Central serous chorioretinopathy and antiphospholipid syndrome: Three cases report.

Antiphospholipid syndrome (APS) is an acquired autoimmune disease characterized by recurrent venous or arterial thrombotic events and pregnancy morbidity, with persistently presence of antiphospholipid antibodies (aPL). We report three cases of central serous chorioretinopathy (CSC) associated with APS.

PERIPHERAL EXUDATIVE HEMORRHAGIC CHORIORETINOPATHY-A NEW ADDITION TO THE SPECTRUM OF PACHYCHOROID DISEASE?

PURPOSE: To demonstrate choroidal vascular changes and report a novel choroidal thickness contour in eyes with peripheral exudative hemorrhagic chorioretinopathy (PEHCR). METHODS: Retrospective, observational, comparative case series. Fourteen eyes of nine patients with PEHCR and 14 eyes of 14 age-matched and sex-matched controls underwent swept-source optical coherence tomography. Choroidal thickness was measured from posterior edge of the retinal pigment epithelium-Bruch membrane to choroidoscleral interface at 11 points 1,000 µm apart. Large choroidal vessel thickness was also measured. RESULTS: In PEHCR group, the choroid was thinnest at 3 mm nasal to fovea (mean 95.3 ± 33.5 µm) and thickest at 7 mm temporal to fovea (mean 272.7 ± 80.2 µm), with gradual increase in choroidal thickness from nasal to temporal periphery. The choroid was thickest subfoveally (259.7 ± 63.8 µm) in the control group. The choroid was significantly thicker in temporal periphery in PEHCR eyes as compared to controls (P = 0.0002). The mean large choroidal vessel thickness was 202.4 ± 50.8 µm in the PEHCR group and 160.6 ± 40.5 µm in the control group (P = 0.0235). CONCLUSION: Peripheral exudative hemorrhagic chorioretinopathy eyes showed progressively increasing choroidal thickness toward the temporal periphery, compared with age-matched and sex-matched controls. This gave rise to a club-shaped choroidal contour compared with the bowl-shaped contour seen in control eyes. Thicker choroid and pachyvessels favor inclusion of PEHCR in the pachychoroid disease spectrum.

Increased choroidal blood flow and choroidal thickness in patients with hypertensive chorioretinopathy.

PURPOSE: To investigate the alterations of central choroidal thickness (CCT) and macular choroidal blood flow in patients with hypertensive chorioretinopathy treated with antihypertensive agents. METHODS: In retrospective observational case series, ten eyes of 9 patients with hypertensive chorioretinopathy were enrolled (5 men and 4 women; 43.1 ± 19.6 years of age). CCT and mean blur rate (MBR) had been observed during follow-up using enhanced depth imaging optical coherence tomography and laser speckle flowgraphy, respectively. RESULTS: With the medication for hypertension, serous retinal detachment (SRD) disappeared in all the eyes (mean period, 57.8 ± 50.4 days), and the mean blood pressure decreased (122.7 ± 13.0 mmHg and 93.4 ± 13.2 mmHg at the initial visit and at the day of subretinal fluid absorption, respectively; P < 0.01). The mean logMAR value of best corrected visual acuity showed a tendency toward improvement with the resolution of SRD (0.15 ± 0.30 and 0.08 ± 0.28, P = 0.15). The average MBR significantly decreased when SRD was absorbed (11.4 ± 4.5 and 7.7 ± 2.2, P < 0.01). Similarly, the mean values of CCT decreased (473.2 ± 218.0 µm and 325.7 ± 112.0 µm, P < 0.01). The changing rates of CCT and MBR showed a significant positive correlation (P < 0.01, R = 0.88). CONCLUSION: The current study demonstrated a novel finding that choroidal blood flow velocity and thickness concurrently increased in the acute phase of hypertensive chorioretinopathy, suggesting the role of choroidal hyperperfusion in the pathogenesis of hypertensive chorioretinopathy.

CHOROIDAL THICKENING AND PACHYCHOROID IN CUSHING SYNDROME: Correlation With Endogenous Cortisol Level.

