Lateral Canthal Sling Procedure for Meibomian Gland Dysfunction? Results of a Pilot Study.

Background: Involutional changes of lid structures often induce horizontal lid laxity; this can result in a reduction of Meibomian gland expression, potentially leading to symptoms of dry eye. The aim of this study was to evaluate the effect of tightening the lower eyelid via a lateral canthal sling (LCS) procedure on dry eye parameters.Methods: Patients with Meibomian Gland Dysfunction (MGD), lower lid laxity (positive Snap-back Test and positive Pinch Test) and no previous lid surgery were evaluated before and 3 months after LCS procedure for symptoms by OSDI. The fellow eye without surgery functioned as a control group. MGD parameters included lipid layer thickness (LLT), non-invasive breakup time (NIBUT), tear meniscus height, loss of Meibomian glands, lid margin parallel conjunctival folds (LIPCOFs), Schirmer's test, the number of expressible Meibomian glands as well as quality of Meibum.Results: Fourteen patients (8 men and 6 women; 79.2 ± 4.0 years) were enrolled in this prospective clinical study. After 3 months, the OSDI showed a significant reduction (preop 42.9 ± 24.7; postop 23.8 ± 21.6; p = .002); NIBUT (5.5 ± 2.6 s to 9.9 ± 6.8 s p = .08) and LLT (64.3 ± 30.4 to 74.1 ± 27.8; p = .025) improved, while Schirmer Test (15.3 ± 4.7 mm to 11.9 ± 2.9 mm; p = .03) and tear meniscus height were reduced (0.8 ± 0.3 to 0.6 ± 0.2; p = .05). Meibomian gland loss scored by the meiboscale slightly increased postoperatively (1.2 ± 0.9 to 1.4 ± 0.9; p = .18). The number of expressible Meibomian glands improved (4.4 ± 2.6 to 6.8 ± 2.1, p = .002) as well as the quality of Meibum (0.9 ± 1.0 to 0.5 ± 0.8, p = .04). Snap back test as well the pinch test were negative in all patients postoperatively.Conclusion: Addressing lower lid laxity with an LCS procedure simultaneously enhances tear drainage, reduces tear film volume parameters and increases tear film stability results with an improvement of dry eye symptoms. It is likely that increased lower eyelid tension and thus excretory pressure on the Meibomian glands is responsible for these alterations.

Gender-Specific Differences in Signs and Symptoms of Dry Eye Disease.

INTRODUCTION: Dry eye disease (DED) is a multifactorial disease of the ocular surface characterized by loss of homeostasis of the tear film. Epidemiological studies suggest it occurs more frequently in women than men. The aim of this retrospective analysis was to determine whether patients with DED show gender-specific differences in symptoms and signs. MATERIAL AND METHODS: A retrospective analysis of 107 patients was conducted at the Department of Ophthalmology of the University of Düsseldorf - Germany. After completing the OSDI questionnaire, the patients underwent objective refraction and were asked about the following symptoms: burning, itching, foreign body sensation, epiphora, sticky eyes, pain, red eyes and swollen eyelids. Furthermore, they underwent an evaluation of the following signs: lipid layer thickness, non-invasive break-up-time (NI-BUT), conjunctival hyperemia, lid parallel conjunctival folds, meibography, Schirmer test and tear meniscus height, ocular surface staining, expressibility of meibomian glands. RESULTS: Of the 107 patients (56.2 ± 17.3 years) 75 were women and 32 men. Women reported significantly more often eyelid swelling (p = .03) and showed a tendency to complain more about red eyes (p = .051), while men tended to complain more often about epiphora (p = .053). In an age-matched sample taken from the cohort, significant differences were found for OSDI-scores (p = .025) showing women reporting more symptoms compared to men, while men showed more conjunctival hyperaemia (p = .004) than women. Women showed a significant thicker lipid layer (p = .0009) in the full cohort, but not in the age-matched sample (p = .43). CONCLUSION: Although the majority of the investigated parameters did not show gender-specific differences, women reported higher OSDI scores. These findings could be explained by an increased frequency of neuropathic symptoms or ocular surface sensitivity in women or higher resilience of men to consult an ophthalmologist. Sex may have an effect on the clinical characteristics of DED, but further studies are needed to confirm these findings.

