As most patients of central serous retinopathy (CSC), the symptoms of acute onset will alleviate by oneself after 4-6 months. About 30%-50% of patients with CSC experience chronic or recurrent cases. Resulting in persistent neurosensory detachments and subretinal fluid, causing significant vision loss. Mineralocorticoid receptor (MR) is a kind of nuclear hormone receptors, plays a role in theregulation of water and electrolyte balance. Excessive MR signaling is associated with many diseases. Study found that MR antagonists decreased the thickness of the retina and improved in vision, there was no serious adverse reactions during the period of treatment for chronic CSC. Initial dose of MR antagonists was 25 mg per day, 1 week later, dosage was increased to 50 mg per day, and treatment for about 3 months. There is no conclusive effective treatment and the dosage are still unknown. MR antagonists may be a safe and effective way to treat chronic CSC, though evidence is scant. Prospective, multicenter, large-scale trials is required.
Objective To observe the morphologic alterations of retina pigment epithelial around fluorescein leakage site using spectral-domain optical coherence tomography (SD-OCT) in acute central serous chorioretinopathy (CSC) before and after laser coagulation. Methods Twenty-four eyes of 24 consecutive patients with unilateral acute CSC who underwent laser photocoagulation (wave length: 561 nm, energy: 80-100 mW, spot diamteter: 100 μm, exposure time: 0.1 s) were enrolled in this retrospective study. The patients included 22 males and 2 females, with an average age of (39.2±7.2) years and an average duration of (1.1±0.6) months. The fluorescein leakage outside the foveal avascular zone and serous retinal detachment were found in all the eyes by fluorescein fundus angiography (FFA) and SD-OCT. Twenty-six leakage sites were observed in 24 eyes on FFA. There were 5 smoky leakage points (19.2%), 21 inky leakage points (80.8%). The retinal pigment epithelial (RPE) layer at leakage points were scanned by SD-OCT before and at 2 weeks, 2 months after treatment. SD-OCT showed that the RPE abnormalities were detected at all of the fluorescein leakage points, including serous pigment epithelial detachment (PED) in 16 leakage points (64.5%), protruding RPE in 5 leakage points (19.5%), and irregular RPE in 5 leakage points (19.2%). The morphologic alterations of RPE around fluorescein leakage point before and after laser coagulation were observed. Results At 2 weeks after laser photocoagulation, PED existed in 7 leakage points (26.9%), protruding RPE in 3 leakage points (11.5%), and irregular RPE in 16 leakage points (61.5%). At 2 months after laser photocoagulation, protruding RPE existed in 3 leakage points (11.5%), and irregular RPE in 5 leakage points (19.2%), including RPE defect in 2 leakage points. And there were undetectable RPE abnormalities in other 16 leakage points (61.5%). Conclusions In acute CSC, SD-OCT shows that morphologic features of the RPE layer are changed, including PED, protruding RPE, irregular RPE around the leakage point. After laser photocoagulation the abnormalities of RPE can also be altered from PED to irregular RPE, and RPE defect is observed in restricted regions.
