Eplerenone for the Treatment of Chronic Recurrent Chorioretinopathy Centralis Serosa. / Eplerenon zur Behandlung der chronisch rezidivierenden Chorioretinopathia centralis serosa.

BACKGROUND: Chorioretinopathy centralis serosa (CCS) is a common disease that leads to the loss of retinal ion/fluid homeostasis due to decompensation of the retinal pigment epithelium, resulting in fluid accumulation with detachment of the neurosensory retina and/or retinal pigment epithelium. We investigated the effect of eplerenone, a mineralocorticoid receptor inhibitor, on chronic recurrent CCS (cr-CCS). METHODS: A retrospective study with data analysis of 17 eyes (12 patients) between 2014 - 2021 with cr-CCS in whom other methods were not effective, not applicable, or not desired, was performed. Duration of CCS was at least 12 months with at least one recurrence. Each patient received 25 mg (1st week) and 50 mg (from the 2nd week) for at least 3 months. In each case, best-corrected visual acuity and central and peripheral retinal thickness were measured by spectral-domain optical coherence tomography. Side effects were rated by the patients as "none", "mild" (noticeable but not affecting), "tolerable" (affecting but acceptable because of good effect), and "not tolerable" (then discontinuation of therapy). RESULTS: There was no significant reduction in central or peripheral retinal thickness. A complete reduction of subretinal fluid was achieved in 5 of 17 eyes (29.4%). In 12 eyes, no effect of eplerenone could be detected, so other therapies were required. Visual acuity change was mainly dependent on the duration of CCS and the degree of photoreceptor damage. Side effects were reported by 11 patients as "none" and 1 patient as "mild" (arterial hypotension). No other side effects were observed. CONCLUSION: The response rates of therapy with eplerenone were relatively low, and no significant effect could be demonstrated. Eplerenone should not (no longer) be routinely used in the therapy of cr-CCS.

Current diagnosis and treatment practice of central retinal artery occlusion: results from a survey among German stroke units.

BACKGROUND: Central retinal artery occlusion (CRAO) is a neuro-ophthalmological emergency whose optimal management is still under debate and due to the absence of definite guidelines, practice is expected to vary. We aimed to characterize early evaluation as well as acute treatment and diagnostic approaches in German hospitals with a stroke unit (SU). METHODS: In 07/2021, all 335 certified German SUs were invited to participate in an anonymous online survey endorsed by the German Stroke Society on emergency department care organization, diagnostic procedures, and treatment of patients with unilateral vision loss (UVL) subsequently diagnosed with CRAO. RESULTS: One hundred and sixty-three (48.6%) of the 335 eligible centers responded. Most (117/135; 86.7%) stated that UVL patients were treated as an emergency, in 62/138 (44.9%) hospitals according to specific guidelines. First-line evaluation was performed by neurologists in 85/136 (62.5%) hospitals, by ophthalmologists in 43/136 (31.6%) hospitals. Seventy of 135 (51.9%) respondents indicated a lack of on-site ophthalmological expertise. Seventy-four of 129 (57.4%) respondents performed thrombolysis in CRAO and 92/97 (94.8%) stated that patients with CRAO-if admitted to neurology-were treated on a SU. CONCLUSIONS: Our findings reflect notable heterogeneity in early intrahospital care of CRAO in German SUs but demonstrate a preference for work-up and management as acute stroke by the involved neurologists. Streamlining interdisciplinary emergency evaluation is essential for ongoing and future prospective trials.

Early Onset of Neovascular Glaucoma After Intra-Arterial Thrombolysis for Central Retinal Artery Occlusion: A Possible Complication?

Purpose: To report on four cases of central retinal artery occlusion (CRAO) treated with intra-arterial thrombolysis with early onset neovascular glaucoma in the further disease course. Patients and Methods: Retrospective analysis of the medical records of six consecutive patients treated with intra-arterial thrombolysis for CRAO of which four developed neovascular glaucoma. Results: All six patients were diagnosed with acute CRAO and treated with intra-arterial thrombolysis 4.5-6 hours after symptom onset. The patients had no significant carotid artery stenosis and unremarkable ophthalmic history. No visual improvement could be achieved after treatment. Four to seven weeks after CRAO onset, four of these patients developed severe painful neovascular glaucoma. Conclusion: Early onset of aggressive neovascular glaucoma following intra-arterial thrombolysis for CRAO might be a complication of CRAO itself, still possible association with intra-arterial thrombolysis in our patients should be discussed.

