Coats disease is a relatively rare and idiopathic disorder characterized by retinal telangiectasia and massive intra-retinal and (or) sub-retinal lipid accumulation, resulting in complications including retinal detachment and neovascular glaucoma. Previous reports have revealed that Coats disease can be associated with other disorders, especially some inherited diseases, such as retinitis pigmentosa (RP) and facioscapulohumeral muscular dystrophy (FSHD). Coats disease associated with other inherited disorders is generally called Coats-like retinopathy, which has some unique features that differs from the classic Coats disease, for example there is no sex and age preference, more bilateral cases, more severe cases and more genetic factors involved. Patients of Coats-like retinopathy with RP and FSHD may have mutations in Crumbs homologue gene 1 and D4Z4 genes.
ObjectiveTo observe the multimodal imaging characteristics of choroidal metastasis.MethodsA retrospective clinical observation study. From January 2016 to November 2018, 28 patients with choroidal metastasis diagnosed in Department of Ophthalmology in the Second People’s Hospital of Yunnan Province were included in the study. There were 12 males and 16 females, with the mean age of 50.8±6.9 years. There were 18 unilateral patients and 10 bilateral patients. The lesion of choroidal metastasis was regressed after systemic antitumor therapy in 3 patients (4 eyes). All patients underwent ultra-wide-angle fundus photography, infrared fundus imaging, fundus autofluorescence, FFA, frequency-domain OCT, and B-ultrasound examinations.ResultsIn the ultra-wide-angle fundus photography, metastatic tumors were located in the posterior or middle part of the retina, of which 26 were isolated lesions and 12 were multifocal. A yellow-white bulge lesion with (11 eyes) or without pigmentation (27 eyes). There were 12 eyes with exudative retinal detachment. Infrared photography of the fundus showed that the tumor area showed varying degrees of mottled brightness change, and the infrared photograph of the exudative retinal detachment area was relatively low. Fundus autofluorescence showed that 14 eyes had plaque-like strong autofluorescence in the tumor, 13 eyes had a mottled autofluorescence formed by strong and weak fluorescence in the tumor; 3 eyes of old lesions showed " leopard-like” autofluorescence. Among the 38 eyes in the fluorescein angiography, 32 eyes of the early lesions showed low fluorescence, and the venous phase showed a needle-like high fluorescence point, and the post-leakage fluorescence gradually increased. Two eyes with old lesions showed a " leopard-like” change. In 38 eyes, OCT showed wavy ridges of the choroid and pigment epithelium, and a large number of fine-grained or cluster-like high-reflector accumulations were observed between the retinal neuroepithelial layer and the pigment epithelial layer. B-ultrasound showed substantial lesions in the posterior pole and uniform internal echo. There were 23 eyes with flat shape, 12 eyes with flat hemisphere, and 3 eyes with irregular shape.ConclusionsColor photography of the fundus showed the size, location, pigmentation and peripheral retinopathy of the metastatic lesions. Infrared photography showed different reflex signals in the tumor, exudation, and atrophy. The autofluorescence of the fundus showed the damage of pigment epithelium in the lesion. In the fluorescein angiography, the fresh tumor showed fluorescence leakage, while the atrophic tumor showed transmitted fluorescenc. OCT reflected the height of the lesion and the change of pigment epithelium.