[Epithelial Dystrophies of the Cornea]. / Epitheliale Dystrophien der Hornhaut.

In 2015, the first revision of the international classification of corneal dystrophies (IC3D) has been published. According to this latest version of the IC3D the dystrophies of the cornea are divided into · epithelial and subepithelial dystrophies,. · epithelial-stromal TGFBI dystrophies,. · stromal dystrophies, and. · Descemet-membrane and endothelial dystrophies.. This article summarizes the epithelial and subepithelial dystrophies of the cornea, which, according to IC3D are the following: · epithelial basement membrane dystrophy (EBMD),. · epithelial recurrent erosion dystrophy (ERED),. · subepithelial mucinous corneal dystrophy (SMCD),. · Meesmann corneal dystrophy (MECD),. · Lisch epithelial corneal dystrophy (LECD),. · gelatinous drop-like corneal dystrophy (GDLD).. The main problem concerning almost all dystrophies of the corneal epithelium are epithelial defects (erosion) associated with pain, epiphora and red eyes. In addition, all dystrophies of the epithelium tend to relapse.While therapy is usually initiated with topical therapeutics, in the course of the disease invasive procedures like phototherapeutic keratectomy (PTK) (possibly with the administration of mitomycin C) or in severe cases even keratoplasty (preferably as deep anterior lamellar keratoplasty; DALK) have to be used. Due to the origin of the disease in the epithelial stem cells at the limbus, the replacement of these cells can also be discussed.

[Recurrent Corneal Erosions in Epithelial Corneal Dystrophies]. / Rezidivierende Hornhauterosion bei epithelialen Hornhautdystrophien.

The corneal epithelium is the most important structure of the ocular optical system. Recurrent corneal erosions can result from inflammation, trauma, degeneration and dystrophies. Epithelial basement membrane dystrophy (EBMD), epithelial recurrent erosion dystrophy (ERED) and Francheschetti and Meesmann's epithelial corneal dystrophy (MECD) can all - besides other signs and symptoms - result in more or less frequent corneal erosions. The pathomechanisms involved however are different. In EBMD, corneal erosions are facultative and clinical signs are often subtle. Aberrant basement membrane structures are associated with thinning of the epithelium and can be clinically identified as maps or fingerprints. In ERED, recurrent corneal erosions are - predominantly in the first decades of life - always present. A defect in the COL17A1 gene results in a dysfunctional hemidesmosome. In MECD, punctate corneal erosions are less frequent and result from intraepithelial microcysts which open spontaneously onto the ocular surface. Usually lubricants, therapeutic contact lenses and sometimes epithelial debridement and phototherapeutic keratectomy are the mainstay for treating corneal erosions in these three dystrophies.

Optical coherence tomography for the diagnosis and monitoring of idiopathic intracranial hypertension.

The objectives of the study were to investigate the value of optical coherence tomography in detecting papilledema in patients with idiopathic intracranial hypertension (IIH), a disease which is difficult to monitor and which can lead to permanent visual deficits; to analyze retinal changes over time. In this non-interventional case-control study, spectral-domain optical coherence tomography (SD-OCT) was used to analyze the retinal and optic nerve head (ONH) morphology of 21 patients with IIH and 27 age- and sex-matched healthy controls over time. We analyzed the ONH volume using a custom-made algorithm and employed semi-automated segmentation of macular volume scans to assess the macular retinal nerve fiber layer (RNFL) and ganglion cell layer and inner plexiform layer complex as well as the total macular volume. In IIH patients, the ONH volume was increased and correlated with cerebrospinal fluid (CSF) pressure. The ONH volume decreased after the initiation of treatment with acetazolamide. The macular RNFL volume decreased by 5% in 3.5 months, and a stepwise multivariate regression analysis identified CSF pressure as the main influence on macular RNFL volume at diagnosis. The only factor predicting macular RNFL volume loss over time was ONH volume. SD-OCT can non-invasively monitor changes in retinal and ONH morphology in patients with IIH. Increased ONH volume leads to retinal atrophy in the form of macular RNFL volume loss, presumably due to mechanic jamming of the optic nerve at the disc and subsequent axonal loss.

