Clinical Characterization of Korean Patients with Pseudoxanthoma Elasticum and Angioid Streaks.

This study aimed to characterize Korean patients with pseudoxanthoma elasticum (PXE) presenting with angioid streaks. Retinal phenotypes were longitudinally evaluated by multimodal ophthalmic imaging, and targeted gene panel sequencing for inherited retinal diseases was conducted. Seven subjects from unrelated families (median age, 51.2 years) were enrolled and followed for a median of 3.2 years. Four asymptomatic patients were significantly younger than three symptomatic patients with decreased visual acuity at presentation (mean age; 38.1 vs. 61.5 years, p = 0.020). The asymptomatic patients maintained good vision (20/32 or better) and had no choroidal neovascularization (CNV) over the observation period. The symptomatic patients showed additional reduction in visual acuity and bilateral CNV occurrence during the longitudinal follow-up. Pathogenic ABCC6 variants were identified in all patients, leading to a diagnosis of PXE. Heterozygous monoallelic variants were identified in four patients and compound heterozygous variants were detected in three patients. Nine ABCC6 variants were identified, including one novel variant, c.2035G>T [p.Glu679Ter]. This is the first genetic study of Korean patients with PXE.

PSEUDOXANTHOMA ELASTICUM: SUCCESSFUL LONG-TERM MANAGEMENT OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO ANGIOID STREAKS WITH PRO RE NATA INTRAVITREAL BEVACIZUMAB INJECTIONS.

PURPOSE: To demonstrate how a patient with recurrent episodes of choroidal neovascularization (CNV), secondary to angioid streaks, can be managed successfully with a pro re nata regime of intravitreal bevacizumab injection over an eight-year period. METHOD: A 32-year-old white woman with pseudoxanthoma elasticum has been followed up over an eight-year period for management of recurrent episodes of CNV in both eyes. She was educated to recognize the early signs and symptoms of CNV. Physical examination including visual acuity and slit-lamp examination as well as investigations such as macula optical coherence tomography and optical coherence tomography angiography were performed. Bevacizumab injections were given to her when she was diagnosed with CNV. RESULTS: Multiple episodes of CNV were successfully treated with pro re nata regimes of intravitreal bevacizumab injections. The patient was able to maintain excellent visual acuity of 0 logarithm of the minimum angle of resolution even after suffering recurrent episodes of CNV. CONCLUSION: This case report supports that a pro re nata regime of intravitreal bevacizumab injection therapy can be used successfully to treat recurrent episodes of CNV in a patient with pseudoxanthoma elasticum over an eight-year period. Early diagnosis through patient education and the use of appropriate diagnostic tools such as optical coherence tomography angiography have enabled us to deliver early treatment, resulting in an excellent outcome for this patient.

Switching from ranibizumab to aflibercept in choroidal neovascularization secondary to angioid streaks.

PURPOSE: To evaluate the efficacy of switching from intravitreal ranibizumab to intravitreal aflibercept in choroidal neovascularization secondary to angioid streaks. DESIGN: Multicenter retrospective interventional case series. METHODS: Patients previously treated with intravitreal ranibizumab with at least 12-month follow-up (M12) after switching (M0) to intravitreal aflibercept. Switch to intravitreal aflibercept was decided in cases of refractory or recurrent choroidal neovascularization. Primary endpoint: Change of best-corrected visual acuity using the Early Treatment Diabetic Retinopathy Study letters. Secondary endpoints: Mean change of central macular thickness, absence of intraretinal/subretinal fluid on spectral domain optical coherence tomography and the percentage of eyes with absence of leakage on fluorescein angiography. RESULTS: Fourteen eyes of 13 patients were included. Mean best-corrected visual acuity was 65.0 ± 21.03 letters at M0 and 63.5 ± 17.30 letters at M12 (p = 0.5). Secondary endpoints: Mean central macular thickness was 344 ± 194.65 µm at M0 and 268 ± 79.97 µm at M12 (p = 0.008). Absence of intraretinal/subretinal fluid was observed in 71%. Fluorescein angiography (nine eyes) showed absence of leakage in 77% (seven eyes). CONCLUSION: Switching from intravitreal ranibizumab to intravitreal aflibercept represents a therapeutic option in patients with refractory or recurrent choroidal neovascularization secondary to angioid streaks.

