Dear Editor,We present a case of acute zonal occult outer retinopathy(AZOOR)complex in a myopic patient with angioid streaks(ASs).A 19-year-old female has been experiencing visual field defects in her left eye for mor...Dear Editor,We present a case of acute zonal occult outer retinopathy(AZOOR)complex in a myopic patient with angioid streaks(ASs).A 19-year-old female has been experiencing visual field defects in her left eye for more than 3y.She was diagnosed with ASs and choroiditis at a local hospital.She has a seven-year history of bilateral high myopia.A fundus examination confirmed the presence of ASs and myopic fundus changes in both eyes.Multimodal imaging revealed an AZOOR complex in the left eye.展开更多
AIM: To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision. METHODS: A case report RESULTS: A 48-year-old woman presented with bilateral blurry vision an...AIM: To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision. METHODS: A case report RESULTS: A 48-year-old woman presented with bilateral blurry vision and right eye metamorphopsia. The patient had a history of angioid streaks in the left eye ten years ago for which she had received laser surgery and had poor residual vision. Visual acuity was 20/60 in the right eye and count fingers at 6 feet in the left. Fundus examination showed subretinal hemorrhage and macular thickening on the right and a disciform macular scar with focal atrophic pigment epithelial lesions on the left. Both eyes had angioid streaks and peau d’orange pigmentary pattern of the retina. External examination showed several, yellow skin papules and plaques on the lateral and posterior neck, as well as prominent mental creases. Pathologic examination of skin biopsy confirmed the diagnosis of PXE, showing calcium deposition and fragmented, clumped elastic fibers in the deep reticular dermis. She responded well to intravitreal bevacizumab injections and visual acuity improved to 20/25 OD. Preventative care was emphasized and the patient was referred to cardiology, gastroenterology and human genetics for counseling. CONCLUSION: PXE is a multisystem disorder affecting the dermatologic, ocular, and cardiovascular systems. Ophthalmic findings of angioid streaks and choroidal neovascularization in the presence of stereotypical skin changes and prominent mental creases should prompt evaluation for PXE.展开更多
Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 - 100,000. PXE patients also carry...Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 - 100,000. PXE patients also carry an increased risk for cardiovascular and gastrointestinal diseases. Early diagnosis can play a crucial role in preventing early loss of vision and systemic complications in these patients however despite having characteristic clinical features;it is still to a large extent being under-diagnosed. Here we present two cases of PXE with reduced vision.展开更多
AIM:To evaluate the corneal biomechanical properties in patients with angioid streaks(AS),and to compare these parameters with those of healthy subjects.METHODS:A retrospective study was conducted,enrolling AS patient...AIM:To evaluate the corneal biomechanical properties in patients with angioid streaks(AS),and to compare these parameters with those of healthy subjects.METHODS:A retrospective study was conducted,enrolling AS patients and healthy participants as controls.The collected corneal tomographic parameters included flat keratometry,steep keratometry,mean keratometry,maximum keratometry,topographic cylinder value,central corneal thickness,and the total deviation value of the Belin-Ambrósio enhanced ectasia display measured with Pentacam tomography.Corvis ST was used to measure corneal biomechanical parameters,non-contact tonometry intraocular pressure(IOPnct),and biomechanically corrected intraocular pressure(bIOP).RESULTS:The study comprised 18 eyes from 10 male and 8 female AS patients with a mean age of 48.83±10.66y,and the controls included 31 eyes from 12 male and 19 female healthy participants with a mean age of 47.87±10.69y.All corneal tomographic parameters were comparable between the two groups(all P>0.05),and no corneal ectasia was observed in any AS patient.Compared with the controls,AS patients exhibited statistically significant increases in applanation 1(A1)time and stiffness parameter A1(SP-A1),along with significant decreases in applanation 2(A2)velocity,deformation amplitude(DA),DA ratio(2 mm),and Corvis biomechanical index(CBI;all P<0.05),which indicated higher corneal stiffness in AS.The IOPnct value in AS was significantly elevated,while no significant difference was found in the bIOP value compared to controls(P=0.031 and P=0.095,respectively).CONCLUSION:Eyes with AS exhibit normal corneal tomographic characteristics and increased corneal stiffness.展开更多
基金Supported by the National Natural Science Foundation of China(No.82171073).
