Evaluation of Structural and Functional Retinal Changes in Children With Intermittent Exotropia Versus Healthy Children.

PURPOSE: To detect structural and functional changes in children with intermittent exotropia using optical coherence tomography, pattern electroretinography (ERG), and multifocal ERG. METHODS: The study included 26 patients with intermittent exotropia and 26 healthy individuals matched for age and sex with a mean age of 9.23 and 11.20 years in the intermittent exotropia and control groups, respectively (P = .310). All patients underwent full ophthalmic examination including measurement of the angle of strabismus at near and far, and assessment of the macula and optic nerve using optical coherence tomography, pattern ERG, and multifocal ERG. RESULTS: All patients had 6/6 best corrected visual acuity with no fixation preference. A significantly prolonged P and N wave latency was found in all measured rings of multifocal ERG of patients with intermittent exotropia, P wave amplitude was lower in rings 1 and 5, and N wave amplitude was lower in rings 1 and 2. Regarding pattern ERG, the amplitude of P50 wave was lower and N95 latency was prolonged in the intermittent exotropia group. The upper and lower ganglion cell complex and the superior retinal nerve fiber layer were significantly thinner in the intermittent exotropia group compared to controls. CONCLUSIONS: Children with intermittent exotropia without any associated amblyopia or refractive error showed a subnormal pattern and multifocal ERG response in addition to a thinner ganglion cell layer and retinal nerve fiber layer compared to normal controls. This signifies that subtle structural and functional retinal changes are found in patients with intermittent exotropia. [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XXX-XXX.].

New insights into amiodarone induced retinal and optic nerve toxicity: functional and structural changes.

Background: Amiodarone is widely used for heart arrhytmia. Previous studies have suggested the possibility of optic neuropathy with the chronic use of this drug. Objectives: To identify structural or functional changes in the retina and optic nerve in patients on chronic amiodarone therapy without visual complaints. Methods: This observational study included 15 eyes of 15 patients with cardiac arrythmia on chronic amiodarone treatment and 15 healthy matched subjects as a control group. All subjects underwent electrophysiological tests [pattern visual evoked potential (PVEP), pattern electroretinogram (PERG), multifocal electroretinogram (mfERG), and optical coherence tomography (OCT) and angiography (OCTA)]. Results: There were no statistically significant differences between the two groups regarding the PVEP, PERG, and the mfERG parameters. Macular and optic nerve head OCT and OCTA have not shown statistically significant differences except for the morphological parameters of the optic disc (p = 0.008 for the horizontal and p = 0.013 for vertical cup/disc ratio and p = 0.045 for rim area). Conclusion: Patients on chronic amiodarone therapy have not shown evident structural or functional changes in the retinal or optic nerve as demonstrated by electrophysiological tests, OCT, and OCTA results compared to controls.

Bacillary layer detachment with malignant choroidal tumors: a case series.

PURPOSE: To study the incidence and characteristics of bacillary layer detachment (BALAD) occurring with the two most common choroidal malignancies, choroidal metastasis and choroidal melanoma. METHODS: A retrospective multicentric record analysis. Eyes with a diagnosis of choroidal melanoma or choroidal metastasis that had good-quality fundus photography and spectral domain optical coherence tomography (OCT) scans of the macular and tumor regions allowing for delineation of the retinal layers were included for analysis. Qualitative image evaluation was done by two independent graders for the presence, location, and OCT features of BALAD, as well as any associated intraretinal or subretinal fluid. Demographic and clinical data were also retrieved. RESULTS: Of the 11 eyes with choroidal metastasis and 7 eyes with choroidal melanoma that were included in the final analysis, 6 (54.5%) and 1 (14.3%) had BALAD, respectively. The BALAD co-localized with the subretinal fluid in all cases and with the intraretinal fluid in 1/3 cases (33.3%), was foveal in location in 3 eyes (42.9%), was overlying the tumor in 6 eyes (85.7%), and varied in number and size. Reflectivity within the BALAD was consistently higher than the vitreous and adjacent subretinal fluid, and discernable suspended hyperreflective particles were noted in 5 eyes (71.4%). CONCLUSION: BALAD is relatively common with choroidal metastasis. The OCT features described supplement our recognition of this new entity.

Revisiting the Robustness of Pentacam HR Indices Against Soft Contact Lens Wear.

