Stargardt's disease: Clinical presentation and fundus fluorescein angiographic findings

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 angiography. results: thirty patient were found to have stargardt's disease. mean age was 18.46.9 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.