Retinal sensitivity in angioid streaks.

PURPOSE: To investigate retinal sensitivity in eyes with angioid streaks (AS). METHODS: This study was a retrospective observational case series. A total of 13 eyes from 9 patients with AS underwent ophthalmologic examination, including fundus autofluorescence (FAF), spectral domain optical tomography (SD-OCT), and microperimetry. Retinal pigment epithelium (RPE) atrophy was assessed using FAF. Outer retinal atrophy, more specifically, disruption of the ellipsoid zone, was evaluated using SD-OCT images. The association between retinal sensitivity, RPE atrophy or outer retinal atrophy, and patient demographic characteristics or ophthalmologic findings were investigated. RESULTS: The mean area of outer retinal atrophy was 14.5 ± 12.9 mm2, significantly larger than the mean area of RPE atrophy (9.0 ± 9.0 mm2; P = 0.0028). The average retinal sensitivity in the area of RPE atrophy was 2.4 ± 5.6 dB, that in the area of outer retinal atrophy outside RPE atrophy was 14.6 ± 7.5 dB, and that in the area without any atrophy was 25.6 ± 5.1 dB. Reticular pseudodrusen (RPD) was observed in 5 eyes. Eyes with RPD showed a greater extent of both outer retinal atrophy and RPE atrophy than those without RPD and a greater decrease in mean retinal sensitivity. CONCLUSIONS: In eyes with AS, especially in those with RPD, large areas of outer retinal atrophy and RPE atrophy were observed, and the retinal sensitivity in the areas was correspondingly decreased.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FEATURES OF ANGIOID STREAKS.

PURPOSE: To analyze the optical coherence tomography angiography features of eyes affected with angioid streaks (AS) and to evaluate their ability to predict choroidal neovascularization (CNV) activity. METHODS: Angioid streaks were individuated from a pool of consecutive patients. Eyes with and without CNV were evaluated by multimodal imaging. RESULTS: Thirty-eight eyes of 19 consecutive patients diagnosed with AS were included. Thirty of 38 eyes with CNV and 8 of 38 eyes without CNV were included. In the majority of cases, CNV showed on optical coherence tomography angiography tangled appearance always associated with signs of neovascular inactivity on multimodal imaging (100%-0%, inactive-active, respectively). Choroidal neovascularization cases showing interlacing appearance were often associated with signs of neovascular activity on multimodal imaging (71.4%-28.6%, active-inactive, respectively). Optical coherence tomography angiography revealed a total of 27 AS, of which 20 appeared as a choriocapillary rarefaction, and in 7 AS, optical coherence tomography angiography choriocapillary segmentation revealed an irregular vascular network, possibly representing fibrovascular tissue over the crack-like breaks in Bruch membrane. CONCLUSION: Optical coherence tomography angiography is a noninvasive tool to detect the presence of CNV secondary to AS and to evaluate CNV activity. Optical coherence tomography angiography is able to add a novel element to the multimodal imaging characterization of AS.

Angioid streaks in aagenaes syndrome.

