Estrías angioides bilaterales con membranas neovasculares coroideas / Bilateral angioid streaks with choroidal neovascular membranas

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Polypoidal choroidal vasculopathy in a patient with angioid streaks treated by bevacizumab.

OBJECTIVE: We report the association of polypoid choroidal vasculopathy (PVC) with angioid streaks (AS) secondary to pseudoxanthoma elasticum (PXE) treated by intravitreal injection of bevacizumab. CASE REPORT: A 50-year-old patient, followed in dermatology for a PXE, who consulted for a consulted for a decreased vision in his right eye (RE) for a month. On examination, best corrected visual acuity (BCVA) was at 1/20 P14 in the RE and at 8/10 P2 in the left eye (LE). Fundus examination revealed AS in both eyes, large patches of exudate at the posterior pole with retinal hemorrhages in the RE. Fluorescein angiography (FA) showed constant hypofluorescence by mask effect due to exudates and macular early hyperfluorescence with late diffusion associated with small hyperfluorescent lesions in the RE. We suspected CNV type 2. Macular Spectral Domain optical coherence tomography (SD-OCT) showed significant retinal infiltration with pre-epithelial hyper-reflectivity bracing the diagnosis of type 2 CNV. In view of the importance of exudates and intra-retinal infiltration, we completed by indocyanine green angiography (ICGA) which showed hypercyanecent vascular dilations grouped in clusters of grapes relevant to an associated VPC. The patient received eight monthly intravitreal (IVT) injections of bevacizumab with good anatomical evolution. At 10 months, visual acuity was 1/10 with disappearance of exudates and retinal infiltration. CONCLUSION: Patients with AS should receive regular follow-up given the risk of CNV but also of VPC, especially in cases of PXE. Multimodal imaging is of great help and ICGA remains inescapable.

Evaluation of lamina cribrosa in angioid streaks using spectral-domain optical coherence tomography enhanced depth imaging.

PURPOSE: To investigate retinal nerve fiber layer (RNFL) thickness, anterior lamina cribrosa depth (ALD) and laminar thickness (LT) in eyes with angioid streaks (AS) in comparison with healthy eyes. METHODS: Patients with AS (n=32 eyes) and age-matched healthy subjects (n=42 eyes) underwent enhanced depth imaging with spectral domain optical coherence tomography of the optic nerve head. RNFL thickness was obtained automatically by using the Heidelberg software. The ALD was defined as the vertical distance between the Bruch's membrane openings as reference plane and the anterior border of the lamina cribrosa. The LT was measured as the distance between the anterior and posterior borders of the lamina cribrosa. RNFL thickness, ALD and LT were compared between the AS and healthy eyes adjusting for axial length. RESULTS: The mean age was 51.7±8.0 years for AS patients and 53.8±10.2 years for control subjects (P=0.34). The mean RNFL thickness was 88.6±17.6µm in AS eyes and 102.1±8.3µm in control eyes; the difference between the groups was significantly significant. ALD was 350.9±70.8µm in AS eyes and 432.5±82.1µm in control eyes, while LT was 166.3±41.0µm in AS eyes and 241.3±43.2µm in control eyes. ALD and LT were also significantly thinner in AS eyes than control eyes (P=0.003, P<0.001; respectively). CONCLUSION: Enhanced depth imaging revealed that eyes with AS demonstrate thinner RNFL, ALD and LT compared with normal eyes. Further studies with a larger sample size are needed to understand the relationship between these findings and the morphologic changes and pathogenesis of AS.

Optical coherence tomography angiography features of choroidal neovascularization secondary to angioid streaks.

