Macular Degeneration: Haimovici R

Haimovici R, Owens SL, Fitzke FW, Bird AC. · Institute of Ophthalmology, University College, London, UK. · Graefes Arch Clin Exp Ophthalmol. · Pubmed #11931085 No free full text.

Abstract: BACKGROUND: Abnormalities of dark adaptation have been documented in patients with age-related macular degeneration (AMD), but the relationship with the various forms of this disorder has not been studied systematically. METHODS: Dark-adapted retinal sensitivities and kinetics of dark adaptation were studied using a Humphrey visual field analyzer adapted for these purposes in patients over 64 years of age. One eye per patient was studied. Study eyes had a normal visual acuity and macular drusen only. The fellow eye was categorized as follows: group I, pigment epithelial detachments and tears of the retinal pigment epithelium (RPE); group II, choroidal neovascularization; and group III, drusen only. The results of psychophysical tests of the study eyes (group I and II and one eye of group III patients) were compared with one another and with older patients without evidence of AMD (group IV). RESULTS: Retinal sensitivity was found to be most consistently abnormal nearest the fovea. The time course of dark adaptation was prolonged beyond 45 min in 10/11 patients (91%) in group I, 6/10 patients (60%) in group II, and 6/10 (60%) in group III and 1/11 (9%) in group IV. CONCLUSION: In a high proportion of patients with visual loss from AMD in one eye, the fellow eye shows abnormal dark adaptation. These changes appear to be most pronounced in patients with detachments of the RPE in the fellow eye.

Haimovici R, Wroblewski J, Piguet B, Fitzke FW, Holder GE, Arden GB, Bird AC. · Institute of Ophthalmology, London, UK. · Eye. · Pubmed #11913893 links to  free full text

Abstract: PURPOSE: To investigate the nature of symptomatic visual disturbance in patients with EFEMP1 retinal dystrophy in the absence of geographic atrophy or choroidal neovascularization. METHODS: Patients presenting to a tertiary referral centre underwent clinical evaluation, fluorescein angiography, colour contrast sensitivity, focal, pattern, and standard electroretinography, electrooculography, scotopic threshold perimetry and dark adaptometry. RESULTS: Clinical features included reduced central vision, difficulty passing from light to dark, and diffuse submacular and peripapillary deposits, which were hyperfluorescent by fluorescein angiography. Colour contrast thresholds were abnormal in all six patients studied and both pattern and focal electroretinograms were abnormal in five of six patients. The scotopic and mixed rod-cone single flash ERG was normal but two patients demonstrated reduced oscillatory potentials and one had borderline delayed 30 Hz responses. Scotopic thresholds were elevated and rod-mediated dark adaptation kinetics were markedly prolonged in all six patients when measured over the central visible confluent deposits. CONCLUSIONS: In patients with EFEMP1 retinal dystrophy with confluent macular deposits, scotopic sensitivity is reduced and dark adaptation kinetics are prolonged over the macular deposits but are normal elsewhere. These results emphasize the localised nature of functional deficits in some patients with EFEMP1 retinal dystrophy and correlate well with the patient's visual symptoms. Symptomatic visual dysfunction may precede the development of clinically evident geographic atrophy or choroidal neovascularization in this disorder.

Haimovici R, D'Amico DJ, Gragoudas ES, Sokol S, Anonymous00093. · Department of Ophthalmology, Boston University School of Medicine, DOB-10 720 Harrison Avenue, Boston, MA 02118, USA. · Ophthalmology. · Pubmed #11772599 No free full text.

Abstract: OBJECTIVE: To describe early and unusual features in 16 patients with deferoxamine-induced retinal toxicity and to assess the role of diagnostic tests in the diagnosis and management of patients with the disorder. DESIGN: Retrospective, observational case series. PARTICIPANTS: Sixteen patients with deferoxamine retinopathy identified from members of the Vitreous, Retina, and Macula societies of the United States. INTERVENTION/TESTING: The patients underwent complete ophthalmologic examination. Most patients were also evaluated by fluorescein angiography and electrophysiologic testing. The diagnosis was based on the medical history, systemic and ocular findings, and the results of electrophysiologic tests. MAIN OUTCOME MEASURES: Ocular symptoms, ophthalmoscopic, fluoroangiographic, and electrophysiologic findings. RESULTS: We confirmed previously reported findings in patients with established disease, including macular and/or peripheral pigmentary changes, reduced electroretinographic (ERG) amplitudes, and reduced electrooculographic (EOG) light-peak to dark-trough ratios. Peripapillary, papillomacular, and paramacular patterns of retinal pigment epithelial (RPE) degeneration were each observed in one patient. Diffuse RPE or outer retinal fluorescence by fluorescein angiography was a marker for active retinopathy both at the onset of disease and during recurrence and preceded the development of RPE pigment mottling. CONCLUSIONS: Unusual patterns of deferoxamine retinopathy may occur in addition to the foveomacular and/or peripheral patterns previously described. Fluorescein angiography is particularly useful for determining whether there is ongoing retinal/RPE injury. ERG and EOG testing may indicate earlier or more widespread injury than is suggested by fundus examination alone. Patients who do not discontinue deferoxamine after the development of retinopathy risk further retinal/RPE injury and visual deterioration.

Haimovici R, Gantz DL, Rumelt S, Freddo TF, Small DM. · . Department of Ophthalmology, Biophysics, and. Pathology, Boston University School of Medicine, Massachusetts, USA. · Invest Ophthalmol Vis Sci. · Pubmed #11381066 links to  free full text

Abstract: PURPOSE: To detect and identify, in situ, the lipid composition of drusen, diffuse Bruch's membrane deposits, and sclera in aging human eyes using hot-stage polarizing microscopy (HSPM), a method that allows qualitative determination of lipid subtypes within histologic sections based on morphology and melting temperatures of liquid crystals as monitored by birefringence during heating and cooling. METHODS: Full-thickness buttons of the central macula and the periphery of human eyes from 17 patients were fixed in 5% calcium-buffered formalin. Frozen sections were stained with oil red O or Sudan black or were analyzed by HSPM. RESULTS: Birefringent anisotropic droplets ("maltese crosses") with melting characteristics of cholesterol esters were identified within diffuse Bruch's membrane deposits, drusen, and sclera. Deposits that melted from crystal to oil without any maltese cross formation when cooled were present in the sclera and are consistent with triglyceride-rich deposits. Deposits with optical properties consistent with phospholipids were identified in a single aged eye. Eyes from young donors did not show these changes. CONCLUSIONS: HSPM is a valuable technique for evaluating the nature of lipid deposits in aging eyes. Further studies are warranted to determine whether similar changes are also present in eyes with age-related macular degeneration.