Aagenaes syndrome, also called lymphoedema cholestasis syndrome 1 (LSC1), is characterized by neonatal intrahepatic cholestasis, often lessening and becoming intermittent with age and severe chronic lymphoedema, mainly affecting the lower extremities. The condition is autosomal recessively inherited, and the gene is located on chromosome 15q. The locus, LCS1, was mapped to a 6.6 cM region on chromosome 15. Angioid streaks are visible irregular crack-like dehiscences in bruch's membrane that are associated with atrophic degeneration of the overlying retinal pigment epithelium. Angioid streaks have been described to be associated with pseudoxanthoma elasticum, paget's disease, sickle-cell anaemia, acromegaly, Ehlers-Danlos syndrome, and diabetes mellitus, but also appear in patients without any systemic diseases. Patients with angioid streaks are generally asymptomatic, unless the lesions extend towards the foveola or develop complications such as traumatic bruch's membrane rupture or macular choroidal neovascularization.

LOCATING AND CHARACTERIZING ANGIOID STREAKS WITH EN FACE OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: To characterize angioid streaks (AS) with en face optical coherence tomography (OCT). METHODS: Case report of a patient with myopia presenting with choroidal neovascularization secondary to AS. Swept-source en face OCT ability to image the streaks was compared with spectral-domain and swept-source B-scans as well as color and red-free pictures. A 48-year-old man with myopia presented with sudden central visual loss. Choroidal neovascularization secondary to AS was diagnosed and intraocular anti-vascular endothelial growth factor given with clinical and OCT features improvement. RESULTS: Angioid streaks were visualized as less dark than the overlying retinal and the underlying choroidal vasculature. En face OCT located the changes at the level of Bruch membrane. An AS was found to be interrupted by the choroidal neovascularization, what was not captured by other modalities. CONCLUSION: En face OCT allows to assess the extent of changes in Bruch membrane and their spatial relationship to choroidal neovascularization.

Efecto del traumatismo por aceleración/desaceleración en estrías angioides: hipótesis patogénica / The effect of the acceleration/deceleration trauma in angioid streaks: A pathogenic hypothesis

CASO CLÍNICO: Varón de 59 años, sufrió traumatismo por aceleración/desaceleración (TAD), de localización craneal, causado por accidente de tráfico; un mes después presentó pérdida de agudeza visual (AV) del ojo derecho (OD). Mediante angiografía fluoresceínica (AGF) se detectaron líneas hiperfluorescentes centrífugas desde la papila, imagen compatible con el diagnóstico de estrías angioides (EA). El hallazgo de una membrana neovascular coroidea (MNVC) yuxtafoveal justificó la pérdida de AV. CONCLUSIÓN: El TAD puede provocar la hiperextensión del globo ocular en su eje ecuatorial, y producir la rotura de estructuras frágiles como la membrana de Bruch (MB) en pacientes con EA, con la subsiguiente formación de una MNVC

CASE REPORT: A 59-year-old male with acceleration/deceleration cranial trauma (ADT), caused by a car accident. After one month, he presented with loss of visual acuity in the right eye. A fluorescein angiography test was performed and it detected centrifugal hyperfluorescent lines from the optic nerve head, a characteristic compatible with the diagnosis of angioid streaks. The loss of visual acuity was demonstrated by the discovery of a juxtafoveal choroidal neovascular membrane (CNV). CONCLUSION: ADT can cause hyper-extension of the eyeball in its equator line, producing the rupture of fragile structures such as the Bruch membrane (MB) in patients with angioid streaks and the subsequent formation of CNV

The effect of the acceleration/deceleration trauma in angioid streaks: A pathogenic hypothesis. / Efecto del traumatismo por aceleración/desaceleración en estrías angioides: hipótesis patogénica.

CASE REPORT: A 59-year-old male with acceleration/deceleration cranial trauma (ADT), caused by a car accident. After one month, he presented with loss of visual acuity in the right eye. A fluorescein angiography test was performed and it detected centrifugal hyperfluorescent lines from the optic nerve head, a characteristic compatible with the diagnosis of angioid streaks. The loss of visual acuity was demonstrated by the discovery of a juxtafoveal choroidal neovascular membrane (CNV). CONCLUSION: ADT can cause hyper-extension of the eyeball in its equator line, producing the rupture of fragile structures such as the Bruch membrane (MB) in patients with angioid streaks and the subsequent formation of CNV.

