Choriocapillaris thickness and density in axially elongated eyes.

PURPOSE: Axial myopia is characterized by a thinning of the choroid. We examined whether the myopic choroidal thinning also includes a thinning of the choriocapillaris. METHODS: Using light microscopy, we measured thickness and density of the choriocapillaris at the posterior pole, posterior pole-equator midpoint (PPEMP), equator and close to the ora serrata on histological sections of 58 enucleated human globes (mean age: 62.4 ± 17.8 years; range: 24-88 years; mean axial length: 27.8 ± 4.0 mm; range: 22.0-37.0 mm). RESULTS: Choriocapillaris thickness decreased (p < 0.001) from the posterior pole (median: 3.9 µm; interquartile range (IQR): 3.3-6.0) to the equator (median: 2.7 µm; IQR: 1.5, 4.2). It was not significantly associated with axial length, neither at the posterior pole (p = 0.25), the PPEMP (p = 0.81), equator (p = 0.80) or ora serrata (p = 0.50). Mean choriocapillaris density decreased from the posterior pole to the equator (198 µm/300 µm; IQR: 152/300, 246/300 versus 156 µm/300 µm; IQR: 72/300, 216/300; p < 0.001). Choriocapillaris density was not significantly associated with axial length (posterior pole: p = 0.07; PPEMP: p = 0.33; equator: p = 0.22; ora serrata: p = 0.36). CONCLUSIONS: The choriocapillaris thickness and density, decreasing from the posterior pole to the fundus periphery, were not significantly associated with axial length. These findings may be of interest for the understanding of high myopia and pathologic myopia.

Histology of myopic posterior scleral staphylomas.

PURPOSE: Since histomorphometric descriptions of posterior scleral staphylomas, although forming a major part of myopic maculopathy, have been scarce so far, we histomorphometrically examined scleral staphylomas in enucleated human eyes. METHODS: Using light microscopy, we histomorphometrically examined sagittal histological sections of human globes enucleated due to malignant choroidal melanomas or secondary angle-closure glaucoma. RESULTS: Out of 246 globes included into the study, posterior scleral staphylomas were detected in 10 eyes (mean length: 31.4 ± 3.0 mm; range: 28.0-37.0 mm). In the staphylomatous region in the study group as compared with the corresponding region of a control group adjusted for age and axial length, scleral thickness was significantly lower (109 ± 25 µm versus 319 ± 161 µm; p = 0.001). The study group in the staphylomatous region as compared to the highly myopic control group in the corresponding region did not differ significantly in retinal pigment epithelium (RPE) cell density (19.6 ± 4.9 cells/300 µm versus 21.1 ± 5.7 cells/300 µm; p = 0.84) and RPE height (8.2 ± 2.8 µm versus 6.1 ± 2.5 µm; p = 0.13), Bruch's membrane (BM) thickness (3.5 ± 1.3 µm versus 4.2 ± 2.3 µm; p = 0.40) and choriocapillaris thickness (5.3 ± 2.8 µm versus 4.4 ± 2.8 µm; p = 0.49) and density (164 ± 99 µm versus 226 ± 38 µm; p = 0.13). All staphylomatous regions showed a localized BM defect. CONCLUSIONS: Marked scleral thinning and spatially correlated BM defects histologically characterized myopic scleral staphylomas, while thickness and density of the choriocapillaris and RPE and BM thickness did not differ significantly between staphylomatous versus non-staphylomatous eyes in the respective regions. These findings support the notion that a locally reduced scleral resistance against a backward pushing BM led to a local scleral outpouching. The outpouching-associated increase in curvature length may stretch BM with the sequel of a localized BM rupture.

Retinal Pigment Epithelium Cell Density and Bruch's Membrane Thickness in Secondary versus Primary High Myopia and Emmetropia.

