OBJECTIVE: To document the clinical presentation and fundus fluorescein angiographic findings
in Stargardt’s disease.
METHODOLOGY: This descriptive study was conducted at the Department of Ophthalmology
Unit-I Civil Hospital Karachi and Unit III Lyari General Hospital Karachi of Dow University of
Health Sciences from June 2004 to May 2008. Patients were selected from the out patient department
of Sindh Govt. Lyari General Hospital and Civil Hospital Karachi fulfilling the inclusion
criteria. Sociodemographic data and family history were obtained and patients subjected to
complete ophthalmic examination of anterior and posterior segment and Fundus Fluorescein
RESULTS: Thirty patient were found to have Stargardt's disease. Mean age was 18.4±6.9 years.
Twenty two (73.4%) patients were found to be 20 years of age or below. Males [18 (60%)] outnumbered
the females [12(40%)]. Visual acuity at presentation revealed a symmetric loss of vision
in right and left eyes (P=0.410 at 99% CI). Macula showed atrophic lesion in 12 cases (40%),
beaten bronze in 13 (43.3%) cases and varnished appearance in 5 (16.7%) cases. Retinal flecks
were found in 18 (60%) patients only. Fundus fluorescein angiography revealed dark choroids
and elliptical hyperfluorescent lesion at the macula in all the patients. Tiny hyperfluorescent
areas not corresponding to the area of flecks were found only in 18 (60%) patients.
CONCLUSION: We conclude that Stargardt’s disease has varied presentation and there is a familial
and genetic predilection.
Stargardt's, Autosomal recessive, hyperfluorescence, Macular Dystrophy.