PURPOSE: To investigate subfoveal choroidal thickness and pachychoroid and their correlation with hormone level in patients with endogenous Cushing syndrome (CS). METHODS: We enrolled a consecutive series of patients with CS and healthy controls. All participants had swept-source optical coherence tomography. All patients with CS had hormone test including morning plasma-free cortisol, 24-hour urine-free cortisol (24UFC), and plasma adrenocorticotropic hormone. We compared subfoveal choroidal thickness and pachychoroid changes between two groups. We performed univariate and multivariate analysis to study correlation between hormone level and choroid thickness as well as pachychoroid in patients with CS. RESULTS: Compared with control group, Cushing group had significantly greater subfoveal choroidal thickness (371.6 ± 114.9 and 320.0 ± 74.0, P = 0.002) and higher proportion of eyes with pachychoroid (53.1 and 14.3%, P < 0.001). Subfoveal choroidal thickness was significantly correlated with 24UFC (P = 0.007) but not with plasma-free cortisol (P = 0.48) or adrenocorticotropic hormone (P = 0.56). Pachychoroid was significantly correlated with 24UFC (P = 0.03) but not with plasma-free cortisol (P = 0.24) or adrenocorticotropic hormone (P = 0.32). CONCLUSION: There was a positive correlation between elevated 24UFC and choroid thickening as well as pachychoroid, indicating the importance of normal endogenous cortisol level in maintaining the human choroid vasculature.

ASSOCIATION OF OBSTRUCTIVE SLEEP APNEA WITH CENTRAL SEROUS CHORIORETINOPATHY AND CHOROIDAL THICKNESS: A Systematic Review and Meta-Analysis.

PURPOSE: Obstructive sleep apnea (OSA) has been associated with an array of ocular disorders. This systematic review aims to investigate the association of OSA with central serous chorioretinopathy (CSCR) and subfoveal choroidal thickness changes on enhanced depth imaging optical coherence tomography. METHODS: Systematic review and meta-analysis of all articles published up to November 2017 examining rate of OSA in patients with CSCR versus controls or examining subfoveal choroidal thickness measurements on enhanced depth imaging optical coherence tomography in patients with OSA versus controls. Pooled odds ratios and weighted mean difference with 95% confidence intervals (CIs) were calculated. RESULTS: For the CSCR/OSA analysis, 7,238 patients (1,479 with CSCR and 5,759 controls) from 6 studies were eligible. For the choroidal thickness/OSA analysis, 778 eyes of 778 patients (514 with OSA and 264 controls) from 9 studies were eligible. Patients with CSCR had a 1.56 increased odds of having OSA than controls (odds ratio, 1.56; 95% CI, 1.16-2.10). There was no statistically significant difference in choroidal thickness between mild OSA subjects and controls (weighted mean difference = -3.17; 95% CI, -19.10 to 12.76). Patients with moderate OSA (weighted mean difference = -24.14; 95% CI, -42.16 to -6.12) and severe OSA (weighted mean difference = -51.19; 95% CI, -99.30 to -3.08) had thinner choroidal thickness measurements than controls. CONCLUSION: In summary, our results suggest that patients with CSCR are more likely to have OSA, and that moderate/severe OSA is associated with smaller subfoveal choroidal measurements on enhanced depth imaging optical coherence tomography.

The association of Helicobacter pylori with choroidal and retinal nerve fiber layer thickness.

PURPOSE: To investigate the effect of Helicobacter pylori (H. pylori) infection on choroidal thickness (CT) and retinal nerve fiber layer thickness (RNFLT). METHODS: The study included 25 patients with H. pylori infection and 25 healthy individuals as the control group. Helicobacter pylori patients were classified as the pre-treatment (Group 1; n: 25) and the post-treatment (Group 2; n: 25). RNFLT and CT were measured before and after treatment of H. pylori infection, using enhanced depth imaging (EDI) spectral-domain optical coherence tomography (Spectralis, Heidelberg Engineering, Heidelberg, Germany). The axial length and intraocular pressure were also measured. RESULTS: The mean subfoveal CT was 320.96 ± 29.15 µm in Group 1 and 287.48 ± 49.17 in the control group (p = 0.007), while the mean subfoveal CT did not show any difference between Group 2 and the control group (p > 0.05). No statistically significant difference was determined between the H. pylori patients and the control group in respect of RNFLT values (p > 0.05). CONCLUSIONS: CT increases during H. pylori infection and returns to the normal range within 6 weeks of treatment. RNFLT does not show any change during H. pylori infection. The data related to the subfoveal CT may be useful in understanding the pathogenesis of central serous chorioretinopathy developing in H. pylori patients.

