ObjectiveTo observe and investigate the related factors that might affect clinical features of familial exudative vitreoretinopaty (FEVR) patients. MethodsA retrospective chart study. From January 2012 and December 2021, 42 patients with 84 eyes with a diagnosis of FEVR from Department of Ophthalmology, Peking University People's Hospital were included in the study. The patients came from 42 separate families. There were 31 males and 11 females, with an average age of first diagnosis was 16.6±33.7 months. There were 21 patients referred from other hospitals for the fundus disease found in eye screening after birth, 21 patients were first seen in our hospital. There were 4 and 38 premature and full-term infants, respectively. Two patients with a positive family history of FEVR. All patients are FEVR stages 1-5. The wide-angle digital pediatric retinal imaging system after general anesthesia for fluorescein fundus angiography (FFA) examination were performed for patients aged ＜5 years. If patients ≥ 5 years old, routine FFA examination was performed. Sixty-eight first-degree relatives from 28 families undergo routine fundus examinations and FFA examination. Genetic examination was performed for 26 families, including 26 probands and 57 first-degree relatives. Genetic examination were performed on gene the coreceptor of low density lipoprotein receptor-associated protein 5 (LRP5), Wnt receptor coiled protein 4 (FZD4), Norrie disease (NDP), tetraporin 12 (TSPAN12), catenin β1 (CTNNB1) genes known to be involved in FEVR. The clinical features and the genotype of FEVR were observed in relation to the clinical phenotype. ResultsAmong the 42 patients, 13 patients were first observed by strabismus and nystagmus, with the median age of 12 months. Eight patients were complained non-chasing or vision-related symptoms. Among the 84 eyes, FEVR stage 1 or 2, 3 or 4, and 5 were 50 (59.5%, 50/84), 31 (36.9%, 31/84), and 3 (3.6%, 3/84) eyes, respectively. Among the 23 patients who were > 3 months at first diagnosis, 16 patients had at least one eye severer than stage 3 (69.6%, 16/23). Of the 68 first-degree relatives, 22 (32.4%, 22/68) had FEVR-like changes. Among the 26 families that underwent genetic detection, 13 families (50%, 13/26) of 16 variants of FEVR-related genes were detected, of which 10 mutations of LRP5 gene were the most common. There were 10 families with single gene mutations, including 6, 2 and 2 families of LRP5, FZD4 and CTNNB1 genes, respectively. One family of LRP5 gene mutations were compound heterozygous mutations, 1 family with LRP5 gene mutaition combined with NDP gene mutation, and 1 family with LRP5 and TSPAN12 gene mutation. Among the proband with FEVR pathogenic genes, 6 cases with similiar stage of both eyes, and 7 cases with inconsistent disease stages, and there was no obvious correlation between gene mutations and clinical phenotypes. ConclusionIn addition to the age of first diagnosis, no exact factors affecting the clinical manifestations of FEVR are found, and the association between clinical phenotypic and genetic heterogeneity still needs to be further explored.
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 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.