Alterations of the outer retina in non-arteritic anterior ischaemic optic neuropathy detected using spectral-domain optical coherence tomography.

BACKGROUND: A characteristic disease pattern may be reflected by retinal layer thickness changes in non-arteritic anterior ischaemic optic neuropathy measured using spectraldomain optical coherence tomography. Retinal layer segmentation is enabled by advanced software. In this study, retinal layer thicknesses in acute and chronic non-arteritic anterior ischaemic optic neuropathy were compared. DESIGN: A single-centre cross-sectional analysis was used. PARTICIPANTS: A total of 27 patients (20 age-matched healthy eyes) were included: 14 with acute (<7 days) and 13 patients with chronic non-arteritic anterior ischaemic optic neuropathy. METHODS: Macular volume and 12° peripapillary ring optical coherence tomography scans were used. MAIN OUTCOME MEASURES: The peripapillary thicknesses of the following layers were determined by manual segmentation: retinal nerve fibres, ganglion cells + inner plexiform layer, inner nuclear layer + outer plexiform layer, outer nuclear layer + inner segments of the photoreceptors and outer segments of the photoreceptors to Bruch's membrane. Macular retinal layer thicknesses were automatically determined in volume cubes centred on the fovea. RESULTS: Peripapillary retinal swelling in acute nonarteritic anterior ischaemic optic neuropathy was attributable to retinal nerve fibre layer, ganglion cell layer/inner plexiform layer and outer nuclear layer/segments of the photoreceptors thickening. In chronic cases, peripapillary retinal nerve fibre layer, macular ganglion cell layer and inner plexiform layer thinning were observed. CONCLUSIONS: In acute non-arteritic anterior ischaemic optic neuropathy, the inner and outer peripapillary retinal layers are affected by thickness changes. In chronic cases, atrophy of the ganglion cells and their axons and dendrites is evident by inner retinal layer thinning.

A novel C8orf37 splice mutation and genotype-phenotype correlation for cone-rod dystrophy.

BACKGROUND: To identify the disease-causing mutation in a consanguineous family of Morrocan origin with syndromic autosomal recessive (ar) cone-rod dystrophy (CRD) in two patients and describe genotype-phenotype correlations. MATERIALS AND METHODS: Genome-wide homozygosity mapping and direct sequencing of C8orf37, located in a homozygous interval, was performed in the family. mRNA analysis revealed the effect of the newly identified splice-site mutation. For a comparative analysis phenotypic and genetic data of C8orf37 mutations were extracted from published cases. RESULTS: The new splice-site mutation c.155+2T>C identified in the family results in a skipping of 82 bp. The CRD phenotypes of our patients were consistent with previous reports. Non-ocular findings in our patients and two previously described patients were postaxial polydactyly present at birth. Both families with additional postaxial polydactyly had splice site mutations affecting intron 1 of C8orf37, one at the slice donor and one at the splice acceptor site. CONCLUSIONS: This report extends the genotypic spectrum of C8orf37-associated retinal dystrophies and demonstrates for the first time a genotype-phenotype correlation between an arCRD-polydactyly-association and truncating germline mutations affecting the N-terminal region of the protein. Furthermore, our findings underline the ciliary function of C8orf37 protein.

Acetazolamide therapy in a case of fingolimod-associated macular edema: early benefits and long-term limitations.