PURPOSE: To evaluate various selected Pentacam HR indices in a cohort of soft contact lens (SCL) wearers before and after SCL discontinuation. METHODS: Ninety right eyes of 90 SCL wearers (group 1) were compared to healthy controls (group 2). For group 1 eyes, Pentacam HR images were captured within one hour of SCL removal and after one month of discontinuation. The evaluated indices included curvature, elevation, and pachymetric parameters. RESULTS: Elevation indices and two pachymetric indices (central corneal thickness and thinnest corneal thickness) did not show significant differences, either between the two groups or in group 1 after SCL discontinuation. Some pachymetric indices (average and maximum corneal pachymetry progression indices, and maximum Ambrosio's relational thickness index) showed statistically significant (P-value <0.001), yet clinically irrelevant differences. Contrarily, changes in curvature indices were both statistically significant (P-value <0.001) and clinically relevant. CONCLUSION: From a clinical point of view, many elevation and pachymetric indices are robust against SCL wear, and can be relied upon for corneal ectasia screening of SCL wearers, even without abstaining from wearing SCLs.

Evaluation of Corneal Epithelial Thickness and Dry Eye Disease Tests in Thalassemic Adolescents.

PURPOSE: To assess dry eye disease (DED) in thalassemic adolescents by evaluating corneal epithelial thickness (CET) and various dry eye clinical tests and correlate them to tissue iron overload. METHODS: The study included 120 Beta-thalassemia patients (11 to 18 years) and 120 matched controls. CET maps were captured using anterior segment optical coherence tomography. OSDI questionnaire was completed. Dry eye tests included Schirmer test, tear film breakup time (TBUT), and ocular surface staining (OSS) with fluorescein and lissamine green. We recorded serum ferritin level, and liver iron concentration (LIC) measured by magnetic resonance imaging. RESULTS: Superior and inferior CET was thinner, while map standard deviation (MSD) was higher in thalassemics compared to controls (all P <0.001 ). Thalassemic group also showed higher OSDI scores (P <0.001), shorter TBUT (P <0.001 ), and higher OSS grades (P <0.001 ). Both superior and inferior CET was correlated positively with TBUT, and negatively with OSS (all P < 0.001). Serum ferritin and LIC showed negative correlations with CET (superior and inferior, both P< 0.001), positive correlations with MSD, P< 0.001, as well as with TBUT (P< 0.001), OSS (P< 0.001), and OSDI scores (P< 0.001). CONCLUSION: Thalassemic adolescents had thinner CET with higher thickness' variability, shorter TBUT and more marked OSS than controls. Correlation of higher serum ferritin and hepatic iron overload with irregular epithelial thinning and more affected dry eye tests results supports our hypothesis that high tissue iron levels could play a pivotal role in DED pathogenesis in thalassemic patients.

Ganglion Cell Complex Thinning in Young Gaucher Patients: Relation to Prodromal Parkinsonian Markers.

BACKGROUND: Patients with Gaucher disease (GD) have an increased risk for parkinsonism. Retinal thinning has been described in parkinsonism as an early nonmotor feature. Scarce reports have addressed retinal thickness changes in GD. OBJECTIVES: The objectives of this study were to compare ganglion cell complex (GCC) thickness in adolescents and young adults (AYAs) with GD with healthy control subjects, and to correlate it with the presence of parkinsonian features (PFs), clinical prodromal markers of parkinsonism, severity score index (SSI), and glucosylsphingosine (Lyso-GL-1). METHODS: This study included 48 AYAs with GD (11-29 years), 11 with manifest PFs (Group 1) and 37 with no PFs (Group 2), and 48 matched healthy control subjects (Group 3). Age of GD onset, disease duration, medication history, history of constipation, SSI, and hematological assessment were done. Neurocognitive evaluation included Parts I, II, and III of the Unified Parkinson's Disease Rating Scale (UPDRS), Wechsler Adult and Intelligence Scale and Wechsler Intelligence Scale for Children, Beck Depression Inventory (BDI), rapid eye movement sleep behavior disorder (RBD) scale, Munich Parasomnia Screening scale, and the olfactory dysfunction scale. Molecular analyses of the acid GBA gene and Lyso-GL-1 were done. Participants underwent full ophthalmological examination and optical coherence tomography with GCC thickness measurement. RESULTS: GCC was significantly thinner in Group 1 than in Groups 2 and 3 (P < 0.001), whereas no significant difference was found between Groups 2 and 3 (P = 0.977). In addition, a significant interocular GCC thickness difference was found among the studied AYAs with GD (P = 0.007). GCC correlated positively with total intelligence quotient (P < 0.001) and negatively with Lyso-GL-1 (P = 0.019), UPDRS (P = 0.004), and BDI (P = 0.029), but not with SSI (P = 0.874), GD type (P = 0.85), or genotype (P = 0.842). A significant negative relationship was found between GCC thickness and PFs (P = 0.001), parasomnia (P = 0.003), constipation (P = 0.031), RBD (P = 0.044), and hyposmia (P = 0.033). CONCLUSIONS: GCC thinning may be a promising biomarker for central nervous system neurodegeneration that has the potential to monitor early PFs among people with GD. © 2020 International Parkinson and Movement Disorder Society.

