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.

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.

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.

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.

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.

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.

Angioid streak-related choroidal neovascularization treated by intravitreal ranibizumab.

PURPOSE: The purpose of this study was to report the visual outcome of intravitreal therapy with ranibizumab of choroidal neovascularization secondary to angioid streaks after 1-year follow-up. METHODS: Nine patients (age, 58 +/- 4 years; range, 53-65 years) were treated with off-label intravitreal injections of 0.3 mg ranibizumab. Primary outcomes were best-corrected visual acuity changes (Early Treatment Diabetic Retinopathy Study logarithm of the minimum angle of resolution and letters) and optical coherence tomography macular thickness changes. RESULTS: Mean follow-up was 14 months (+/-2; range, 12-18 months). Mean visual acuity was 0.52 logarithm of the minimum angle of resolution and 30 letters (range, 0.2-1.2; 0-47 letters) at baseline and 0.37 logarithm of the minimum angle of resolution (P = 0.014) and 37 letters (range, 0-1.2; 2-55 letters) (P = 0.01) at the last examination. Seven of 9 patients (78%) gained vision (mean, 2 lines), 1 patient (11 %) was stable, and 1 patient (11 %) lost 1 line of vision (5 letters). Two patients (22%) gained >or=3 lines of visual acuity, no patient lost >1 line. Mean optical coherence tomography macular thickness was 262.4 microm (+/-34.4 standard deviation) at baseline and 216.4 microm (+/-19 standard deviation) at the last examination (P = 0.05). The mean number of injection was 5 (range, 3-7); 78% of patients needed to be retreated after the loading dose of 3 monthly injections. CONCLUSION: Ranibizumab can be considered as an effective therapy in angioid streak-related neovascularization, even if in an off-label setting.

Long-term control of choroidal neovascularisation secondary to angioid streaks treated with intravitreal bevacizumab (Avastin).

AIM: To evaluate the efficacy of intravitreal bevacizumab (IB) in the long-term control of subfoveal choroidal neovascularisation (CNV) associated with angioid streaks (AS). METHODS: Patients with unilateral active CNV associated with AS were enrolled. Exclusion criteria were previous treatment for CNV and comorbidity. Postoperative visual acuity was defined as a gain or loss of two or more lines of best-corrected visual acuity (BCVA). Post-treatment CNV size was dichotomised into "increased," if the CNV area had grown by > or =200 microm(2), and "stable/reduced" if it had decreased by > or =200 microm(2) or had not changed by more than 200 microm(2). Patients were retreated if no further improvement or worsening was noted. RESULTS: Patients were five males and six females aged 33 to 58 years (mean 46.8 (SD 9.2)), who received a mean number of 3.5 (1.3) IB treatments (min: 2; max: 6). The mean retreatment interval was 3 (1.5) months (min: 1; max: 6). The mean follow-up duration was 23.8 (2.9) months. At 20 months all patients had stable/reduced CNV size and stable/improved BCVA. The mean BCVA rose significantly from 0.28 (0.2) at baseline to 0.56 (0.29) at 20 months (p<0.0001). CONCLUSION: IB is a promising tool for the long-term control of CNV in AS. Further studies are required to validate these findings.