Foveal crack sign as a predictive biomarker for development of macular hole in fellow eyes of patients with full-thickness macular holes.

To investigate the prevalence and predictive value of the foveal crack sign (FCS) in fellow eyes of patients with full-thickness macular holes (FTMH) regarding future macular hole (MH) formation. In a retrospective observational case series, 113 fellow eyes of 113 patients with FTMH have been observed during a mean follow-up time of 21 months. According to baseline SD-OCT images, patients were divided into 4 separate groups: patients with FCS and vitreous adhesion, patients with FCS and vitreous detachment, patients without FCS with vitreous adhesion, patients without FCS with vitreous detachment. Progression rate to MH formation, predictive value of FCS and of vitreous interface status were calculated and compared across the four groups. FCS was observed in 19 of 113 fellow eyes (17%) of patients with FTMH, 10 of them with progression to MH during the mean follow up time of 21 months. 2 other eyes with progression to MH showed no FCS at baseline. Progression rate was shown to be 77% (10 of 13 eyes) in patients with FCS and vitreous adhesion, 0% (none of 6 eyes) in patients with FCS and vitreous detachment, 4% (2 of 48 eyes) in patients without FCS with vitreous adhesion, 0% (none of 46 eyes) in patients without FCS with vitreous detachment. FCS had sensitivity of 83.3% (95% CI 50.9-97.1%) and specificity of 91.1% (95% CI 83.3-95.6%) in predicting MH formation, positive predictive value of FCS was 52.6% (95% CI 29.5-74.8%) and negative predictive value 97.9% (95% CI 91.8-99.6%). Having simultaneously FCS and vitreous adhesion showed 83.3% (95% CI 50.9-97.1%) sensitivity and 97.1% (95% CI 91.1-99.2%) specificity in predicting macular hole formation; positive predictive value was 76.9% (95% CI 46.0-93.8%) and negative predictive value was 98.0% (95% CI 92.4-99.7%). Fellow eyes of patients with FTMH with foveal crack sign are at a very high risk (77%) of FTMH development, as long as posterior vitreous adhesion is present.

Hyperreflectivity of Inner Retinal Layers as a Quantitative Parameter of Ischemic Damage in Acute Retinal Vein Occlusion (RVO): An Optical Coherence Tomography Study.

PURPOSE: To investigate the reflectivity changes of inner retinal layers in acute retinal vein occlusion (RVO) on spectral-domain optical coherence tomography (SD-OCT) and to correlate these values with other known parameters of acute ischemic damage. PATIENTS AND METHODS: In this retrospective and observational case series, 230 eyes from 115 patients with acute RVO (central or branch) were categorized as ischemic or non-ischemic depending on fluorescein angiography (FA) images at baseline. Thickness and reflectivity of selected retinal layers were measured from SD-OCT images at baseline. Reflectivity values were correlated with other parameters of acute ischemic damage (best-corrected visual acuity (BCVA), retinal thickness, extent of macular edema, ischemic area on fluorescein angiography). The data were compared with contralateral eyes (controls). Prominent middle limiting membrane sign (p-MLM) was also registered. RESULTS: RVO reflectivity values differed significantly in all retinal layers compared to controls (P<0.001). Ischemic RVO eyes had higher optical intensity values for the innermost retinal layer (IMRL; P=0.008) and inner retinal layer (P=0.019) compared to non-ischemic cases. For all RVO eyes as well as central RVO, severity parameters like BCVA, central and total retinal thickness showed a strong correlation with the IMRL reflectivity. In branch RVO, BCVA remained significantly correlated with the IMRL reflectivity, while the thickness values showed significant correlation only for central foveal thickness in non-ischemic branch RVO type. The p-MLM was seen on OCT in 94% of the ischemic and in 66% of the non-ischemic RVO cases. CONCLUSION: Acute RVO leads to increased reflectivity of inner retinal layers with significantly higher values in the ischemic vs non-ischemic type. Increased inner retinal layers' reflectivity correlated significantly with BCVA, retinal thickness of separate retinal layers, as well as ischemic area on FA. Quantitative non-invasive measurement of inner retinal layers' reflectivity might be used to determine the extent of acute ischemic retinal damage in RVO.

ACUTE RETINAL ISCHEMIA IN CENTRAL VERSUS BRANCH RETINAL ARTERY OCCLUSION: Changes in Retinal Layers' Thickness on Spectral-Domain Optical Coherence Tomography in Different Grades of Retinal Ischemia.

