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      Splinter Cataract in Patients with Keratoconus and Frequent Eye Rubbing: A Novel Finding

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          Abstract

          Case series

          Patients:—

          Final Diagnosis: Splinter cataract

          Symptoms: None

          Clinical Procedure: —

          Specialty: Ophthalmology

          Objective:

          Unknown etiology

          Background:

          Patients with keratoconus are at increased risk of developing cataract at a younger age compared to the normal population. Predisposing factors include atopy and topical steroid use. In this case series we report the novel observation of splinter-shaped cortical cataract in 16 eyes of 14 patients with keratoconus in the absence of other risk factors of cataract development from a single center in Riyadh, Saudi Arabia.

          Case Report:

          This is a retrospective review of 16 eyes of 14 patients diagnosed with keratoconus and found to have splinter cortical cataract. Twelve patients had unilateral and 2 patients had bilateral splinter cortical cataract in the inferotemporal quadrant of the crystalline lens. Thirteen eyes (81.25%) had clinically proven keratoconus and 3 eyes (18.75%) were keratoconus suspects. All patients affirmed frequent eye rubbing and 62.5% of eyes had a history of vernal keratoconjunctivitis. Best corrected visual acuity (BCVA) by the logarithm of the minimum angle of resolution (LogMAR) was reported between 0 to 0.2 in 69% of eyes (11 eyes) and 4 eyes (25%) had BCVA of 0.3 to 0.6, and 1 eye (6%) had BCVA of 1.3.

          Conclusions:

          Splinter-shaped cortical cataract could be a sign of frequent eye rubbing. Careful examination of the crystalline lens with dilated pupil may reveal peripheral cortical opacities in the inferotemporal quadrant, which can alert the physician that the patient has habitual eye rubbing and is at increased risk of keratoconus development or progression.

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          Most cited references25

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          Keratoconus: an inflammatory disorder?

          Keratoconus has been classically defined as a progressive, non-inflammatory condition, which produces a thinning and steepening of the cornea. Its pathophysiological mechanisms have been investigated for a long time. Both genetic and environmental factors have been associated with the disease. Recent studies have shown a significant role of proteolytic enzymes, cytokines, and free radicals; therefore, although keratoconus does not meet all the classic criteria for an inflammatory disease, the lack of inflammation has been questioned. The majority of studies in the tears of patients with keratoconus have found increased levels of interleukin-6 (IL-6), tumor necrosis factor-α(TNF-α), and matrix metalloproteinase (MMP)-9. Eye rubbing, a proven risk factor for keratoconus, has been also shown recently to increase the tear levels of MMP-13, IL-6, and TNF-α. In the tear fluid of patients with ocular rosacea, IL-1α and MMP-9 have been reported to be significantly elevated, and cases of inferior corneal thinning, resembling keratoconus, have been reported. We performed a literature review of published biochemical changes in keratoconus that would support that this could be, at least in part, an inflammatory condition.
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            Atopy and keratoconus: a multivariate analysis.

            The primary goal of this study was to determine if atopy is a risk factor for keratoconus. Other potential risk factors were also studied and included age, sex, race, eye rubbing, mitral valve prolapse, handedness, collagen vascular disease, ocular trauma, pigmentary retinopathy, Marfan's syndrome, Down's syndrome, and a history of contact lens wear. A case-control study was designed (n=120) with incident cases assembled from the years 1985-99. Controls were chosen from the same person-time experience as cases and were picked from a source population with multiple outcomes ensuring that none was knowingly related to any of the potential exposures being studied. Atopy was defined based on the UK working group 1994 definition (at least 4/6 criteria = complete, 3/6 criteria = incomplete, and at least 1/6 criteria = partial). Keratoconus was defined based on clinical criteria and previously published I-S values. Multiple logistic regression was used in the analysis to obtain the odds ratios as the measure of association. In the univariate associations, there was an association between keratoconus and atopy as well as eye rubbing and family history of keratoconus. However, in the multivariate analysis, only eye rubbing was still a significant predictor of keratoconus (odds ratio = 6.31 p = 0.001). This study supports the hypothesis that the most significant cause of keratoconus is eye rubbing. Atopy may contribute to keratoconus but most probably via eye rubbing associated with the itch of atopy. No other variable measured was significantly associated with the aetiology of keratoconus.
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              Mechanisms of rubbing-related corneal trauma in keratoconus.

              Corneal scarring in keratoconus, which is observed prior to contact lens wear and in association with a chronic habit of abnormal rubbing, suggests a keratocyte change to a repair phenotype in response to rubbing trauma. This review examines known and putative mechanisms for rubbing-related corneal trauma and cone formation. Responses to eye rubbing (and possible causal links) may include increased corneal temperature, epithelial thinning, increased concentrations of inflammatory mediators in the precorneal tears, abnormal enzyme activity, large intraocular pressure spikes, high hydrostatic tissue pressure, thixotropically reduced ground substance viscosity, temporary displacement of ground substance from the corneal apex, buckling and flexure of fibrils associated with waves of corneal indentation, biomechanically coupled curvature transfer to the cone apex, slippage between collagen fibrils at the cone apex, and changes to keratocytes due to mechanical trauma and/or high hydrostatic pressure, in addition to scar formation. Cone formation appears to depend on a loss of shear strength and may be a consequence of a reduction in ground substance viscosity and glue function, which could allow the cornea to bend and yield to intraocular pressure. For some forms of keratoconus, a reduction in shear strength and cone-forming deformation may be responses to rubbing trauma. Some of the mechanisms for corneal rubbing trauma may be relevant to post-laser-assisted in situ keratomileusis ectasia or complications following other types of corneal surgery. There appear to be indications for the control of chronic habits of abnormal rubbing.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2023
                28 April 2023
                : 24
                : e939082-1-e939082-6
                Affiliations
                Anterior Segment Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
                Author notes
                Corresponding Author: Sara AlHilali, e-mail: SaraAlHilali@ 123456gmail.com

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Financial support: None declared

                Conflict of interest: None declared

                Author information
                https://orcid.org/0000-0002-8050-2953
                https://orcid.org/0000-0002-0105-7055
                Article
                939082
                10.12659/AJCR.939082
                10152507
                37113025
                b1bd2d3c-418a-4739-9360-b380c4bf1250
                © Am J Case Rep, 2023

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 27 November 2022
                : 20 March 2023
                : 24 March 2023
                Categories
                Articles

                cataract,keratoconus
                cataract, keratoconus

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