Unilateral acute idiopathic maculopathy. 1991.

This is a report of nine patients who experienced sudden, severe, unilateral central vision loss following a flulike illness. Each patient had an exudative detachment of the macula. All patients experienced a spontaneous resolution of the acute macular manifestations with near-complete recovery of vision. A characteristic "bull's-eye" appearance in the macula persisted. The acute manifestations of the disorder did not recur in any of the patients during the period of follow-up. The constellation of findings was suggestive of an inflammatory disease of the retinal pigment epithelium, but a specific causative agent could not be identified. The acute clinical and angiographic features, the natural course, and the residual pigment epithelial derangement were not consistent with any previously described disorder.

Perifoveal function in patients with North Carolina macular dystrophy: the importance of accounting for fixation locus.

PURPOSE: To quantify the extent of visual function losses in patients with North Carolina Macular Dystrophy (NCMD) and to demonstrate the importance of accounting for eccentric fixation when making comparisons with normal data. METHODS: Five patients with NCMD who were from a single family were examined. Multifocal electroretinograms (mfERGs) and psychophysical assessments of acuity and luminance visual field sensitivities were measured throughout the central retina. Comparisons of responses from equivalent retinal areas were accomplished by shifting normal templates to be centered at the locus of fixation for each patient. RESULTS: Losses of psychophysically measured visual function in patients with NCMD extend to areas adjacent to the locations of visible lesions. The multifocal ERG amplitude was reduced only within the area of visible lesion. Multifocal ERG implicit times were delayed throughout the entire central retinal area assessed. CONCLUSIONS: ERG timing is a sensitive assay of retinal function, and our results indicate that NCMD has a widespread effect at the level of the mid and outer retina. The findings also demonstrated that it is necessary to account for fixation locus and to ensure that equivalent retinal areas are compared when testing patients with macular disease who have eccentric fixation.

Comprehensive functional vision assessment of patients with North Carolina macular dystrophy (MCDR1).

PURPOSE: Previous studies indicated abnormal development of fixation toward the optic nerve head in patients with the inherited retinal disease North Carolina macular dystrophy (NCMD). The implication of this development on functional vision and structural characteristics has not been described. METHODS: The anatomical characteristics of five NCMD-affected individuals were assessed by measuring the retinal thickness of the macula using optical coherence tomography. The underlying physiologic health of the retina was assessed using the multifocal ERG. Psychophysical assessment of remaining vision in the affected areas was done with a new microperimetry system that measures functional visual acuity at 27 discrete locations and the Humphrey visual field analyzer. RESULTS: All patients had better areas of visual sensitivity toward the nasal macula. Follow-up examination showed no changes in the clinical appearance of the retina. Visual acuities ranged from -0.10 logMAR (Snellen equivalent, approximately 20/16) to 0.50 logMAR (Snellen equivalent, approximately 20/63) in the better eye. No significant changes in visual acuity were found over time. Local multifocal electroretinogram deficits were found in all patients. Patients with grade 2 or 3 disease had large patches of decreased amplitudes and delayed implicit times. Results of the anatomical, electrophysiological, and psychophysical tests were consistent. CONCLUSION: The electrophysiological and psychophysical deficits found in patients with more severe disease were consistent with an abnormal development of fixation from the anatomical fovea toward the optic nerve head with the placement of the lesion temporal to fixation (into the nasal visual field).

A randomized clinical trial of feeder vessel photocoagulation of proliferative sickle cell retinopathy. I. Preliminary results

A randomized prospective clinical trial of feeder vessel photocoagulation for proliferative sickle cell retinopathy was performed. In Chicago, there were 34 argon laser-treated eyes and 30 control eyes. In Kingston, there were 53 xenon arc treated eyes and 50 control eyes. Prolonged loss of visual acuity was rare in both photocoagulated and control eyes. Argon laser and xenon arc feeder vessel photocoagulation reduced the incidence of vitreous hemorrhage and visual loss from vitreous hemorrhage. However, photocoagulation was associated with an increased risk of choroidal neovascularization (xenon arc greater than argon laser) and retinal detachment (argon laser), although visual loss from these complications has not been in these patients so far. (AU)

Macular vasculature, visual acuity, and irreversibly sickled cells in homozygous sickle cell disease

Observations of visual acuity and the conjunctival, macular, and perimacular vascularity have been assessed in patients with homozygous sickle cell (SS) disease. There were 17 matched pairs, each consisting of one patient with a high count (>15 percent) and one with a low count (<5 percent) of irreversibly sickled cell (ISCs). The macular vascular bed was assessed by measurements of the foveal avascular zone (FAZ), perimacular zones, and counts of perimacular vascular abnormalities (perimacular counts). Small foveal avascular zones and high perimacular counts were commoner in younger than older patients and there was a significant inverse correlation between size of the FAZ and the perimacular count. These observations were compatible with the hypothesis that perimacular vessel anomalies represent the early vaso-occlusive phase which progresses to ischaemia and the formation and enlargement of avascular areas. Visual acuity was assessed by Snell's test type and by measuring contrast sensitvity. There was no obvious relationship between acuity measured by the 2 methods and no relationship between acuity and observations of mascular vascularity. High ISC counts were significantly related to abnormalities of conjunctival vasculature, but no relationship was noted with abnormalities of the macular vasculature or with visual acuity (AU)