PURPOSE: To compare changes in thickness of separate retinal layers between acute central and branch retinal artery occlusion (RAO) regarding the severity grade of retinal ischemia using spectral-domain optical coherence tomography. METHODS: Design: retrospective, observational case-control series. SETTING: institutional. PATIENTS: 114 patients with acute RAO. Examinations were conducted with spectral-domain optical coherence tomography at the first visit (baseline). OBSERVATIONS: RAO was categorized as branch (43 patients) or central RAO (71 patients) clinically. Retinal artery occlusion was categorized into the 3 grades "incomplete," "subtotal," and "total," based on optical coherence tomography findings. Thickness of several retinal layers was evaluated on spectral-domain optical coherence tomography images. Obtained data were compared between the 3 grades and between central and branch RAO. The contralateral eyes were used as controls. MAIN OUTCOME MEASURES: thickness of selected retinal layers. RESULTS: Thickness of selected inner retinal layers and central foveal thickness increased statistically significant according to the severity grade (healthy = incomplete < subtotal < total). In each severity grade, there was no significant difference in retinal layers' thickness between branch and central RAO, except for central foveal thickness. CONCLUSION: Thickness increase of selected retinal layers follows-except for central foveal thickness-the same pattern in central and branch RAO. Retinal layers' thickness increases significantly with the higher ischemia grade in both central and branch RAO.

Central retinal venous pressure is higher than intraocular pressure during amateur trumpet playing.

BACKGROUND: It has been shown in the literature that the Valsalva manoeuvre influences ocular perfusion by changing intraocular pressure and central retinal venous pressure (CRVP). High-resistance wind instrument (HRWI) playing is a common situation resembling a Valsalva manoeuvre. The aim of this investigation was to explore the influence of amateur trumpet playing on CRVP. METHODS: The left eyes of 20 healthy non-professional trumpet players (median age 26, range 19-52 years; 17 males, 3 females) were included in this investigation. Subjects, sitting at a slit lamp, were asked to play the tone b' flat with their own mouthpiece on the same trumpet for at least 30 s with moderate loudness. The following data were obtained: intraocular pressure (IOP) by applanation tonometry before and during playing, CRVP by contact lens dynamometry before and during playing, airway pressure (AirP) using a pressure sensor during playing and blood pressure and heart rate using the common cuff method before and during playing. RESULTS: The results are presented as the medians before vs during playing: a calculated mean ophthalmic artery pressure of 66 vs 72 mmHg, heart rate of 76 vs 82 beats per minute, airway pressure of 0 vs 17 mmHg, IOP 12 vs 13 mmHg and CRVP of 24 vs 55 mmHg (Wilcoxon test: p = 0.00009), respectively. A correlation between the CRVP during playing and the height of the spontaneous CRVP is noted (Spearman rank correlation coefficient: ρ = 0.68). CONCLUSIONS: Amateur trumpet playing increases CRVP, airway pressure and IOP. The increase in CRVP is greater than that of the intraocular pressure. The increase in CRVP seems to be more important for retinal perfusion changes during trumpet playing than the increase of IOP. It can be hypothesised that high airway pressure during playing may cause a permanent increase in CRVP, at least in a subgroup of trumpet players.

Hair cortisol concentration in patients with active central serous chorioretinopathy is elevated - a pilot study.

PURPOSE: To investigate hair cortisol concentration (HCC), a biochemical correlate of long-term cortisol output patterns, and its relationship to active central serous chorioretinopathy (CSC). METHODS: Twenty-six participants were included in this observational pilot study (11 patients with active CSC and 15 healthy controls). Hair cortisol concentrations (HCCs) were determined from 3 cm hair strands collected near the scalp from patients and controls as an index of cumulative cortisol secretion over the 3-month period prior to hair sampling. RESULTS: Patients with CSC exhibited higher HCCs (mean value: 20.14, 95% CI: 14.89-27.16 pg/mg) than healthy controls (mean value: 11.06, 95% CI: 8.63-14.22 pg/mg, p = 0.008). Group differences were not affected by relevant covariates (BMI, smoking status, sex). CONCLUSION: Patients with active CSC have increased HCC, supporting the fact that cortisol is a major player in CSC pathogenesis.

Rapid progressive visual decline and visual field defects in two patients with the Heidenhain variant of Creutzfeld-Jakob disease.

