Angioid Streaks

IntroductionThe purpose of the study is to characterize the rate of progression of geographic atrophy (GA) areas in patients with age-related macular degeneration (AMD) with subclinical angioid streaks (AS), compared to patients with AMD without subclinical AS.MethodsThis is a retrospective, longitudinal, case–control study. Among a cohort of patients with AMD, we selected patients with GA with subclinical AS and followed them for a 2-year follow-up. An age- and sex-matched control group with GA secondary to AMD without subclinical AS was selected. Demographics and differences in the GA progression between the two groups were analyzed.ResultsAmong 60 eyes of 60 patients affected by GA secondary to AMD, 20 eyes of 20 patients (mean age 82 ± 5 years old) were included in the subclinical AS group, whereas 40 eyes of 40 patients (mean age 79 ± 6 years old, p = 0.077) were in the control group.All 20 eyes of subclinical AS group showed reticular pseudodrusen at the baseline compared to 73% of patients without AS (p = 0.002). In the subclinical AS group, 90% of eyes showed peripapillary atrophy in comparison to 63% in the control group (p = 0.026). Subclinical AS eyes showed a significantly lower subfoveal choroidal thickness in comparison to the control group (124 ± 60 μm vs. 161 ± 84 μm, respectively, p = 0.043). At 2-year follow-up, the rate of progression was higher in the patients with subclinical AS; the yearly growth rate was 0.41 ± 0.17 mm/year after the square root transformation in the subclinical AS group, in comparison to 0.32 ± 0.14 mm/year in the control group (p = 0.017).ConclusionsPatients with subclinical AS showed a more aggressive phenotype of GA in comparison to AMD patients without subclinical AS, characterized by a higher rate of progression of GA areas during a 2-year follow-up.

Purpose: to quantitatively analyze choriocapillaris perfusion using swept-source optical coherence tomography angiography (SS-OCTA) in eyes presenting with angioid streaks in comparison with control eyes. Methods: Macular 6 × 6 mm SS-OCTA scans were retrospectively analyzed in eyes with angioid streaks and in control eyes. En face choriocapillaris flow images were compensated with en face choriocapillaris structure images, followed by the Phansalkar local thresholding method (with a window radius of four and eight pixels). Quantitative analysis was performed in the four peripheral 1 × 1 mm corners of the 6 × 6 mm SS-OCTA image to include equidistant and comparable regions. The percentage of flow deficits (FD%), the number and size of the flow deficits (FDs) and the total area of FDs were then calculated. Results: 54 eyes of 31 patients were included in the study: 27 eyes diagnosed with angioid streaks and 27 controls. Analysis of the four 1 × 1 mm peripheral corners of the 6 × 6 mm SS-OCTA image showed that eyes with angioid streaks had a higher FD% compared to the control group (47.62 ± 8.06 versus 38.90 ± 6.38 using a radius of four pixels (p < 0.001); 48.37 ± 7.65 versus 39.66 ± 6.51 using a radius of eight pixels (p < 0.001). The average size of FDs as well as the total area size of the FDs were significantly higher in eyes with angioid streaks compared to control eyes (p < 0.001). Eyes with angioid streaks present reduced choriocapillaris flow compared to control eyes. Decreased choriocapillaris perfusion may contribute, among other factors, to the development of neovascularization and atrophy in patients with angioid streaks.

To evaluate the corneal biomechanical properties in patients with angioid streaks (AS), and to compare these parameters with those of healthy subjects. 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). 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). Eyes with AS exhibit normal corneal tomographic characteristics and increased corneal stiffness.