To assess differences between secondary high myopia (SHM) due to congenital glaucoma and primary high myopia (PHM) and non-highly myopic eyes (NHM) in the relationships between axial length and Bruch's membrane (BM) thickness and retinal pigment epithelium (RPE) density. The histomorphometric study included human globes enucleated for reasons such as malignant uveal melanoma, end-stage painful secondary angle-closure glaucoma and congenital glaucoma. BM thickness and RPE cell density were measured upon light microscopy. The investigation included 122 eyes (mean axial length: 26.7 ± 3.7 mm; range: 20.0-37.0 mm): 7 eyes with SHM (axial length: 33.7 ± 2.1 mm; range: 31.0-37.0 mm), 56 eyes with PHM (mean axial length: 29.1 ± 2.4 mm; range: 26.0-36.0 mm) and 59 eyes in the NHM-group (axial length: 23.5 ± 1.3 mm; range: 20.0-25.5 mm). In the SHM group, longer axial length was associated with lower RPE cell density at the posterior pole (standardized regression coefficient beta: 0.92; non- standardized regression coefficient B: -2.76; 95% confidence interval (CI): -4.41, -1.10;P = 0.01), at the midpoint posterior pole/equator (beta: -0.87; B: -3.60; 95% CI: -6.48, -0.73;P = 0.03), and at the equator (beta: -0.88; B: -0.95; 95% CI: -1.68, -0.23; P = 0.02), but not at the ora serrata (P = 0.88). In the PHM-group and NHM group, RPE cell density at the posterior pole (P = 0.08) and ora serrata (P = 0.88) was statistically independent of axial length, while at the midpoint posterior pole/equator (P = 0.01) and equator (P < 0.001), RPE cell density decreased with longer axis. BM thickness in the SHM group decreased with longer axial length at the posterior pole (beta: -0.93;B: -0.29; 95% CI: -0.39, -0.14; P = 0.003), midpoint posterior pole/equator (beta: -0.79; B: -0.22; 95% CI: -0.42, -0.02; P = 0.035) and equator (beta: -0.84; B: -0.21; 95% CI: -0.37, -0.06; P = 0.017), while in the PHM-group and NHM-group, BM thickness at any ocular region was not statistically significantly correlated with axial length (all P > 0.05). In the SHM-group, but not in the PHM-group or NHM-group (P = 0.98), lower BM thickness was associated with lower RPE cell density (beta: 0.93; B: 0.09; 95% CI: 0.04, 0.14; P = 0.007), while in the eyes without congenital glaucoma the relationship was not statistically significant. In SHM in contrast to PHM, BM thickness and RPE cell density decrease in a parallel manner with longer axial length. The findings fit with the notion of BM being a primary driver in the process of axial elongation in PHM as compared to SHM.

Peripapillary border tissue of the choroid and peripapillary scleral flange in human eyes.

PURPOSE: To assess dimensions and associations of the peripapillary border tissue of the choroid (PBT-C) and peripapillary scleral flange (PBT-S). METHODS: The histomorphometric investigation included histological sections of enucleated eyes of Caucasian patients. Using light microscopy, the PBT dimensions were measured. RESULTS: The study included 85 eyes (85 patients) with an age of 62.0 years (14.1 years) (mean (SD)) (range:37-87 years) and mean axial length of 26.7 mm (3.5 mm) (range:21.0-37.0 mm). Thicker PBT-C thickness (mean: 68.8 µm (35.7 µm)) was associated with shorter axial length (p < 0.001; standardized regression coefficient beta: -0.50), and longer PBT-C length (mean: 531 µm (802 µm)) was correlated with longer axial length (p < 0.001;beta:0.66). PBT-C cross-sectional area (mean 17 050 µm2 (10 420 µm2 )) was not significantly associated with axial length (p = 0.37). Decreasing with longer axial length (p < 0.001;beta:0.64), the angle between PBT-C and Bruch's membrane was approximately 90° in non-highly myopic eyes without overhanging Bruch's membrane (BM), it ranged between 100° and 180° in eyes with BM overhanging into the intrapapillary region, and it was close to 0° in eyes with parapapillary gamma zone. Thicker thickness of PBT-S (mean:83 µm (21 µm)) was correlated with presence of glaucoma (p = 0.02). Optic nerve pia mater thickness (mean:109 µm (44 µm)) increased with glaucoma presence (p = 0.046;beta:0.31) but not with axial length (p = 0.34). CONCLUSIONS: Peripapillary border tissue of the choroid (PBT-C) and PBT-S as continuation of the optic nerve pia mater are distinct structures, with PBT-C remodelling during myopic axial elongation and PBT-S being mostly independent of axial elongation. PBT-C and PBT-S may be of importance for the optic nerve head biomechanics and PBT-C for separation of the choroidal space from the intrapapillary compartment.