Corneal Viscoelastic Properties in Patients with Angioid Streaks.

PURPOSE: The aim of this study is to investigate the possible corneal biomechanical changes in patients with angioid streaks and to understand if the calcified and thickened Bruch's membrane associated with angioid streaks influences elasticity of the eye and intraocular pressure. MATERIALS AND METHODS: Twelve eyes of 12 patients with angioid streaks (six males and six females) and 12 eyes of 12 age- and sex-matched healthy volunteers were enrolled in the study. Corneal hysteresis (CH), corneal resistance factor (CRF), corneal compensated intraocular pressure (IOPcc) and Goldmann-correlated intraocular pressure (IOPg) were measured with an Ocular Response Analyzer (ORA). Central corneal thickness (CCT) was measured with an ultrasound pachymeter. RESULTS: Mean CRF and IOPg values in eyes with angioid streaks (12.10 ± 1.27 and 17.76 ± 2.73, respectively) were significantly higher than those in matched control eyes (10.70 ± 1.28 and 14.67 ± 2.72, respectively; p = 0.01 for CRF, p = 0.007 for IOPg). There was no statistically significant difference between eyes with angioid streaks and matched control eyes in measured CH, IOPcc and CCT values (p = 0.29, p = 0.09 and p = 0.86, respectively). CONCLUSIONS: This study revealed that angioid streaks can affect corneal biomechanical properties by increasing CRF, as compared to the healthy eyes. Increased CRF means increased resistance for effect of IOP on eye so it can be speculated that these patients tend to be more protected for glaucoma.

Visual Impairment in Pseudoxanthoma Elasticum: A Survey of 40 Patients.

BACKGROUND: Pseudoxanthoma elasticum is an inherited disorder of connective tissue characterized, among other symptoms, by impaired vision. OBJECTIVE: To evaluate the nature and age of onset of ophthalmologic manifestations in pseudoxanthoma elasticum. PATIENTS AND METHODS: Forty consecutive patients affected with pseudoxanthoma elasticum underwent measurements of their refractive error and visual acuity, together with slit-lamp examination. RESULTS: The mean age of the patients (8 M, 32 F) was 43.35 years. Fifty-seven eyes (33 patients, mean age: 40.75 years) had a BCVA >20/50 whereas 23 eyes in 16 patients (mean age: 53.31 years) had ≤20/50. Seven patients (17.50%), all but one over 52 years old, were visually disabled. BCVA ≤20/50 in at least one eye was observed in 73.33% of patients of 52 years old or older and in 20.00% of patients younger than 52, respectively. Angioid streaks were observed in 75 eyes (93.75%) and extended toward the macula in 51 eyes from 29 patients. Macular involvement was observed for the first time at a mean age of 44.28 years. Neovascularization was observed in 28 eyes (17 patients; mean age: 51.70 years), all with poor BCVA. CONCLUSION: Macular choroidal neovascularization is frequent in pseudoxanthoma elasticum, and accounts for the poor ophthalmologic natural history of the disease. Patients should be advised to self-monitor their visual acuity using the Amsler grid. The frequency of choroidal neovascularization appears age-dependent, suggesting that bi-yearly fundus examination is appropriate in young patients whereas patients older than 40 should be examined twice a year.

Intravitreal ranibizumab for the treatment of choroidal neovascularisation secondary to angioid streaks.