Objective To observe visual field outcome and refractive status of patients with retinopathy of prematurity (ROP) treated by laser photocoagulation. Method The data of 39 ROP patients (73 eyes) who received laser photocoagulation were retrospectively analyzed and compared with 13 normal control subjects (25 eyes) whose age and sex were matched with ROP group. There were 24 males (45 eyes) and 15 females (28 eyes) in ROP group, with an average age of (7.0±1.28) years. The first laser treatment was carried out at postnatal age (PA) of (38.74±3.82) weeks, the birth weight (BW) of (1402.33±369.61) g and the number of laser burns was (517.86±277.40). The control group included 7 females (13 eyes) and 6 males (12 eyes), with an average age of (7.17±0.96) years. The age (t=0.691) and gender (χ2=1.425) were comparable between the two groups (P=0.491, 0.233). The data of patients and controls were retrospectively analyzed including best corrected visual acuity, refractive examination, automated perimetry test. The differences of the mean deviation (MD) of visual field and the spherical equivalent (SE) between these two groups were comparatively observed. ROP patients were divided into no VF loss group (MD≤2 dB) and VF loss group (MD＞2 dB), mild VF loss group (MD≤6 dB) and moderate VF loss group (MD＞6 dB) according to the results of automated perimetry test, the differences of gestational age (GA), PA, BW, number of laser burns and SE between these groups were comparatively observed. Results The MD in ROP group and control group were 4.87±5.12 dB and 1.27±3.34 dB, respectively; the difference between the two groups was statistically significant (t=–4.01,P＜0.001). The subgroup analysis showed that BW, number of laser burns, and SE were significantly different between no VF loss group and VF loss group (t=2.074, –1.996, –2.162;P=0.042, 0.026, 0.034); while the GA was not significantly different between these two groups (t=1.973,P=0.052). The difference of PA was not statistical significant different between mild VF loss group and moderate VF loss group (t=2.03,P=0.051) and SE was significantly different between the above two groups (t=3.283,P=0.002). For refractive outcomes, the BW and ROP stage correlated with SE significantly (r=–0.304, –0.387;P=0.015, 0.002). The mean BCVA in ROP group was 0.84±0.23, and 59 eyes (91.2%) with BCVA better than 0.5. Conclusion Laser treatment for ROP tends to have less effect on long term refractive status and VF loss, with good visual outcome.
ObjectiveTo explore the outcome of extended internal limiting membrane (ILM) peeling combined with releasing the edge technique for primary failed idiopathic macular hole (IMH) surgery.MethodsA retrospective analysis was performed. The data of 18 eyes of 18 IMH patients who were failed in primary surgery from August 2013 to June 2019 in Peking University People’s Hospital were enrolled in the study. Among them, 5 patients were males and 13 patients were females. The average age was 66.2±6.4 years. The BCVA were measured by ETDRS charts. The minimum macular hole size was measured on OCT B-scan image. The average preoperative BCVA and minimum macular hole size of primary surgery was 32.6±13.1 letters and 621.1±161.8 μm. The average preoperative BCVA and minimum macular hole size of second surgery was 34.4±12.3 letters and 499.0±148.6 μm. Average interval period of first and second surgery was 3.4±1.3 weeks. The surgical technique used in the reoperation included the extended ILM peeling combined with releasing the MH edges. The extended ILM peeling area ranged from 4 DD diameter to vascular arcades. The technique of releasing the macular hole edges was performed by using a silicone soft-tip extrusion cannula, with which tapping the edges softly or aspirated vacuum the edges concentricly. The average follow-up was 9.3±5.2 months. The clear OCT image can be obtained for confirming MH closure which was considered as the closure time in the first time. The comparison of preoperative and postoperative was performed by paired t-test.ResultsThe closure rate of second surgery was 94.4% (17/18), and average closure time was 2.1±1.0 weeks. Only 1 eye experienced the second surgical failure and received the third surgery to achieved macular hole closure. Average final follow-up BCVA was 51.5±13.0 ETDRS letters, with average improvement of 18.9 ETDRS letters (＞3 lines) compared with preoperative BCVA of primary surgery. There was significantly statistical difference between the final BCVA and preoperative BCVA (t=5.412, P＜0.001). Eleven patients (61.1%) had 3 lines BCVA improvement, 15 patients (83.3%) had more than 1 line improvement, and 3 patients (16.7%) had on improvement. The final BCVA of patients significantly improved compared with preoperative BCVA of the second surgery (t=7.595, P＜0.001), with average improvement of 17.1±9.5 letters.ConclusionThe extended ILM peeling combined with releasing macular hole edges technique is effective to improve the closure rate and BCVA of primary failed IMH eyes.