BACKGROUND: Heidenhain variant of Creutzfeldt-Jakob (HvCJD) is a rare disease, patients presenting with loss of visual acuity and a decline in visual fields. CASE PRESENTATION: Two patients with rapid loss of visual acuity and declining visual fields presented with homonymic hemianopsia over several weeks. Cranial MRI showed neither stroke nor other morphological changes explaining the severe visual field defects. Neurological examination revealed no pathologies. However, lumbar puncture showed an increase in total protein in cerebrospinal fluid (CSF). Visual field testing revealed further deterioration during follow-up. Several weeks later, patients' behaviour changed markedly, exhibiting aggression, declining memory function and physical degeneration. The suspected diagnosis was the Heidenhain variant of Creutzfeld-Jakob disease (HvCJD). CSF analysis showed evidence of PrPSc and 14-3-3 protein. Both patients died within 8 weeks of the CJD diagnosis. CONCLUSIONS: Loss of visual acuity and a decline in visual fields without corresponding MRI findings and marked changes in behaviour should lead to a diagnosis of HvCJD. Corresponding diagnostic tests should be performed for confirmation. The prognosis for survival is poor and should be immediately communicated to affected patients and their relatives.

Bilateral vision loss due to Leber's hereditary optic neuropathy after long-term alcohol, nicotine and drug abuse.

PURPOSE: Leber's hereditary optic neuropathy is relatively rare, and no clinical pathognomonic signs exist. We present a rare case of bilateral vision loss of a patient with multiple drug abuse in the history. OBSERVATION: A 31-year-old man presented with a history of progressive, decreased vision in both eyes for 6 month. On examination, his visual acuity was hand motion in both eyes. Funduscopy demonstrated a temporal pallor of the optic disc. Goldmann visual field perimetry showed a crescent visual field in the right eye and a circular decrease to less than 50 ° in the left eye. Electroretinogram showed a scotopic b-wave amplitude reduction. Optical coherence tomographies, Heidelberg Retina tomography, visual evoked potentials, and magnetic resonance imaging with contrast as well as blood tests were normal. The patient reported to consume various kinds of drugs as well as recreational drug use and alcohol consumption since he was 16 years old. We started a hemodilution therapy, believing the patient suffered from a bilateral, toxic optic neuropathy due to his lifestyle. Laboratory results later on showed Leber's hereditary optic neuropathy. CONCLUSION AND IMPORTANCE: Leber's hereditary optic neuropathy is a rare disease without a typical, pathognomonic presentation. Even though the patient gave good reasons for a toxic optic neuropathy, one should never stop to test for other diseases.

Retinal venous pressure is higher than the airway pressure and the intraocular pressure during the Valsalva manoeuvre.

PURPOSE: The aims of this prospective experimental study were to explore the influence of the Valsalva manoeuvre (VM) on retinal venous pressure (RVP) in human volunteers in a university setting and to establish correlations for RVP with the increase in airway pressure (∆AirP) and in intraocular pressure (∆IOP). METHODS: In total, 31 healthy young volunteers (age: 24 ± 1.7 years) were investigated. The instruments used included a dynamic contour tonometer, a contact lens dynamometer (Imedos) and an electronic pressure transducer for measuring airway pressure. The following measurements were successively performed in left eyes: tonometry, dynamometry, repeated simultaneous dynamometry and airway pressure measurement during the VM and tonometry during the VM. The pressures obtained during the VM were determined at 10, 20 and 30 seconds after onset of the VM by linear interpolation. RESULTS: The pressures (in mmHg) at baseline and during the VM (median and range with outliers) were as follows: ∆AirP: 10 seconds: 10.0 (7.5); 20 seconds: 12.5 (11.0); and 30 seconds: 11.0 (10.0); and RVP: Start: 17.1 (2.4); 10 seconds: 26.0 (7.5); 20 seconds: 25.0 (6.5); and 30 seconds: 24.0 (6.0). During the VM, the RVP was significantly increased compared with the ∆AirP (p = 0.0017). The IOP during the VM was 13.5 (2.7), and the increase in IOP (∆IOP) was 0.8 (5.6). CONCLUSION: During the VM, the RVP was increased compared with the ∆AirP. The increase in RVP (∆RVP) was significantly greater than the ∆IOP. During the VM, the calculated retinal perfusion pressure may be more strongly reduced by the ∆RVP than by the ∆IOP. These properties may influence retinal and optic nerve head pathophysiology.

[Results of Re-switch from Intravitreal Aflibercept to Ranibizumab in Patients with Exudative Age-related Macular Degeneration]. / Resultate nach Rückumstellung von intravitrealem Aflibercept auf Ranibizumab bei persistierender Aktivität einer altersbedingten Makuladegeneration.