UNLABELLED: Fingolimod is a potent drug in relapsing forms of multiple sclerosis. Visual impairment due to fingolimod-associated macular edema (FAME) usually leads to discontinuation of fingolimod therapy. METHODS: We report on a 24-year old woman with bilateral FAME. RESULTS: We continued fingolimod and added oral acetazolamide, which led to recovery of visual acuity and regression of macular edema. However, fingolimod had to be discontinued when fluorescein angiography revealed an enlarged foveal avascular zone. DISCUSSION AND CONCLUSION: Oral acetazolamide might be a treatment option for FAME, while ischemic conversion may be limiting. Ophthalmologic assessments are mandatory for follow-up when fingolimod therapy is continued after onset of FAME.

Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography.

OBJECTIVE: The aim of this non-interventional study was to characterize retinal layer pathology in Susac syndrome (SuS), a disease with presumably autoimmune-mediated microvessel occlusions in the retina, brain, and inner ear, in comparison to the most important differential diagnosis multiple sclerosis (MS). METHODS: Seventeen patients with SuS and 17 age- and sex-matched patients with relapsing-remitting MS (RRMS) and healthy controls (HC) were prospectively investigated by spectral-domain optical coherence tomography (OCT) including intraretinal layer segmentation in a multicenter study. Patients with SuS additionally received retinal fluorescein angiography (FA) and automated perimetry. RESULTS: Patchy thinning of the retinal nerve fiber layer, ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer plexiform layer compared to corresponding sectors in RRMS and HC eyes (p < 0.003 for SuS vs RRMS and HC) was observed in 23/34 (68%) SuS eyes, particularly in temporal quadrants. The outer nuclear layer (ONL) and photoreceptor layers (PRL) were not affected. FA performed in 15/17 patients with SuS was negative for disease-specific branch retinal artery occlusions in all but 1 eye at the time of OCT examination and revealed no additional vascular abnormalities, even in severely damaged OCT areas. In a subset of patients with SuS, associations of visual field data with distinct retinal layers were observed. CONCLUSION: Distinct OCT patterns of scattered, scar-like intraretinal pathology in SuS eyes, sparing the ONL and PRL, suggest a retinal, but not choroidal, vascular pathomechanism and clearly differentiate SuS from RRMS. Depending on the disease stage, OCT and FA provide specific complementary diagnostic information in SuS.

Retinal pathology in idiopathic moyamoya angiopathy detected by optical coherence tomography.

OBJECTIVE: To investigate whether patients with moyamoya angiopathy without obvious retinal pathologies such as retinal infarctions or the congenital morning glory anomaly may have subtle subclinical retinal changes. METHODS: In this cross-sectional study, spectral domain optical coherence tomography was used to analyze the retinal morphology of 25 patients with idiopathic moyamoya angiopathy and 25 age- and sex-matched healthy controls. We analyzed the retinal vasculature with blue laser autofluorescence, lipofuscin deposits with MultiColor confocal scanning laser ophthalmoscopy, and the optic nerve head (ONH) volume with a custom postprocessing algorithm. In addition to the total retinal thickness, semiautomated segmentation was used for segmentation of retinal layers in macular cross scans, macular volume scans, and peripapillary ring scans. RESULTS: The main finding was a pronounced reduction of the ONH volume in moyamoya angiopathy compared with controls (0.76 ± 0.45 mm(3) and 1.47 ± 0.50 mm(3), respectively; p < 0.0001), which was associated with a less pronounced reduction of the retinal nerve fiber layer in macular volume scans (0.97 ± 0.11 mm(3) and 1.10 ± 0.10 mm(3), respectively; p < 0.001). Autofluorescence and MultiColor confocal scanning laser ophthalmoscopy images revealed no pathologies except for one branch retinal artery occlusion. CONCLUSION: Our results indicate that even patients with moyamoya angiopathy who do not have obvious retinal abnormalities have retinal abnormalities. These can be detected by spectral domain optical coherence tomography, and the association of ONH abnormalities with the vascular changes may suggest that idiopathic moyamoya angiography is a systemic disease involving abnormalities of the early mesodermal development.

