Loss of vision associated with angioid streaks in β-thalassemia intermedia

Aessopos, Athanasios; Floudas, Charalampos S.; Kati, Maria; Tsironi, Maria; Giakoumi, Xanthoula; Livir-Rallatos, Charalampos; Farmakis, Dimitrios

doi:10.1007/s12185-007-0014-y

Cited by 15 publications

(9 citation statements)

References 14 publications

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“…12 ; 41 ; 61 Angioid streaks are associated with pseudoxanthoma elasticum, Paget's disease of bone, acromegaly, Ehlers-Danlos syndrome, diabetes mellitus, sickle cell anemia and β-thalassemia. 2 ; 3 ; 4 ; 6 ; 12 ; 21 ; 41 ; 42 ; 61 ; 62 …”

Section: Retinal Abnormalities In β-Thalassemia Major and Intermediamentioning

confidence: 99%

Retinal abnormalities in β-thalassemia major

Dunaief

2016

Survey of Ophthalmology

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Patients with beta (β)-thalassemia (β-TM: thalassemia major, β-TI: thalassemia intermedia) have a variety of complications that may affect all organs, including the eye. Ocular abnormalities include retinal pigment epithelium degeneration, angioid streaks, venous tortuosity, night blindness, visual field defects, decreased visual acuity, color vision abnormalities, and acute visual loss. Patients with β-TM are transfusion dependent and require iron chelation therapy (ICT) in order to survive. Retinal degeneration may result from either retinal iron accumulation from transfusion-induced iron overload or retinal toxicity induced by ICT. Some who were never treated with ICT exhibited retinopathy, and others receiving ICT had chelator-induced retinopathy. We will focus on retinal abnormalities present in individuals with β-TM viewed in light of new findings on the mechanisms and manifestations of retinal iron toxicity.

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Section: Retinal Abnormalities In β-Thalassemia Major and Intermediamentioning

confidence: 99%

Retinal abnormalities in β-thalassemia major

Dunaief

2016

Survey of Ophthalmology

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“…1,2 Other changes, including optic neuropathy and decreased visual acuity, have been linked to the use of deferoxamine. 1,3 Although optic neuropathy and retinal toxicity are known complications of deferoxamine, there is no report of any ocular complication after deferiprone, an oral substitute for deferoxamine.…”

mentioning

confidence: 99%

Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption

Mehdizadeh

Nowroozzadeh

2009

Clinical and Experimental Optometry

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“…Angioid streaks, caused by linear crack-like dehiscences in the collagenous and elastic portion of Bruch’s membrane, are a common finding in patients with inherited pseudoxanthoma elasticum and may be associated with other funduscopic observations 4,5. Widespread diffuse mottling detected as multiple, indistinct, confluent lesions at the level of the retinal pigment epithelium are known as “peau d’orange” changes.…”

Section: Discussionmentioning

confidence: 99%

New optical coherence tomography fundus findings in a case of beta-thalassemia

Eleftheriadou¹,

Theodossiadis²,

Rouvas³

et al. 2012

OPTH

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Patients with beta-thalassemia may present with an acquired diffuse elastic tissue defect due to degeneration of elastic tissue along with vaso-occlusive findings in the retinal microvasculature. Here we report the case of a patient with granular-like accumulation presenting as black sunburst lesions detected by optical coherence tomography (OCT). A 38-year-old man with beta-thalassemia intermedia associated with angioid streaks complained of deterioration of vision in both eyes. Funduscopic examination revealed small, round, hyperpigmented lesions bilaterally. During the early and late phases of fluorescein angiography, granular hyperfluoresence was present, associated with pigment decompensation and mottled-like hypofluorescence. The main OCT finding was the presence of granuloid-like accumulations at the retinal pigment epithelium level. Granule penetration was also noticed at the photoreceptor layer, while isolated granuloid-like accumulations were found in the inner layers of the macula and choroid. In this case, the new OCT finding was the granular-like hyperpigmented accumulations in the macula located at the level of the retinal pigment epithelium. To the best of our knowledge, our OCT findings show for the first time granuloid-like accumulations representing black sunburst lesions.

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Loss of vision associated with angioid streaks in β-thalassemia intermedia

Cited by 15 publications

References 14 publications

Retinal abnormalities in β-thalassemia major

Retinal abnormalities in β-thalassemia major

Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption

New optical coherence tomography fundus findings in a case of beta-thalassemia

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