Angioid Streaks

Hagedoorn, A.

doi:10.1001/archopht.1939.00860050030004

Cited by 50 publications

(14 citation statements)

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“…Clinical examination with subsequent histopathological findings by Bock4 in 1938 in two patients with pseudoxanthoma elasticum confirmed that the underlying abnormality was not vascular in nature but rather a structural alteration in Bruch’s membrane. A few years later similar histopathological results were found in patients suffering pseudoxanthoma elasticum, Paget’s disease, but also from systemic diseases 5. Despite the fact that many systemic diseases like acromegaly, Ehlers–Danlos syndrome and diabetes mellitus, have been associated with angioid streaks, the most common diseases related to angioid streaks are pseudoxanthoma elasticum,6 Paget’s disease7,8 of bone and sickle-cell9 anemia.…”

Section: Introductionmentioning

confidence: 53%

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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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Section: Introductionmentioning

confidence: 53%

“…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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“…It took more than two further decades before Kofler, based on meticulous clinical observations, eventually suggested that angioid streaks are due to breaks in BM (Kofler, 1917). His observations were later supported by histological studies (Böck, 1938; Hagedoorn, 1939; Verhoeff, 1948; McWilliam, 1951; Jensen, 1977; Dreyer and Green, 1978) and were more recently confirmed by in vivo imaging (Charbel Issa et al, 2009). In 1941, Scholz provided an excellent thorough review of the early literature on angioid streaks, analyzing a total of 188 reported cases (Scholz, 1941).…”

Section: Historical Notes and Terminologymentioning

confidence: 85%

“…As pointed out by Hagedoorn (Hagedoorn, 1939), Hallopeau and Laffitte were the first to report a possible relation between PXE and retinal disease (Hallopeau and Laffitte, 1903). However, it took until 1929 for the Swedish ophthalmologist Ester Grönblad together with the dermatologist James Strandberg to recognize the syndromic association between the characteristic ocular and skin phenotypes (Grönblad, 1929; Strandberg, 1929).…”

Section: Historical Notes and Terminologymentioning

confidence: 99%

“…Several authors noted a patchy transition zone towards the rather normal appearing peripheral BM anterior to the equator (Böck, 1938; Hagedoorn, 1939; Klien, 1947; Verhoeff, 1948; Jensen, 1977). This transition zone may be the histopathological correlate for peau d’orange, although no direct evidence has been provided for this interpretation.…”

Section: Histologymentioning

confidence: 99%

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An update on the ocular phenotype in patients with pseudoxanthoma elasticum

Gliem¹,

Zaeytijd²,

Finger³

et al. 2013

Front. Genet.

124

206

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Pseudoxanthoma elasticum (PXE) is an inherited multi-system disorder characterized by ectopic mineralization and fragmentation of elastic fibers in the skin, the elastic laminae of blood vessels and Bruch’s membrane in the eye. Biallelic mutations in the ATP-binding cassette (ABC) transporter gene ABCC6 on chromosome 16 are responsible for the disease. The pathophysiology is incompletely understood. However, there is consent that a metabolic alteration leads to dysfunction in extracellular calcium homeostasis and subsequent calcification of connective tissues rich in elastic fibers. This review summarizes and aims at explaining the variety of phenotypic ocular findings in patients with PXE. Specialized imaging techniques including white light fundus photography, blue light autofluorescence, near-infrared confocal reflectance imaging, high resolution optical coherence tomography, fluorescein and indocyanine green (ICG) angiography have revealed characteristic lesions at the ocular fundus of PXE patients. These include the classic signs of angioid streaks, peau d’orange, comet lesions, and choroidal neovascularizations (CNVs), but also the more recently recognized features such as chorioretinal atrophy, subretinal fluid independent from CNV, pattern dystrophy-like changes, debris accumulation under the retinal pigment epithelium, reticular drusen and a decreased fluorescence on late phase ICG angiography.

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Angioid Streaks

Starr

Chen

et al. 2022

Albert and Jakobiec's Principles and Practice of Ophthalmology

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Angioid Streaks

Cited by 50 publications

References 0 publications

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

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

An update on the ocular phenotype in patients with pseudoxanthoma elasticum

Angioid Streaks

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