ObjectiveTo observe the OCT angiography (OCTA) features of adult-onset foveomacular vitelliform dystrophy (AFVD).MethodsRetrospective clinical observational study. Twelve patients (22 eyes) diagnosed as AFVD by multi-modal imaging in Ophthalmology Department of Yunnan Second People’s Hospital from March 2018 to May 2019 were included in this study. There were 8 males (16 eyes) and 4 females (6 eyes). The patients aged from 33 to 62 years, with the mean age of 48.7±8.9 years. Ten patients were binocular, 2 patients were monocular. The visual acuity was 0.08-0.6. In 22 eyes, the vitelloid-like substance was relatively complete in 8 eyes, the vitelloid-like substance had different degrees of rupture in 14 eyes, secondary choroidal neovascularization (CNV) was observed in 10 eyes. The Heidelberg OCTA instrument was used for OCTA examination. The central wavelength was 840 nm, the acquisition speed was 85,000 times/s. A 3 mm × 3 mm scan was obtained. In the scanning process, eye-tracking technology was adopted to select images with better image quality and position for marking and saving. The image characteristics of vitelloid-like substance, fundus vascular changes and secondary CNV in OCTA were analyzed.ResultsIn 8 eyes with a relatively complete vitelloid-like substance, B-scan images showed dense vitelloid-like substance under the retinal neurocortical layer, which was located between the RPE layer and the ellipsoid zone and had a uniform density. Blood flow signals at the vitelloid-like substance can be seen in the en-face image, which was the artifact of the vitelloid-like substance reflecting the blood vessels above. In the 14 eyes with different degrees of vitellin-like material rupture, the signal of vitellin-like substance between the ellipsoid zone and the RPE layer in the B-scan image was not uniform, and some weak reflected signal lacunae could be seen. In the image of en-face, the relatively intact areas of vitelloid-like substance still showed the artifact of the blood vessels above the reflection, while there was no blood flow signal at the rupture of vitelloid-like substance. In 22 eyes, the morphology of retinal small blood vessels in the superficial and deep capillary arch ring region of retina was abnormal in 10 eyes. Some small blood vessels could be seen to have branch and shape changes, and the anastomosis failed to show a complete arch ring structure.No significant structural changes in retinal capillaries were observed in 12 eyes. Among the 10 eyes with secondary CNV, 8 eyes showed the non-active CNV which was as thick as "wild branches", and 2 eyes showed the active CNV which was composed of dense and small vascular branches.ConclusionAFVD in OCTA can be manifested as abnormal retinal vascular morphology caused by the vitelliform material pushing, vascular artifacts reflected by the vitelliform material itself, and the presence of CNV under the vitelliform material.
ObjectiveTo observe the multimodal imaging features of the eyes with acute syphilitic post-polar squamous chorioretinitis (ASPPC) at different stages of disease.MethodsA retrospective case study. From July 2016 to March 2019, 8 patients (11 eyes) of ASPPC patients diagnosed in the ophthalmological examination of Yunnan Second People's Hospital were included in the study. Among them, there were 7 males (10 eyes) and 1 female (1 eye); the average age was 48.7±8.9 years; the average course of disease was 13.24 ±11.30 months. All patients underwent fundus color photography, infrared photography (IR), FAF, FFA, OCT, OCT angiography (OCTA). According to the stage and characteristics of the disease, the affected eyes were divided into acute phase and absorption phase, with 7 and 4 eyes respectively. We observed the color fundus images of ASPPC, IR, FAF, FFA, OCT, OCTA image characteristics of different disease stages.ResultsIn the acute phase, the posterior pole subretinal yellow-white squamous lesions, neuroepithelial detachment, and yellow-white exudates were observed in fundus color photography; uneven infrared reflections can be seen in the lesion area by IR; the posterior pole was round or scaly with strong autofluorescence in FAF, the range was larger than the fundus color photography; FFA arteriovenous stage lesions showed fuzzy weak fluorescence, the fluorescence gradually increased with time, the late stage showed a round-shaped strong fluorescence, surrounded by a weak fluorescence ring, and the area with thick exudation was covered by fluorescence; the neuroepithelium of the diseased area was detached, the uniform strong reflection signal can be seen in it by OCT. In the absorption phase, fundus color photography showed the yellow-white scaly lesions under the posterior retina absorption, and the pigment was slightly depleted; IR showed the mottled infrared reflection in the lesion area was significantly reduced compared with the acute phase; FAF showed the posterior spot-like strong autofluorescence, including "leopard spot-like changes" 3 eyes; FFA showed mottled fluorescent staining in the lesion, and no fluorescein leakage or accumulation; OCT showed needle-like protrusions in the RPE layer, and the outer membrane and ellipsoid zone were unclear; OCTA showed weakened choroidal capillary blood flow signal, the signal was missing in some areas.ConclusionsIn the acute phase of ASPPC, the posterior pole subretinal shows yellow-white squamous lesions, neuroepithelial detachment, yellow-white exudate, FFA shows late fluorescein leakage in the lesion area; in the absorption period, the fundus shows yellow-white lesions have been absorbed, and FFA shows fluorescence dyed without any leakage. OCT indicates that the RPE, outer membrane and ellipsoid zone are damaged to varying degrees. OCTA indicates that the choroid of the diseased area had weakened blood flow signal.