ObjectiveTo analyze the associations between the choroidal vasculature and submacular fluid (SMF) in central serous chorioretinopathy (CSC). MethodsA retrospective study. A total of 29 CSC patients (31 eyes) with complete records who visited the Department of Ophthalmology in Peking University People's Hospital from August 1, 2021 to March 1, 2023 were included in this study. The patients were divided into complete absorption and incomplete absorption groups according to the status of SMF in the last visit. All the patients underwent ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) with a scanning range of 24 mm × 20 mm. The UWF SS-OCTA images were automatically analyzed in 9 regions (superotemporal, superior, superonasal, temporal, central, nasal, inferotemporal, inferior, and inferonasal). Alterations of choroidal vasculature in the nine subfields after SMF absorption were described, including choroidal thickness (CT), flow density of choriocapillaris layer, vessel density of large choroidal vessel layer, three-dimensional choroidal vascularity index (CVI), the mean choroidal vessel volume (mCVV), and the mean choroidal stroma volume (mCSV). The relevant factors affecting the complete absorption of SMF were additionally evaluated. ResultsAt baseline, CT (Z=2.859, P=0.004), mCVV (t=2.514, P=0.018), and mCSV (Z=2.958, P=0.003) in the superotemporal region of the affected eyes in the incomplete absorption group were significantly higher than those in the complete absorption group. Compared with baseline, at the last visit, the proportion of asymmetric vortex veins in the complete absorption group was significantly decreased (χ2=6.000, P=0.014), CVI in the superotemporal, superonasal, temporal, central, nasal, inferotemporal, and inferonasal regions (t=－4.125, t=－3.247, Z=－3.213, t=－2.994, t=－3.417, t=－3.733, t=－3.795; P=0.001, 0.006, 0.001, 0.010, 0.005, 0.003, 0.002), the mCVV of 9 regions (t=－2.959, t=－2.537, t=－2.235, t=－3.260, t=－3.022, t=－2.796, t=－2.747, Z=－2.107, t=－2.935; P=0.011, 0.025, 0.044, 0.006, 0.010, 0.015, 0.017, 0.035, 0.012) were significantly decreased. Compared to the complete absorption group, the choroidal blood flow changes in the non-complete absorption group were more limited, and CT in the upper region increased significantly at the last follow-up (t=2.272, P=0.037). Multivariate logistic regression analysis revealed that baseline CT in the superotemporal region may be an independent risk factor affecting the complete absorption of SMF (odds ratio=0.981, 95% confidential interval 0.965-0.997, P=0.021). ConclusionsIn the process of SMF absorption in CSC, significant reductions of choroidal blood flow were found in the large choroidal vessel layer, and there may be a locally compensatory increase in CT. In addition, baseline CT in superotemporal region is an independent risk factor affecting SMF absorption.
Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a peripheral retinal disease characterized by subretinal hemorrhage and/or subretinal pigment epithelial hemorrhage or exudation. It is often misdiagnosed as age-related macular degeneration, polypoidal chorioretinopathy or choroidal melanoma. With the development of multimodal imaging, PEHCR has different features under different examinations, such as B-scan ultrasound, fluorescein fundus angiography, optical coherence tomography and so on, which contributes to differention from other diseases. Clinical treatments for the disease include intravitreal injection of retinal photocoagulation therapy, anti-vascular endothelial growth factor, pars plana vitrectomyand so on, but there is still no universal consensus. In order to gain a deeper understanding of the clinical features, treatment options and prognosis of PEHCR, minimize missed diagnoses and misdiagnoses, and improve treatment efficiency, further research is required.
Objective To observe and analyze the clinical characteristics of children with autosomal dominant hereditary microcephaly with or without chorioretinopathy, lymphedema, or intellectual disability syndrome (MCLMR). MethodsA retrospective clinical study. In September 2023, the first patient and three family members (parents, brother) of MCLMR who were diagnosed through ophthalmic examination and genetic testing at Department of Ophthalmology of Henan Children's Hospital were included in the study. Clinical data were collected, inquired about medical history and family history in detail, and performed best corrected visual acuity (BCVA), optical coherence tomography (OCT), fluorescein angiography (FFA), flash visual evoked potential (F-VEP), full field electroretinogram (ERG), cranial magnetic resonance imaging (MRI), and systemic examination. 