PURPOSE: To characterize the features of choroidal neovascularization (CNV) secondary to angioid streaks (AS) with optical coherence tomography angiography (OCT-A) and to assess its sensitivity in CNV detection in this particular context. METHODS: Consecutive patients, both with treatment-naïve and recurrent CNV associated with angioid streaks were prospectively analyzed. All patients underwent macular imaging by fluorescein angiography (FA), indocyanine green angiography (ICGA), spectral-domain (SD)-OCT, and OCT-A (AngioVue, Optovue, Optovue Inc., Freemont, CA, USA). OCT-A detection rate of CNV associated to AS was evaluated by two independent observers. We studied the association between OCT-A feature and either exudative or active status using Fisher exact test. RESULTS: A total of 32 eyes of 18 consecutive patients were included in the analysis. OCT-A was able to detect CNV associated with angioid streaks in 87.5 % (28/32) eyes. OCT-A phenotypes of CNV were classified into interlacing pattern in 9 eyes, pruned vascular tree pattern in 7 eyes, and combined pattern in 12 eyes (Interuser agreement: 0.871 ± 0.071). CNV were not detectable in 4/32 eyes. There was a statistically significant association between the presence in OCT-A of densely ramified networks with both previous treatment status in the last 6 months (p < 0.001) and with exudative signs on SD-OCT (p = 0.014). CONCLUSION: OCT-A appears as a sensitive tool for detection of CNV secondary to AS. The interlacing pattern was significantly associated with active and exudative features.

To laser or not?

A 64-year-old male patient presented to the clinic with diminished vision in his right eye for the past 2 years. Examination revealed a best-corrected visual acuity (BCVA) of counting fingers at 1 m with a scarred choroidal neovascular membrane (CNVM) in the right eye while the left eye had a BCVA of 20/20 with a small peripapillary subretinal hemorrhage along with angioid streaks in both eyes. Multimodal imaging revealed the presence of a CNVM in the left eye which was treated with focal thermal laser. Regression was noted on optical coherence tomography angiography at 1 month post-laser, but at 3-month follow-up, exacerbation of the CNVM was observed. Subsequently, four intravitreal ziv-aflibercept injections were given, and scarring of CNVM was noted on OCT. Thermal laser in the background of angioid streaks has worsened the breaks in the Bruch's membrane leading to worsening of the CNVM.

Mujer de 60 años con problemas de visión y lesiones cutáneas / A 60-year-old woman with visual impairment and cutaneous lesions

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Silent polypoidal choroidal vasculopathy in a patient with angioid streaks.

We present a case of silent polypoidal choroidal vasculopathy (PCV) in a patient with angioid streaks. PCV was detected during a routine ophthalmic examination and confirmed by fluorescein angiography, indocyanine green angiography, and optical coherence tomography. After 2 years of follow-up, the PCV remained silent without any complications. We report this rare coexistence and review literature on this topic.

Silent polypoidal choroidal vasculopathy in a patient with angioid streaks / Vasculopatia polipoidal de coróide quiescente em um paciente com estrias angióides

ABSTRACT We present a case of silent polypoidal choroidal vasculopathy (PCV) in a patient with angioid streaks. PCV was detected during a routine ophthalmic examination and confirmed by fluorescein angiography, indocyanine green angiography, and optical coherence tomography. After 2 years of follow-up, the PCV remained silent without any complications. We report this rare coexistence and review literature on this topic.

RESUMO Nós apresentamos um relato de vasculopatia polipoidal de coroide (PCV) em paciente com estrias angióides. Vasculopatia polipoidal de coroide detectada em exame oftalmológico de rotina e confirmado por angiofluoresceinografia, angiografia com indocianina verde e tomografia de coerência óptica. Após 2 anos de seguimento a vasculopatia polipoidal de coroide permaneceu quiescente, sem qualquer complicação. Nós relatamos esta coexistência rara e apresentamos revisão da literatura.

Prevalence of reticular pseudodrusen in newly presenting adult onset foveomacular vitelliform dystrophy.