ObjectiveTo observe the imaging characteristics of fundus choroidal nodules in patients with neurofibromatosis type 1 (NF1). MethodsA retrospective clinical study. From January 2018 to August 2022, 20 eyes of 10 patients with NF1 combined with choroidal nodules who were diagnosed by ophthalmology examination at the Affiliated Hospital of Yunnan University were included in the study. Among them, there were 6 male cases with 12 eyes and 4 female cases with 8 eyes; both eyes were affected. Age was (28.0±6.9) years old. Both eyes were involved. All patients underwent color fundus photography, infrared fundus photography (IR), fundus autofluorescence (FAF), fluorescein fundus angiography (FFA), and optical coherence tomography (OCT). Nine eyes underwent multi-wavelength color imaging (MC) and 5 eyes underwent OCT angiography (OCTA). ResultsIn 20 eyes, fundus color photography showed "spiral-like" changes in the small retinal blood vessels on the surface of the choroidal nodules in 1 eye. FAF and FFA examination showed no abnormalities in all affected eyes. On IR examination, choroidal nodules appeared as strong reflective lesions of varying sizes and numbers, in the form of spots and/or sheets, and were partially fused. In the 9 eyes that underwent MC examination, patchy red signals was observed in standard MC images. OCT examination showed that all affected eyes had strong choroidal reflective mass lesions under the retinal pigment epithelium, which were flat patchy or slightly raised “dome-like”, corresponding to IR strong reflective lesions. The choriocapillaris layer was squeezed and thinned, and the large choroidal vessels show weak reflection. Five eyes underwent OCTA examination, there was no loss of blood flow density at the choroidal nodules and the of the superficial an deep retinal capillary plexus in 3 eyes. The choroidal capillary blood flow density was reduced in 2 eyes. ConclusionIR of choroidal nodules is characterized by strong reflection lesions of varying sizes and numbers, which appear in spots and/or sheets. OCT shows enhanced reflection of the choriocapillaris layer corresponding to the strong IR reflection lesions.
ObjectiveTo enhance the understanding of pulmonary epithelioid hemangioendothelioma (PEH), and improve the diagnostic rate of the disease. MethodsThe experience of diagnosis and treatment of a case of PEH in August 2013 was reported in the present study and the related literature was reviewed. The etiology, clinical manifestations, pathological diagnosis, differential diagnosis, therapy and prognosis of this disease were described. ResultsThe etiology of PEH remained unknown. Symptoms of PEH were usually nonspecific and mild. Chest radiograph or computed tomography usually revealed calcification in multiple nodules of half or both lungs. Primitive lumen formed by a single cell was the pathologic feature. Immunohistochemical stains showed that the malignant cells were of endothelial type. Genetic analysis was relatively limited currently. There was no effective treatment for this disease and the prognosis was poor. ConclusionPEH is a low potential malignancy occurring exclusively in young women. Symptoms of PEH are usually nonspecific and variable which can easily lead to misdiagnosis. So, pathological diagnosis should be emphasized.