Reactive uveitis, retinal vasculitis and scleritis as ocular end-stage of Acanthamoeba keratitis: a histological study.

We analysed histologically two Acanthamoeba keratitis (AK) eyes with anterior and posterior segment inflammation and blindness. Two enucleated eyes of 2 patients (age 45 and 51y) with AK (PCR of epithelial abrasion positive) were analysed. Histological analysis was performed using hematoxylin-eosin, periodic acid-Schiff and Gömöri-methenamine silver staining. We could not observe Acanthamoeba trophozoites or cysts neither in the cornea nor in other ocular tissues. Meanwhile, we found uveitis, retinal vasculitis and scleritis in these eyes, due to the long-standing, recalcitrant AK. So in this stage of AK, systemic immune suppression may be necessary for a longer time period.

Corrugated Bruch's membrane in high myopia.

PURPOSE: To assess the appearance of Bruch's membrane (BM) in axially elongated eyes. METHODS: The light-microscopical investigation included histological anterior-posterior sections of human eyes. Using a light microscope, we assessed whether BM in the posterior segment was straight or locally corrugated. Corrugation of BM was defined as an elevation of BM with a height >20 µm over a basis of 50 µm without collateral proliferations of retinal pigment epithelium or choroidal swelling. RESULTS: The investigation included 85 eyes (age: 62.0 ± 14.1 years; axial length: 26.7 ± 3.5 mm). In multivariate analysis, the presence of a corrugated BM, detected in eight eyes (9.4%), was strongly associated with the presence of macular BM defects [p = 0.001; odds ratio (OR): 418; 95% confidence interval (CI): 1 215 000], but not with axial length (p = 0.54). Bruch's membrane (BM) corrugation was detected in seven (54%) of 13 eyes with macular BM defects. The single eye with BM corrugation and without macular BM defect showed the corrugated BM located in the parapapillary region at the peripheral end of a large parapapillary gamma zone. CONCLUSION: Bruch's membrane (BM) corrugation can be present in the vicinity of macular BM defects in highly myopic eyes, perhaps due to differences in the tension within BM in various regions at the margin of the BM defect. Bruch's membrane (BM) corrugation may also develop at the papillary end of BM in eyes with a large parapapillary gamma zone, potentially due to a disinsertion of BM at the end of the peripapillary choroidal border tissue of Jacoby. The observation of BM corrugation may help elucidating the aetiology of axial myopia.

Association between axial length and horizontal and vertical globe diameters.