Editor, Angioid streaks are irregular, radiating, jagged, tapering lines that extend from the peripapillary area into the peripheral fundus that may occur in isolation or as the ocular manifestation of a systemic disease. Linear breaks or dehiscence in a thickened, calcified and abnormally brittle Bruch's membrane can be associated with choroidal neovascularization (CNV) and poor vision. Several treatments have been attempted with limited success and high rates of recurrence (Costa et al. 2003; Shaikh et al. 2003; Aras et al. 2004). We report a case of subfoveal CNV due to angioid streaks treated with intravitreal bevacizumab 1.25 mg. A 23-year-old man with angioid streaks was referred for subfoveal CNV in his right eye (OD). He had suffered visual loss in the left eye (OS) after minor trauma. Best corrected visual acuity (BCVA) was counting fingers at 2 m OD and 20/400 OS. Fundus examination revealed peripapillary angioid streaks in both eyes, a grey subfoveal lesion with subretinal haemorrhage and subsensory fluid OD and a large pigmented macular scar OS. Fluorescein angiography (FA) revealed a subfoveal classic CNV associated with leakage and peripapillary hyperfluorescence corresponding to the angioid streaks OD and a staining of disciform scarring with no active leakage in the macula OS. Optical coherence tomography (OCT) showed neurosensory serous detachment and type 2 CNV OD (1, 3). The patient was counselled as to the prognosis of his condition and treatment options. Photodynamic therapy was offered but not accepted and an intravitreal injection of bevacizumab 1.25 mg was administered. (A) Early and (B) late-phase fluorescein angiography images at baseline. Optical coherence tomography scans at 6-mm at (A) baseline and (B) 18 weeks post-injection. After 7 weeks, VA was 20/40 and some residual leakage was seen at the lesion margin on FA. The subject underwent a second injection. After 12 weeks VA was 20/30, fundus examination showed no subsensory fluid, and OCT and FA revealed no leakage (2, 3). (A) Early and (B) late-phase fluorescein angiography images at 18 weeks after treatment Choroidal neovascularization occurs in 70–86% of patients with angioid streaks and more than half have vision of 20/200 or worse after the age of 50 years. It has been suggested that laser photocoagulation might resolve CNV and help to stabilize VA or slow down visual loss, given the very high frequency of recurrence (Lim et al. 1993). Other therapies such as photodynamic therapy, Indocyanine green-mediated photothrombosis (IMP) and transpupillary thermotherapy have recently been proposed as alternative treatments for CNV associated with age-related macular degeneration (AMD) and others types of CNV but they do not appear to change the course of the disease and the visual prognosis is poor (Lim et al. 1993; Costa et al. 2003; Shaikh et al. 2003; Aras et al. 2004). This is the first case report of intravitreal injection of bevacizumab in a patient with subfoveal CNV with angioid streaks. Our patient demonstrated a significant improvement at 18 weeks, with VA of 20/30. There was no evidence of inflammation by either OCT or FA at this time-point. Vascular endothelial growth factor (VEGF) has been implicated as the major angiogenic stimulus responsible for neovascularization in AMD. Bevacizumab has shown promising results in off-label intravenous injections administered as salvage treatment (Rosenfeld et al. 2005). A formal prospective study is necessary to determine the safety and efficacy of intravitreal bevacizumab in the treatment of CNV.

Editor, Angioid streaks are irregular, greyish lines that surround the optic disc and extend radially outwards. They are associated with different disorders, the most frequent being pseudoxanthoma elasticum or Grönblad−Strandberg syndrome. In a large proportion of cases, they are associated with choroidal neovascularization (CNV). The treatment most frequently employed for CNV is photodynamic therapy (PDT). However, the current use of antiangiogenic agents for CNV secondary to age-related macular degeneration (AMD) opens a new avenue to treatment for patients affected by CNV secondary to other disorders. Texeira et al. (2006) reported a case of CNV-associated angioid streaks treated with bevacizumab with very good results. We report a case of subfoveal CNV-associated angioid streaks treated with intravitreal pegabtanib sodium. A 41-year-old man presented with a sudden decrease in visual acuity (VA) in the left eye. His best corrected VA was 45 letters (ETDRS). In direct ophthalmoscopy, angioid streaks were observed in both eyes and a lesion suspected of being a neovascular membrane was seen in the foveal area of the left eye (LE). Fluorescein angiography (FA) showed an occult CNV in the LE. Optical coherence tomography (OCT) showed evidence of pigment epithelial detachment (PED) and intraretinal oedema (Fig. 1). Treatment was started with intravitreal injections of sodium pegabtanib (Macugen® 0.3 mg/0.5 ml; ) at regular intervals, with 6 weeks between injections. Four injections of pegabtanib were administered. After 36 weeks, BCVA in the LE was 79 letters. No leakage was seen on FA. Examination by OCT showed a dramatic improvement in the PED and complete disappearance of the intraretinal oedema. No systemic or ocular side-effects were reported. Up figures: fluorescein angiography (FA) and optical coherence tomography (OCT) before treatment; Down figures: FA and OCT post treatment. The most frequent cause of vision loss in patients with angioid streaks is CNV, which occurs in 86% of cases. Photodynamic therapy with verteporfin (Visudyne®) had been the most broadly applied treatment for this type of CNV until the new antiangiogenic therapies emerged. Various authors have found differing results with PDT (Arias et al. 2006; Browning et al. 2002; Karacorlu et al. 2002), but some of the series reported were affected by significant limitations (Arias et al. 2006; Browning et al. 2002; Karacorlu et al. 2002). Antiangiogenic agents are a new therapy for AMD and depend on their inhibitory effect on vascular endothelial growth factor (VEGF). Pegabtanib avoids significant visual loss in patients affected with AMD (Chakravarthy et al. 2006). In addition, bevacizumab and ranibizumab have appeared as alternatives. Texeira et al. (2006) reported a case of CNV associated-angioid streaks treated with two injections of bevacizumab (Avastin®), in which a dramatic visual recovery was seen, along with lack of leakage in OCT and FA. The fundamental difference between this case and the one we present concerns the type of CNV. Texeira et al. (2006) described a classical neovascular membrane; by contrast, our case involved a neovascular membrane of the occult type, which is less frequently associated with angioid streaks. Another difference concerns the number of injections: our case required four injections, whereas Texeira et al. (2006) administered two. Best corrected VA at the end of treatment was very good in both cases. Further research is required, involving detailed studies with extended follow-up to determine the efficacy and safety of intravitreal sodium pegabtanib (Macugen®; pfizer) in the treatment of CNV associated with angioid streaks.