AIMS: To assess the medium to long-term efficacy and safety of intravitreal ranibizumab for the treatment of choroidal neovascularisation (CNV) secondary to angioid streaks (AS). METHODS: A total of 12 eyes of nine patients treated with intravitreal ranibizumab (0.5 mg in 0.05 ml) for CNV secondary to AS were retrospectively identified. Efficacy of treatment was determined by changes in best-corrected LogMAR visual acuity (BCVA) and optical coherence tomography. Changes with respect to baseline BCVA were defined as improved or reduced with a gain or loss of more than 10 letters, respectively, or stable if remaining within 10 letters. RESULTS: Over a mean follow-up of 21.75 months (range: 1-54), patients received mean 5.75 (range: 2-15) intravitreal ranibizumab injections per affected eye. BCVA improved in three eyes (25%), stabilised in eight eyes (66.67%), and deteriorated in one eye (8.33%). There was no significant change in central retinal thickness (CRT) over the follow-up period (P=0.1072). No drug-related systemic side effects were recorded. CONCLUSION: The long-term treatment of CNV secondary to AS with intravitreal ranibizumab showed a stabilisation in CRT and an improvement or stabilisation of BCVA. The absence of systemic side effects was reassuring. Further long-term prospective studies are required to validate these findings.

Monthly ranibizumab for choroidal neovascularizations secondary to angioid streaks in pseudoxanthoma elasticum: a one-year prospective study.

PURPOSE: To evaluate the efficacy and safety of monthly intravitreal ranibizumab for the treatment of choroidal neovascularizations (CNV) secondary to angioid streaks (AS) in pseudoxanthoma elasticum (PXE). DESIGN: Twelve-month prospective, open-label, uncontrolled, nonrandomized interventional clinical trial. METHODS: In 7 patients, 1 eye with an active CNV was injected with 0.5 mg ranibizumab monthly over 1 year. Distance and reading visual acuity, reading speed, angiographic findings, and central retinal thickness (CRT) on optical coherence tomography were assessed at each visit. Central retinal light increment sensitivity (LIS) was assessed by microperimetry at baseline, at 6 months, and 3 to 4 months after the last injection. RESULTS: Best-corrected visual acuity increased significantly from baseline to month 12 (20/63 or 61 ETDRS letters to 20/32 or 73 ETDRS letters; P = .012). The effect was maintained 3 months later (61 ETDRS letters to 72 ETDRS letters; P = .055). Reading acuity and speed could be maintained throughout the study. Central LIS improved (6.6 dB, SD ± 5.9 at baseline to 7.4 dB, SD ± 6.2 at last follow-up; P < .001). Leakage from active CNVs subsided. Mean change in CRT from baseline to month 12 and 15 was -86 µm (P = .074) and -65 µm (P = .182), respectively. No serious adverse events occurred. CONCLUSIONS: Efficacy outcomes indicate a beneficial therapeutic effect of intravitreal ranibizumab on central visual function including retinal LIS. Both the functional and morphologic response based on angiographic and OCT findings to ranibizumab treatment implicate an important pathophysiological role of vascular endothelial growth factor in CNVs secondary to AS in PXE. Intravitreal ranibizumab appears to be a safe and efficacious treatment in these patients.

Management of CNV in angioid streaks by intravitreal use of specific anti-VEGF165 Aptamer (pegaptanib sodium): long-term results.

PURPOSE: To investigate the effectiveness of intravitreal pegaptanib sodium injection in patients with choroidal neovascularization (CNV) secondary to angioid streaks. METHODS: Five eyes of four patients with angioid streaks with CNV underwent uneventful intravitreal injection of pegaptanib sodium (0.3 mg/90 µL). Patients were followed up with Snellen visual acuity testing, optical coherence tomography, and fundus fluorescein angiography. RESULTS: The median follow-up time was 18 months (range: 15 to 24 months). Visual acuity improved in two eyes, and stabilized in three out of five eyes. At final examination, CNV regressed with resolution of subretinal fluid in all but one patient with bilateral CNV from angioid streaks. CONCLUSION: Intravitreal pegaptanib sodium for CNV associated with angioid streaks led to inactivation of most of the CNV lesions, stabilizing or improving visual acuity in all eyes.

Combination treatment with intravitreal injection of ranibizumab and reduced fluence photodynamic therapy for choroidal neovascularization secondary to angioid streaks: preliminary clinical results of 12-month follow-up.