PURPOSE: To assess relationships between axial length and the horizontal and vertical globe diameters. MATERIAL AND METHODS: The study consisted of enucleated human eyes. The horizontal, vertical, and sagittal diameters were measured. RESULTS: The study included 135 globes removed because of malignant uveal melanoma (111 globes) or end-stage painful glaucoma (n = 24 eyes). Mean axial, horizontal, and vertical diameters were 24.6 ± 2.6 mm (range: 20-35 mm), 23.7 ± 1.4 mm (range: 21-29 mm) and 23.7 ± 1.4 mm (range: 20-29 mm) respectively. The horizontal diameter and vertical diameter did not differ significantly (P = 0.92), while both were significantly (P < 0.001) shorter than the axial diameter. The horizontal diameter was significantly and linearly correlated with the vertical globe diameter (P < 0.001; regression line: vertical globe diameter = 0.84 × horizontal globe diameter + 3.69). The axial diameter was significantly (P < 0.001) associated with the horizontal diameter and vertical diameters in a bipartite manner. In eyes with an axial length ≤24 mm, horizontal and vertical diameters increased by 0.44 and 0.51 mm, respectively, for each mm increase in axial diameter, while in eyes with an axial length >24 mm, the horizontal and vertical globe diameter increased by a lower amount of 0.19 and 0.21 mm, respectively, for each mm increase in axial diameter. CONCLUSIONS: Myopic enlargement of the globe beyond an axial length of 24 mm takes place predominantly in the sagittal axis, leading to a change in the globe form from a sphere to an elongated form. It fits with the notion that myopic elongation may occur by an elongation of the eye walls in regions close to the globe's equator.

Retinal pigment epithelium cell density in relationship to axial length in human eyes.

PURPOSE: To assess associations between axial length and density of retinal pigment epithelium (RPE) cells in various ocular regions. METHODS: The histomorphometric investigation included histological sections of enucleated eyes of Caucasian patients. Using a light microscope, we counted the number of RPE cells on Bruch's membrane at the ora serrata, in the pre-equatorial region, the equatorial and retro-equatorial region, at the midpoint equator/posterior pole, and at the posterior pole. RESULTS: The study included 65 globes with a mean axial length 25.9 ± 3.5 mm (range: 21.0-34.0 mm). Retinal pigment epithelium (RPE) cell count in the equatorial to retro-equatorial region (p < 0.001; correlation coefficient r2 : 0.44), in the pre-equatorial region (p < 0.001; r2 : 0.39) and at the midpoint equator/posterior pole (p = 0.03; r2 : 0.12) decreased with increasing axial length. Retinal pigment epithelium (RPE) cell count at the ora serrata (p = 0.49) and posterior pole (p = 0.44) was not significantly correlated with axial length. As a corollary, mean RPE cell density was higher (p < 0.001) at the posterior pole than at the midpoint equator/posterior pole or at the ora serrata region, where it was higher than in pre-equatorial region (p < 0.001) and in the equatorial to retro-equatorial region (p < 0.001). CONCLUSIONS: The decrease in the RPE cell density mainly in the equatorial to retro-equatorial region in association with longer axial length suggests a region of enlargement of Bruch's membrane in the equatorial to retro-equatorial area in association with axial elongation. The finding may be of interest to elucidate the process of emmetropization/myopization.

Retinal Thickness and Axial Length.

PURPOSE: To examine the relationships between axial length and foveal and peripheral retinal thickness. METHODS: Using optical coherence tomography, foveal retinal thickness was measured in participants of the population-based Beijing Eye Study without optic nerve or macula diseases. Inner and outer nuclear layer thickness as surrogate for retinal thickness was assessed in the fundus periphery in human globes enucleated due to malignant uveal melanoma or painful glaucoma. RESULTS: The study included 1117 individuals with a mean age of 64.2 ± 9.7 years (range: 50-93 years) and mean axial length of 23.4 ± 1.04 mm (range: 20.29-28.68 mm). In multivariate analysis, thicker central foveal thickness was associated with male sex (P < 0.001; standardized regression coefficient beta: -0.13; nonstandardized regression coefficient B: -5.84; 95% confidence interval (CI): -8.56, -3.13); urban region of habitation (P = 0.02; beta: 0.07; B: 3.56; 95% CI: 0.55, 6.57); thinner lens thickness (P = 0.01; beta: -0.08; B: -5.11; 95% CI: -9.01, -1.21); thinner subfoveal choroidal thickness (P = 0.04; beta: -0.07; B: -0.01; 95% CI: -0.03, -0.001); and longer axial length (P < 0.001; beta: 0.18; B: 3.79; 95% CI: 2.41, 5.17). In the same multivariate model, superior, inferior, and temporal foveal thickness was not significantly associated with axial length (P = 0.26, P = 0.19, P = 0.08, respectively), while thicker nasal foveal thickness was associated with longer axial length (P = 0.009; beta: 0.09; B: 1.50; 95% CI: 0.37, 2.62). In the histomorphometric part of the study including 32 eyes (sagittal diameter: 27.0 ± 4.2 mm; range: 22-37 mm), mean thickness of the inner and outer nuclear layers at the equator and at the midpoint equator/posterior pole decreased with longer axial length (P = 0.004; beta: -0.48; and P = 0.02; beta: -0.44, respectively). CONCLUSIONS: Myopic axial globe elongation was associated with retinal thinning in the equatorial and pre-equatorial region, while foveal retinal thickness was mostly unaffected by axial length. It suggests that axial elongation takes place predominantly in the equatorial and pre-equatorial region of the eye.

