Recurrence of paraproteinemic keratopathy after penetrating keratoplasty and its assessment with confocal microscopy

Purpose To report on a case of recurrence of paraproteinemic keratopathy (PPK) associated with monoclonal gammopathy after bilateral penetrating keratoplasty. Observations Penetrating keratoplasty was performed on both eyes of a 45-year-old man due to bilateral progressive corneal stromal clouding. Recurrence of the corneal stromal opacities accompanied by a decrease in visual acuity was observed on slit-lamp examination already two years after penetrating keratoplasty. Confocal laser scanning microscopy (CLSM) of the corneal grafts performed three years after penetrating keratoplasty showed bilateral morphological changes identical to that found in the patient's corneas prior to penetrating keratoplasty. A hematological work-up revealed monoclonal gammopathy of type IgG kappa. The histochemical examination of the explanted corneas confirmed the diagnosis of PPK. Conclusions and importance Paraproteinemic keratopathy is an underdiagnosed ophthalmological condition, which may be associated with potentially life-threatening hematologic disorders. A hematological workup should be performed in patients with corneal opacities of uncertain etiology. Penetrating keratoplasty should be performed with caution in patients with monoclonal gammopathy due to the possibility of a very fast recurrence of PPK in the corneal graft. This is the first presentation of the recurrence of flake like PPK after penetrating keratoplasty assessed with CLSM

Corneal topometric, aberrometric and biomechanical parameters in mucopolysaccharidosis patients

AIMS: To report corneal topometric and aberrometric values in mucopolysaccharidosis (MPS) and to investigate their correlation with biomechanical corneal parameters. METHODS: One randomly chosen eye of 20 MPS patients with no to moderate corneal clouding and one eye of 23 healthy controls with comparable age were prospectively included into this study. Corneal surface regularity was assessed by index of surface variance (ISV), -vertical asymmetry (IVA), -height asymmetry (IHA), -height decentration (IHD); keratoconus index (KI), central keratoconus index (CKI) and Zernike indices of anterior and posterior corneal surface using Scheimpflug imaging (Pentacam). Corneal resistance factor (CRF) and corneal hysteresis (CH) were assessed by Ocular Response Analyzer. Statistical analyses were performed using Mann-Whitney-Test and Spearman Correlation Coefficients. RESULTS: IVA, ISV, IHD, IHA, but not KI and CKI were significantly higher in MPS patients compared to age matched healthy controls. Spherical aberration and asphericity coefficients either at the anterior or at the posterior corneal surface differed significantly between both groups. The grade of the MPS-associated corneal opacity correlated significantly with ISV (rho = 0.52), IVA (rho = 0.54), IHA (rho = 0.57) and IHD (rho = 0.48). Density of the MPS-affected corneas correlated significantly with ISV (rho = 0.52), IVA (rho = 0.72), IHA (rho = 0.57), IHD (rho = 0.69), 3rd order horizontal trefoil aberration at the posterior (rho = 0.62) and anterior surface (rho = 0.48) as well as with CH (rho = 0.55) and CRF (rho = 0.57). Spherical aberration at the back surface correlated with CRF and CH in MPS and in healthy controls. CONCLUSIONS: This is the first study analyzing shape of the corneal surface in MPS patients. Topometric indices of corneal asymmetry are significantly increased and correlate with MPS-related corneal opacity and density. Spherical aberration and asphericity coefficient at the front and at the back corneal surface differ significantly between MPS and healthy controls

Impact of cross-linking procedure on perioperative quality of life in keratoconus patients

