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Evolution of angioid streaks.

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.

Adolescent↗

Angioid streaks.

Angioid streaks are often associated with a systemic condition, most frequently pseudoxanthoma elasticum, Paget's disease of the bone, or one of the sickle hemoglobinopathies. The clinical manifestations of angioid streaks and those three systemic conditions are reviewed. A diagnostic survey is suggested for patients discovered to have angioid streaks with no known systemic disease. The results of such a survey in 50 patients are presented. In addition, separate studies of patients with Paget's disease of the bone (50 patients) and of the sickle hemoglobinopathies (100 patients) are described, and the characteristics of patients with angioid streaks as well as the incidence of streaks in these conditions is reviewed. The histopathologic and fluorescein angiographic characteristics of angioid streaks, as well as the possible benefit of photocoagulation for complications of angioid streaks is discussed.

Age Factors↗

Angioid streaks.

Angioid streaks were first described by Doyne in 1889. Gronblad proposed in 1929 that they followed disruption of the elastic layer of Bruch's membrane, having noted the association between angioid streaks and pseudoxanthoma elasticum. Several other diseases have been associated with angioid streaks, including Paget's disease and sickle cell disease. Angioid streaks are found predominantly in the 20 to 50 year age-group and may be associated with minimal visual loss, but the problem is the high risk of rapid development of subretinal neovascularisation at the macula with resultant haemorrhage and scarring. Prophylactic light coagulation along the angioid streaks to prevent subretinal neovascularisation is not recommended. However, light coagulation treatment to foci of subretinal neovascularisation is possible if the network is not too close to fixation. As recurrence of neovascularisation is to be expected, very careful follow-up is necessary.

Adolescent↗

Diagnosis and management of angioid streaks.

Angioid streaks are asymptomatic breaks in Bruch's membrane developing later in life. Secondary macular degeneration and other fundus abnormalities often accompany their development. Angioid streaks are frequently associated with systemic diseases such as pseudoxanthoma elasticum, Paget's disease of bone, and the sickle cell hemoglobinopathies. The clinical manifestations of angioid streaks, related fundus changes, and these three systemic disorders are discussed as well as principles of treatment and management. Two case reports are presented which illustrate some of the important characteristics of angioid streaks.

Aged↗

Ocular melanocytosis and angioid streaks.

Unilateral angioid streaks of the ocular fundus with homolateral ocular melanocytosis in a 49-year-old white woman are reported. The findings are substantiated by fluorescein angiography. The local changes related to ocular melanocytosis probably precipitated the formation of angioid streaks in the genetically predisposed patient. Two greyish-yellow patches were also found on ophthalmoscopy of the involved eye. One of these, located in the paramacular region, exhibited definite progression, and transformed into a disciform lesion without implicating the macula. The presence of these likely precursors of angioid streaks about the macula is more ominous. A comment about the relationship between melanocytosis, angioid streaks, and phakomatoses is added.

Adult↗

Understanding angioid streaks.

BACKGROUND: Angioid streaks are defined as a series of linear, cracked-line dehiscences of Bruch's membrane, with secondary changes in the retinal pigment epithelium and choriocapillaris. They may be progressive or degenerative, with varied presentation, color, distribution, and retinal involvement. METHODS: The epidemiology, pathophysiology, diagnosis, and management of angioid streaks are described. In addition, the systemic diseases most commonly associated with the disease are reviewed. RESULTS: Optometrists need to be able to differentially diagnose angioid streaks and to refer for evaluation of underlying systemic disease. CONCLUSIONS: Angioid streaks are considered a rare disorder, associated with pseudoxanthoma elasticum, Paget's disease of the bone, sickle hemoglobinopathies. Marfan syndrome, and Ehlers-Danlos syndrome. Ocular complications include subretinal choroidal neovascular membrane formation. Clinicians should be aware of the disease's subtle ocular appearance, its association with systemic diseases, its potential for producing subretinal ocular complications, and correct management protocols and treatments.

Angioid Streaks↗

Angioid streaks in homozygous beta thalassemia.

One hundred patients with homozygous beta thalassemia (62 had beta thalassemia major and 38 had beta thalassemia intermedia) were examined by ophthalmoscopy for angioid streaks. Angioid streaks were found in 20 patients from both the beta thalassemia major and beta thalassemia intermedia groups (nine and 11 patients, respectively). A positive correlation was found between age and angioid streaks (P = .0017), as was a difference in the prevalence of angioid streaks between the two forms of the disease (P = .079). Additionally, a significant correlation was noted between chelating therapy and the prevalence of angioid streaks (P = .039). However, using multivariate analysis to correct for the effects of age, the correlation of angioid streaks with the form of disease disappeared, whereas the level of significance between chelation therapy and angioid streaks was reduced (P = .05). The high frequency of angioid streaks observed in patients with beta thalassemia and the severe complications observed in one patient render a thorough ophthalmoscopic examination and follow-up of such patients necessary for both early diagnosis and possible therapeutic intervention.

