlike typical Stargardt's disease. These findings suggest a potential overlap with retinitis pigmentosa, impacting retinal function.

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Area of Science:

  • Ophthalmology
  • Medical Genetics
  • Retinal Diseases

Background:

  • Stargardt's disease typically affects central vision, sparing peripheral retinal function.
  • Bull's-eye maculopathy is characterized by macular degeneration, often associated with Stargardt's disease/fundus flavimaculatus.

Observation:

  • Four patients presented with bull's-eye macular dystrophy but exhibited significantly impaired peripheral retinal function.
  • One patient initially showed normal peripheral findings but later developed peripheral bone spicule pigmentation and optic disc atrophy.
  • Three patients from another family displayed both macular and peripheral pigment degeneration.

Findings:

  • These cases present a phenotype distinct from classic Stargardt's disease, suggesting a possible rare combination with retinitis pigmentosa.

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  • Peripheral retinal degeneration and electroretinogram abnormalities were observed in some patients over time.
  • The probable mode of inheritance in the majority of affected individuals was autosomal recessive.

    • Implications:

    • This research expands the understanding of macular dystrophy phenotypes and their potential progression.
    • It highlights the importance of comprehensive retinal evaluation, including peripheral function, in diagnosing macular diseases.
    • The findings may aid in differentiating rare genetic retinal disorders and inform genetic counseling.