Background: To evaluate the effect of crosslinking (CXL) with riboflavin for keratoconus (KC) therapy on quality of life (QoL): comparison of keratoconus patients with and without treatment. Methods: Prospective monocentric study. We recruited patients with progressive KC and with stable disease. Patients with progressive disease received cross-linking treatment; patients with stable disease were monitored. We compared QoL in both groups over 6 months and detected the influence of cross-linking treatment on QoL. QoL was assessed by NEI-VFQ-25, EQ-5D 5L, and EQ-Visual analog scale (VAS). In the evaluation of the Nei VFQ, the subgroups LFVFS and LFSES were calculated. Results: We enrolled 31 eyes of 31 patients in the intervention group and 37 eyes of 37 patients in the control group. Medians with standard deviations (SD) were calculated. All QoL-tests showed equal scores at baseline in both groups. At V2, one day after the treatment, EQ-VAS (56.4), LFVFS (57.4), and EQ5D5L (0.59) were significantly reduced. At V3 (one week after treatment), all results returned to baseline level. LFSES was not affected by the treatment. It remained stable (V2 85.4, V3 84.3). Comparing the baseline scores with the follow-up scores at month 6, we found a significant increase in QoL in all tests in the intervention group. Otherwise, the quality of life in the control group did not change over time. Conclusions: Cross-linking led only to a short-term reduction in QoL. Although the treatment is painful for a few days, no effect on general quality of life LVSES has been demonstrated. QoL already returned to baseline after one week and the patients were not limited anymore

A lower birth weight percentile is associated with central corneal thickness thinning : results from the Gutenberg Prematurity Eye Study (GPES)

Purpose: Prematurity, prenatal growth restriction, and retinopathy of prematurity (ROP) are associated with altered ocular geometry, such as a steeper corneal shape in childhood, but it is unclear whether perinatal history affects corneal thickness development, so this study investigated whether corneal thickness in adulthood is affected by perinatal history. Marterials and Methods: The Gutenberg Prematurity Eye Study (GPES) is a retrospective cohort study with a prospective ophthalmologic examination in Germany. The corneal thickness was measured by Scheimpflug imaging (Pentacam HR, Oculus Optikgeräte GmbH, Wetzlar, Germany), and the relationship between perinatal parameters respective birth weight percentile and corneal thickness at different locations was assessed using uni- and multivariable linear regression models. Covariates included age, sex, mean corneal radius, white-to-white distance, gestational age, birth weight percentile, ROP occurrence, and treatment. The main outcome measures were corneal thickness at the apex, the pupil center, and the corneal periphery. Results: The corneal thickness was measured in 390 participants (754 eyes, mean age 29.7+/-8.7 years, 224 females). In multivariable analyses, a lower birth weight percentile was associated with a lower corneal thickness at the apex (B = 0.20, p = 0.003) and the pupil (B = 0.19, p = 0.007). These effects diminished towards the corneal periphery and were not observed beyond the 4-mm diameter circle around the thinnest corneal position. Neither gestational age, ROP occurrence, or ROP treatment affected the corneal thickness. Conclusion: A lower birth weight percentile in subjects born preterm as a proxy for restricted fetal growth is associated with corneal thickness thinning in adults aged 18 to 52 years, indicating that corneal thickness development, particularly in the corneal center, may originate in the fetal stage

The role of preterm birth, retinopathy of prematurity and perinatal factors on corneal aberrations in adulthood : results from the Gutenberg prematurity eye study

Introduction Prematurity and retinopathy of prematurity (ROP) are associated with altered corneal shape and reduced visual acuity in childhood, but their long-term effects on corneal shape in later life are still unclear. This study evaluated whether prematurity and related perinatal factors are associated with corneal aberrations in adulthood. Methods The Gutenberg Prematurity Eye Study (GPES) is a cohort study using Scheimpflug imaging of the cornea. Associations were assessed between corneal Zernike aberrations and gestational age (GA), birth weight (BW), BW percentile, ROP occurrence, ROP treatment and other perinatal factors using univariate and multivariable linear regression analyses. Results This study involved 444 eyes of 256 individuals born preterm (aged 28.1 ± 8.4 years, 146 females) and 231 eyes of 132 individuals born full-term (aged 29.8 ± 8.9 years, 77 females). Multivariable analyses revealed an association between corneal higher-order aberrations and lower birth weight percentile (B = −0.001, p < 0.001) as well as ROP treatment (B = 0.120, p = 0.03). Corneal lower-order aberrations were also associated with lower birth weight percentile (B = −0.004; p = 0.001) and ROP treatment (B = 0.838, p = 0.01) but not with ROP occurrence. Increased corneal aberrations were correlated with lower visual acuity and the spherical equivalent refractive error. Conclusions Perinatal factors, particularly low birth weight percentile and ROP treatment lead to a more irregular corneal shape in adulthood, thereby reducing optical image quality and potentially contributing to reduced visual acuity and altered refractive error

