Ocular complications in Sweet's syndrome.
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Biomedical subjects
J B Jonas
Publications and source records attributed to J B Jonas.
Glaucomatous parapapillary atrophy. Occurrence and correlations.
Glaucomatous optic nerve damage is typically associated with intrapapillary changes, such as neuroretinal rim loss. In this study, parapapillary chorioretinal atrophy was evaluated in 691 normal eyes, 1081 glaucomatous eyes, and 31 eyes with ocular hypertension. It was significantly larger and occurred more often in the glaucomatous eyes (parapapillary atrophy area, 1.07 +/- 0.83 mm2) (mean +/- SD) than in the normal eyes (0.55 +/- 0.64 mm2) or in the eyes with ocular hypertension (0.55 +/- 0.37 mm2). These differences were significant also for eyes with moderate glaucomatous damage (0.86 +/- 0.62 mm2). Parapapillary chorioretinal atrophy was associated with shallow glaucomatous cupping, diffuse nerve fiber loss, a marked tessellated fundus, and only moderately elevated intraocular pressure. It increased with a decreasing neuroretinal rim area. It showed a spatial correlation to neuroretinal rim loss inside the optic disc. In unilateral glaucoma, it was larger in the affected eye than in the unaffected eye. Parapapillary chorioretinal atrophy is associated with glaucoma.
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Optic disc histomorphometry in normal eyes and eyes with secondary angle-closure glaucoma. I. Intrapapillary region.
The intrapapillary region of the optic disc shows ophthalmoscopical changes in glaucoma. In search of a histological correlate, this region was examined histomorphometrically in serial sections of 21 human eyes with secondary angle-closure glaucoma and 28 control eyes with malignant choroidal melanoma. The lamina cribosa was significantly (P less than 0.05) thinner, the optic cup deeper and wider, the peripapillary scleral ring finer, and the corpora amylacea count was lower in glaucoma eyes than in control eyes with normal optic nerves. There was no significant difference in optic disc diameter. The decrease in lamina cribrosa thickness may be one of several factors leading to glaucomatous optic nerve fiber loss. Due to a decrease in the relative height the inner limiting membrane should not be taken as the reference level for optic-cup-depth measurement. A high corpora amylacea count may point to a normal optic nerve fiber population.
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Optic disc histomorphometry in normal eyes and eyes with secondary angle-closure glaucoma. II. Parapapillary region.
Parapapillary atrophy has been reported to occur in glaucoma eyes. Seeking the microscopical equivalent, we evaluated histomorphometrically serial sections of 21 human eyes enucleated due to secondary angle-closure glaucoma and 28 nonglaucomatous eyes with malignant choroidal melanoma. In the parapapillary region two zones were differentiated: in zone "B" adjacent to the optic disc, Bruch's membrane was denuded of retinal pigment epithelium cells; zone "A" peripheral to zone "B" showed pigment irregularities in the retinal pigment epithelium. Both zones "B" and "A" were significantly larger and zone B occurred more frequently in glaucomatous eyes than in the control group. Additionally, the outer and inner retinal layers and the parapapillary retina as a whole were significantly thinner in the glaucoma eyes than in the control eyes. Photoreceptors were completely lost or markedly decreased in number in zone "B." The findings may indicate that zones "B" and "A" represent the histological correlate of the glaucomatous parapapillary chorioretinal atrophy.
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Count and density of human retinal photoreceptors.
This investigation was directed at determining the count and regional distribution of photoreceptors in the eyes of 21 human cornea donors aged between 2 and 90 years. Mean count of rods was 60,123,000 +/- 12,907,000, and mean cone count was 3,173,000 +/- 555,000. Determined 40 microns away from the foveola, cone density measured 125,500 cones/mm2. Extrapolating the distribution curve, cone concentration in the foveal center can be assumed to be about 150,000 cells/mm2 to 180,000 cones/mm2. Towards the retinal periphery, cone density decreased from 6000 cones/mm2 at a distance of 1.5 mm from the fovea to 2500 cells/mm2 close to the ora serrata. Comparing different fundus regions, cone concentration was significantly highest in the nasal region. Cone diameter increased from the center towards the periphery. At a distance of 40 microns away from the foveola, it measured about 3.3 microns, and in the outer retinal regions about 10 microns. Rod density was highest in a ring-like area at a distance of about 3-5 mm from the foveola with a mean of 72,246 +/- 17,295 cells/mm2. Rod density peaked at 150,000 rods/mm2. It decreased towards the retinal periphery to 30,000-40,000 rods/mm2. Rod diameter increased from 3 microns at the area with the highest rod density to 5.5 microns in the periphery. The hexagonal rod and cone inner segments were regularly arranged in a honey-comb fashion.
