Fundus autofluorescence in Stargardt macular dystrophy-fundus flavimaculatus.

PURPOSE: To quantify autofluorescence (AF) levels in patients with Stargardt macular dystrophy-fundus flavimaculatus (STGD-FFM), and to identify patterns of AF. DESIGN: Observational, comparative study. METHODS: Prospective study. SETTINGS: Patients were recruited at Moorfields Eye Hospital. STUDY POPULATION: Forty-three STGD-FFM patients aged 20 to 40 years and 35 age-matched normal volunteers. The right eye was chosen arbitrarily for measures of AF. INTERVENTION: The AF images were obtained using a confocal scanning laser ophthalmoscope. Levels of AF across the macula were measured. The distribution of AF was also evaluated. In 36 patients (84%) pattern electroretinogram (PERG) and full-field ERG were obtained and results were evaluated with respect to levels of AF. MAIN OUTCOME MEASURES: Values of AF, AF distribution, PERG, and ERG. RESULTS: Normal or high AF at the center of the macula with high AF temporally or nasally or both was detected in 17 patients (39%). In nine (21%), low AF at the center of the macula with normal or low AF temporally or nasally or both was found. Levels of AF were normal throughout the macula in six patients (14%). In 11 (26%), high, normal, and low levels of AF were found. All patients tested with low AF at the center of the macula and normal or low AF temporally or nasally or both had peripheral cone/rod dysfunction. None of the patients tested that had normal or high AF at the fovea and high AF temporally or nasally, or normal AF throughout the macula, had peripheral cone/rod dysfunction. CONCLUSION: AF is not universally high in STGD-FFM. Some patients have normal or low AF. Autofluorescence patterns appear to relate to functional abnormalities.

Retinal pigment epithelium translocation after choroidal neovascular membrane removal in age-related macular degeneration.

PURPOSE: To test the feasibility of a new surgical technique and to assess visual function over the translocated retinal pigment epithelium (RPE) cells in patients operated on for subfoveal choroidal neovascularization (CNV) secondary to age-related macular degeneration (ARMD). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Nine patients with previously untreated exudative ARMD underwent surgical excision of the subfoveal CNV with RPE translocation and were observed for 12 to 32 months. METHODS: The surgery consisted of a standard three-port pars plana vitrectomy, excision of the CNV, and RPE translocation. Pre- and postoperative ocular examination included best-corrected visual acuity measurement, fundus color stereo photography, and fundus fluorescein angiography. Optical coherence tomography and confocal laser scanning ophthalmoscopy (cLSO) were performed after surgery. A crossfixation target and a single-point flashing light were projected on different areas of the posterior pole using a cLSO. Photopic 10 to 2 perimetry, photopic fine matrix mapping, and cLSO microperimetry were also performed after surgery in six patients. MAIN OUTCOME MEASURES: Optical coherence tomography cross-sectional scans and cLSO RPE autofluorescence were recorded to detect the presence of viable translocated RPE. Visual acuity, fixation, photopic 10 to 2 perimetry, photopic fine matrix mapping, and cLSO microperimetry were used to test central visual function. RESULTS: Retinal pigment epithelium was translocated successfully at the time of CNV removal from the edge of the RPE defect to a subfoveal location in seven of nine patients. One patient experienced proliferative vitreoretinopathy, but significant hemorrhage was not a feature. Optical coherence tomography showed the translocated RPE as an area of increased optical reflectivity with optical shadowing external to it. Confocal laser scanning ophthalmoscopy showed autofluorescence of the translocated RPE. The crossfixation target was seen when projected on the translocated RPE. During eccentric fixation, the patients could see a flashing point-target projected on the translocated RPE. Photopic 10 to 2 perimetry, photopic fine-matrix mapping, and cLSO microperimetry showed the presence of central visual function. CONCLUSIONS: The authors propose that translocation of RPE at the time of CNV removal, from the edge of the RPE defect to a subfoveal location, may have a role in the surgical management of ARMD.

Clinical variations in assessment of bull's-eye maculopathy.

OBJECTIVES: To evaluate the phenotypic variation in bull's-eye maculopathy and seek possible correlations between functional loss and clinical appearance. METHODS: From January 1, 1999, to September 30, 2000, we prospectively examined patients with bull's-eye lesions. Age of onset, duration of symptoms, visual acuity, clinical appearance, and autofluorescence images were recorded, the area of atrophy measured, and electrophysiologic investigations performed. RESULTS: Forty-seven patients, including 6 sibling pairs, met the study entry criteria. On the basis of autofluorescence imaging, 3 distinct groups were identified. Group 1 showed a distinct ring of increased autofluorescence surrounding an area of decreased autofluorescence. In group 2, the ring of increased autofluorescence was not present. Group 3 displayed a speckled appearance within the affected area. All patients had evidence of central sparing in an area of centrally increased autofluorescence. There was significant correlation with the age of onset, visual acuity, and duration of disease. Electrophysiologic tests revealed that 28 patients had macular dysfunction only, 14 had cone-rod dystrophy, 3 had rod-cone dystrophy, and only 2 (monozygotic twins) had cone dystrophy. The correlation between electrophysiologic and autofluorescence data was poor. The sibling pairs had concordant autofluorescence appearance, but electrophysiologic grouping differed in 2 pairs. CONCLUSIONS: Bull's-eye maculopathy represents a heterogeneous group of disorders. The clinical appearance was not helpful in assessing the degree of retinal dysfunction. The difference in qualitative characteristics of functional loss between siblings implies that these attributes do not necessarily reflect the influence of the primary mutation.