BACKGROUND: The purpose of this study was to investigate the effectiveness of re-switch from intravitreal aflibercept to ranibizumab in patients with exudative age-related macular degeneration. MATERIALS AND METHODS: This retrospective case series included 17 eyes of 17 patients who had previously switched from ranibizumab to aflibercept and finally back to ranibizumab. Main outcomes were change of visual acuity (VA) and assessment of central macular thickness (CMT). Secondary outcomes included predictive factors which had a beneficial effect as VA and CMT before re-switch, number of previous injections and gender. RESULTS: The mean VA was 0.64 ± 0.36 logMAR before the switch, and 0.87 ± 0.40 logMAR before the re-switch, and gained with a slight but not significantly improvement up to 0.85 ± 0.58 logMAR after the re-switch (p = 0.896). The average CMT before the switch was 448.6 µm ± 181.5. This decreased to 343.8 µm ± 161.3 after the switch (p = 0.614) to 299.1 µm ± 155.8 at switchback (p = 0.133). Overall, 8 patients (47%) had an improvement of vision, whereas in 5 patients (30%) VA deteriorated. Further analysis of predictive factors revealed a mean improvement of VA in male patients after re-switch, while female patients lost VA, with statistical significance between after the switch and after the re-switch to the benefit of male patients (p = 0.016). CONCLUSIONS: A re-switch from aflibercept to ranibizumab may enable improvement in morphological parameters and stabilization of VA in patients with exudative age-related macular degeneration who achieved no more benefit from the initial switch.

Retinal Changes in Different Grades of Retinal Artery Occlusion: An Optical Coherence Tomography Study.

Purpose: To investigate layer-by-layer changes in retinal thickness and reflectivity regarding the severity grade of acute retinal artery occlusion (RAO) using spectral-domain optical coherence tomography (SD-OCT). Methods: This study is a retrospective, observational case-control series that took place in an institutional setting and included 148 eyes from 74 patients diagnosed with acute RAO (central or branch). SD-OCT examinations were taken at baseline. Based on OCT findings, RAO was categorized into three grades (incomplete, subtotal, total). The thickness and reflectivity of selected retinal layers were measured from SD-OCT images. The data were compared across the three grades and against the contralateral eyes (controls). The main outcome measures were thickness and reflectivity of selected retinal layers. Results: The thickness of the inner and middle retinal layers differed significantly across the three RAO groups (P < 0.001), whereas the outer retinal layer thickness remained not significantly different. Reflectivity values showed statistically significant differences in the inner, middle, and outer retinal layers, but not in the vitreous body (P < 0.001). Conclusions: The reflectivity changes of selected retinal layers differ significantly regarding different grades of RAO. SD-OCT reflectivity measurement may be used as a noninvasive method to estimate the grade of retinal ischemia in RAO.

Intraocular methotrexate can induce extended remission in some patients in noninfectious uveitis.

PURPOSE: To assess the outcomes of the intravitreal administration of methotrexate in uveitis. METHODS: Multicenter, retrospective interventional case series of patients with noninfectious uveitis. Thirty-eight eyes of 30 patients were enrolled, including a total of 54 intravitreal injections of methotrexate at a dose of 400 µg in 0.1 mL. The primary outcome measure was visual acuity. Secondary outcome measures included control of intraocular inflammation and cystoid macular edema, time to relapse, development of adverse events, and levels of systemic corticosteroid and immunosuppressive therapy. RESULTS: Methotrexate proved effective in controlling intraocular inflammation and improving vision in 30 of 38 eyes (79%). The side effect profile was good, with no reported serious ocular adverse events and only one patient having an intraocular pressure of >21 mmHg. Of the 30 eyes that responded to treatment, 8 relapsed, but 22 (73%) entered an extended period of remission, with the Kaplan-Meier estimate of median time to relapse for the whole group being 17 months. The eight eyes that relapsed were reinjected and all responded to treatment. One eye relapsed at 3 months, but 7 eyes again entered extended remission. Of the 14 patients on systemic therapy at the start of the study, 8 (57%) were able to significantly reduce this following intravitreal methotrexate injection. CONCLUSION: In patients with uveitis and uveitic cystoid macular edema, intravitreal MTX can effectively improve visual acuity and reduce cystoid macular edema and, in some patients, allows the reduction of immunosuppressive therapy. Some patients relapse at 3 to 4 months, but a large proportion (73%) enter an extended period of remission of up to 18 months. This larger study extends the results obtained from previous smaller studies suggesting the viability of intravitreal methotrexate as a treatment option in uveitis.