This report describes a case of bilateral central serous chorioretinopathy (CSCR) in a patient with angioid streaks. A 39-year-old male was referred for worsening vision. Best-corrected visual acuity (BCVA) at presentation was 5/10 in his right eye and 4/10 in his left eye. Fundus examination, fluorescein angiography, and optical coherence tomography were compatible with angioid streaks in both eyes associated with macular atrophy due to previous CSCR attack in the right eye and active nonresolving CSCR in the left eye. Macular laser photocoagulation was done in the left eye. After 3 months, subretinal fluid was absorbed and BCVA improved to 7/10. CSCR may occur rarely in patients with angioid streaks and ophthalmologists should consider CSCR in cases with angioid streaks and vision deterioration.

Contribution of OCT angiography in angioid streaks

Purpose: The objective of the present investigation was to define the indocyanine green (ICG) angiographic features of angioid streaks (AS) in young patients with Grönblad-Strandberg syndrome and to compare them with findings on fluorescein angiography (FA) and red-free photographs. Methods: Complete ophthalmological examination, red-free photography, FA and ICG angiography were performed on 6 patients, 4 women and 2 men, ranging in age from 21 to 33 years, with Grönblad-Strandberg syndrome. Results: ICG angiography showed AS in the form of hypofluorescent lines with numerous associated hyperfluorescent foci. The AS were more clearly visualized and were seen to be more numerous and larger by ICG angiography. Choroidal neovascularization (CNV) and macular involvement had become bilateral in all of the cases. ICG angiography allowed precise localization of CNV in some cases, but it was not much superior to FA in the determination of CNV and the visualization of ‘peau d’orange’. Conclusions: ICG angiography provides some information different from FA in the evaluation of AS, but usually neovascular complications and peau d’orange appearance could be seen more clearly in the red-free photographs and FA. The hypofluorescent AS pattern was significantly observed in young patients with Grönblad-Strandberg syndrome.

Angioid streaks of the fundus are not apparent at birth. In order to study their evolution, we examined in a retrospective manner the fundus pictures of 111 subjects with angioid streaks. The earliest form of angioid streaks became apparent at age 8 with findings of narrow short radial discontinuous hypopigmented streaks. Thereafter angioid streaks enlarged in length and width. The end-stage was disciform macular degeneration, helicoid peripapillary atrophy, or diffuse choroidal sclerosis with obscuration of the angioid streaks. We conclude that angioid streaks represent a dynamic manifestation of an underlying retinochoroidal degenerative process.

Angioid streaks are irregular, peripapillary brown to red curvilinear bands that extend in a weblike manner from the optic disc (Fig 1). Often confused with blood vessels, angioid streaks are wider, with tapering ends, lie below the normal retinal vasculature, and are readily discerned by direct ophthalmoscopy. Their importance lies in their association with a host of systemic disorders. Pseudoxanthoma elasticum (PXE), Paget's disease of the bone, and sickle-cell disease account for the majority of diseases linked to angioid streaks. PXE can be demonstrated in more than half the patients with angioid streaks 1,2 ; in one large series, 85% of patients with PXE demonstrated angioid streaks. 3 Additional associated conditions include acromegaly, abetalipoproteinemia, diabetes mellitus, Ehlers-Danlos syndrome, hemochromatosis, facial hemochromatosis, hemolytic anemia, hypercalcinosis, hyperphosphatemia, neurofibromatosis, senile elastosis, Sturge-Weber syndrome, tuberous sclerosis, lead poisoning, and myopia. 1 Additional ocular findings in PXE include a diffuse, yellowish mottling of the retinal pigment