Histologic differences between primary high myopia and secondary high myopia due to congenital glaucoma.

PURPOSE: To search for histomorphometric differences between eyes with primary high myopia (PHM) or secondary high axial myopia (SHM) caused by congenital glaucoma, and non-highly myopic eyes (NHM). METHODS: Histologic anterior-posterior sections were histomorphometrically examined. RESULTS: The investigation included 58 human globes (mean age: 61.5 ± 18.5 years; axial length: 27.3 ± 4.0 mm; range: 21.0-39.0 mm). Bruch's membrane thickness was thinner in SHM than in PHM (posterior pole: p = 0.007; parapapillary region: p = 0.007); midpoint posterior pole/equator = 0.05) and thinner in SHM than in NHM (all p < 0.04), while PHM and NHM did not differ (all p > 0.50). Choroidal thickness did not differ (all p ≥ 0.40) at any measurement location between SHM and PHM, and was thinner (p < 0.05) in both myopic groups than in NHM. Posterior sclera was thinner (p < 0.001) in both myopic groups than in NHM, with no significant difference between both myopic groups. Pars plana scleral thickness was thinner (p = 0.02) in SHM than in PHM after adjusting for axial length. Scleral volume (p = 0.41) and choroidal volume (p = 0.74) did not differ between any of the groups. CONCLUSIONS: Thinning of Bruch's membrane overall is typical for SHM while eyes with PHM have a normal Bruch's membrane thickness. It may point to Bruch's membrane as an active part in the process of emmetropization/myopization. SHM in contrast to PHM showed scleral thinning in the pars plana region suggesting that the process of emmetropization/myopization takes place posterior to the pars plana. Both SHM and PHM unspecifically showed an axial length associated with thinning of choroid and posterior sclera, while both myopic groups did not differ with NHM in choroidal and scleral volume.

Eyelid aging: pathophysiology and clinical management.

Life expectancy is increasing in most countries. With increasing age, many individuals may develop involutional ophthalmic diseases, such as eyelid aging. Dermatochalasis, ptosis, ectropion, and entropion are common disorders in middle-aged and older adults. This review outlines the pathophysiology and clinical management of these involutional eyelid disorders. Recently, a decrease in elastic fibers with ultrastructural abnormalities and an overexpression of elastin-degrading enzymes have been demonstrated in involutional ectropion and entropion. This may be the consequence of local ischemia, inflammation, and/or chronic mechanical stress. Eyelid aging with progressive loss of tone and laxity may affect the ocular surface and adnexal tissues, resulting in different clinical symptoms and signs. Surgical management depends on the appropriate correction of the underlying anatomical defect.

Eyelid aging: pathophysiology and clinical management / Envelhecimento palpebral: fisiopatologia e considerações clínicas

ABSTRACTLife expectancy is increasing in most countries. With increasing age, many individuals may develop involutional ophthalmic diseases, such as eyelid aging. Dermatochalasis, ptosis, ectropion, and entropion are common disorders in middle-aged and older adults. This review outlines the pathophysiology and clinical management of these involutional eyelid disorders. Recently, a decrease in elastic fibers with ultrastructural abnormalities and an overexpression of elastin-degrading enzymes have been demonstrated in involutional ectropion and entropion. This may be the consequence of local ischemia, inflammation, and/or chronic mechanical stress. Eyelid aging with progressive loss of tone and laxity may affect the ocular surface and adnexal tissues, resulting in different clinical symptoms and signs. Surgical management depends on the appropriate correction of the underlying anatomical defect.

