Objective To test the hypothesis that the macular pigment may be a marker of foveal cone function and consequently the structural integrity of foveal cones.Methods Sixteen patients (32 eyes) diagnosed to have Stargardt dystrophy and three patients with full thickness macular holes by clinical criteria were studied with a scanning laser ophthalmoscopy (SLO) comparing argon laser blue and infrared images for the presence or absence of macular pigment (MP) in the fovea. An C++ computer based program was used to evaluate the density of MP. Eyes were graded into three categories: those without foveal macular pigment, those with partial pigment and those with normal amounts of macular pigment. These categories were compared with visual acuity determined by the Snellen chart. Results Thirteen eyes with a visual acuity of 20/200 or worse had no macular pigment in the fovea. Eleven eyes with visual acuity of 20/40 or better had a normal amount of macular pigment in the fovea and 1 eye had partial macular pigment. Eleven eyes with partial macular pigment had intermediary acuity value.Conclusions Foveal macular pigment is closely related to foveal cone acuity and therefore may be a marker for the presence of foveal cones. Infrared light is a sensitive indicator of early macular diseases.(Chin J Ocul Fundus Dis，2003，19：201-268)
Stargardt disease (STGD) is one of the most prevalent inherited macular dystrophy, and most often occurs in child or adolescence. Irreversible vision loss is observed in almost all cases. Type 1 (STGD1) is one of the most common type. It is an autosomal recessive condition, caused by mutations in the Abca4 gene. In recent years, encouraging progress has been made in the treatment of STGD1. C20-D3-retinyl acetate (ALK-001), fenretinide and ICR-14967 (A1120) as visual cycle modulators, StarGen as gene supplementation therapies, and the stem cell transplantation of human embryonic stem cell-derived retinal pigment epithelium cells are the most promising therapies. With the development of studies and clinical trials, the clinical application of various treatments of STGD1 are expected in the near feature, which are expected to save the vision of most patients.