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Size of glaucomatous optic discs.
In normal eyes, the optic disc size shows a high interindividual variability. In the diseased eye, some optic nerve anomalies and diseases occur preferentially in small optic discs, and some are more common in large optic nerve heads. We conducted a study to determine whether glaucoma subtypes are correlated with the optic disc size or not. Stereo optic disc photographs of 997 eyes with primary open-angle glaucoma, 44 eyes with normal-tension glaucoma, 129 eyes with pseudoexfoliative glaucoma, and 850 normal eyes were examined. Compared to eyes with primary open-angle glaucoma (mean optic disc area: 2.63 +/- 0.61 mm2), the optic disc was significantly larger in eyes with normal-tension glaucoma (2.96 +/- 0.73 mm2; P < 0.05), and it was smaller but not significantly smaller in eyes with pseudoexfoliative glaucoma (2.53 +/- 0.51 mm2). Eyes with primary open-angle glaucoma and normal eyes (2.69 +/- 0.69 mm2) did not differ significantly in optic disc size. The abnormally large optic disc in eyes with normal-tension glaucoma may be important diagnostically and pathogenetically.
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[Inner nuclear layer of the retina in eyes with secondary angle-block glaucoma].
Glaucomatous changes have been known to occur in the inner retinal layer all over the fundus and in the deep retinal layer and retinal pigment epithelium close at the optic disc border. This study was conducted to address the question as to whether the middle retinal layers are altered by the glaucomatous process. The study included histological slides of 23 eyes enucleated due to painful secondary traumatic angle-closure glaucoma and 14 nonglaucomatous eyes removed because of a malignant choroidal melanoma. We evaluated the cell count and thickness of the inner nuclear layer via histomorphometry. The inner nuclear layer contained significantly fewer cells and was thinner (P < 0.05) in the glaucoma group compared to the control group. Since this study contains eyes with possible secondary changes due to the initial trauma, further investigations on eyes with primary open-angle glaucoma are necessary to confirm the results. This could be important for psychophysical testing in glaucoma.
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Human optic nerve fiber count and optic disc size.
In the optic nerve head, the optic nerve fibers are represented by the neuroretinal rim. The rim area showing a high interindividual variability is positively correlated with the optic disc size. This study was performed to address the question of whether, in addition to having a larger neuroretinal rim, eyes with large optic discs also have a higher count of optic nerve fibers compared to eyes with small optic nerve heads. Histologic semithin sections of 72 optic nerves of 56 cornea donors were histomorphometrically evaluated using a computerized image analyzer. The optic nerve fiber count increased significantly (P = 0.01) with enlarging optic disc size. The nerve fiber count was positively correlated with the retrobulbar optic nerve cross section area. It decreased with advancing age, with a mean annual loss of about 4,000 fibers. The nerve fiber density per disc area decreased with increasing optic disc area. Mean and median of the minimal nerve fiber diameter was larger in older subjects. The results may indicate that the optic nerve fiber count, and the anatomic reserve capacity in progressive optic neuropathies, are higher in eyes with large optic discs than in eyes with small optic nerve heads. The optic nerve fiber population decreased with advancing age. This is important for progression, pseudoprogression, and prognosis of optic neuropathies. Optic nerve fiber crowding is more marked in eyes with small optic discs than in eyes with large optic nerve heads. The age-related loss of predominantly small optic nerve fibers can potentiate the optic nerve atrophy in glaucoma and Alzheimer's disease, with both damaging preferentially large axons.
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Decreased photoreceptor count in human eyes with secondary angle-closure glaucoma.