RESUMOA expectativa de vida está aumentando na maioria dos países. Com o envelhecimento, muitos indivíduos desenvolverão doenças oculares crônicas e involucionais, tais como o envelhecimento palpebral. Dermatocálase, ptose, ectrópio e entrópio são doenças frequentes em adultos e idosos. Esta revisão destaca a fisiopatologia e a clínica de doenças palpebrais involucionais. Recentemente, uma diminuição de fibras elásticas com anormalidades ultraestruturais e um aumento de enzimas degradantes de elastina foram demonstrados em ectrópio e entrópio involucionais. Isto pode ser consequência de isquemia local, inflamação e/ou estresse mecânico crônico. O envelhecimento palpebral com perda progressiva de tônus e flacidez pode afetar a superfície ocular e os anexos oculares, resultando em sinais e sintomas clínicos diferentes. O tratamento cirúrgico depende da correção apropriada do defeito anatômico subjacente.

Bilateral Orbital IgG4-Related Disease with Systemic and Corneal Involvement Showing an Excellent Response to Steroid and Rituximab Therapy: Report of a Case with 11 Years Follow-Up.

IgG4-related disease is a newly recognized fibro-inflammatory condition. The purpose of this report is to present a patient with 11 years of follow-up, who revealed characteristic features of IgG4-related disease with systemic, orbital and corneal involvement and showed a favorable response to steroids and rituximab treatment.

Scleral cross section area and volume and axial length.

PURPOSE: To examine whether the scleral cross sectional area and estimated scleral volume are associated with a longer axial length in human eyes. METHODS: Histologic anterior-posterior sections running through the pupil and the optic nerve head were examined. Using a light microscope, we measured the thickness of the sclera at the limbus, ora serrata, equator, midpoint between equator and posterior pole (MPEPP), peripapillary region and posterior pole. Additionally we determined the length and the cross section area of the sclera. RESULTS: The histomorphometric study included 214 human globes of 214 subjects (mean age: 62.5±13.9 years) (147 eyes enucleated due to malignant choroidal melanoma or due to other non-glaucomatous reasons; 67 eyes enucleated due to secondary angle-closure glaucoma). Mean axial length was 25.1±1.8 mm (median: 24.0 mm; range: 20-35 mm). Scleral thickness measurements decreased with increasing axial length for values taken at the equator (P = 0.008; correlation coefficient r = -0.18), MPEPP (P<0.001;r:-0.47), optic nerve head border (P<0.001;r = -0.47) and posterior pole (P<0.001;r = -0.54). Scleral cross section area decreased significantly with increasing axial lengths for the regions at or behind the equator (P = 0.002;r = -0.21), at or behind the MPEPP (P = 0.001;r = -0.25), and at or behind the optic nerve head border (P = 0.001;r = -0.24). Scleral volume measurements were not significantly associated with axial length. CONCLUSIONS: Despite an associated increase in surface area, eyes with longer axial length do not have an increase in scleral volume. It may point against a scleral volume enlargement to play a role in the process of axial elongation.

Bruch's membrane thickness in high myopia.