Risk factors for central serous chorioretinopathy in pregnant Japanese women.

AIM: This study was performed to determine risk factors for central serous chorioretinopathy (CSC) in pregnant women. METHODS: This retrospective observational study was performed in a cohort of all 1881 women giving birth at a single center. The hospital database was searched to abstract all women diagnosed with pre-eclampsia (PE) as well as those visiting the eye clinic during the current pregnancy. Medical chart review was performed in all women diagnosed with CSC and PE. RESULTS: PE developed in 73 (3.9%) women, six (8.2%) of whom visited the eye clinic for problems occurring in the current pregnancy; 47 of 1808 (2.6%) women without PE visited the eye clinic (P  = 0.015). Four women were identified as having developed CSC after onset of PE, and none of those without PE developed CSC (5.5% [4/73] vs 0.0% [0/1808], respectively, P <  0.0001). Stepwise regression analysis selected four risk factors for CSC: hematocrit value > 38.0% (odds ratio [OR], 22.9; 95% confidence interval [CI], 2.12-247), serum creatinine > 0.7 mg/dL (OR, 21.7; 95%CI, 1.12-422), time interval from diagnosis of PE until delivery > 14 days (OR, 20.0; 95%CI, 1.87-214), and urine protein : creatinine ratio (mg/mg) > 4.5 (OR, 15.7; 95%CI, 0.81-304). Hematocrit value > 38.0% was finally identified as the only independent risk factor (OR, 22.9; 95%CI, 2.12-1716) for CSC in PE women. CONCLUSION: CSC was likely to occur in PE women, especially in those with hemoconcentration as a result of plasma leakage from the circulating blood due to increased vascular permeability.

Efficacy of reduced-fluence photodynamic therapy for central serous chorioretinopathy associated with combined serous retinal detachment and fovea-involving pigment epithelial detachment.

The purpose of the study was to evaluate the effect of reduced-fluence photodynamic therapy (RFPDT) for chronic central serous chorioretinopathy (CSC) associated with serous retinal detachment (SRD) and fovea-involving pigment epithelial detachment (PED). Ten eyes of ten patients with chronic CSC associated with combined SRD and PED involving the fovea were included. RFPDT was applied to the hyperfluorescent area identified at the mid-to-late phase of indocyanine green angiography, which indicated the area of leakage. We evaluated the changes in best-corrected visual acuity (BCVA) and optical coherence tomography at month 1 and final follow-up visit. The mean age of the participants was 47.4 ± 7 years. The mean follow-up duration was 6.3 ± 4 months. At month 3, the SRD had resolved completely in all ten eyes (100 %), and PED had resolved in seven eyes (70 %). The initial mean BCVA improved from 20/50 at baseline to 20/32 at the last visit (P > 0.05). The mean central retinal thickness was reduced from 534 ± 279 µm at baseline to 194 ± 46 µm at the last examination (P < 0.001). The mean subfoveal choroidal thickness decreased from 461 ± 57 at baseline to 369 ± 75 at the final visit (P < 0.001). Reduced-fluence PDT appears as an effective treatment for chronic CSC cases associated with SRD and fovea-involving PED.

Central serous chorioretinopathy in primary hyperaldosteronism.

