Choroidal Neovascularization in Angioid Streaks and Pseudoxanthoma Elasticum: 1 Year Follow-Up
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Purpose To report the efficacy of intravitreal injection of bevacizumab 1.25 mg (IVB) in the treatment of choroidal neovascularization (CNV) secondary to angioid streaks (AS). Methods Case review of two patients with CNV secondary to AS treated with three IVB with a 1-year follow-up. Results In both patients after 1 year fluorescein angiography and optical coherence tomography showed complete inactivity of the CNV and the ophthalmologic examination revealed stabilization of the visual acuity. Conclusions The IBV appeared to be an effective and safe treatment for CNV secondary to AS resulting in a long-term CNV inactivation. Further long-term studies in this type of lesion are desirable.Keywords:
Angioid streaks
Pseudoxanthoma Elasticum
Choroidal neovascularization (CNV) belongs to the rare retinal disorders in children. Authors present 3 cases of CNV, which were diagnosed in patients between 12-15 years of age (2 females and 1 male). Complete ophthalmological examination including fluorescein angiography, indocyanine angiography and optical coherence tomography were performed in all cases. Laboratory tests to exclude the systemic diseases were also done. CNV in young people maybe a result of high myopia, angioid streaks, ocular histoplasmosis, trauma or it could be idiopathic. In our series, we found idiopathic CNV in 2 eyes and 1 CNV after traumatic choroidal break. Stabilization of visual acuity was achieved after argon laser treatment in 2 cases.
Angioid streaks
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DURING the past two years angioid streaks of the retina have been seen in 3 patients, in 2 of whom pseudoxanthoma elasticum was also present. In these 2 patients severe vascular disturbance was found in all the extremities. Since no mention of a relationship of these three conditions has been found in the available literature and since the association is probably significant, the cases are being reported. The condition referred to as angioid streaks of the retina was first described by Doyne1in 1889. Since that time this condition has been found to be related clinically to two other degenerative diseases, (1) pseudoxanthoma elasticum and (2) osteitis deformans (Paget's disease). The relation to pseudoxanthoma elasticum was first noticed by Groenblad and Strandberg2in 1929. Pseudoxanthoma elasticum is a degenerative disease of the skin, first reported by Balzer,3in 1884. Scholz,4reviewing the cases of angioid streaks
Angioid streaks
Pseudoxanthoma Elasticum
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Purpose: To report on the five-year follow-up of ranibizumab therapy for choroidal neovascularization (CNV) secondary to angioid streaks (AS) in pseudoxanthoma elasticum (PXE).
Angioid streaks
Pseudoxanthoma Elasticum
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Purpose To report the efficacy of intravitreal injection of bevacizumab 1.25 mg (IVB) in the treatment of choroidal neovascularization (CNV) secondary to angioid streaks (AS). Methods Case review of two patients with CNV secondary to AS treated with three IVB with a 1-year follow-up. Results In both patients after 1 year fluorescein angiography and optical coherence tomography showed complete inactivity of the CNV and the ophthalmologic examination revealed stabilization of the visual acuity. Conclusions The IBV appeared to be an effective and safe treatment for CNV secondary to AS resulting in a long-term CNV inactivation. Further long-term studies in this type of lesion are desirable.
Angioid streaks
Pseudoxanthoma Elasticum
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Pseudoxanthoma elasticum (PXE) is a rare, systemic disease with typical ophthalmological findings including angioid streaks (AS) (represent breaks in the Bruch's membrane). A serious and common complication of AS is the development of choroidal neovascularization (CNV), which can result in significant and irreversible vision loss.