PURPOSE: To measure the thickness of Bruch's membrane (BM) and assess its associations with axial length. METHODS: Using a light microscope, we measured the thickness of Bruch's membrane, choroid and sclera on horizontal anterior-posterior histological sections of enucleated human globes. RESULTS: The study included 54 eyes (42 with glaucomatous optic nerve damage; 23 globes with axial length ≥26.5 mm) with a mean axial length of 26.6 ± 3.3 mm (range: 21.0-34.0 mm). In the non-highly myopic eyes, BM was significantly thicker at the pars plana region (6.6 ± 1.1 µm) than at the equator (4.1 ± 1.5 µm; p < 0.001), the midpoint between equator and posterior pole (4.1 ± 1.5 µm; p < 0.001), the parapapillary region (4.5 ± 1.0 µm; p = 0.006) or the posterior pole (4.4 ± 1.7 µm; p = 0.001). At any measurement location at or behind the ora serrata, BM thickness did not differ significantly (all p ≥ 0.40) between the non-glaucomatous non-highly myopic group and the glaucomatous non-highly myopic group, or between the non-highly myopic group and the highly myopic group. BM thickness at any location was not significantly (all p > 0.45) related to axial length. The ratio of scleral thickness to BM thickness and the ratio of choroidal thickness to BM thickness significantly decreased with longer axial length for measurements taken behind the equator. CONCLUSIONS: BM is physiologically thickest in the pars plana, with no major thickness differences between locations at or behind the ora serrata. In contrast to choroidal and scleral thickness, BM thickness is not significantly related to axial length. Axially elongated eyes have a normal BM thickness, despite a thinned choroid and sclera.

Peripapillary ring: histology and correlations.

PURPOSE: To assess the histologic correlate of the peripapillary ring (PPR) as the optic disc boundary, to measure it histomorphometrically and to assess its associations with glaucoma and axial length. METHODS: Using a light microscope, we measured the width of the PPR and optic nerve pia mater. We additionally had measured the axial length, length of parapapillary beta and gamma zones, and thickness of the sclera and choroid. RESULTS: The study included 83 human globes with a mean axial length of 26.1 ± 3.3 mm (range: 20.0-35.0 mm). In the non-highly myopic (axial length <26.5 mm) non-glaucomatous eyes, mean PPR width was 55 ± 14 µm (range: 26-88 µm) and mean thickness of the optic nerve pia mater was 52 ± 22 µm (range: 20-84 µm). PPR width and pia mater thickness were significantly associated with each other (p = 0.001; correlation coefficient r = 0.37). PPR width increased significantly with longer axial length (p = 0.001; r = 0.35) and with the length of the peripapillary scleral flange (p = 0.02; r = 0.34). Pia mater thickness increased significantly with longer axial length (p = 0.03; r = 0.24) and with presence of glaucoma (p = 0.03). In eyes without parapapillary gamma zone, PPR was covered or was at least touched by the end of Bruch's membrane and separated the peripapillary choroid from the prelaminar optic nerve head tissue. In eyes with parapapillary gamma zone, PPR was the continuation of the optic nerve pia mater and reached the undersurface of the peripapillary retinal nerve fibre layer. In these eyes, Bruch's membrane started in a distance of up to 5 mm from PPR, and the thin space of peripapillary choroid ended by an attachment of Bruch's membrane to the thinned sclera. CONCLUSIONS: Peripapillary ring width (mean: 55 ± 14 µm) increases slightly with longer axial length and is independent of glaucoma. It is strongly correlated with the pia mater thickness and is the continuation of the pia mater to the ocular surface.

Peripapillary arterial circle of Zinn-Haller: location and spatial relationships with myopia.