Glaucoma has been known to be associated with a loss of retinal ganglion cells and their axons throughout the fundus and a decreased count of photoreceptors and retinal pigment epithelial (RPE) cells in the parapapillary region. This study investigated whether glaucomatous changes of the deep retinal layer occur outside the parapapillary region. The nuclei of the retinal photoreceptors and RPE cells were counted in histologic slides of 23 eyes with painful secondary angle-closure glaucoma resulting from perforating corneal injuries. Fourteen eyes with malignant choroidal melanoma not involving the ciliary body or trabecular meshwork served as the control group. No surgical procedure, including laser treatment, had been performed posterior to the ora serrata. There were no hints of retinal vessel occlusion and localized traumatic retinopathy, historically, ophthalmoscopically, or histologically. Photoreceptor count was significantly lower (P less than 0.05) in the glaucoma eyes than in the control group. Count of RPE cells did not differ between the two groups. This may indicate that glaucoma can be associated with a loss of photoreceptors. This could be important for psychophysical testing and may point to a more widespread involvement of ocular tissues in glaucoma than believed.
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Correlation of the blind spot size to the area of the optic disk and parapapillary atrophy.
We evaluated the relationship between the optic disk and the blind spot area. Using kinetic Goldmann perimetry in 23 patients with open-angle glaucoma and 19 normal subjects, the blind spot size was correlated significantly with the total area of the optic disk, peripapillary scleral ring, and parapapillary chorioretinal atrophy. Zone beta of the parapapillary atrophy with a visible sclera was attributed to an absolute scotoma, and zone alpha with irregular pigmentation was attributed to a relative scotoma. The blind spot was significantly larger in the glaucomatous eyes than in the normal eyes, which corresponded with a larger zone beta in the glaucomatous eyes. The intrapapillary and parapapillary region of the optic nerve head correlated to the size of the blind spot, which included the parapapillary chorioretinal atrophy and a significant size difference between normal and glaucomatous eyes.
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Correlation of the optic disc size to glaucoma susceptibility.
Previous studies have suggested that a larger optic disc size in blacks as compared with whites is related to the increased glaucoma susceptibility in blacks. In an intraindividual bilateral comparison of 245 white patients with open-angle glaucoma, the authors evaluated whether the glaucomatous optic nerve damage was greater or less in the eye with the larger optic nerve head. Highly myopic eyes were excluded. The difference in optic disc area of one eye as compared with the contralateral eye was not significantly correlated to the differences in visibility of retinal nerve fiber bundles and mean visual field defect between the two eyes. Mean perimetric loss and the retinal nerve fiber layer index were not significantly higher in the eye with the larger or smaller optic nerve head. This indicates that in whites, high myopes excluded, the susceptibility to glaucomatous optic nerve fiber loss may be independent of the optic disc size.
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[Neuroretinal rim in eyes with simple optic atrophy].
In comparison with normal eyes, eyes affected by glaucomatous optic nerve damage are characterized by a decreased neuroretinal rim area and an increased optic cup size. This study was performed to find whether eyes with nonglaucomatous optic nerve atrophy and normal eyes differ with respect to the neuroretinal rim. In all, 143 eyes with nonglaucomatous descending optic nerve atrophy and 563 normal eyes were biomorphometrically examined. The area and form of the neuroretinal rim and optic cup and the horizontal and vertical cup-to-disc ratios did not differ significantly between the two groups. These results are in part not supported by data in the literature. If they are confirmed by further studies, they will indicate that measurement of the neuroretinal rim and estimation of the cup-to-disc ratios will not be useful for the diagnosis of damage to the descending nonglaucomatous optic nerve. It will be helpful for the differentiation of eyes with glaucomatous and nonglaucomatous optic nerve atrophy.
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Parapapillary atrophy and retinal vessel diameter in nonglaucomatous optic nerve damage.
