Assessment of corneal topographic, tomographic, densitometric, and biomechanical properties of Fabry patients with ocular manifestations

Cankurtaran, Veysel; Tekin, Kemal; Çakmak, Ayşe; İnanç, Merve; Turgut, Faruk

doi:10.1007/s00417-019-04593-8

Cited by 8 publications

(3 citation statements)

References 37 publications

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“…Biomechanical studies have provided evidence that the accumulation of sphingolipids in the cornea affects static and dynamic responses, in eyes with VK. Cankurtaran et al, using corneal tomography, demonstrated that FD patients have statistically significant higher corneal densitometry values in all corneal concentric zones and layers, except the posterior 0–2 mm and posterior 2–6 mm zones, as compared to healthy eyes; thus, VK is associated with increased light backscattering and reduced corneal transparency [ 58 ]. Moreover, corneal biomechanical analysis conducted with Corvis ST (Oculus Optikgerate Gmbh, Wetzlar, Germany) showed increased corneal stiffness in VK, probably due to the stromal accumulation of sphingolipids [ 58 ].…”

Section: Corneal Manifestationsmentioning

confidence: 99%

“…Cankurtaran et al, using corneal tomography, demonstrated that FD patients have statistically significant higher corneal densitometry values in all corneal concentric zones and layers, except the posterior 0–2 mm and posterior 2–6 mm zones, as compared to healthy eyes; thus, VK is associated with increased light backscattering and reduced corneal transparency [ 58 ]. Moreover, corneal biomechanical analysis conducted with Corvis ST (Oculus Optikgerate Gmbh, Wetzlar, Germany) showed increased corneal stiffness in VK, probably due to the stromal accumulation of sphingolipids [ 58 ]. Koh et al, through the use of quantitative contrast sensitivity evaluations, found clear functional deficits in contrast sensitivity measurements, despite normal visual acuities, in patients with VK [ 59 ].…”

Section: Corneal Manifestationsmentioning

confidence: 99%

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Ophthalmic Manifestations in Fabry Disease: Updated Review

Gambini

Scartozzi

Giannuzzi

et al. 2023

JPM

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Fabry disease (FD) is an X-linked lysosomal storage disorder, causing Gb-3 (globotriaosylceramide) buildup in cellular lysosomes throughout the body, in particular in blood vessel walls, neuronal cells, and smooth muscle. The gradual accumulation of this glycosphingolipid in numerous eye tissues causes conjunctival vascular abnormalities, corneal epithelial opacities (cornea verticillata), lens opacities, and retinal vascular abnormalities. Although a severe vision impairment is rare, these abnormalities are diagnostic indicators and prognostics for severity. Cornea verticillata is the most common ophthalmic feature in both hemizygous men and heterozygous females. Vessel tortuosity has been linked to a faster disease progression and may be useful in predicting systemic involvement. New technologies such as optical coherence tomography angiography (OCTA) are useful for monitoring retinal microvasculature alterations in FD patients. Along with OCTA, corneal topographic analysis, confocal microscopy, and electro-functional examinations, contributed to the recognition of ocular abnormalities and have been correlated with systemic involvement. We offer an update regarding FD ocular manifestations, focusing on findings derived from the most recent imaging modalities, to optimize the management of this pathology.

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Section: Corneal Manifestationsmentioning

confidence: 99%

Section: Corneal Manifestationsmentioning

confidence: 99%

Ophthalmic Manifestations in Fabry Disease: Updated Review

Gambini

Scartozzi

Giannuzzi

et al. 2023

JPM

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“…Optical aberrations result in reduced visual quality and blurred images. HOA impairs retinal image quality in the form of glare and halos, yet they cannot be corrected with lenses neither sphere nor cylinder (27).HOA is a minor part of ocular aberrations 10% (28)..In the current study we investigated the anterior segment properties in different thyroid hormon diseases, we also investigated aberrometric values in hyperthyroidi,hypothyroidi and healthy individuals,interestingly, in our study, HOA values in the central 6-mm corneal zone were higher in patients with hyperthyroidism.this can be explained with the effect of the thyroid hormone on keratan sulfate and glycosaminoglycan (18,19).However further studies needed to explain this mechanism.…”

Section: Konuk Et Al Investigated Cct Values Of Patients With Graves'...mentioning

confidence: 99%

The Effect of Thyroid Hormone Level on Topographic, Densitometric and Aberrometric Values Of the Cornea

Alakuş,

Kozan,

Çağlayan

et al. 2023

Preprint

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Purpose : To investigate the topographic measurements,aberrometrics and densitometrics values ofcorneas in hypothyroid , hyperthyroid patients compared to healthy individuals. Material and Methods: This cross-sectional comparative study included 20 hypothyroid patients(group I), 20 hyperthyroid patients (group II), and 20 age-matched controls (group III). The Pentacamhigh resolution imaging system is used to determine corneal topographic measurements,aberrometryand densitometry. Results: Mean age group I, II and III 42.11±12.84(21-65), 44.52±10.11(20-66), 44.04±8.06(20-65)years was respectively (p =0.091). There was no significant difference between the groups nostatistically significant differences were found between the groups in terms of the 0-2 mm and 2-6 mmdensitometry values. At 6-10 mm corneal zone at all layers, including anterior, mid-stroma, posteriorand total, were significantly higher in the hyperthyroidism group compared to the control group(p=0.002, p=0.001, p=0.002 and p=0.001, respectively). At the 10-12 mm peripheral corneal zone,corneal densitometry values in all layers were significantly higher in the hyperthyroidism groupcompared to both the control group and hypothyroidism group (p<0.05 for all values). For theaberrometric measures, higher order aberrations were greater for the hyperthyroidism group than thecontrol group” (p=0.012). Conclusion : Hyperthyroidism may affect corneal densitometric and aberometric values,which may alter the corneal transparency.

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Cornea and Sclera

Yanoff,

Sassani

2025

Ocular Pathology

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Assessment of corneal topographic, tomographic, densitometric, and biomechanical properties of Fabry patients with ocular manifestations

Cited by 8 publications

References 37 publications

Ophthalmic Manifestations in Fabry Disease: Updated Review

Ophthalmic Manifestations in Fabry Disease: Updated Review

The Effect of Thyroid Hormone Level on Topographic, Densitometric and Aberrometric Values Of the Cornea

Cornea and Sclera

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