Adolescent↗

Angioid streaks associated with abetalipoproteinemia.

Angioid streaks were observed in two patients with abetalipoproteinemia. The progression of the angioid streaks was minimal over the years that these patients received vitamin A and E supplementation, though in one patient the development of subretinal neovascular membranes within the angioid streaks was the cause of rapid central visual loss. The simultaneous appearance of two rare entities in unrelated individuals strengthens the relationship between these two disorders that has been suggested by previous case studies. The authors propose a common metabolic pathway involving trace element deficiencies that may account for this relationship as well as the association of angioid streaks with other rare disorders such as Paget's disease, hypoparathyroidism, lead poisoning, hyperphosphatemia, and a number of hemoglobinopathies. Their study of these two patients underscores the need for further investigations as to the role of copper, zinc and omega-3 fatty acids in the pathogenesis of retinopathy in abetalipoproteinemia.

Abetalipoproteinemia↗

Angioid streaks in sickle-thalassemia.

Angioid streaks have been described in a diverse group of diseases including hemoglobinopathies such as sickle cell anemia and beta-thalassemia. We investigated the prevalence of angioid streaks and pseudoxanthoma elasticum in the rare situation of patients who had compound heterozygous traits for hemoglobin S and beta-thalassemia. We examined 58 consecutive patients with sickle-thalassemia. Of these, 25 were men and 33 were women, and they ranged in age from 19 to 58 years (mean, 32.6 years). Angioid streaks were identified in six of 58 patients (10%), and of these three also displayed the cutaneous lesions of pseudoxanthoma elasticum, which were confirmed by skin biopsy. An expanded study on several relatives of the patients with angioid streaks failed to identify any similar cases. Statistical evaluation of the main hematologic and biochemical parameters in the patients with and without angioid streaks did not demonstrate any significant differences, except that the thalassemic component in all six patients with angioid streaks was beta(0) (that is, did not allow the synthesis of hemoglobin A). We conclude that angioid streaks and pseudoxanthoma elasticum skin lesions occur with an increased frequency in patients with sickle-thalassemia.

Adult↗

Angioid streaks associated with abetalipoproteinemia.

Angioid streaks were observed in a 31-year-old man with abetalipoproteinemia. Angioid streaks have now been described in hereditary spherocytosis, beta thalassemia, alpha thalassemia, sickle cell anemia, and acanthocytosis secondary to abetalipoproteinemia and hypobetalipoproteinemia. The nature of the association of angioid streaks and structural defects of hemoglobin and the red blood cell is not understood.

Abetalipoproteinemia↗

Indocyanine green videoangiography of angioid streaks.

PURPOSE: The fluorescein angiographic features of angioid streaks are variable, and angioid streaks and their main complication, choroidal neovascularization, can sometimes be difficult to visualize in the presence of diffuse pigment migration, diffuse atrophy of the retinal pigment epithelium, or hemorrhage. The objective of the present investigation was to define the indocyanine green angiographic features of angioid streaks and to compare them with findings on fluorescein angiography. METHODS: For this prospective study, we recruited 22 consecutive patients, 21 of whom had angioid streaks and one who had typical peau d'orange appearance of the fundus. Complete ophthalmologic examination, fluorescein angiography, and indocyanine green videoangiography by the means of scanning laser ophthalmoscope were performed on all patients. RESULTS: In 21 patients with angioid streaks and in one patient with peau d'orange appearance of the fundus, indocyanine green videoangiography showed angioid streaks in the form of hyperfluorescent lines with numerous associated hyperfluorescent foci. The angioid streaks were more clearly visualized and were seen to be more numerous and larger by indocyanine green videoangiography than with red-free images or fluorescein angiography. Choroidal neovascularization was suspected in six eyes but could be precisely localized by fluorescein angiography in only three eyes. Indocyanine green angiography allowed precise localization of choroidal neovascularization in all six of these eyes. CONCLUSIONS: These findings indicate that indocyanine green videoangiography provides different information than fluorescein angiography in the evaluation of angioid streaks and can more precisely localize their neovascular complications.

Adolescent↗

[Angioid streaks. Pathogenesis and the clinical picture].

Angioid streaks were first described by Doyne in 1889. Since that time histopathology and diagnostic methods have been greatly improved. Angioid streaks of the fundus are not apparent at birth. The earliest form is known as "peau d'orange". The end stage is disciform macular degeneration, helicoid peripapillary atrophy or diffuse choroidal sclerosis. Moreover, macular hemorrhage and precipitation of angioid streaks have frequently been noted after trauma. Angioid streaks have been described in a diverse group of diseases including pseudoxanthoma elasticum, Paget's disease, hemoglobinopathies such as sickle cell anemia and beta-thalassemia.