BACKGROUND: To evaluate combination treatment with intravitreal ranibizumab injection and reduced fluence photodynamic therapy for choroidal neovascularization associated with angioid streaks. METHODS: This is an interventional case series of 10 previously untreated eyes of 10 patients with choroidal neovascularization secondary to angioid streaks. All eyes were treated with reduced fluence photodynamic therapy using 25 J/cm, immediately followed by intravitreal ranibizumab injection at baseline, and subsequent injections were performed on an as-needed basis thereafter. Treatment efficacy was assessed based on best-corrected visual acuity and optical coherence tomography findings. RESULTS: After 12 months of follow-up, the best-corrected visual acuity improved by >2 lines in 6 eyes (60%), remained within 2 lines of baseline in 3 eyes (30%), and decreased by ≥ 3 lines in only 1 eye (10%). The mean central foveal thickness decreased significantly from 332.2 µm at baseline to 235.7 µm at the last follow-up (P < 0.001), as measured by optical coherence tomography. CONCLUSION: The preliminary results of this prospective study indicate that combination treatment with intravitreal ranibizumab injection and reduced fluence photodynamic therapy for choroidal neovascularization associated with angioid streaks seems to be effective in reducing or eliminating retinal edema, regression of neovascularization, and improving or stabilizing visual acuity without any complications. Large controlled studies are needed to evaluate the long-term effects of this combination regimen.

Long-term effectiveness of intravitreal bevacizumab for choroidal neovascularization secondary to angioid streaks in pseudoxanthoma elasticum.

PURPOSE: To investigate the long-term effectiveness of intravitreal bevacizumab for treating active choroidal neovascularizations in pseudoxanthoma elasticum (PXE). METHODS: Fourteen patients (16 eyes) received intravitreal bevacizumab (1.5 mg) and were investigated monthly. Further treatments were administered depending on disease activity. Examinations included best-corrected visual acuity, biomicroscopy, optical coherence tomography, fluorescein angiography and indocyanine green angiography, fundus autofluorescence, and digital fundus photography. Areas of atrophy of the retinal pigment epithelium and retinal fibrosis were quantified using semiautomated detection on fundus autofluorescence images. RESULTS: Mean age of the cohort was 55 ± 13 years, and mean best-corrected visual acuity at baseline was 20/80 (logarithm of the minimum angle of resolution, 0.56, SD, 0.51). At last follow-up, after an average of 6.5 ± 5.7 injections over 28 months, best-corrected visual acuity was 20/40 (logarithm of the minimum angle of resolution, 0.31, SD, 0.32; P = 0.04). Central retinal thickness was reduced from 254 ± 45 µm to 214 ± 40 µm (P = 0.035). The size of retinal pigment epithelial atrophy and retinal fibrosis measured on fundus autofluorescence images increased in both the treated eye and the fellow eye (P < 0.05). Best-corrected visual acuity of patients with early disease compared with that of those with advanced disease improved significantly more over the treatment course (20/25 vs. 20/63; P = 0.008). CONCLUSION: Intravitreal bevacizumab therapy demonstrates long-term effectiveness by preserving function in advanced disease and improving function in early disease. Best results of treating active choroidal neovascularizations in PXE are achieved when treatment starts the earliest possible.

Intravitreal bevacizumab for the treatment of choroidal neovascularization secondary to angioid streaks: one year of follow-up.