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SCHOLZ (1941), reviewing the literature on angioid streaks and pseudoxanthoma elasticum, collected a total of 188 cases, only seventeen of which were reported from Great Britain.A further case is described in this article.Angioid streaks were first described by Doyne (1889) and then by Plange (1891); Knapp (1892) gave them their present name.The fundus appear- ance in this disease was described by Verhoeff (1928) as follows:A more or less broken peripapillary ring of pigment from which pigmented streaks extend out into the fundus.The appearance of the streaks varies from dark red to brown or even black.The edges of the streaks are serrated and along their course there may be reddish dots.They lie beneath the retinal vessels and above the choroidal ones.Usually there is a complete or incomplete greyish zone about the papilla.The entire fundus may have a mottled appearance or the degenerative changes may appear only in the periphery.Both eyes are involved.However, since Verhoeff published this account, two cases of monocular angioid streaks have been described: one by Gronblad (1932) and one by Isayama (1940).The most common presenting symptoms of patients with angioid streaks is diminution of vision.This is due to associated macular abnormalities which were present in 140 out of the 188 cases collected by Scholz (1941).These abnormalities may be haemorrhage, scarring, abnormal pigmentation, hole at the macula, exudate, or a specific type of retinitis such as disciform degeneration or retinitis circinata.Flame-shaped and blot-haemorrhage may occur in any part of the fundus.A peculiar type of haemorrhage caused by trauma described by Marchesani and Wirz (1931) comprises large tear-drop-like haemorrhages in the layer between the retina and choroid, rather similar to pre-retinal haemorrhages but at a deeper level.The diagnosis is revealed by examining the untraumatized eye.These haemor- rhages are absorbed completely, but the authors suggest that they leave behind them white streaks in the retina similar to those which remain after a choroidal detachment.Other abnormalities of the retina may also be seen, such as pigment and hyaline bodies, especially drusen of the optic disc.
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Pseudoxanthoma Elasticum
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PURPOSE: To investigate the effect of indocyanine green-mediated photothrombosis on choroidal neovascularization secondary to angioid streaks. METHODS: Six eyes of 5 patients with an average age of 70 years were diagnosed to have subfoveal choroidal neovascularization secondary to angioid streaks. Therapy was indicated if choroidal neovascularization was considered to be active, according to evidence of fluorescein leakage on angiograms, as well as presence of intra- or subretinal fluid on optical coherence tomography. Patients received photothrombosis at baseline with retreatment as necessary at 3, 6 and 9 months follow-up. Fluorescein leakage was assessed at 3, 6 and 9 months, along with foveal thickness measured by optical coherence tomography. Indocyanine green angiography was performed initially and whenever retreatment was considered. Retreatment was performed whenever there was evidence of increased leakage on fluorescein angiograms at follow-up visits. RESULTS: The mean follow-up was 13.3 months. All eyes had at least 12 months follow-up. Two eyes underwent retreatment. Three eyes had visual acuity improvement and three eyes had stabilization. All patients showed less fluorescein leakage on final angiograms and reduced foveal thickness upon optical coherence tomography measurement. CONCLUSION: Photothrombosis is a feasible procedure for choroidal neovascularization secondary to angioid streaks. Lesion analysis showed anatomical improvement in most cases in this series. Further studies are warranted to evaluate long-term results of this treatment.
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* OBJECTIVE: To evaluate image quality of oral fluorescein angiography in patients with age-related macular degeneration and choroidal neovascularization. * MATERIALS AND METHODS: Twenty four patients with proven choroidal neovascularization were examined in a prospective observational case series. Confocal oral and intravenous fluorescein angiography were performed using the Heidelberg Retina Angiograph. Oral and intravenous image quality were compared in a masked fashion. * RESULTS: Choroidal neovascularization could be diagnosed in all patients using intravenous angiograms. Oral fluorescein angiography accurately detected the presence or absence of choroidal neovascularization in all patients and could determine the classification of choroidal neovascularization, location, and extent and borders in 20 (83.3%) of 24 patients. Early, mid, and late phases of oral angiograms occurred at 8, 15, and 26 minutes, respectively. * CONCLUSION: Oral fluorescein angiography using the confocal Heidelberg Retina Angiograph allowed detection of choroidal neovascularization in all patients in this study. Visualization of extent and type of choroidal neovascularization was possible in most eyes with choroidal neovascularization. These findings suggest oral angiography is an excellent screening tool for choroidal neovascularization and allows guidance of treatment in the majority of cases. [Ophthalmic Surg Lasers Imaging 2003;34: 17-24]
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