Adult↗

[Laser photocoagulation for choroidal neovascularization developed in a patient with optic disc drusen and angioid streaks].

BACKGROUND: Optic disc drusen accompanied by angioid streaks is rarely seen in Japan. Laser photocoagulation for choroidal neovascularization (CNV) in patients with angioid streaks is controversial. CASE: A 45-year-old woman presented with bilateral papilledema. Clinical examination revealed optic disc drusen and angioid streaks. During the follow-up period, juxtapapillary CNV developed in both eyes. CNV in the right eye developed progressively towards the fovea and was eventually treated by laser, but the CNV in the left eye regressed spontaneously. CONCLUSION: These are the first precise clinical records on optic disc drusen with angioid streaks to be reported. The outcome of laser treatment for CNV in this case was favorable, because alteration of Bruch's membrane was mild, in the form of angioid streaks.

Angioid Streaks↗

Angioid streaks in pituitary tumor.

The diagnosis of angioid streaks on fundus examination allows the examiner to focus on a limited number of commonly associated conditions, such as pseudoxanthoma elasticum, Paget's disease, and sickle cell disease. There have been sporadic reports of pituitary tumors associated with angioid streaks. This is the first case report of pituitary tumor associated with angioid streaks. This is the first case report of pituitary tumor associated with angioid streaks documented by fluorescein angiography, EMI scan, and histopathologic diagnosis of the tumor, and also in which pseudoxanthoma elasticum was ruled out, both clinically and by a negative skin biopsy.

Adenoma↗

[Angioid streaks and associated lesions: ophthalmoscopic and fluorescein angiographic interpretation].

Angioid streaks are characteristic and well known lesions of the fundus of the eye. They are frequently associated with other less known chorioretinal lesions. In order to investigate the incidence and the clinical importance of these latter lesions, the photographs and the fluorescein angiographies of the 24 fundi of 12 patients with angioid streaks were studied. The lesions associated with the angioid streaks are light areas along the streak, red paired spots, fundus peau d'orange, salmon spots, optic disc drusen and macular atrophic and exudative-hemorrhagic degenerations. The comparison between the photographs and the fluorescein angiographies of the fundus allows to deduce, in many cases, the anatomic lesion that causes the clinical appearance of the various kinds of angioid streaks and the associated lesions. Although the misinterpretation of these results sometimes led to inaccurate speculations about the nature and the pathogenesis of the angioid streaks and the associated lesions, it is important to point out that lacking histopathological reports that would clarify the true nature of the lesions associated with the angioid streaks, ophthalmoscopy and fluorescein angiography are the only methods helpful to reveal the basic lesions.

Angioid Streaks↗

Comparison of fluorescein and indocyanine green angiography in angioid streaks.

BACKGROUND: The main cause of vision loss in patients with angioid streaks is choroidal neovascularization and subsequent macular degeneration. Indocyanine green angiography allows visualization of the choroidal circulation and may be superior to fluorescein angiography in the evaluation of patients with angioid streaks. METHODS: The ophthalmoscopic, fluorescein and indocyanine green angiographic characteristics of angioid streaks were studied in 34 patients with such streaks. Nineteen patients had pseudoxanthoma elasticum and 15 patients had isolated angioid streaks. The fluorescence characteristics of the 'peau d'orange' and of choroidal neovascularization, when present, were also analyzed. RESULTS: Angioid streaks may be hyperfluorescent, hypofluorescent or invisible on indocyanine green angiography. Hyperfluorescent streaks were found in 88% of eyes, hypofluorescent streaks in 11%; in 18% of eyes some streaks were not visualized by indocyanine green angiography. The peau d'orange stained as a speckled pattern in the midperiphery; the flecks were concentrated temporal to the macula. Eighteen eyes presented classic and 6 occult choroidal neovascularization. In several eyes a plaque-like lesion was seen on indocyanine angiography that did not correspond to occult choroidal neovascularization on fluorescein angiography. CONCLUSION: Indocyanine angiography outlines angioid streaks as well as the peau d'orange appearance better than fluorescein angiography in the majority of cases. In some cases, however, funduscopically visible streaks can not be visualized. Sometimes classic choroidal neovascular membranes are not visualized by conventional indocyanine green angiography. Occult choroidal neovascularization is better defined by indocyanine green angiography. The fluorescence of angioid streaks and of plaque-like lesions makes the interpretation of indocyanine green angiography difficult.

Adult↗

Angioid streaks and sickle haemoglobinopathies.

Five patients had angioid streaks associated with sickle cell haemoglobinopathy. Other diseases associated with angioid streaks were ruled out, as was elastic tissue degenegation in sickle cell patients. After studying over 350 patients, we believe the incidence of angioid streaks in sickle cell disease to be between 1 and 2 per cent.

Adult↗