Comparison of two different scheimpflug devices in the detection of keratoconus, regular astigmatism, and healthy corneas.

Aim. The aim of this study was to determine the intra- and interobserver variability of two Scheimpflug based camera systems, Pentacam and Sirius. In addition, the comparability of the measurements was tested in healthy subjects, subjects with regular astigmatism, and keratoconus patients. Methods. Intra- and interobserver variability were assessed in 20 healthy corneas. Pachymetry values were also compared with ultrasound pachymetry as a reference measurement. To detect possible differences between the two devices, 82 eyes with clinically established keratoconus, 30 eyes with regular astigmatism (>1.5 D), and 60 eyes without corneal pathologies were included in this prospective study. Results. Pachymetry and keratometry showed good intra- and interobserver variability for both devices. Pachymetry values obtained with the Sirius system (579 ± 58 µm) were significantly higher compared to the Pentacam system (551 ± 40 µm, P < 0.001) and to ultrasound pachymetry (550 ± 43 µm, P < 0.001). Significant interdevice differences were found regarding the majority of the detected keratometry parameters. Conclusions. Both devices show almost perfect reproducibility in healthy subjects. However, pachymetry and keratometry values of the two devices should not be used interchangeably.

Keratin films for ocular surface reconstruction: evaluation of biocompatibility in an in-vivo model.

Amniotic membrane (AM) transplantation is the clinical standard for ocular surface reconstruction, however recently keratin film (KF) has been proposed as an alternative material. Aim of the current study was to evaluate corneal biocompatibility of KF in a rabbit model. Forty-six New Zealand white rabbits underwent dissection of a corneal intrastromal pocket in which an AM or KF implant was inserted and observed for 10 days and for 4 weeks. Half of animals received topical steroids, while the other half were left without. At the end of the follow-up clinical and histology examinations were performed to evaluate transparency, inflammation and degradation. After 10 days the clinical and the histology results appeared to be comparable in KF implanted eyes treated with and without steroids. After 4 weeks, comparable clinical results were observed in all KF implanted eyes, while the inflammation score was lower in non-steroid compared to steroid treated eyes along with a higher degradation rate of the keratin films. In conclusion, keratin films from human hair show a good biocompatibility and transparency in vivo. The administration of topical steroids seems to slow down implant degradation which might be important for the modulation of tissue integration and matrix regeneration.

Evaluation of Meibomian Gland Dysfunction and Local Distribution of Meibomian Gland Atrophy by Non-contact Infrared Meibography.

AIMS: The main purpose of this study was to investigate the relationship between Meibomian gland atrophy (meiboscore) and Meibomian gland expressibility. In addition, the local distribution of Meibomian gland loss was analyzed. METHODS: A retrospective analysis of 128 patients (92 women and 36 men, 57 ± 17 years) from our dry eye clinic was performed. Infrared meibography was performed using the Keratograph 5 M (Oculus, Wetzlar, Germany) and evaluated with a scoring system introduced by Arita et al. RESULTS: A significant inverse correlation between Meibomian gland atrophy measured by meibography and expressible Meibomian glands (r = -0.197, p = 0.003) as well as between meiboscore and TBUT (r = -0.1615, p = 0.012) was found. There also was a significant correlation between the total meiboscore and the age (r = 0.33, p < 0.0001). We could find a strong and highly significant correlation between the total meiboscore and the individual meiboscore of the upper eyelid (r = 0.905, p < 0.0001) and the lower eyelid (r = 0.892, p < 0.0001). There was no significant difference of Meibomian gland atrophy between the individual thirds of the upper eyelid, but for the lower eyelid, we could find a higher degree of Meibomian gland atrophy in the nasal third compared with the middle and the temporal third (Dunn's post hoc test, p < 0.0001). CONCLUSIONS: Meibomian gland atrophy seems to be not constant over the tarsal plate but the examination of the lower tarsus might be sufficient in most of the cases. The correlation of the meiboscore with functional dry eye parameters suggest that in patients with detectable Meibomian gland atrophy there is also an impaired Meibomian gland function. However, meibography seems not to be sufficient as a single test for the diagnosis of MGD. For the future larger, prospective studies are needed to confirm these results and further evaluate the potential of meibography in the diagnosis of MGD.