3 ml of peripheral venous blood were collected from the proband, her parents and younger brother, and extracted whole genome DNA. Second generation sequencing technology was used for gene sequencing. For suspected pathogenic sites, Sanger sequencing was used for validation, and bioinformatics analysis was performed to determine the pathogenicity of the genetic variant sites. The relevant literature of PubMed of the National Library of Medicine and Wan Fang Med Online by computer were searched. The genetic characteristics and conducted literature review were summarized. ResultsThe proband (Ⅱ-1) was an 8-year-old and 5-month-old female. Her head was relatively small, the lower jaw was small, the ears protrude, the nose was wide, the eyelid was tilted upwards, philtrum was long. Mild intellectual disability, no history of lymphedema. The BCVA values for the right and left eyes were 0.08 and 0.1, respectively. Bilateral nystagmus. Atrophic lesioned in the macular area and below choroid retina of both eyes. FFA examination showed mottled fluorescent staining in the macular area and the below retina, with no obvious fluorescein leakage in the late stage. OCT examination revealed shallow macular fovea morphology, absence of ellipsoidal bands, unclear layers, thinning of the entire retina, and significant atrophy of the choroid and retina beneath the macula. F-VEP examination, no waveform was detected in both eyes. Full field ERG examination showed severe reduction in amplitude of a wave and b wave in both eyes. Head magnetic resonance imaging showed widening of the subarachnoid space in the left temporal region, with no significant abnormal signals observed in the brain parenchyma. Her father (Ⅰ-1) had mild nystagmus and strabismus. The phenotypes of the eyes of the mother (Ⅰ-2) and brother (Ⅱ-2) were not significantly abnormal. The genetic testing results showed that the proband (Ⅱ-1) had a heterozygous missense mutation c.895A>G (p.Ile299Val) in exon 8 of the KIF11 gene, which was a known mutation. Her parents (Ⅰ-1, Ⅰ-2) and younger brother (Ⅱ-2) were both wild-type. The bioinformatics analysis results indicated that this mutation is a potentially pathogenic variant. A total of 109 cases were retrieved from 20 relevant literatures. Among them, 55 were male, 54 were females. There were 61 cases with family history and 48 cases without family history, respectively. Among the 109 cases, 98 cases (89.9%, 98/109) had microcephaly, 2 cases had premature closure of cranial sutures, and 11 patients underwent cranial MRI, which showed 11 cases of small head with simplified development of the cerebral gyrus. 50 cases (45.9%, 50/109) of lymphedema. 83 cases (76.1%, 83/109) of intellectual developmental disorders. 92 cases (84.4%, 92/109) had ocular abnormalities, 69 cases (63.3%, 69/109) had chorioretinopathy, 20 cases (18.3%, 20/109) had retinal folds, 10 cases (9.2%, 10/109) had nystagmus, and 17 cases (15.6%, 17/109) had retinal detachment. ConclusionsThe main clinical manifestations of MCLMR are microcephaly, chorioretinopathy, with or without lymphedema, and intellectual disability. The main manifestations of eye diseases are low vision, nystagmus, and chorioretinopathy. The heterozygous missense mutation c.895A>G (p.Ile299Val) in exon 8 of KIF11 gene is the pathogenic variant of this family.
ObjectiveTo observe the characteristic variation of the patients' inner and outer retina who had chronic central serous chorioretinopathy (CSC) after being treated of photodynamic therapy (PDT). MethodsNineteen patients with chronic CSC were recruited, including 15 eye of men and 4 eye of women, logMAR BCVA was 0.1-1.0, 0.39±0.30. Meanwhile, 24 healthy people were located in the control group. All the patients received PDT for the first time. All subjects including 24 healthy people underwent fourier domain optical coherence tomography (FD-OCT). Retinal thickness were investigated before PDT and 1, 4, 12, 20 weeks after PDT respectively. Data were recorded including inner layer and outer layer. Retinal thickness were compared in fovea (1 mm), parafovea (3 mm)and perifovea(5 mm). Paired-samples t test was used to compare retinal thickness before and after PDT. The statistical differences of patients and control group were evaluated by independent-samples t test. The correlations between the best logMAR corrected visual acuity (BCVA) was analyzed by Pearson statistical analyses. ResultsThe inner(F=13.814, 10.095, 4.689) and outer(F=9.354, 5.878, 3.978)layer fovea thickness of CSC subjects in 1, 4, 12 week was thinner, the difference was statistically significant (P < 0.05). The outer layer fovea thickness at P12(t=-3.725), parafovea of inner and outer retinal(t=-3.198, -2.722)was reduced when compared with control group, and differences have statistical sense, respectively (P < 0.05). There was correlation between logMAR BCVA and outer retinal thickness in fovea and parafovea (r=0.465, -0.728, -0.687; P < 0.05). ConclusionIn our study, the inner and outer layer retinal thickness decreased generally after the first time PDT in CSC patients.