文摘Dear Editor,We present a case of acute zonal occult outer retinopathy(AZOOR)complex in a myopic patient with angioid streaks(ASs).A 19-year-old female has been experiencing visual field defects in her left eye for more than 3y.She was diagnosed with ASs and choroiditis at a local hospital.She has a seven-year history of bilateral high myopia.A fundus examination confirmed the presence of ASs and myopic fundus changes in both eyes.Multimodal imaging revealed an AZOOR complex in the left eye.
文摘AIM: To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision. METHODS: A case report RESULTS: A 48-year-old woman presented with bilateral blurry vision and right eye metamorphopsia. The patient had a history of angioid streaks in the left eye ten years ago for which she had received laser surgery and had poor residual vision. Visual acuity was 20/60 in the right eye and count fingers at 6 feet in the left. Fundus examination showed subretinal hemorrhage and macular thickening on the right and a disciform macular scar with focal atrophic pigment epithelial lesions on the left. Both eyes had angioid streaks and peau d’orange pigmentary pattern of the retina. External examination showed several, yellow skin papules and plaques on the lateral and posterior neck, as well as prominent mental creases. Pathologic examination of skin biopsy confirmed the diagnosis of PXE, showing calcium deposition and fragmented, clumped elastic fibers in the deep reticular dermis. She responded well to intravitreal bevacizumab injections and visual acuity improved to 20/25 OD. Preventative care was emphasized and the patient was referred to cardiology, gastroenterology and human genetics for counseling. CONCLUSION: PXE is a multisystem disorder affecting the dermatologic, ocular, and cardiovascular systems. Ophthalmic findings of angioid streaks and choroidal neovascularization in the presence of stereotypical skin changes and prominent mental creases should prompt evaluation for PXE.
文摘Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elastic tissues of skin, eyes and blood vessels. Its estimated prevalence is thought to be 1:25,000 - 100,000. PXE patients also carry an increased risk for cardiovascular and gastrointestinal diseases. Early diagnosis can play a crucial role in preventing early loss of vision and systemic complications in these patients however despite having characteristic clinical features;it is still to a large extent being under-diagnosed. Here we present two cases of PXE with reduced vision.
文摘AIM:To evaluate the corneal biomechanical properties in patients with angioid streaks(AS),and to compare these parameters with those of healthy subjects.METHODS:A retrospective study was conducted,enrolling AS patients and healthy participants as controls.The collected corneal tomographic parameters included flat keratometry,steep keratometry,mean keratometry,maximum keratometry,topographic cylinder value,central corneal thickness,and the total deviation value of the Belin-Ambrósio enhanced ectasia display measured with Pentacam tomography.Corvis ST was used to measure corneal biomechanical parameters,non-contact tonometry intraocular pressure(IOPnct),and biomechanically corrected intraocular pressure(bIOP).RESULTS:The study comprised 18 eyes from 10 male and 8 female AS patients with a mean age of 48.83±10.66y,and the controls included 31 eyes from 12 male and 19 female healthy participants with a mean age of 47.87±10.69y.All corneal tomographic parameters were comparable between the two groups(all P>0.05),and no corneal ectasia was observed in any AS patient.Compared with the controls,AS patients exhibited statistically significant increases in applanation 1(A1)time and stiffness parameter A1(SP-A1),along with significant decreases in applanation 2(A2)velocity,deformation amplitude(DA),DA ratio(2 mm),and Corvis biomechanical index(CBI;all P<0.05),which indicated higher corneal stiffness in AS.The IOPnct value in AS was significantly elevated,while no significant difference was found in the bIOP value compared to controls(P=0.031 and P=0.095,respectively).CONCLUSION:Eyes with AS exhibit normal corneal tomographic characteristics and increased corneal stiffness.