Six-month effects of a thermodynamic treatment for MGD and implications of meibomian gland atrophy.

PURPOSE: The aim of this study was to evaluate the 6-month effect of a single automated thermodynamic treatment (LipiFlow) and implications of meibomian gland atrophy on treatment efficacy 6 months after application. METHODS: We analyzed the data of 26 subjects with meibomian gland dysfunction before and 6 months after treatment. Investigated parameters included subjective symptoms, lipid layer thickness, meibomian gland assessment, tear osmolarity, corneal and conjunctival staining, lid margin parallel conjunctival folds, Schirmer test values, bulbar redness, tear meniscus height, meibomian gland atrophy, and noninvasive tear break-up time. RESULTS: Subjective symptoms (mean Ocular Surface Disease Index, 42 ± 19 to 33 ± 21; P = 0.004, mean Standard Patient Evaluation of Eye Dryness 16 ± 7 to 12 ± 7; P = 0.0001), lipid layer thickness (44.0 ± 15.6 to 51.3 ± 20.4; P = 0.014), number of expressible glands (2.9 ± 1.6 to 6.4 ± 4.6; P < 0.0001), lid margin parallel conjunctival folds (2.3 ± 1.0 to 2.0 ± 0.9; P = 0.04), and bulbar redness (1.4 ± 0.5 to 1.2 ± 0.5; P = 0.0001) were all improved 6 months after treatment. Symptomatic improvement was higher in patients with less severe meibomian gland atrophy compared with patients with more dropout at treatment. There was no change of meibomian gland atrophy 6 months after treatment. CONCLUSIONS: In summary, the results showed that a single thermodynamic treatment is effective in the treatment of meibomian gland dysfunction and that the effects last for at least 6 months. We suggest performing meibography in every patient before treatment for better prediction of therapeutic effects.

Evaluation of an automated thermodynamic treatment (LipiFlow®) system for meibomian gland dysfunction: a prospective, randomized, observer-masked trial.

PURPOSE: To compare the effectiveness of a single LipiFlow(®) treatment with combined lid warming and massage in patients with meibomian gland dysfunction (MGD). METHODS: In this prospective, randomized, crossover, observer-masked clinical trial, subjects were randomized to receive either a single 12-min LipiFlow-LipiFlow Thermal Pulsation (LTP) system treatment or to perform combined twice-daily lid warming and massage for 3 months. All subjects were examined before, and 1 and 3 months after initiation of treatments. Investigated parameters included subjective symptoms, lipid layer thickness, meibomian gland assessment, tear break-up time, tear osmolarity, corneal and conjunctival staining, Schirmer test values, and tear meniscus height. RESULTS: A total of 31 subjects completed the 3-month follow-up. At 1 and 3 months, patients in the LipiFlow treatment group had a significant reduction in Ocular Surface Disease Index (OSDI) scores compared with those in the lid-margin hygiene group. Both treatments produced a significant improvement in expressible meibomian glands compared to the baseline parameters, but no significant difference was noted between the two groups. The other investigated objective parameters did not show a significant difference. CONCLUSION: Results of our study show that a single LipiFlow treatment is as least as effective as a 3-month, twice-daily lid margin hygiene regimen for MGD. However, the present study was observer-masked only, and therefore a placebo effect may have confounded any improvements in subjective symptoms and other parameters in both groups.

