Unilateral Roth spots as an initial presentation in Essential Thrombocythemia.

PURPOSE: To describe a 25-year-old female patient with unilateral Roth spots as the initial presentation of Essential Thrombocythemia. METHOD: Case report. RESULTS: A 25 -year-old female presented with visual complaints of seeing flashing lights and mild blurred vision in her left eye. Dilated fundus exam revealed intra-retinal hemorrhages with a pattern compatible with Roth spots. Further work up showed thrombocythemia with positive JAK2 (V617F) mutation, confirming the diagnosis of Essential Thrombocythemia (ET). The symptoms and fundus findings resolved in one month after initiation of the treatment with low dose oral aspirin.ET which is defined as repeated platelet count of ≥ 450,000 per microliter without a secondary cause, can present with highly variable symptoms from being asymptomatic to chest pain, cerebral venous sinus thrombosis, retinal vascular occlusion, and granuloma annulare.In the presented case Roth spots hastened the need for more complete workup to determine the systemic cause of this ocular finding, ultimately leading to the diagnosis. CONCLUSION: Finding of Roth spots may warrant a systemic work up to find out the underlying pathologic condition, which in the present case was Essential Thrombocythemia, a potentially life-threatening condition. DECLARATION: Authors declare no conflict of interest.

The efficacy of 2 different phakic intraocular lens implant in keratoconus as an isolated procedure or combined with collagen crosslinking and intra-stromal corneal ring segments: a systematic review and meta-analysis.

PURPOSE: To compare the efficacy of phakic intra-ocular lenses in isolation or in combination with corneal crosslinking (CXL) and/or intra-stromal corneal ring segments (ICRS) in keratoconus. METHODS: Data extracted from the publications meeting the selection. The outcome parameters included mean pre- and post-operative uncorrected distance visual acuity, corrected distance visual acuity (CDVA), sphere and cylinder of refraction and complications. Available data analyzed with Cochrane Review Manager. RESULTS: A total of 23 studies including 464 eyes were included. All the parameters showed significant improvement in all subgroups other than CDVA in ACPIOL + CXL subgroup and cylinder in PIOL + CXL subgroups. There was not a significant difference between PCPIOL and ACPIOL in the outcomes, exception was more improvement of CDVA in "ACPIOL only" than" PCPIOL only" subgroup. CONCLUSION: Both PCPIOLs and ACPIOLs are comparably safe and efficient options in management of KCN and their efficacy significantly improves when combined with CXL/ICRS.

Selective effects of estradiol on human corneal endothelial cells.

Fuchs endothelial corneal dystrophy (FECD) results from genetic and environmental factors triggering mitochondrial and oxidative stress in corneal endothelial cells (CEnCs) leading to CEnC death and corneal opacification. FECD is more common in women than men, but the basis for this observation is unknown. Because FECD is commonly diagnosed around the time of the menopausal transition in women when estrogen levels decrease precipitously, we studied the effects of the potent estrogen,17-ß estradiol (E2) on growth, oxidative stress, and metabolism in primary cultures of human CEnCs (HCEnCs) under conditions of physiologic 2.5% O 2 ([O 2 ] 2.5 ) and under hyperoxic stress ([O 2 ] A : room air + 5% CO 2 ). We hypothesized that E2 would counter the stresses of the hyperoxic environment in HCEnCs. HCEnCs were treated ± 10 nM E2 for 7-10 days at [O 2 ] 2.5 and [O 2 ] A followed by measurements of cell density, viability, reactive oxygen species (ROS), mitochondrial morphology, oxidative DNA damage, ATP levels, mitochondrial respiration (O 2 consumption rate [OCR]), and glycolysis (extracellular acidification rate [ECAR]). There were no significant changes in HCEnC density, viability, ROS levels, oxidative DNA damage, OCR, and ECAR in response to E2 under either O 2 condition. We found that E2 disrupted mitochondrial morphology in HCEnCs from female donors but not male donors at the [O 2 ] A condition. ATP levels were significantly higher at [O 2 ] 2.5 compared to [O 2 ] A in HCEnCs from female donors only, but were not affected by E2. Our findings demonstrate the overall resilience of primary HCEnCs against hyperoxic stress. The selective detrimental effects of hyperoxia and estradiol on HCEnCs from female but not male donors suggests mechanisms of toxicity based upon cell-sex in addition to hormonal environment.

Recurrence of Herpetic Keratitis after COVID-19 Vaccination: A Report of Two Cases.

Background: Recurrence of herpetic keratitis following vaccination has been documented following vaccination with the Zostavax, trivalent flu, hepatitis A, and rabies vaccines. The USFDA and WHO have acknowledged that the novel COVID-19 vaccines similarly have a risk of reactive immunologic-based inflammation, namely, myositis, pericarditis, and Guillain-Barré syndrome. Case Presentation. We present two patients with latent herpetic keratitis who experienced reactivation of keratitis within weeks of COVID-19 vaccination despite prolonged periods of prior latency. A 52-year-old healthy male with no herpes simplex virus (HSV) keratitis recurrences in two years developed visual decline and patchy stromal haze within 24-48 hours of receiving the second Pfizer-BioNTech (COVID-19 BNT162b2) vaccine. A 67-year-old female with chronic neurotrophic keratitis developed her most severe exacerbation of herpes zoster keratitis in over 10 years occurring 2-3 weeks after her first Moderna (mRNA-1273) vaccine, which was later complicated by bacterial superinfection. Conclusions: The COVID-19 vaccines work by generating both adaptive humoral and cellular immune responses in humans, including elevation of anti-spike neutralizing antibody titers, antigen-specific CD4+ and CD8+ T-cell responses, and increased levels of proinflammatory cytokines such as interferon gamma (IFNγ). The general activation of the T-cell-mediated immune response and proinflammatory cytokines such as IFNγ may underlie the role of the COVID vaccines in reactivation of herpetic stromal keratitis and the clinical findings in our reported cases.