Objective To observe the OCT angiography imaging features of choroidal neovascularization (CNV) with different activity in age-related macular degeneration (AMD). Methods A retrospective case analysis. Forty-two eyes of 33 patients (21 males and 12 females, aged 65.3±8.61 years) who were diagnosed with AMD by multi-mode fundus imaging examination at the Ophthalmology Department of Yunnan Second People's Hospital during January 2017 and October 2018 were enrolled in this study. All patients underwent BCVA, slit-lamp biomicroscopy, indirect ophthalmoscopy, fundus colorized photography, FAF, FFA and OCT examinations. The patients were divided into active CNV (27 eyes of 19 patients) and inactive CNV (15 eyes of 14 patients) by comprehensive analysis of fundus imaging characteristics and treatment process. The imaging features of OCTA in the two groups were compared. The number of eyes of each active or inactive indicator in the active CNV group and the inactive CNV group was calculated, and the composition ratio of each group of the indicators was subjected to the χ2 test. Results Among the 27 eyes of active CNV, 22 eyes (81.5%) of OCTA showed abundant small capillary branching structure, while 13 eyes (13.3%) of 15 eyes of inactive CNV showed more coarse blood vessel. Among the 27 eyes of active CNV, 26 eyes (96.3%) of OCTA showed that the marginal vascular end points of CNV lesions were "arcaded" or "ring", while 12 eyes (80.0%) of 15 eyes of inactive CNV showed the presence of isolated branches of peripheral vessels. Among the 27 eyes with active CNV lesions, there were no large feeder vessels inside the lesions, and 8 (53.3%) of the 15 inactive CNV lesions showed feeder vessels in the center of the lesion. Among the 27 eyes with active lesions, 23 eyes (85.2%) of OCTA showed a low-reflection "halo" around the CNV lesion, and no low-reflection "halo" structure was observed in the 5 eyes of the inactive CNV lesion. The statistical results showed that there were abundant small blood vessel branches (χ2=22.759, P=0.000), annular anastomosis around the lesion (χ2=31.704, P=0.000), low-reflection halo (χ2=32.327, P=0.000), and large nourishing blood vessels (χ2=26.063, P=0.000), dilated choroidal vessels (χ2=32.912, P=0.000). All the above indicators were statistically different between the two groups. Conclusion The abundant small vessel branches in OCTA, the surrounding anastomosis in a ring structure and the low reflex halo around the lesion are markers of active CNV, while the large feeding vessels and dilated choroidal vessels are indicators of inactive CNV.
ObjectiveTo analyze the OCT angiography (OCTA) features of eyes affected with angioid streaks.MethodsRetrospective analysis of 26 patients (52 eyes) diagnosed as angioid streaks by multi-modal imaging in Ophthalmology Department of Yunnan Second People’s Hospital from May 2017 to February 2019 were included in this study. There were 18 males and 8 females, with the mean age of 50.8±6.9 years. All the patients were binocular. There were 34 eyes in 20 patients with CNV. Among them, 13 eyes had a course of disease within 1 month, 16 eyes had a course of disease over 1 month, and 5 eyes were treated with anti-VEGF drugs. All patients were examined by ultra-wide-angle fundus photography, infrared fundus imaging (IR), spectral-domain OCT, FAF and FFA. At the same time, the Heidelberg OCTA instrument was used for OCTA examination. The central wavelength was 840 nm, the acquisition speed was 85,000 times/s, and the width was 45 nm. A 3 mm × 3 mm scan was obtained. Each cube consisted of two 304 B scans of repeated volumes, and motion correction was performed using two orthogonally captured image volume registration. The results of fundus color photography, IR, FAF, FFA, OCT and OCTA were compared and analyzed to summarize the image features of AS and its secondary CNV in OCTA.dus color photography, IR, FAF, FFA, OCT and OCTA were compared and analyzed to summarize the image features of AS and its secondary CNV in OCTA.ResultsAmong 52 eyes, 40 eyes showed choroidal capillary shadows in OCTA, and no obvious abnormal OCTA images in 12 eyes. Ten eyes OCTA showed a vascular network beseide the optic disc, FFA showed fluorescent staining instead of fluorescein leakage. Among the 52 eyes, 34 eyes had secondary macular CNV, and 34 eyes had different forms of CNV. Different forms had a certain correlation with the length of medical history and treatment history. Among them, 13 eyes with short course of disease (less than 1 month) and untreated patients had smaller flower-ring morphology, 16 eyes with longer course (more than 1 month) but no treatment had larger fan-shaped segments, and 5 eyes with anti-VEGF therapy were trimmed after the dendritic shape.ConclusionsAS streaks appeared no blood flow signal area in OCTA, and repairing vascular network beside the optic disc can be observed in some AS patients. The CNV performance with different disease course and treatment experience is different.