Les bêtathalasso-drépanocytoses pourvoyeuses de rétinopathies ischémiques graves

Fanny, A.; Coulibaly, F.; Gbé, K.; Meite, M.; Adjorlolo, C.; Konan-Toure, M. L.; Berete, R.; Boni, S.; Ouattara, A.; Diallo, M.

doi:10.1016/s0181-5512(05)81070-8

Cited by 8 publications

(3 citation statements)

References 5 publications

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“…In sickle thalassemia, angioid streaks have been encountered [ 60 ], and a frequency of 10 % was reported in a group of 58 cases, by Aesopos et al [ 61 ]. In another study, by Fanny et al, out of the 18 patients studied, 13 (72.2 %) presented with sickle cell retinopathy [ 62 ]. Overall, retinopathy in beta thalassemia appears similar to sickle cell retinopathy.…”

Section: Discussionmentioning

confidence: 99%

β-Thalassemia and ocular implications: a systematic review

et al. 2016

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BackgroundBeta-thalassemia is a severe genetic blood disorder caused by a mutation in the gene encoding for the beta chains of hemoglobin. Individuals with beta-thalassemia major require regular lifelong Red Blood Cell transfusions to survive. Ocular involvement is quite common and may have serious implications.MethodsExtensive review of observational studies on beta-thalassemia, to determine the prevalence and spectrum of ocular abnormalities, by clinical examination and multimodal imaging, and to investigate risk factors for their development.ResultsFrequency of ocular involvement differs among various studies (41.3–85 %, three studies). Ocular findings in beta-thalassemia may correlate to the disease itself, iron overload or the chelating agents used. Beta-thalassemia ocular manifestations include ocular surface disease, as demonstrated by tear function parameters (two studies). Lens opacities are present in 9.3–44 % (five studies). Lenticular opacities and RPE degeneration correlated positively with use of desferrioxamine and deferriprone respectively (two studies). Ocular fundus abnormalities characteristic of pseudoxanthoma elasticum (PXE), including peau d’orange, angioid streaks, pattern dystrophy-like changes, and optic disc drusen are a consistent finding in seven studies. Patients with PXE-like fundus changes were older than patients without these fundus changes (two studies). Age (two studies) and splenectomy (one study) had the strongest association with presence of PXE-like fundus changes. Increased retinal vascular tortuosity independently of the PXE-like fundus changes was found in 11–17.9 % (three studies), which was associated with aspartate amino transferase, hemoglobin and ferritin levels (two studies). Fundus autofluorescence and electrophysiological testing (ERG and EOG) may indicate initial stages or more widespread injury than is suggested by fundus examination (two studies).ConclusionsBeta-thalassemia may present with various signs, both structural and functional. Pseudoxanthoma elasticum like fundus changes are a frequent finding in patients with b-thalassemia. These changes increase with duration or severity of the disease. Retinal vascular tortuosity may be an additional disease manifestation related to the severity and duration of anemia and independent of the PXE-like syndrome. Patients with long-standing disease need regular ophthalmic checkups because they are at risk of developing PXE-like fundus changes and potentially of subsequent choroidal neovascularization.

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Section: Discussionmentioning

confidence: 99%

β-Thalassemia and ocular implications: a systematic review

et al. 2016

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“…Ophthalmologic involvement with variable gravity according to age and type of sickle cell disease is progressing insidiously, for a long time without any functional signs, and exposes to sometimes serious complications [8].…”

Section: Discussionmentioning

confidence: 99%

Retinopathy Revealing Cerebral Venous Thrombosis in Sickle Cell Disease in Niger

Adam¹,

Soumana²,

Ali³

et al. 2019

OJOph

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Cerebral venous thrombosis (CVT) in sickle cell disease has been rarely described in the literature. Some authors consider sickle cell disease as a risk factor for CVT. We report the case of a 20-year-old boy, known as sickle cell, followed at the National Reference Center for Sickle Cell Disease in Niger. Admitted in consultation for a decrease of acuity with the right eye with perception of a black spot evolving since about three (3) days, ophthalmological examination revealed sickle cell retinopathy associated with cerebral venous thrombosis on MRI. The rest of the balance showed normochromic anemia, ionic disturbance and a D-dimer at 1500 μg/l. Rehydration, strict rest, antibiotic therapy and analgesics were started with an internal medicine opinion for further treatment. There was an improvement in visual acuity of 6/10 in 48 hours with disappearance of black spot perception, after three weeks, an ad integrum recovery of retinal lesions to control imaging. Despite the ocular complications of sickle cell disease involving visual prognosis, cerebral venous thrombosis is another complication to be investigated in any sickle cell retinopathy.

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“…Fanny et al, studied 18 patients suffering from sickle beta thalassemia and found that 13 of the patients had sickle cell retinopathy with 3 of them having proliferative disease. It appears that retinopathy in sickle thalassemia shares similar characteristics with sickle cell retinopathy [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

Pattern dystrophies in patients treated with deferoxamine: report of two cases and review of the literature

et al. 2018

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BackgroundDeferoxamine (DFO) is one of the most commonly used chelation treatments for transfusional hemosiderosis. Pattern dystrophies constitute a distinct entity of retinal disorders that has been occasionally identified in association with deferoxamine.Case presentationWe report two cases of bilateral macular pattern dystrophy in transfusion dependent patients undergoing chronic chelation therapy with deferoxamine due to thalassemias. Our patients were evaluated with multimodal imaging and the results are presented. Both patients had normal cone and rod responses in the full-field electroretinogram and continued the prescribed chelation therapy, after hematology consult. The patients were followed up every 3 months for 2 and 4 years respectively for possible deterioration. Their best corrected visual acuity remained stable with no anatomic change on Optical Coherence Tomography findings.ConclusionMultimodal imaging of our patients allowed a better evaluation and possibly earlier detection of the DFO-related changes. Screening and close follow up of patients under chronic chelating therapy is important in order to promptly diagnose and manage possible toxicity either with discontinuation of the offending agent or dose modification.

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Les bêtathalasso-drépanocytoses pourvoyeuses de rétinopathies ischémiques graves

Cited by 8 publications

References 5 publications

β-Thalassemia and ocular implications: a systematic review

β-Thalassemia and ocular implications: a systematic review

Retinopathy Revealing Cerebral Venous Thrombosis in Sickle Cell Disease in Niger

Pattern dystrophies in patients treated with deferoxamine: report of two cases and review of the literature

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