PURPOSE: To describe ophthalmological characteristics of 13 patients with primary hyperaldosteronism (PA). METHODS: Cross-sectional study. All patients underwent extensive ophthalmological examination. RESULTS: Thirteen PA patients (9 male, 4 female) were diagnosed with arterial hypertension for 11.0 ± 11.2 years. Ophthalmological imaging revealed macular serous subretinal fluid (SRF) on optical coherence tomography in 2 patients (15 %). In one of these patients, bilateral chronic central serous chorioretinopathy (CSC) with polypoidal choroidal neovasculopathy was diagnosed, which was effectively treated with full-dose photodynamic therapy. In the other patient with SRF and bilateral diffuse hyperfluorescent areas on fluorescein angiography, the SRF had decreased spontaneously after 6 weeks of follow-up. In 5 of the remaining patients (38 %), retinal pigment epithelium alterations resembling findings characteristic for CSC were seen on multimodal imaging. The mean subfoveal choroidal thickness was 290.2 ± 65.0 µm. CONCLUSIONS: Retinal abnormalities resembling (subclinical) CSC are common in patients with PA. These findings indicate that mineralocorticoid-mediated pathways are involved in the pathogenesis of CSC. In CSC patients with hypertension of unknown origin, a diagnosis of PA should be considered.

Circadian disturbance and idiopathic central serous chorioretinopathy.

BACKGROUND: This present retrospective case control study was designed to evaluate circadian disturbance in patients with chronic idiopathic central serous chorioretinopathy (ICSC). METHODS: Between January 1st, 2012, and November 30th, 2014, 29 consecutive patients with chronic ICSC examined in a referral setting were compared with a gender-matched and age-matched control group of 29 patients. A history of pharmacologic medication (including corticosteroid treatment), sleep disturbance, irregular working hours, cardiovascular risk factors, and depressive anxiety disorders was noted. RESULTS: The median age of the patients was 52, and in the control subjects it was 50. The male-female ratio for both groups was 4.8:1. Patients with chronic ISCS were more likely to be exposed to irregular working hours (p < 0.01, OR 9.3 [2.29-37.6]) and to present with overweight than the control subjects (p = 0.016). No significant differences were found for sleeping disturbances, pharmacological medication, cardiovascular risk factors, or depressive anxiety disorders. CONCLUSIONS: In this preliminary study, the exposition of irregular working hours as a risk factor for chronic ICSC was identified, which had not been previously reported. If further studies confirm these findings, then employment with regular working hours could be recommended for chronic ICSC patients.

RISK FACTORS FOR CENTRAL SEROUS CHORIORETINOPATHY: A Systematic Review and Meta-Analysis.

PURPOSE: Central serous chorioretinopathy (CSC) is a common retina disease and has a relative high recurrence rate, etiology, and pathogenesis of which remains ambiguous. The systematic review and meta-analysis aimed to measure risk factors for CSC in a quantitative method, providing some precautions and interventions on this disease and preventing further recurrences. METHODS: A comprehensive literature review relating to risk factors for CSC through PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure (CNKI), and VIP databases was conducted before March 2015. Odds ratio (OR) with 95% confidence interval (CI) was calculated after data combination to assess the associations between risk factors and CSC. RESULTS: A total of 9839 patients in 17 studies were included and risk factors with significant differences found between CSC and control groups were listed as follows: hypertension (OR = 1.7; 95% CI: 1.28-2.25), Helicobacter pylori (H. pylori) infection (OR = 3.12; 95% CI: 1.81-5.40), steroid usage (OR = 4.29; 95% CI: 2.01-9.15), sleeping disturbance (OR = 1.90; 95% CI: 1.28-1.83), autoimmune disease (OR=3.44; 95% CI: 1.90-6.26), psychopharmacologic medication use (OR = 2.69; 95% CI: 1.63-4.45), and Type-A behavior (OR = 2.53; 95% CI: 1.08-5.96). CONCLUSION: The authors concluded that hypertension, H. pylori infection, steroid usage, sleeping disturbance, autoimmune disease, psychopharmacologic medication use, and Type-A behavior were possible risk factors relating to the occurrence of CSC.

Investigation of the Etiology of Central Serous Chorioretinopathy Using En-Face Optical Coherence Tomography and Indocyanine Green Angiography.