PurposeTo report the association and prevalence of reticular pseudodrusen (RPD) in eyes with newly presenting adult onset foveomacular vitelliform dystrophy (AFVD). To compare the strength of association with other pathologies resulting from dysfunction of the choroid-Bruch's membrane-retinal pigment epithelium (RPE) complex, including eyes with geographic atrophy (GA) and angioid streaks.MethodsRetrospective single-centre review of all consecutive newly presenting AFVD. Multimodal imaging with spectral domain optical coherence tomography, fundus photographs, red-free/blue light images, and fundus fluorescein angiograms were graded for the presence of RPD. For comparison, all consecutive newly presenting cases of GA and eyes with angioid streaks were studied.ResultsFifteen (15) patients were identified with AFVD (mean age of 77.3 years; 73.3% female). Mean age of patients with AFVD and RPD was 80.5 years (SD 3.7), whereas that of patients with AFVD without RPD was 75.1 years (SD 7.0). This age difference did not reach statistical significance, P=0.1. Six (40%) had identifiable RPD; being a bilateral finding in 100% of patients. No males with AFVD and RPD were identified. A total of 92 eyes presented with GA. Twenty-three (23) of these (25.0%) had RPD. Twelve (12) patients presented with identifiable angioid streaks, with 4 (36.4%) having RPD.ConclusionRPD are a frequent finding in eyes with newly presenting AFVD; not being restricted to AMD, but a finding common among diseases where pathophysiological mechanisms involve damage to Bruch's membrane and the RPE, whether genetic or degenerative. Our study supports the concept that they occur with high but variable frequencies in eyes with various pathologies.

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.

[Indocyanine green angiography in angioid streaks]. / Angiografía verde indocianina en las estrías angioides.

PURPOSE: To define the findings of an indocyanine green angiographic study of angioid streaks and compare them with the ophthalmoscopic and fluorescein angiographic findings. PATIENTS AND METHODS: Twelve eyes of six patients with bilateral angioid streaks were examined by ophthalmoscopy, fluorescein angiography, and indocyanine green angiography. RESULTS: In the ophthalmoscopy, the streak colors ranged from brown in the early phases to orange in severe stages. The streaks already presented a hyperfluorescent pattern from the arterial phases in all of the eyes with the fluorescein angiography. Using the indocyanine green angiography filling of the choroidal veins, a hyperfluorescent pattern was observed in eight eyes with severe lesional changes and a hypofluorescent pattern in four eyes with an early stage of the disease. The angioid streaks were more clearly visualized by indocyanine green angiography. CONCLUSION: The indocyanine green angiography contributes to the diagnosis of angioid streaks. The indocyanine patterns seems to be correlated with the ophthalmoscopic features and degree of evolution of the disease. The indocyanine green angiography allows for the early detection of angioid streaks and diagnosis in young patients.

[Subretinal neovascularization and hemorrhages in angioid streaks]. / Neovascularización y hemorragias subretinianas asociadas a estrías angioides.

PURPOSE: To study the development of subretinal neovascularization and/or subretinal hemorrhages in patients with angioid streaks. METHODS: Fifty two eyes from twenty six patients with angioid streaks were included in our study. The diagnosis of angioid streaks was established by ophthalmoscopic and florescein angiographic features. All the patients underwent a complete ophthalmological study that included an examination of the ocular fundus and a retinography every six months. RESULTS: Subretinial neovascularization was found in 38% in this group of patients. Subretinal hemorrhages, either with or without neovascularization, were found in 59%. CONCLUSIONS: There is no known treatment capable of preventing these conditions from appearing, however the patients should be informed about the risk of subretinal hemorrhages after relatively minor ocular trauma. These results may be helpful in the evaluation, prognosis and surgical planning for patients with angioid streaks.

Detection of choroidal calcium by computed tomography scan quantitative computerized tomography: a clinically useful diagnostic tool.

OBJECTIVE: Quantitative computerized tomography (QCT), a scanning method routinely used to classify tissues based on their radio density, was used to detect choroidal calcification. DESIGN: Case reports. PARTICIPANTS: Two patients with known or suspected choroidal calcium-choroidal osteoma (CO) and pseudoxanthoma elasticum (PXE) and five normal control subjects were assessed. INTERVENTION: The CT scan of choroid, B-scan ultrasound, fluorescein angiography. MAIN OUTCOME MEASURES: The QCT scan used to detect the presence of calcium based on a readout greater than 90. RESULTS: Calcium was detected bilaterally, as predicted, in PXE, and in both eyes of CO, although only one eye had a clinically noted lesion. No control eye gave a scale readout consistent with calcium. No findings suggestive of calcium were noted with B-scan ultrasound or fluorescein angiography in PXE or CO. CONCLUSION: The QCT can detect calcium in the choroid when B-scan ultrasonography and CT scan fine sectioning fail to identify calcium despite high clinical suspicion.