Clinical application of digital indocyanine green angiography in choroidal neurofibromatosis.

Indocyanine green angiography (ICGA) was used to investigate 2 cases of type 1 systemic neurofibromatosis that had appeared at birth with café-au-lait skin spots, gradually developing into multiple cutaneous neurofibromas. Patients underwent periodical visual acuity examinations, the fundus was checked and fluorescein angiography (FA) was done; all findings appeared extremely stable. In 1995 these 2 patients underwent ICGA to check for pathological choroidal involvement. In both cases the initial examination stages showed multiple extensive areas of hypofluorescence, their morphology and extension coinciding with the retinal pigment epithelium (RPE) lesions shown by FA and by ophthalmoscopic examination. In later stages the hypofluorescent areas became smaller, generally shrinking to small isolated dots in the middle of the original areas. These initially hypofluorescent areas appeared to be due to slow focal choroidal filling caused by deep alterations to the walls of the choroidal arterioles induced by the disease. Chronic hypoperfusion of the choriocapillaris results in impairment of the overlying RPE, causing it to atrophy. The late hypofluorescent areas could be either persistent nonperfused lobules of choriocapillaris or neurofibromatose choroidal nodules. ICGA examination showed that the FA lesions described in the literature as choroidal nodules are in fact alterations to the RPE secondary to areas of hypoperfusion in the choriocapillaris.

Clinical application of digital indocyanine green videoangiography in senile macular degeneration.

Digital indocyanine green videoangiography (ICGV) was done in 34 eyes of 24 patients with aging macular degeneration (AMD), including drusen, either alone (6 eyes) or in association with other AMD changes (9 eyes), geographic atrophy of the retinal pigment epithelium (2 eyes), well-defined choroidal neovascularization (CNV; 3 eyes), occult CNV (12 eyes) and recurrent CNV (11 eyes). Of the 11 eyes with soft drusen, 10 showed abnormal fluorescence in the late ICGV picture. ICGV of the 4 eyes with hard drusen showed no abnormality. The geographic atrophy of the retinal pigment epithelium and choriocapillaris remained hypofluorescent with sharply demarcated boundaries throughout the study. ICGV confirmed the presence of CNV in all 3 eyes with well-defined CNV and in 11 of the 12 eyes with occult CNV. Additionally, all but 1 eye with primary occult CNV and 6 of the 8 eyes with recurrent occult CNV could be reclassified in this study as having well-defined CNV. Overall, ICGV yielded additional information in 17 of the 20 eyes with primary and recurrent occult CNV. Its clinical importance for the evaluation of early stages of AMD has still to be confirmed by future investigations. ICGV is recommended as a diagnostic examination in eyes with CNV poorly defined by fluorescein angiography.

Dominant inherited tilted disc syndrome and lacquer cracks.

Three patients with the tilted disc syndrome from one family were examined. The presence of the trait in three consecutive generations suggests an autosomal dominant mode of inheritance, although in these patients with variable expression. The propositus showed bilateral inferonasal retinal ectasia, with atrophic subretinal scars. Linear-like lacquer cracks, radiating from the central scars, were also present running parallel to the margin of the optic nerve head. The linear streaks were very similar to those usually seen in traumatic tears of Bruch's membrane. The mechanical stretching of the ectatic area, and its abnormal location inferonasal to the optic disc might have been responsible for the unusual pattern of the lacquer cracks in our patient.

Presumed ocular histoplasmosis syndrome and linear streak lesions.

Five cases of subretinal neovascular membranes in the macula associated with punched out chorioretinal scars and linear streaks were seen in five Dutch patients. Clinically the fundus lesions are consistent with those of presumed ocular histoplasmosis syndrome (POHS) seen in the United States of America. Cutaneous serological testing for histoplasmin reactivity was negative in the three patients tested. Of special interest is the presence of linear streaks in association with POHS. They have not been previously described in patients from Europe with this syndrome.

Neuroretinal rim area in normal eyes: a study on a randomized group of 30 subjects.

This study was undertaken in order to evaluate the range of the neuroretinal rim area in a normal sample and the repeatability of manual photogrammetric measurements. Thirty randomly chosen eyes from 30 subjects without ocular disease were examined: the mean disc area (2.20 +/- SD 0.58 mm2), the mean cup area (0.36 +/- 0.29 mm2) and the mean rim area (1.83 +/- 0.37 mm2) were evaluated. All the measurements were performed by two independent observers and were corrected to the actual size by measuring refraction and axial length of each eye. A linear correlation among disc areas (r = 0.71), cup areas (r = 0.93) and rim areas (r = 0.74) between the two observers was found. Furthermore, a highly significant correlation between disc area and rim area was observed (r = 0.90) together with a correlation between the disc and cup area (r = 0.82). A correlation between disc area and axial length was finally found (r = 0.59). The neuroretinal rim area has been expressed as a percentage of the total disc area. Percent values (+/- SD) were clustered around a mean of 84.83 +/- 8.8%. Despite their high degree of repeatability, our data are slightly different from those previously reported by other authors.

Rip of the retinal pigment epithelium: report of an atypical case.

A 71-year-old man presented with a large disciform scar in the right eye and hard (nodular) as well as soft (granular) drusen scattered throughout the posterior pole of the left eye. A large serous pigment epithelial detachment was evident in the drusen area of the left fundus. Six months later a tear of the retinal pigment epithelium (RPE) occurred in the left eye. It was located exactly along the peripheral border of the drusen area instead of the border of the RPE detachment where it would have been expected. It is considered that reduced adhesion between the RPE and Bruch's membrane as well as the sharp change in tissue cohesiveness at the border of the drusen area and surrounding healthy tissue were relevant in the pathogenesis of this case.

Idiopathic sub-retinal neovascular membranes in the macula (hemorrhagic macular choroidopathy of young adults). Clinical report and effectiveness of laser treatment.

Twenty-six patients with idiopathic sub-retinal neovascular membranes (INVMs) located within the posterior pole were examined. Clinical and fluorescein angiographic features of these patients are described. Follow-up ranged from 2 to 96 months (mean 16.5 months). Laser treatment was performed according to the location of the neovascular membrane: 7 out of 9 eyes (77%) increased their final visual acuity or stabilized after Argon blue-green laser photocoagulation (INVMs further than 200 microns from the center of the fovea). Krypton red light laser treatment was performed in 13 eyes with juxta-foveal and sub-foveal INVMs: following treatment 10 eyes (77%) improved by 2 or more Snellen lines or maintained their pre-treatment visual acuity. Because of the fact that the Krypton laser was not yet available, 5 patients were not treated: all of them had sub-foveal (3) or juxta-foveal INVMs (2). The natural history of 4 cases (80%) showed an increase in the final visual acuity in 3 eyes and a steady state in one eye. Pathogenesis and treatment outcome of the disease are finally discussed.