PURPOSE: To identify locations of hypofluorescent lesions on late-phase indocyanine green angiography (ICGA) in patients with central serous chorioretinopathy (CSC) using en-face optical coherence tomography (OCT). PROCEDURES: We retrospectively studied 25 consecutive untreated CSC patients, using swept-source OCT and ICGA. En-face swept-source OCT images were automatically segmented and flattened with Bruch's membrane (BrM). We compared the sizes of hyperreflective areas in the 25 CSC and 25 contralateral eyes on en-face images and hypofluorescent areas on ICGA after 30 min. RESULTS: All 25 CSC eyes and 13 contralateral eyes showed abnormal hypofluorescent areas on late-phase ICGA and hyperreflective areas on en-face OCT from BrM to the choriocapillaris, and these findings correlated with the abnormal areas (r = 0.9988; p < 0.001). CONCLUSIONS: In CSC patients, we detected abnormal hypofluorescence on ICGA in the late phase, which corresponded to abnormal hyperreflective areas from BrM to the choriocapillaris level in en-face images.

Multimodal imaging findings in a case of severe Central Serous Chorioretinopathy in an uncomplicated pregnancy.

BACKGROUND: Central Serous Chorioretinopathy (CSC) has been previously reported as an infrequent complication of pregnancy that usually resolves spontaneously after delivery, with minimal or no sequel. We report a case of a severe form of CSC in an uncomplicated pregnancy with extensive subfoveal exudates and severe permanent visual loss. Multimodal imaging techniques, including color and red-free photographs, near-infrared reflectance, fluorescein angiography, and spectral-domain optical coherence tomography, were performed and the findings were correlated to the changes in visual acuity. CASE PRESENTATION: A 35-year-old pregnant woman presented with loss of vision and metamorphopsia in her left eye. Fundus examination showed subfoveal severe exudation with a posterior pole serous detachment. Optical coherence tomography (OCT) showed macular neurosensory detachment with central highly reflective sub-retinal material. Multimodal fundus pictures and angiograms revealed distinct clinical features of the disease during both the acute and final phase. The disease spontaneously resolved after delivery with regression of the subretinal fluid and the disappearance of subfoveal exudates. Nevertheless, because of severe atrophic macular changes and subfoveal fibrosis, no improvement of visual acuity was noted. CONCLUSION: Severe variants of CSC may also present in cases of uncomplicated pregnancy and result in a poor prognosis. Recognising these presentations of CSC is critical to avoid improper management. Multimodal imaging may help to clarify the diagnosis and highlight the clinical features.

Central Serous Chorioretinopathy Treatments: A Mini Review.

Central serous chorioretinopathy (CSC) is a retinal disorder that primarily affects young (20- to 50-year-old) white men, although it is seen occasionally in older patients and females. CSC is characterized by avascular focal leakage through the retinal pigment epithelium (RPE), resulting in serous detachment of the neurosensory retina. The course is usually self-limiting and in most cases resolves spontaneously within a 3-month period, with visual acuity usually recovering to 20/30 or better. However, chronic CSC may develop as a consequence of recurrences or persistent neurosensory detachment, and can result in progressive RPE atrophy and permanent visual loss. A primary involvement of the RPE and choroidal vascularization play a significant role in the pathogenesis of CSC and the current treatment options attempt to restore the functions of the RPE and the normal choroidal vasculature. The aim of the current review is to provide an overview of the current therapeutical approaches to CSC, including observation, laser treatment, photodynamic therapy with verteporfin, intravitreal anti-vascular endothelial growth factor therapy and the mineralocorticoid receptor antagonists.

Central serous chorioretinopathy in Susac syndrome.

We report central serous chorioretinopathy (CSC) in a patient with Susac syndrome. The diagnosis of Susac syndrome was based on the results of funduscopy, brain magnetic resonance imaging, and audiometric testing. Our case demonstrates that possible choroidal involvement in Susac syndrome may lead to the development of CSC.

Central serous chorioretinopathy.

The pathogenesis of central serous chorioretinopathy (CSC) is still not fully understood. The involvement of corticosteroids is undisputed, although their exact role has not been clarified; other parts of the underlying mechanism of CSC have been mainly elucidated by imaging techniques such as fluorescein and indocyanine green angiography. Even though most cases of CSC are self-limiting, severe as well as recurrent courses exist, and for these patients only a limited number of treatment options are available: laser photocoagulation, with a risk of scotoma and choroidal neovascularization, and photodynamic therapy. In this review article, we give an overview of its epidemiology, the current understanding of its pathogenesis as well as systemic and ocular risk factors. We illuminate modern diagnostic tools as well as current treatment options in the context of CSC, particularly in the light of a better understanding of corticosteroids and their receptors involved in its pathogenesis.