Peripheral corneal thickness and associated factors : results from the population-based German Gutenberg Health Study

Purpose Changes in peripheral corneal thickness are described in various corneal diseases such as corneal ectasia. However, few data exist describing the increase in corneal thickness from central to peripheral and reporting the normal distribution of corneal thickness in rings around the corneal centre. The aim of this study was to report these cornea characteristics and investigate associated factors in a population-based setting. Methods The Gutenberg Health Study is a prospective, population-based study examining participants in a 5-year follow-up (age range 40–80 years) using Scheimpflug imaging. Corneal thickness was assessed in each participant at the apex, as well as in the corneal centre (thinnest corneal thickness) and in rings with 2, 4, 6, 8 and 10 mm diameter around the corneal centre, and the increase in corneal thickness towards the periphery. The relationship between corneal thickness at these locations and possible associated factors was determined using linear regression models. For this purpose, general and ocular parameters were included. Results A total of 9729 participants were included in the present analysis (4874 women, age 59.2 ± 10.8 years). Multivariable analysis showed a correlation between the increase in corneal thickness in the circles from 0 to 10 mm (diameter) and the following parameters: age (B = −0.24 µm per year, p < 0.001); body height (B = −0.04 µm, p = 0.005); smoking (B = −0.72 µm, p < 0.001); spherical equivalent (B = −0.70 µm per dioptre, p < 0.001); white-to-white distance (B = −0.75 µm/mm, p < 0.001); mean corneal radius (B = −3.61 µm/mm, p < 0.001); intraocular pressure (B = −0.12 µm/mmHg, p < 0.001); glaucoma (B = −1.94 µm, p < 0.001); and pseudophakia (B = 0.89 µm, p < 0.001). Conclusion The results of the present study suggest that several general and ocular parameters are associated with peripheral corneal thickness. In the context of diagnosing glaucoma, a smaller increase in corneal thickness towards the periphery might be a new additional marker

Fluocinolone acetonide intravitreal implant as a therapeutic option for severe Sjögren’s syndrome-related keratopathy: a case report

Abstract Background In this report, we present the results of a severe case of Sjögren’s syndrome-related keratopathy after fluocinolone acetonide 190-μg intravitreal implant (Iluvien®; Alimera Sciences Inc.) therapy. Case presentation A 52-year-old Caucasian woman with Sjögren’s syndrome secondary to autoimmune hepatitis and primary sclerosing cholangitis was admitted to our emergency department owing to bilateral corneal ulcers and corneal perforation in the left eye following exposure keratopathy in an artificially induced coma. Within the following months, recurrent fulminant keratolysis with perforations required multiple penetrating keratoplasties and amniotic membrane transplants in both eyes. With new signs of severe keratolysis, an intravitreal fluocinolone acetonide implant was injected off-label in the left eye, and a third penetrating keratoplasty was performed 2 weeks later. In the 6 months of follow-up after the last penetrating keratoplasty, no more surgical interventions were needed in the eye with the fluocinolone acetonide implant. The corneal surface remained stable, and intraocular pressure was normal. During this time frame, two further penetrating keratoplasties, one vitrectomy, and five amniotic membrane transplants were performed in the fellow eye owing to relapsing keratolysis and perforations. Conclusions To the best of our knowledge, this is the first report of fluocinolone acetonide intravitreal therapy in a patient with corneal disease. In the 6-month follow-up period, no surgical intervention was needed in the eye with the fluocinolone acetonide implant, whereas further penetrating keratoplasties and amniotic membrane transplants were performed in the fellow eye. Intravitreal fluocinolone acetonide may be considered as a treatment option in severe cases of autoimmune corneal disease