ObjectiveTo investigate and analyze the clinical manifestations and imaging features of the eyes with bullous retinal detachment. MethodsRetrospective case series study. Eleven eyes of 11 patients with bullous retinal detachment diagnosed in Department of Ophthalmology, Peking University People's Hospital from July 2015 to September 2021 were enrolled. There were 10 males and 1 female, with the mean age of (39.27±6.81) years. All patients had monocular bullous retinal detachment, with mean duration ranged from 3 months to 14 years. The basic information and medical history of all patients were collected. All patients underwent best corrected visual acuity (BCVA), indirect ophthalmoscopy, color fundus photography, optical coherence tomography (OCT), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and B-scan ultrasonography. BCVA was performed using a standard logarithmic visual acuity chart, which was converted to logarithm of the minimum angle of resolution (logMAR) visual acuity. The clinical data and imaging features of BCVA, OCT, FFA and ICGA were retrospectively analyzed and summarized. ResultsThe mean logMAR BCVA of the 11 eyes was 0.91±0.45. Nine patients had bilateral disease, but bullous retinal detachment occurred in only 1 eye, and CSC manifestations were present in the contralateral eye. Six patients had received systemic or topical hormone therapy prior to onset. Yellowish-white material was observed in 6 eyes and retinal folds were observed in 5 eyes. OCT examination showed serous retinal detachment in the macular area with granular or patchy hyperreflective signals in the subretinal area in all eyes, and a few granular hyperreflective substances in the neuroretina in 6 eyes. Neuroretina cystoid degeneration was observed in 6 eyes, adhesion between the detached neuroretina and retinal pigment epithelial (RPE) was observed in 6 eyes, RPE tear was observed in 6 eyes, and different forms of retinal pigment epithelial detachment (PED) were observed in 6 eyes. FFA showed multiple fluorescence leakage spots in 10 eyes, and the average number of fluorescence leakage spots in all eyes was 3.82±2.44. There were multiple diffuse RPE lesions in 9 eyes. The results of ICGA examination showed that choroidal vessels were dilated and multiple hyperfluorescent leaks were observed in all eyes. B-scan ultrasonography examination of all affected eyes showed retinal detachment. Retinal reattachment can be achieved at (2.0±1.0) months after photodynamic therapy (PDT), while SRF can be completely absorbed at (2.36±0.81) months. The mean logMAR BCVA can be improved to 0.50±0.33, and no recurrence was found in the follow-up period up to 6 months. ConclusionsBullous retinal detachment is often associated with the use of hormones, while yellow-white material in the subretina and hyperreflective material in the OCT are common. It is characterized by neuroretina cystoid degeneration in the macular area, adhesion between the neuroretina and RPE, RPE tear and PED, with multiple fluorescence leakage spots and diffuse RPE lesions. PDT is an effective treatment for bullous retinal detachment.
Objective To compare the predicted efficiency of macular hole closure index (MHCI) calculated by 2 different methods for postoperative anatomical outcomes after idiopathic macular hole (MH) surgery. Methods This is a prospective exploratory clinical study. A total of 63 patients (63 eyes) with idiopathic MH, who received vitrectomy, inner limiting membrane peeling and gas tamponade, were enrolled in this study. All the patients received optical coherence tomography (OCT) examination at each visit to measure the MHCI using the formula MHCI=（M+N）/BASE, M and N is the distance from outer limiting membrane break points to the beginning points of detached photoreceptor from retinal pigment epithelium of both side of the hole, respectively. BASE is the length of MH base. MHCI1 was measured by built-in caliper of OCT software, MHCI2 was measured by ImageJ software. The minimum macular diameter (MHD) was measured by built-in caliper of OCT software. Based on the OCT images, the anatomical outcomes were classified grade A (bridge-like shape closure), grade B (complete closure) and grade C (poor closure). Grade A and B are considered as good closure, grade C as poor closure. Patients were followed up at 3, 6 and 12 months after surgery. The closure grades at last visit were the final outcome. The relationship between MHCI1, MHCI2 and closure grades was analyzed. And the predicted efficiency of MHD, MHCI1 and MHCI2 for anatomical outcomes after the surgery was studied. Results The mean MHCI1 was 0.68±0.21 (0.30-1.35), MHCI2 was 0.95±0.26 (0.41-1.55), and MHD was (476.24±210.18) μm (127-956 μm). MHCI1 and MHCI2 were both negative correlated with the closure grades (r=−0.665, −0.691; P＜0.001). The receiver operating characteristic (ROC) curve analysis of MHCI1, MHCI2 and MHD for the prediction of good or poor closure showed that area under the curve (AUC) was 0.928, 0.957 and 0.916 respectively, and 0.505, 0.67 and 559 μm were set as the lower cut-off value. The sensitivity was 96.2%, 92.3% and 90.9% respectively, and specificity was 81.8%, 72.7% and 76.9% respectively. Accordingly, the ROC curve analysis for the prediction of grade A or B closure showed that AUC was 0.840, 0.847 and 0.653 respectively, and 0.705, 0.965 and 364 μm were set as the upper cut-off value. The sensitivity was 80.0%, 82.9%, 63.4% respectively and specificity was 75.0%, 85.7%, 65.9%. Conclusion MHCI1 and MHCI2, measured by built-in caliper of OCT software or ImageJ software, both have good predictive efficiency for the anatomical outcomes of MH surgery.