POEMS syndrome in a 20-year-old patient diagnosed following a complaint of reduced visual acuity.

We report a case of POEMS syndrome in a 20-year-old patient diagnosed after visiting an eye clinic with a chief complaint of reduced visual acuity. A male university student aged 20 years was referred to our department complaining of blurred vision in both eyes that had persisted for 1 month. He also noted headache, nausea, and paresthesia in the lower extremities around the same time. The visual acuity of his right and left eye was 20/40 and 20/20, respectively. Optic disc edema and serous retinal detachment were present. Brain magnetic resonance imaging showed no intracranial abnormalities, while elevated cerebrospinal fluid pressure, reduced nerve conduction velocity in both lower extremities, hepatosplenomegaly, M proteinemia, high blood VEGF levels, osteoblastic and osteolytic changes in the spine, and atypical plasma cells in bone lesions were noted. From the above findings, the patient was diagnosed with POEMS syndrome. He received high-dose dexamethasone, thalidomide, and radiotherapy on the sacral mass, followed by high-dose melphalan with autologous stem-cell support, and showed subsequent systemic and ophthalmologic improvement. Here, we report the youngest case ever of POEMS syndrome with ocular manifestation. If patients have optic disc edema in both eyes with no intracranial space-occupying lesion, POEMS syndrome should be considered in differential diagnosis, regardless of age.

Central serous chorioretinopathy following hypobaric chamber exposure.

BACKGROUND: Hypobaric hypoxic exposures are associated with a number of risks, most notably decompression sickness and various ophthalmologic disorders, including high altitude retinopathy. Central serous chorioretinopathy (CSCR) is an idiopathic condition that typically affects young males and is associated with several comorbidities and medications; however, an association with hypoxia or high altitude has not been identified. We present a case of CSCR in an aviator following a simulated flight in a hypobaric chamber. CASE REPORT: A 30-yr-old male U.S. Navy pilot presented with complaints of painless unilateral scotoma, micropsia, and blurred vision 1 h after completing a training exercise in a hypobaric chamber. A dilated fundoscopic examination, macular optical coherence tomography, and intravenous fluorescein angiography confirmed a diagnosis of CSCR. The patient was restricted from flying duty and observed for a period of 1 mo, after which point his symptoms spontaneously resolved and flight status was restored. DISCUSSION: Complaints of visual symptoms immediately following hypobaric exposure should primarily trigger suspicion of decompression sickness; however, once ruled out, patients should be referred to an eye specialist for detailed ocular examination. This case suggests a possible link between CSCR and hypobaric hypoxia as a topic of further investigation.

Potential involvement of mineralocorticoid receptor activation in the pathogenesis of central serous chorioretinopathy: case report.

BACKGROUND: Recently it has been suggested that excessive glucocorticoid-dependent choroidal mineralocorticoid receptor (MR) activation may be involved in the pathogenesis of central serous chorioretinopathy (CSCR). AIM: To present a 38 year-old woman with an impressive improvement of CSCR following MR antagonist eplerenone administration. CASE REPORT: At presentation, visual acuity (VA) was 0.2 in the left eye and 1.0 in the right eye. Optical coherence tomography (OCT) of the left eye showed extended serous retinal detachment including the macular area. RESULTS: After six weeks of treatment with eplerenone (25 mg/day) total resorption of subretinal fluid with an increase in VA to 0.8 was observed. At that point the therapy with eplerenone was discontinued, with no recurrence in the left eye during five months follow-up. Two months after the discontinuation of eplerenone, subretinal fluid accumulation in the right eye was revealed by OCT. Four weeks after reintroducing the treatment with eplerenone (25 mg/day) almost total resorption of subretinal fluid in the right eye was observed. CONCLUSIONS: The effectiveness of MR antagonism in unresolved CSCR supports the hypothesis that excessive choroidal MR activation may be a potential pathological pathway leading to CSCR, and MR blockage may be an effective treatment option for CSCR. Controlled clinical trials are necessary to evaluate this therapeutic approach.