A RARE ASSOCIATION OF BILATERAL STARGARDT'S DISEASE WITH BILATERAL KERATOCONUS

Dr S Anuradha, Dr B Latha, Dr C Sony Shireesha, Dr M Sushma

Abstract


PURPOSE: To report a case of rare association of bilateral stargardt’s disease with bilateral keratoconus

Methods:A 33year old male presented with bilateral progressive vision loss with BCVA of counting fingers at 3 metres in right eye and at 1 metre in left eye.On examination corneal ectasia with thinning,vogt’sstriae and munson’s sign were observed. Scissoring reflex was seen on retinoscopy.Hence corneal topography was done with k values 57.3x59˚ in right eye and 59x149˚ in the left eye and pupils were round,regular,reacting to slight. On fundus examination,retinal pigment epithelial changes of Beaten Bronze appearance  were observed characteristic of stargardt’s disease

Results:High k values on corneal topography suggestive of Keratoconus and retinal pigment epithelial changes suggestive of stargardt’s disease

Conclusion:After an intense search, the association of keratoconus and stargardt’s has not yet reported so far, hence it is a rare association


Keywords


Keratoconus,stargardt’s disease, common associations, rare association

Full Text:

PDF

References


Rabinowitz YS. Keratoconus.Surv Ophthalmol1998;42:297-319.

"Facts About the Cornea and Corneal Disease". NEI. May 2016

"Eye: Cornea, Degenerations and Dystrophies". Robbins Basic Pathology (8th ed.). Philadelphia: Saunders/Elsevier. ISBN 978-1-4160-2973-1.

Freedman J, Gombos GM. Bilateral Macular Coloboma, Keratoconus and retinitis pigmentosa. Ann Ophthalmol.1971; 3: 664-5.

"Stargardt disease/Fundus flavimaculatus - EyeWiki". eyewiki.aao.org

Zhang K, Kniazeva M, Hutchinson A, Han M, Dean M, Allikmets R. The ABCR gene in recessive and dominant Stargardt diseases: a genetic pathway in macular degeneration. Genomics. 1999;60:234–7

Fujinami K, Zernant J, Chana RK, et al. Clinical and molecular characteristics of childhood-onset Stargardt disease. Ophthalmology. 2015;122(2):326-334

Michaelides M, Hunt DM, Moore AT. The genetics of inherited macular dystrophies.J Med Genet. 2003;40:641–650

Cremers FP, van de Pol DJ, van Driel M, et al. Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in Stargardt’s disease gene ABCR. Hum Mol Genet.1998;7:355–362.8


Refbacks

  • There are currently no refbacks.