PURPOSE: To measure histomorphometrically the location of the peripapillary arterial circle of Zinn-Haller (ZHAC) and assess its associations with axial length. METHODS: Using a light microscope, we measured the distance from the ZHAC to the peripapillary ring (optic disc border), the merging point of the dura mater with the posterior sclera ("dura-sclera point"), and the inner scleral surface. In the parapapillary region, we differentiated between beta zone (presence of Bruch's membrane, absence of retinal pigment epithelium) and gamma zone (absence of Bruch's membrane). The peripapillary scleral flange as roof of the orbital cerebrospinal fluid space was the connection between the end of the lamina cribrosa and the posterior full-thickness sclera starting at the dura-sclera point. RESULTS: The study included 101 human globes (101 patients) with a mean axial length of 26.7 ± 3.7 mm (range: 20.0-39.0 mm). The distance between the ZHAC and the peripapillary ring increased significantly with longer axial length (P<0.001; correlation coefficient r=0.49), longer parapapillary gamma zone (P<0.001;r=0.85), longer (P<0.001;r=0.73) and thinner (P<0.001;r=-0.45) peripapillary scleral flange, and thinner sclera posterior to the equator (P<0.001). ZHAC distance to the peripapillary ring was not significantly associated with length of parapapillary beta zone (P=0.33). Including only non-highly myopic eyes (axial length <26.5 mm), the ZHAC distance to the disc border was not related with axial length (P=0.84). In non-highly myopic eyes, the ZHAC was located close to the dura-sclera point. With increasing axial length and decreasing thickness of the peripapillary scleral flange, the ZHAC was located closer to the inner scleral surface. CONCLUSIONS: The distance between the ZHAC and the optic disc border is markedly enlarged in highly myopic eyes. Since the ZHAC is the main arterial source for the lamina cribrosa blood supply, the finding may be of interest for the pathogenesis of the increased glaucoma susceptibility in highly myopic eyes.

Macular Bruch's membrane defects and axial length: association with gamma zone and delta zone in peripapillary region.

PURPOSE: To examine histomorphometrically the macular region of highly myopic eyes. METHODS: On horizontal anterior-posterior histological sections, we examined the posterior pole of 138 human globes (axial length: 20-35 mm). In the parapapillary region, we differentiated between the beta zone (Bruch's membrane without RPE), gamma zone (parapapillary region without Bruch's membrane), and delta zone (elongated and thinned gamma zone). RESULTS: In 12 (8.7%) eyes, a macular Bruch's membrane defect (MBMD) was detected. The MBMD showed a complete lack of RPE and choriocapillaris, and an almost complete lack of photoreceptors. Presence of MBMD was associated with longer axial length (P < 0.001), longer gamma zone (P = 0.04) and delta zone (P < 0.001), thinner peripapillary scleral flange, and thinner sclera just outside of the optic nerve meninges (P < 0.001) and at the posterior pole (P < 0.001). An MBMD was found only in eyes with an axial length of 27 mm or longer. MBMD prevalence in highly myopic eyes was 12/39 or 30.8%. MBMD presence was not significantly related to length of beta zone (P = 0.09). In multivariate binary regression analysis, MBMD presence was significantly (P < 0.001) associated only with axial length. CONCLUSIONS: Highly myopic eyes (axial length ≥27mm) can show an MBMD associated with complete loss of RPE and choriocapillaris, and marked reduction of photoreceptors and large choroidal vessels. MBMD presence was strongly associated with axial length and indirectly with parapapillary gamma zone and delta zone. The myopia-associated secondary MBMDs may occur parallel to the myopia-associated widening of Bruch's membrane opening around the optic nerve head.

Successful remission induction with a combination therapy of rituximab, cyclophosphamide, and steroids in a patient with refractory optic neuritis in Wegener's granulomatosis.

A 56-year old patient with a history of limited Wegener's granulomatosis presented with signs and symptoms of optic neuritis. Radiologic signs of orbital inflammation were absent. Testing of visual acuity and visual field allowed close monitoring of disease activity. Thus, rapid improvement of visual function was achieved with high-dose steroids. Despite maintenance therapy with cyclophosphamide recurrence occurred repeatedly as soon as tapering of steroids was begun. After remission induction with rituximab, the patient retained good visual function under maintenance therapy with azathioprine and low-dose corticosteroids. Optic neuritis in Wegener's granulomatosis without signs of orbital involvement is rare and most likely caused by occlusive vasculitis of the vasa nervorum. In this patient with optic neuritis refractory to conventional therapy, rituximab in combination with cyclophosphamide and corticosteroids was well tolerated and successfully used for remission induction, followed by maintenance therapy with azathioprine and low dose corticosteroids.