Variations in Choroidal and Macular Thickness Maps after Uneventful Phacoemulsification.

OBJECTIVE: To investigate changes in retinal and choroidal thickness maps following uncomplicated phacoemulsification using enhanced depth imaging optical coherence tomography (EDI-OCT). METHODS: A prospective study was done on 66 eyes. EDI-OCT was performed preoperative, 1 week, 1 month, and 3 months postoperative measuring retinal and choroidal thickness at the fovea and the 9 ETDRS subfields. RESULTS: Subfoveal choroidal thickness (SFCT) showed statistically insignificant increase after 1 week ( P = 0.473), but the increase was statistically significant after 1 month (P = 0.014). However, after 3 months, there was non-significant difference from baseline (P = 0.073). Foveal retinal thickness (FT) demonstrated statistically insignificant increase after 1 week (P = 0.094), but statistically significant increase was noted after 1 and 3 months (P = 0.000). CONCLUSION: Uneventful phaco induced statistically significant increases in FT and SFCT from the first postoperative month; however, 3 months postoperative the increase in retinal thickness was maintained but the increase in choroidal thickness became statistically insignificant.

ELECTRORETINOGRAPHIC AND VISUAL-EVOKED POTENTIAL CHANGES IN RELATION TO CHELATION MODALITY IN CHILDREN WITH THALASSEMIA.

PURPOSE: To evaluate possible benefits of using electrophysiological investigations for detecting retinal and visual pathway changes and correlating them with chelation modality in children with thalassemia. METHODS: This study included 60 patients on single oral iron chelator (deferasirox) (Group 1), 60 on deferoxamine chelator (Group 2), and 60 controls (Group 3). Participants underwent full ophthalmologic examination, pattern visual-evoked potential, pattern electroretinogram, and multifocal electroretinogram. RESULTS: Fundus showed no abnormalities. Multifocal electroretinogram mean P1 amplitude showed statistically significant differences in all 5 rings, amplitudes being significantly lower in Groups 1 and 2 than Group 3; moreover, significantly lower in Group 2 than Group 1. There was a statistically significant difference between groups regarding P50 wave latency and N35-P50 of pattern electroretinogram amplitude (P < 0.001 and P < 0.001, respectively). However, there were no statistically significant differences between groups regarding N95 wave of pattern electroretinogram and pattern visual-evoked potential waves' amplitude and latency. Multiple regression analyses illustrated that chelator was the most important determinant for multifocal electroretinogram and P50 parameters. CONCLUSION: Preclinical electrophysiologic changes existed in thalassemics, more obvious in those on deferoxamine. Electrophysiologic studies analysis denotes an early toxic macular insult rather than optic nerve affection. Thus, regular follow-up using multifocal electroretinogram and pattern electroretinogram is recommended.

CORRELATION BETWEEN SUBFOVEAL CHOROIDAL THICKNESS AND FOVEAL THICKNESS IN THALASSEMIC PATIENTS.

PURPOSE: To assess the impact of two iron chelation modalities in thalassemic patients on foveal and subfoveal choroidal thickness. METHODS: The study included 60 ß-thalassemia major patients. They included 30 patients on oral deferasirox after a period of subcutaneous deferoxamine (Group 2) and 30 patients on subcutaneous deferoxamine (Group 3). Thirty age- and sex-matched healthy children were included as a control group (Group 1). All participants underwent a complete ophthalmologic evaluation and Spectral Domain Optical Coherence Tomography. RESULTS: Age, gender, intraocular pressure, best-corrected visual acuity, and refraction were not statistically different between the three studied groups. Ferritin level, pretransfusion hemoglobin, serum iron, and duration of thalassemia were not statistically different between the two thalassemic groups. Foveal thickness in Group 1 (225.15 ± 17.35 µm) was statistically higher than in Group 2 (210.53 ± 21.73 µm) (P < 0.001) and Group 3 (200.15 ± 7.34 µm) (P < 0.001). It was statistically higher in Group 2 than in Group 3 (P = 0.001). Subfoveal choroidal thickness in Group 1 (279.70 ± 32.54 µm) was statistically higher than in Group 2 (255.80 ± 19.20 µm) (P < 0.001) and Group 3 (248.28 ± 20.43 µm) (P < 0.001). It was statistically higher in Group 2 than in Group 3 (P < 0.05). CONCLUSION: Thalassemic patients can develop a significant decrease in foveal thickness because of the inevitable use of chelation therapy. Deferoxamine as a chelating agent can affect foveal thickness more than the oral form (deferasirox).