目的 探讨眼底血管样条纹(angioid streaks,AS)的眼底表现、FFA特点、并发症及预后.方法 分析9例(18只眼)AS患者的临床资料及眼底荧光血管造影(fundus fluorescein angiography,FFA)结果.结果 ①眼底表现:18眼后极部均可见类似血管样的放射状条纹,11眼的后极部条纹通过黄斑,占61.1%,其中8眼眼底可见黄斑部出血、水肿、渗出等改变,占44.4%.②FFA:14眼浅棕褐色的条纹均表现为强荧光带,4眼桔黄色的表现为条纹中心区弱荧光,周围强荧光边;有出血、水肿等改变的区域FFA表现为动脉前期或动脉期出现边界清晰强荧光,晚期增强;或静脉期逐渐出现强荧光团,边界不清.结论 大多数眼底AS患者的眼底所见非常典型,FFA结果有助于其病因分析、诊断分期及指导治疗。

PurposeTo present a case of gigantic idiopathic angioid streaks.Case reportA young male presented with macular choroidal neovascular membrane (CNVM) and peripheral retinal hemorrhages secondary to angioid streaks. Swept source optical coherence tomography (SSOCT) and ultrawide field imaging were performed. The latter revealed extension of the angioid streaks up to the equator in both eyes. SSOCT showed breaks in the retinal pigment epithelium-Bruch's membrane complex in the area of peripheral retinal hemorrhages. The patient was extensively worked up for systemic associations, and the only significant finding was a long history of steroid abuse in the past.ConclusionAdvanced imaging techniques helped to diagnose angioid streaks in this patient. The possible role of steroid abuse in accentuating the presentation of angioid streaks may be explored further.

Present the diagnosis and therapeutic approach in a patient with pseudoxanthoma elasticum and ocular involvement. A 46-year-old patient presented for progressive loss of vision and metamorphopsias in the left eye. The ophthalmological examination showed angioid streaks and secondary choroidal neovascularization. The dermatologist performed an incisional biopsy of a skin area in the right axilla that showed white-yellow, discrete streaks. Histopathological examination confirmed the diagnosis of pseudoxanthoma elasticum. It was decided to start intravitreal injections with aflibercept. Three injections were made in the left eye with subsequent visual acuity improvement. Angioid streaks are a rare retinal condition, and pseudoxanthoma elasticum is the most common association. Other systemic conditions are Ehlers-Danlos syndrome, Paget's disease, and hemoglobinopathies. Definite diagnosis requires skin lesions or calcified elastic fibers on pathological examination and angioid streaks of the retina. Choroidal neovascularization is a frequent complication and leads to vision loss. Intravitreal inhibitors of vascular endothelial growth factor are currently the first line of treatment. Pseudoxanthoma elasticum has numerous systemic manifestations and requires a multidisciplinary team to be monitored and treated.

The blood retinal barrier was investigated in a group of patients with angioid streaks and pseudoxanthoma elasticum, using the technique of vitreous fluorophotometry. Angioid streaks are breaks in the elastic layer of Bruch's membrane with atrophy of the adjacent pigment epithelium. The retinal pigment epithelial cells and their intercellular junctions form part of the blood retinal barrier. In this study, it was found that despite the presence of angioid streaks and extensive areas of pigment epithelial atrophy the blood retinal barrier was intact. In contrast, when disciform degeneration had occurred the blood retinal barrier was abnormal. It is proposed that vitreous fluorophotometry could be used to identify those patients developing disciform degeneration at an early and therefore potentially treatable stage.

Communications between the ciliary and retinal circulations have been described in association with central retinal artery occlusion,^1,2meningioma of the optic nerve,¹and op-tic disc drusen.³We examined a patient with angioid streaks and pseudoxanthoma elasticum who had a congenital communication between cilioretinal and retinal arteries. <h3>Report of a Case.</h3> A 35-year-old woman was examined for decreased vision in the left eye. Her best-corrected visual acuity was 20/20 OD and 20/200 OS. Results of a slit-lamp examination were normal and intraocular pressures by applanation tonometry were 14 mm Hg bilaterally. Ophthalmoscopy disclosed bilateral angioid streaks with associated peau d'orange pigmentary change. In the right eye, a cilioretinal artery coursing inferior and then temporal to the fovea communicated directly with a superotemporal branch retinal artery (<b>Figure 1</b>). Fluorescein angiography confirmed that angioid streaks were present (<b>Figure 2</b>). It showed retrograde filling of the cilioretinal artery from the superotemporal