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A clinical review of Stargardt's disease and/or Fundus Flavimaculatus with follow-up

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Abstract

Out of 49 patients diagnosed as having Stargardt's disease that have been clinically reviewed, 22 of these cases have been followed for a period up to 13 years. The cases have been studied in three subgroups, according to their fundus appearance. Patients from group I were those whose lesions were confined to the macula, showing no flecks in the retina. In group II the macular lesion was surrounded by perimacular flecks and in group III fundus flavimaculatus flecks were seen, diffusely scattered in the posterior pole. It appears that the prognosis of group I is better than the other groups, where surrounding flecks are seen in addition to the macular lesion.

The follow-up indicates that the disease evolves with a rather large spectrum of expressivity. Assessment of a predictive factor for the evolution of the disease is not yet possible. However, the observation of a dark choroid appears to be strongly suggestive for a further centrifugal progression of the disease.

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References

  1. Anmarkrud, N.: Stargardt's disease. Acta Ophthalmol. 57: 172–182 (1979).

    Google Scholar 

  2. Bonnin, P.: Le signe du silence choroidien dans les dégéné-rescences tapéto-rétiniennes postérieures. Doc. Ophthalmol. Proc. 9: 461–463 (1976).

    Google Scholar 

  3. Deutman, A.F.: The hereditary dystrophies of the posterior pole of the eye. Thomas, Springfield, pp. 100–171.

  4. Eagle, C.R., Lucier, A.C., Bernondino, V.B., Yanoff, M.: Retinal pigment epithelial abnormalities in Fundus Flavimaculatus. Ophthalmology, 67: 1189–1200 (1980).

    Google Scholar 

  5. Ernest, T.J., Krill, A.E.: Fluorescein studies in Fundus Flavimaculatus and drusen. Am. J. Ophthalmol. 62: 1 (1966).

    Google Scholar 

  6. Fish, G., Grey, R., Sehmi, K.S., Bird, A.C.: The dark choroid in posterior retinal dystrophies. Br. J. Ophthalmol. 65: 359–363 (1981).

    Google Scholar 

  7. Fishman, G.A.: Fundus Flavimaculatus. A clinical classification. Arch. Ophthalmol. 94: 2061–2067 (1976).

    Google Scholar 

  8. Fishman, G.A., Cunha-Vaz, I.G., Travassos, A.C.: Vitreous fluorophotometry in patients with Fundus Flavimaculatus. Arch. Ophthalmol. 100: 1086–1090 (1982).

    Google Scholar 

  9. Francheschetti, A.: A special form of tapetoretinal degeneration: Fundus Flavimaculatus. Trans. Am. Acad. Ophthalmol. Otolaryng. 69: 1048 (1965).

    Google Scholar 

  10. Francheschetti, A., François, J.: Fundus Flavimaculatus. Arch. Ophthalmol. Paris 25: 505–530 (1965).

    Google Scholar 

  11. François, J.: Juvenile macular degeneration. Jap. J. Ophthalmol. 21: 1–21 (1977).

    Google Scholar 

  12. François, J., De Laey, J.J.: Dégénérescences maculaires juvéniles et fluoroangiographie rétinienne. Bull. Soc. Franç. Ophtalmol. 82: 417–431 (1969).

    Google Scholar 

  13. François, J., De Rouck, A., Verriest, G., De Laey, J.J., & Cambie, E.: Progressive generalized cone dysfunction. Ophthalmologica 169: 255–284 (1974).

    Google Scholar 

  14. François, J., Verriest, G., De Rouck, A., Humblet, M.: Dégénérescence maculaire juvénile avec atteinte prédominante de la vision photopique. Ann. Oculist. 195: 1137–1191 (1962).

    Google Scholar 

  15. François, J., Turut, P.: Maladie de Stargardt et Fundus Flavimaculatus. Arch. Ophthalmol. Paris 35: 817–846 (1975).

    Google Scholar 

  16. Hadden, O.B., Gass, I.D.M.: Fundus Flavimaculatus and Stargardt's disease. Am. J. Ophthalmol. 82: 527–539 (1976).

    Google Scholar 

  17. Klien, B.A., Krill, A.E.: Fundus Flavimaculatus: clinical, functional and histopathologic observations. Am. J. Ophthalmol. 64: 3–23 (1967).

    Google Scholar 

  18. Krill, A.E., Klien, B.A.: Flecked retina syndrome. Arch. Ophthalmol. 74: 496–508 (1965).

    Google Scholar 

  19. Nakano, H.: Fundus Flavimaculatus with typical Stargardt's macula degeneration (clinical, functional and electrophysiological study). Jap. J. Clin. Ophthalmol. 30: 6–12 (1978).

    Google Scholar 

  20. Noble, K.G., Carr, R.E.: Stargardt's disease and Fundus Flavimaculatus. Arch. Ophthalmol. 97: 1281–1287 (1979).

    Google Scholar 

  21. Notting, I.G.A., Deutman, A.F.: Leakage from the retinal capillaries in hereditary dystrophies. Doc. Ophthalmol. Proc. 9: 439–442 (1976).

    Google Scholar 

  22. Stargardt, K.: Über familiare progressive Degeneration in der Maculagegend des Auges. Albrecht von Graefe's Arch. Klin. Ophthalmol. 71: 534–550 (1909).

    Google Scholar 

  23. Verriest, G.: Further studies on acquired deficiency of colour discrimination. J. Optical Soc. Am. 53: 185–195 (1963).

    Google Scholar 

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Gelisken, O., De Laey, J.J. A clinical review of Stargardt's disease and/or Fundus Flavimaculatus with follow-up. Int Ophthalmol 8, 225–235 (1985). https://doi.org/10.1007/BF00137651

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