PURPOSE: To evaluate the efficacy and safety of intravitreal bevacizumab at one year follow-up, for the treatment of choroidal neovascularization (CNV) associated with angioid streaks. METHODS: A retrospective case series of eighteen eyes of 17 patients with CNV secondary to angioid streaks treated with intravitreal bevacizumab between October 2006 and May 2008. Ophthalmic evaluation including best corrected visual acuity (BCVA), slit lamp biomicroscopic examination, optical coherence tomography (OCT) and fluorescein angiography, was performed before and after treatment. Retreatment was given every 4-6 weeks in case of persistent symptoms or CNV activity on OCT. Main outcome measures were changes in BCVA and central retinal thickness on OCT. RESULTS: The mean number of injections was 4.8 at 1 year. Twelve eyes (66.6%) received five injections or more. The mean BCVA at baseline was 20/80 (range 20/400 to 20/32) and improved to 20/44 (range 20/160 to 20/20) at 1 year (p = 0.014). The BCVA improved by three or more lines in eleven eyes (61.11%) and remained within two lines of baseline in seven eyes (38.8%). Mean central retinal thickness was 404.2 µm (range 160-602 µm) at baseline and decreased to 300.5 µm (range 150-523 µm) at 1 year (p = 0.022). No ocular or systemic complications were noted. CONCLUSION: The 1-year outcomes suggest intravitreal bevacizumab to be a promising treatment for CNV associated with angioid streaks, resulting in both functional and anatomical improvements. Repeated injections are needed to maintain these results. Further long term studies are required to confirm these findings.

Intravitreal ranibizumab for choroidal neovascularization in angioid streaks.

PURPOSE: To analyze retrospectively the efficacy of intravitreal ranibizumab injections for the management of choroidal neovascularization (CNV) in patients with angioid streaks. DESIGN: Nonrandomized, double-center, retrospective, interventional case series. METHODS: A consecutive series of patients affected with CNV associated with angioid streaks were treated with intravitreal ranibizumab injections (0.5 mg/0.05 mL). Best-corrected visual acuity, fundus photography results, optical coherence tomography (OCT) results, and fluorescein angiography results were examined before and after treatment. The primary end point was the percentage of eyes with stable or improved visual acuity at the end of follow-up. Secondary end points were the percentage of eyes with stable or decreased macular thickness on optical coherence tomography and the percentage of eyes with persistent leakage on fluorescein angiography at the last follow-up examination. RESULTS: Thirty-five eyes of 27 patients were treated with repeated intravitreal ranibizumab injections (mean, 5.7 injections; range, 2 to 14 injections) for a mean of 24.1 months (range, 6 to 37 months). At the end of follow-up, visual acuity was stabilized or improved in 30 (85.7%) of 35 eyes. Macular thickness had stabilized or decreased in 18 (51.5%) of 35 eyes. At the last follow-up examination, on fluorescein angiography, no further leakage was observed in 23 (65.7%) of 35 eyes. CONCLUSIONS: In this large series of angioid streaks-associated CNV, ranibizumab injections allowed stabilization of visual acuity. Ranibizumab seems to be a safe therapeutic option in CNV associated with angioid streaks.

Clinical course of choroidal neovascularization secondary to angioid streaks treated with intravitreal bevacizumab.

BACKGROUND AND OBJECTIVE: To evaluate visual acuity and anatomical outcomes of choroidal neovascularization (CNV) associated with angioid streaks after treatment with intravitreal bevacizumab injections. PATIENTS AND METHODS: Best-corrected visual acuity, optical coherence tomography measurements (OCT), fluorescein and indocyanine green angiography, and ophthalmoscope examination at baseline and at each follow-up visit were performed. Five patients with CNV associated with angioid streaks were treated with injections of intravitreal bevacizumab (1.25 mg/0.05 mL). Re-treatment was recommended with symptomatic lesions, new subretinal hemorrhages, leakage on fluorescein and indocyanine green angiography, and/or fluid documented by OCT. Follow-up ranged between 18 and 32 months. RESULTS: All eyes showed an improvement of visual acuity and were treated with at least four injections of intravitreal bevacizumab. Reduction of the leakage shown by fluorescein angiography and OCT was noted in all patients. CONCLUSION: Intravitreal bevacizumab appears to be effective in stabilizing and recovering visual acuity in eyes with CNV associated with angioid streaks. Patients with early symptoms might benefit more.