ObjectiveTo observe the changes of retinal microstructure in lamellar macular hole (LMH) after vitrectomy.MethodsA retrospective clinical observational study. Forty patients (41 eyes) with LMH and received vitrectomy in Ophthalmology Department of Peking University People’s Hospital from January 2014 to September 2018 were included in this study. Among them, 14 patients (15 eyes) were males and 26 patients (26 eyes) were females, with an average age of 67.8±8.6 years. There were 37 eyes with a lens and 4 eyes with an IOL. There were 29 eyes with LMH of tractional type, 7 eyes of degenerative type, and 5 eyes of mixed type. All patients underwent BCVA and OCT examinations. The BCVA examination was performed using the international standard visual acuity chart, which was converted into logMAR visual acuity. The average logMAR BCVA was 0.57±0.27; the mean macular retinal thickness (CRT) was 192.3±108.9 μm, the mean macular thickness (MRT) was 427.5±110.2 μm. Among the 29 eyes of tractional type, there were 17 eyes with retinal cavity, 8 eyes with macular retinoschisis, and 3 eyes with incomplete ellipsoid zone. Among the 7 eyes of degenerative type, there were 5 eyes with lamellar hole-associated epiretinal proliferation (LHEP), 5 eyes with retinal cavity, and 5 eyes with incomplete ellipsoid zone. Among the 5 eyes of mixed type, 2 eyes with LHEP, 1 eye with macular epiretinal membrane, and 4 eyes with incomplete ellipsoid zone. The average follow-up time after surgery was 12.8±5.2 months. Among them, 10 eyes were followed up for equal or greater than 24 months. After the surgery, the same equipment and method before the surgery were used for relevant examination. The changes of BCVA, CRT, and MRT before and after surgery were observed. Continuous variables were compared by t test.ResultsAt the last follow-up, the mean logMAR BCVA was 0.37±0.26. Compared with before surgery, the difference was statistically significant (t=5.98, P＜0.01). The mean CRT and MRT were (245.2±90.8) and (347.0±46.7) μm, respectively. Compared with before surgery, the differences were statistically significant (t=-2.49, -5.24; P＜0.05, ＜0.01). CRT and MRT changed greatly within 6 months after surgery, and then tended to be gentle. Among the 3 eyes with incomplete ellipsoid zone of tractional type before surgery, ellipsoid zone recovered in 2 eyes and partially recovered in 1 eye. Among the 17 eyes with retinal cavity and 8 eyes with macular retinoschisis before surgery, there were still 4 eyes with retinal cavity, but all the retinoschisis were disappeared. Among the 5 eyes with retinal cavity of degenerative type before surgery, there were still 2 eyes with retinal cavity and all the eyes with incomplete ellipsoid zone. Among 10 eyes with a follow-up time of equal or greater than 24 months, the macular ganglion cell complex partially atrophied in 6 eyes, and the nerve fiber layer separated in 2 eyes. There was no full-thickness macular hole after surgery.ConclusionFor most LMH patients, vitrectomy can effectively improve the visual acuity and promote the recovery of retinal microstructure.