Angioid Streaks and Sickle Cell Anemia

Paton, David

doi:10.1001/archopht.1959.04220050112018

Cited by 37 publications

(13 citation statements)

References 11 publications

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“…Other funduscopic alterations associated to sickle cell anemia, but not found in our patients, include obstructions of the central retinal vein and/or artery and angioid streaks [16,17]. Table III shows patients with a reduction in visual acuity and corresponding vitreoretinal pathology.…”

Section: Resultsmentioning

confidence: 75%

Funduscopic Alterations in SS and SC Hemoglobinopathies

Bonanomi

Cunha²,

Araújo³

1988

Ophthalmologica

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Fundus changes in sickle cell disease are well studied and documented according to their clinical and angiographic aspects. Sickle cell retinopathy was studied in a Brazilian population of 63 patients (41 with SS hemoglobinopathy, and 22 with SC hemoglobinopathy). All ophthalmoscopic changes observed in our patients were more frequent in the SC group with the exception of atrophic retinal tears, found only in the SS group. Proliferative retinopathy including its complications was seen in 54.54% of the eyes of the SC group, and in only 14.64% of the eyes of the SS group. Decreased vision is consequently greater in the SC group. Despite the great frequency of funduscopic changes in both groups, only 1 patient suffered irreversible visual loss due to retinal detachment operated on without success.

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Section: Resultsmentioning

confidence: 75%

Funduscopic Alterations in SS and SC Hemoglobinopathies

Bonanomi

Cunha²,

Araújo³

1988

Ophthalmologica

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“…For several years the appearance of angioid streaks in sickle-cell hemoglobinopathies was attributed to high level of serum iron 5,13,14. However, other types of anemias with increased iron levels in blood are not associated with angioid streaks.…”

Section: Histopathological Findingsmentioning

confidence: 99%

Angioid streaks, clinical course, complications, and current therapeutic management

Georgalas¹

2008

TCRM

101

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Angioid streaks are visible irregular crack-like dehiscences in Bruch's membrane that are associated with atrophic degeneration of the overlying retinal pigmented epithelium. Angioid streaks may be associated with pseudoxanthoma elasticum, Paget's disease, sickle-cell anemia, acromegaly, Ehlers-Danlos syndrome, and diabetes mellitus, but also appear in patients without any systemic disease. 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 (CNV). The visual prognosis in patients with CNV secondary to angioid streaks if untreated, is poor and most treatment modalities, until recently, have failed to limit the devastating impact of CNV in central vision. However, it is likely that treatment with antivascular endothelial growth factor, especially in treatment-naive eyes to yield favorable results in the future and this has to be investigated in future studies.

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“…Angioid streaks has been reported in sickle cell disease (SCD) since the late 1950s. 13,14 Since then, several papers have been published, presenting a variable frequency of angioid streaks in SCD, ranging from 1% to 22%, depending on patients' age. [15][16][17] Angioid streaks were found to develop in patients with SCD at the age of 25 years on average, although the highest prevalence was reported in a group of 60 Jamaican patients older than 40 years.…”

Section: Pxe-like Manifestations In ␤ Thalassemia and The Sickling Symentioning

confidence: 99%

“…15,17 Accordingly, angioid streaks were primarily thought to be related to the disease itself and pathogenetic mechanisms, such as vascular obstruction affecting the choriocapillary circulation or chronic hemolysis leading to iron deposition were proposed. 13,26 In contrast, another autopsy series of 16 unselected SCD patients included 2 with histopathologic findings identical to PXE in the dermis and the arterial walls of multiple organs. 14 A clearer relationship was found by Lippman et al 27 in 1985.…”

Section: Pxe-like Manifestations In ␤ Thalassemia and The Sickling Symentioning

confidence: 99%

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

Aessopos

Farmakis

Loukopoulos

2002

Blood

139

124

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The development of clinical and histopathologic manifestations of a diffuse elastic tissue defect, resembling inherited pseudoxanthoma elasticum (PXE), has been encountered with a notable frequency in patients with ␤ thalassemia, sickle cell disease, and sickle thalassemia. The PXE-like clinical syndrome, consisting of skin, ocular, and vascular manifestations, has a variable severity in these hemoglobinopathies and it is age-dependent, with a generally late onset, after the second decade of life. The defect is believed to be acquired rather than inherited and related to the consequences of the primary disease. The high prevalence of the findings implicates the elastic tissue injury as one of the main comorbid abnormalities encountered in ␤ thalassemia and the sickling syndromes. In these patients a number of complications, sometimes serious, has been recognized to be related to ocular and vascular elastic tissue defects. Because several organ systems are involved, each medical specialty should be aware of the phenomenon. This coexistence, on the other hand, introduces a novel pathogenetic aspect of PXE and an important research challenge. (Blood. 2002;99:30-35)

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Angioid Streaks and Sickle Cell Anemia

Cited by 37 publications

References 11 publications

Funduscopic Alterations in SS and SC Hemoglobinopathies

Funduscopic Alterations in SS and SC Hemoglobinopathies

Angioid streaks, clinical course, complications, and current therapeutic management

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

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