Parapapillary chorioretinal atrophy and decreased retinal vessel diameter occur in glaucomatous eyes. To evaluate the frequency and degree of these signs in nonglaucomatous optic neuropathy, the authors evaluated morphometrically and compared 47 patients with nonglaucomatous optic nerve atrophy from extraocular causes with 292 patients with primary open-angle glaucoma and 179 normal subjects. Eyes with anterior ischemic optic neuropathy were excluded. The parapapillary atrophy was differentiated into a central zone (beta) with sclera and large choroidal vessels visible by ophthalmoscopy and a peripheral zone (alpha) with irregular pigmentation. Both zones did not differ significantly in the eyes with nonglaucomatous optic neuropathy and the normal eyes. In the glaucomatous eyes, they were significantly larger and occurred more frequently. The retinal vessel diameter was significantly smaller in both groups with optic nerve atrophy than in the normal group. It was concluded that decreased retinal vessel diameters unspecifically suggest optic nerve atrophy. Evaluation of parapapillary chorioretinal atrophy can be helpful in differentiating nonglaucomatous from glaucomatous optic neuropathy.
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Morphometry of the human lamina cribrosa surface.
The lamina cribrosa is a sieve-like perforation in the posterior part of the sclera, that allows passage of the retinal ganglion cell axons and central retinal vessels and preserves a pressure gradient between the intraocular and extraocular space. It has been termed the primary site of glaucomatous damage to the optic nerve. Using electron microscopy, the authors morphometrically evaluated the inner surface of the lamina cribosa in 40 normal human donor eyes. There were 14 men and 21 women with a mean age of 52 +/- 22 yr (10-82 yr). Mean single pore area (0.004 +/- 0.001 mm2) and summed pore area were significantly (P less than 0.05) larger and the ratio of summed pore area to lamina area was higher in the inferior and superior regions than in the temporal and nasal regions. The ratio decreased with increasing lamina cribrosa size. Count, size, form, and density of the pores were statistically independent of age, sex, side, and lamina cribrosa form. Pore count and summed pore area (mean: 0.92 +/- 0.22 mm2) increased significantly with enlarging lamina cribrosa size. The area of the lamina cribrosa openings for passage of the central retinal vessels was independent of the lamina cribrosa size. The high ratio of summed pore area to lamina area and the large single pore area may be pathogenetically important for the increased glaucoma susceptibility in the inferior and superior disc regions. The lack of a correlation between lamina cribrosa size and the area of the lamina cribrosa openings for the retinal vessels may explain why central retinal vessel occlusions occur independently of optic disc size.
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Quantitative pupillometry of relative afferent defects in glaucoma.
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Adult↗
Optic disc topography and short-term increase in intraocular pressure.
Intraocular pressure is intra- and interindividually inconstant. It is influenced by numerous ocular and general factors. We evaluated the question as to whether a short-term increase in intraocular pressure might change the two-dimensional topography of the optic nerve head. Optic disc photographs of 63 glaucomatous eyes in 33 Caucasian patients and 39 normal eyes in 22 subjects were taken at a baseline intraocular pressure of less than 20 mm Hg and at 1 and 8 s after pressure elevations of 10 and 20 mm Hg. No significant differences in the size and form of the optic disc, optic cup, neuroretinal rim, peripapillary scleral ring or parapapillary chorioretinal atrophy were found. The retinal vessels mostly reacted to the intraocular pressure elevation by an initial decrease and subsequent re-increase in their diameter; this change was significant (P less than 0.05) for the pressure elevation of 20 mm Hg. We conclude that the two-dimensional optic disc topography is not significantly changed by a short-term increase in intraocular pressure.
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Dark adaptation in glaucomatous and nonglaucomatous optic nerve atrophy.
Optic nerve damage is associated with impairment of psychophysical functions. We measured dark adaptation in 21 eyes of 14 normal subjects, 35 eyes of 19 patients with primary open-angle glaucoma, and 7 eyes of 4 patients with nonglaucomatous descending optic nerve atrophy. In the normal subjects light thresholds and time of the shoulder in the dark adaptation curve increased significantly with age. In eyes with glaucomatous or nonglaucomatous optic nerve damage light sensitivity was lower than in normal eyes of age-matched control groups. Rod light sensitivity was significantly (P less than 0.05) correlated with neuroretinal rim loss, parapapillary chorioretinal atrophy, and relative afferent pupillary defects. We conclude that velocity and degree of dark adaptation decrease with increasing age. Patients with glaucomatous and nonglaucomatous optic nerve atrophy show decreased light sensitivity especially in the rod part of dark adaptation worsening with advancing optic nerve damage.
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