Corneal collagen cross-linking in pediatric keratoconus with three protocols: a systematic review and meta-analysis.

BACKGROUND: Keratoconus is a bilateral progressive noninflammatory degenerative disease of the cornea characterized by corneal thinning, irregular astigmatism, and subsequent visual impairment. It has an aggressive course in children. This systematic review evaluates the efficacy of available corneal collagen cross-linking (CXL) protocols for stabilizing the cornea in pediatric patients with keratoconus. METHODS: We searched all peer-reviewed publications from 2000 to 2019 indexed in PubMed, Google Scholars, Web of Science, and Cochrane's Database for the terms keratoconus and cross-linking. The following data were extracted from eligible studies: study design, type of intervention, number of the eyes and mean age of patients for each study, duration of follow-up period, mean pre- and postoperative uncorrected and corrected visual acuity, keratometric and aberrometric indices, were analyzed with RevMan 5.3 software. Intra-and intergroup post hoc analyses of outcome variables were performed using t tests. RESULTS: A total of 28 studies, including 1,300 eyes, were reviewed. In conventional and accelerated epithelium-off techniques, there was a significant improvement in uncorrected and corrected visual acuities. Similarly, the keratometric indices improved significantly after CXL. Uncorrected visual acuity did not alter after CXL using transepithelial method. CONCLUSIONS: Both conventional and accelerated collagen CXL of the cornea are effective therapeutic options in management of keratoconus in children.

Posterior pole retinal thickness distribution pattern in keratoconus.

PURPOSE: To evaluate the pattern of retinal thickness distribution in patients with keratoconus (KCN) and its correlation with disease severity. METHODS: For this cross-sectional cohort study, the study subjects with documented keratoconus and normal eyes were prospectively enrolled. All subjects had anterior segment (Pentacam HR) and posterior segment (Spectralis) imaging. Posterior segment imaging by optical coherence tomography included the posterior pole asymmetry analysis map. Data were analyzed with multiple linear regression models and correlation tests to examine the mean and variance of the measured thickness of the retina and its distribution relative to the presence and severity of KCN. RESULTS: A total of 24 subjects with keratoconus (48 eyes) and 14 normal subjects (28 eyes) enrolled in this study. The posterior pole retinal thickness, both superior and inferior hemifields, as well as the overall retinal thickness in KCN patients was greater than the control group. There was a direct correlation between the overall retinal thickness of the posterior pole and the severity of KCN (R2 = 0.422, P < 0.001). However, the variability of the retinal thickness showed no difference between KCN-afflicted and healthy eyes. CONCLUSION: Although KCN is a disease of the anterior segment of the eye, we found an orderly increase in posterior pole retinal thickness that is correlated with the severity of disease in KCN eyes compared to control. These findings suggest that the retina may maintain some degree of plasticity to respond to the degraded optical system of the eye.

Effect of trabeculectomy on ocular higher-order aberrations in patients with open angle glaucoma.

OBJECTIVE: The aim of this study was to evaluate the effect of trabeculectomy on ocular higher-order aberrations following surgery in a group of patients with open angle glaucoma. METHODS: In this prospective study, patients with primary open angle glaucoma underwent wavefront aberrometry before trabeculectomy with mitomycin-C. Coma-like, spherical-like, and total ocular higher-order aberrations were measured as root mean square values. The measurements were repeated 1 month and 3 months after the procedure. RESULTS: A total of 20 eyes from 20 patients were examined. There was a significant decrease in intraocular pressure following surgery at 1 month and 3 months postoperatively. However, there was no statistically significant change in the spherical equivalent of the eyes. One month after surgery, a significant change in coma-like, spherical-like, and total higher-order aberrations of the eyes was observed. However, the repeated measurements performed 3 months after procedure revealed no significant difference compared to the baseline values. CONCLUSION: Ocular higher-order aberrations increased 1 month following trabeculectomy surgery. However, this effect seems to be transient, as the values returned to the preoperative measurement 3 months after the procedure.

Characteristics and clinical presentations of pediatric retinoblastoma in North-western Iran.

AIM: To report the characteristics and clinical presentations of retinoblastoma in a series of pediatric patients from Iran. METHODS: In this retrospective study, profiles of pediatric patients with retinoblastoma archived in a referral eye hospital in north-west of Iran during 7 years (n=40 patients with 57 eyes) were reviewed. Demographics, as well as the laterality, clinical manifestations and the types of treatment were the major endpoints. The Student's t test, Mann-Whitney U-test, Chi-square or Fishers' Exact test was used for analysis where appropriate. RESULTS: There were 23 cases (57.7%) with unilateral and 17 cases (42.5%) with bilateral involvement. The male to female ratio was 1 to 1.4 with a mean admitting age of 24.0±11.3 (range: 5-62) months. The mean diagnosis delay was 7.4±9.6 months (range: 10 days to 13 months). The most common presenting sign was leukocoria (97.5%) followed by proptosis (7.5%), strabismus (7.5%), hyphema (5%), orbital cellulitis (5%) and glaucoma (2.5%). Enucleation was performed in 95.7% of the cases with unilateral involvement and at least one eye of the patients with bilateral disease. A second enucleation was performed in other 3 patients (17.6%) of the latter group. CONCLUSION: This is the first study evaluating a series of Iranian children with retinoblastoma.