Central serous chorioretinopathy (CSC) is one of the representative pachychoroid spectrum disease. Although fundus fluorescein angiography and indocyanine green angiography can be used as the gold standard for the diagnosis of CSC, they are invasive examinations, which may bring certain risks in clinical application and cannot help us obtain quantitative parameters. Optical coherence tomography angiography (OCTA), as a non-invasive and quantitative examination, is an important imaging tool for understanding the pathogenesis, diagnosis and treatment of CSC. With the advancement of OCTA, the swept-source OCTA has a satisfying scanning depth, a wider scanning range and a higher resolution. The development of OCTA broadens the horizons of the pathogenesis of CSC, promotes the understanding of the pathophysiology of CSC, and sheds new light for its clinical diagnosis and treatment. Based on OCTA, the choroid and retina in eyes with CSC are presented with qualitative and quantitative changes in vascular system. OCTA-guided CSC treatment and the discovery of prognostic markers based on OCTA challenge the application of traditional imaging techniques in CSC. With the continuous improvement and progress of OCTA technology, traditional angiography combined with OCTA will bring great benefits to the diagnosis and treatment of CSC. This review summarizes the quantitative application of OCTA in the pathogenesis, diagnosis and treatment of CSC.
ObjectiveTo observe and analyze the clinical and imaging features of eyes with cystoid macular degeneration (CMD) secondary to chronic central serous chorioretinopathy (cCSC). MethodsA retrospective clinical study. From February 2018 to June 2023, 9 patients of 15 eyes with cCSC secondary CMD diagnosed by ophthalmology examination in Yunnan University Affiliated Hospital were included in the study. All patients were male. The age was (53.67±3.83) years. The cases of binocular and monocular were 6 and 3 respectively. The visual acuity of the affected eye ranges from 0.02 to 0.1, which cannot be corrected. Visual acuity decreased and the duration of shadow occlusion was ＞1 year. Half dose photodynamic therapy (PDT) was performed on 8 eyes. All the patients underwent the best corrected visual acuity, posterior mydriatic fundus color photography, infrared fundus photography (IR), fundus autofluorescence (AF), fluorescein fundus angiography (FFA), optical coherence tomography (OCT), and multi-wavelength dazzling imaging (MC). The patients who received half dose PDT were followed up until 3 months after treatment. Patients who did not receive treatment were followed up to 2 years after the first diagnosis. ResultsThe light reflection in macular area decreased or disappeared in all eyes, and abnormal macular pigmentation was observed in 12 eyes. IR examination showed diffuse patchy weak fluorescence in the macular area in all affected eyes, and dotted strong fluorescence in the periphery. Fundus AF examination showed disc-like weak AF in the macular area, and scattered small amounts of strong AF in the middle and margins, among which the retinal pigment epithelium (RPE) atrophy trace in the macular area was observed in 7 eyes. By MC examination, the green signal in the macular area of the posterior pole of all affected eyes was uneven and mottled. FFA examination showed that no abnormal fluorescein leakage was observed in 15 eyes and 8 eyes showed strong fluorescence caused by diffuse permeation fluorescence. A small amount of active fluorescein was found in 7 eyes. OCT examination showed that there were several cystic cavities of different sizes in all the affected eyes, RPE atrophied to different degrees, and RPE cell compensatory ridges and tubular structures in the outer retina were seen in 6 eyes; 7 eyes with CMD and active leakage showed signs of subcortical fluid accumulation. Choroidal hypertrophy was seen in all affected eyes, with significant expansion of the great vascular layer and compression of the middle vascular layer and capillary layer. In 8 eyes treated with half-dose PDT, 6 eyes were ineffective at 3 months after treatment. The treatment was effective in 2 eyes. In 7 eyes that did not receive half-dose PDT, CMD structure did not improve significantly after 2 years of follow-up. The visual acuity decreased with the prolongation of the disease. ConclusionsCMD is more common in cCSC with a long course of disease, which has significant effects on vision and poor prognosis. Fundus color photography shows that the reflection in the macular area of the pole is weakened or disappeared, which may be combined with macular abnormal pigmentation. IR and AF examination show uneven fluorescence in macular area. The green signal in macular area is not uniform according to MC inspection. FFA shows strong fluorescence caused by diffuse permeable fluorescence and fluorescein leakage in active lesions. OCT examination shows that multiple small sacs or connections between sacs were broken and fused, and RPE atrophied to varying degrees.