Subtle retinal pathology in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is characterized by neuro-ophthalmological abnormalities beyond disturbed oculomotor control such as decreased visual acuity and disturbed visual evoked potentials. Here we report retinal alterations in a cohort of 24 patients with clinically definite (n = 20) or probable (n = 4) ALS as compared to matched controls. High-resolution spectral domain optical coherence tomography with retinal segmentation revealed a subtle reduction in the macular thickness and the retinal nerve fiber layer (RNFL) as well as a marked thinning of the inner nuclear layer (INL). Our data indicate an unprecedented retinal damage pattern and suggest neurodegeneration beyond the motor system in this disease.

Evaluation of lipid layer thickness measurement of the tear film as a diagnostic tool for Meibomian gland dysfunction.

PURPOSE: The LipiView interferometer (TearScience Inc, Morrisville, NC) is capable of delivering quantitative values of the tear-film lipid layer thickness (LLT). The purpose of this study was to investigate whether there is a correlation between the LLT and other diagnostic criteria for Meibomian gland dysfunction (MGD). METHODS: We performed a retrospective analysis of 110 patients (199 eyes) from our dry eye clinic. Subjective symptoms, break-up time (BUT), expressible Meibomian glands, and LLT were measured. RESULTS: There was a significant correlation between expressible Meibomian glands and LLT (r = 0.36, P < 0.0001). Also, a possible trend of inverse correlation between subjective symptoms (standard patient evaluation of eye dryness) and the LLT was observed; however, this was not significant (r = -0.13, P < 0.08). Analysis of the whole study collective revealed no correlation between the BUT and the LLT (r = 0.003, P = 0.97). A subgroup analysis of the patients with an LLT ≤ 60 showed a better correlation between the LLT and the BUT, which was marginally not significant (r = 0.21; P = 0.059). For a cut-off value of ≤ 75-nm LLT, we found a sensitivity of 65.8% and a specificity of 63.4% for the detection of an MGD. For a cut-off value of ≤ 60, the sensitivity was 47.9%, and the specificity was 90.2%. CONCLUSIONS: The positive correlation between the LLT and expressible meibomian glands found in this study suggests a higher probability of MGD in patients with a low LLT. This automated assessment of the LLT might be a suitable screening test for detecting MGD. However, further prospective studies are needed to confirm these results and to identify potential confounders.

Retinal neurodegeneration in Wilson's disease revealed by spectral domain optical coherence tomography.

BACKGROUND/OBJECTIVE: In addition to cirrhosis of the liver, Wilson's disease leads to copper accumulation and widespread degeneration of the nervous system. Delayed visual evoked potentials (VEPs) suggest changes to the visual system and potential structural changes of the retina. METHODS: We used the latest generation of spectral domain optical coherence tomography to assess the retinal morphology of 42 patients with Wilson's disease and 76 age- and sex-matched controls. We measured peripapillary retinal nerve fiber layer (RNFL) thickness and total macular thickness and manually segmented all retinal layers in foveal scans of 42 patients with Wilson's disease and 76 age- and sex-matched controls. The results were compared with VEPs and clinical parameters. RESULTS: The mean thickness of the RNFL, paramacular region, retinal ganglion cell/inner plexiform layer and inner nuclear layer was reduced in Wilson's disease. VEPs were altered with delayed N75 and P100 latencies, but the N140 latency and amplitude was unchanged. An analysis of the laboratory parameters indicated that the serum concentrations of copper and caeruloplasmin positively correlated with the thickness of the outer plexiform layer and with N75 and P100 VEP latencies. CONCLUSION: Neuronal degeneration in Wilson's disease involves the retina and changes can be quantified by optical coherence tomography. While the VEPs and the thickness of the outer plexiform layer appear to reflect the current copper metabolism, the thicknesses of the RNFL, ganglion cell/inner plexiform layer, inner nuclear layer and the total paramacular thickness may be the best indicators of chronic neuronal degeneration.