Adult-onset foveomacular vitelliform dystrophy (AFVD) is characterized by gradually loss of vision, subretinal vitelliform macular lesions and retinal pigment epithelium (RPE) atrophy. The electrooculogram (EOG) is usually normal or reduce slightly. On optical coherence tomography (OCT), there are vitelliform substance which deposited between the photoreceptor layer and the RPE layer, and thinner outer nuclear layer above these deposits. OCT angiography can show the superficial and deep retinal capillaries plexus (DCP) above the yolk-like deposits, the choroidal capillary blood flow is reduced and the DCP blood flow is increased near macular. On indocyanine green angiography (ICG), there is choroidal neovascularization under vitelliform substance at early stage and fluorescence leakage in the corresponding retinal region at late stage. There is no effective treatment for AFVD at present, monogenic gene therapy is the most worth looking forward to. Understanding AFVD clinical manifestations, multi-mode imaging features and new advances in treatment can provide a reference for diagnosis and treatment options
Purpose To identify and quantitatively evaluate age-related changes in the retinal pigment epithelium (RPE) and underlying Bruch is membrane and choroid in donor human eyes. Methods 36unpaired human eyes of varying age (3-39 years) from Caucasian donors were supplied by Manchester Eye Bank (UK) or National Disease Research Interchange (Philadephia,USA).Modified Masson is trichrome staining was used to illustrate age-related changes in RPE cell, Bruch is membrane thickness, and density of choriocapillaries and thickness of the choroid. Data were assessed using computer-aided quantitative morphometric analysis method. ResultsThe thickness of Bruch is membrane increased with age while there is a change in morphology of RPE cells including a decrease in number and RPE cell thickening with age. RPE cells decreased at a rate of 8 cells/mm2 middot; year, RPE cell height and thickness of Bruch is membrane increased at rates of 0.01(mu;m/year) and 0.02 (mu;m/year) respectively. The luminal area of choriocapillaries and the thickness of choroid showed no close relation with age. Conclusion RPE cell loss and thickening of Bruch is membrane and RPE cells may be the earlier and primary alteration with age. (Chin J Ocul Fundus Dis,2000,16:236-239)
ObjectiveTo observe the alterations of vascular density of superficial or deep retinal vascular plexus and the area of foveal avascular zone (FAZ) in acute central serous chorioretinopathy (CSC) eyes.MethodsThis is a retrospective study including 22 patients with unilateral CSC in acute stages. The patients were divided into group A (22 affected eyes) and B (22 fellow eyes). Twenty eyes of 20 age and gender-matched healthy volunteers (group C) were enrolled in this study. All subjects underwent optical coherence tomography (OCT) angiography examinations. The observation index included signal intensity of blood flow in choroidal capillary, vessel density and FAZ on the superficial capillary plexus (SCP) and deep capillary plexus (DCP).ResultsThe FAZ on DCP in group A was larger than that in group B and C (t=4.28, 5.57; P=0.00、0.00), but there was no significant difference of FAZ on SCP among 3 groups (t=0.28, 0.80; P=0.78, 0.43). The vessel density of SCP and DCP in group A and B were decreased than those in group C (t=−4.40, −2.91; P=0.00, 0.00). The type of choroidal capillaries dilation had two different manifestations by OCTA, which was circumscribed in 12 eyes and diffuse in 10 eyes. The subretinal fluid in macular area which presented dark chamber with weak reflection was observed by en-face OCT.ConclusionsThe vessel density of SCP and DCP in both eyes of acute CSC are lower than those in normal eyes. The FAZ of DCP in affected eyes of acute CSC is larger than that in fellow eyes and normal eyes. The blood flow of dilated choroidal capillary can be manifested as different morphological enhancements.
ObjectiveTo observe and analyze the image characteristics of eyes with choroidal osteoma using swept-source OCT (SS-OCT).MethodsSeventy-three eyes of 61 patients with choroidal osteoma were enrolled in the study, including 17 males (20 eyes) and 44 females (53 eyes) with an average age of 33.5±12.7 years. Single tumor was found in 71 eyes and multiple tumors were found in 2 eyes. All patients received examinations of slit lamp ophthalmoscope, color fundus photography, fundus autofluorescence, fluorescein angiography and/or indocyanine green angiography, B-scan ultrasonography and/or computerized tomography. Tumor features were characterized using SS-OCT.ResultsAmong 75 tumors of 73 eyes, the osteoma was completely calcified in 7 tumors, completely decalcified in 5 tumors and all the other tumors were composed of calcified and decalcified portions. SS-OCT revealed normal inner retina in all the calcified areas of tumors and abnormal outer retina including external limiting membrane, ellipsoid zone, interdigitation zone and RPE in some cases. Whereas the outer retina and RPE showed abnormality in the decalcified areas of all cases. The choriocapillaris was invisible in all cases and most of the medium and large caliber vessels showed thinning or nonvisibility. Choroidal osteoma revealed a sponge-like appearance (n=39), loofah sponge appearance (n=11), lamellar pattern (n=10), mixed (n=12) and irregular patterns (n=5). Other unique features included horizontal lamellar lines (n=71), hyperreflective horizontal lines (n=47) and hyporeflective tubules (horizontal:n=39, vertical:n=42, circular:n=41) within choroidal osteoma. Hyperreflective tumor-like tissues were also detected above the disrupted Bruch’s membrane. The sclero-choroidal junction was detectable in all tumors and a posterior ciliary vessel penetrating the sclera was also seen.ConclusionsSS-OCT clearly reveals characteristic appearances of choroidal osteoma as sponge-like, loofah sponge, lamellar pattern, mixed and irregular patterns, with unique features of horizontal lamellar lines, hyperreflective horizontal lines and hyporeflective tubules within osteoma. Hyperreflective tumor-like tissues were detected to grow above the disrupted Bruch’s membrane.