Sickle Cell Trait Presenting as Unilateral Proliferative Retinopathy and Macular Thinning in a Pregnant Woman

Ghods, Sepideh; Pour, Elias Khalili; Faghihi, Hooshang; Gharehbaghi, Golnaz; Mirshahi, Ahmad; Ghassemi, Fariba; Inanloo, Bahman; Riazi-Esfahani, Hamid

doi:10.1155/2021/5988889

Cited by 3 publications

(4 citation statements)

References 29 publications

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“…Other researchers have reported the occurrence of PSCR in sickle cell trait (AS). 15,16,17 Because of delays in patient presentation, most eyes presented with advanced stages 4 and 5 (89%).…”

Section: Discussionmentioning

confidence: 99%

Visual outcome of treating proliferative sickle cell retinopathy in 108 eyes

Okonkwo,

Hassan,

Oyekunle

et al. 2023

European Journal of Ophthalmology

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Aim To report treatment methods and visual outcome of treating proliferative sickle cell retinopathy (PSCR). Design Retrospective interventional. Methods Review of PSCR eyes treated between 2017 to 2022. Patient demographics, fundus findings at presentation, genotype, PSCR stage, treatment used, and visual outcome were assessed. Results 108 eyes of 88 consecutive patients were studied. Male: Female 48:40. Mean age: 38.91 (SD:12.52) years. Genotype: sickle cell haemoglobin C (SC) 83 eyes (76.9%), sickle cell haemoglobin S (SS) 19 eyes (17.6%), and sickle cell trait (AS) 6 eyes (5.5%). PSCR stages: 3: 15 eyes (11.0%), 4: 74 eyes (67.0%), and 5: 19 eyes (22.0%). Treatment methods: Intravitreal Injection (IVI) of anti-vascular endothelial growth factor (VEGF) only (27 eyes,25%), scatter retinal laser photocoagulation (SRLP) only (7 eyes, 6.5%), Vitrectomy + SRLP (29 eyes, 26.9%), IVI + SRLP (25 eyes, 23.1%), and Vitrectomy + IVI + SRLP (20 eyes, 18.5%). The treatment used correlated with PSCR stage (p = 0.000). IVI only was mostly used to treat stage 4 (81.4%), and SRLP only was used for stages 3 (42.9%) and 5 (57.1%). IVI + SRLP treated eyes had the best pre- and post-treatment vision. Vitrectomy + SRLP treated eyes had the most improved vision. SRLP only had least visual improvement. Fundus findings correlated with visual outcome (p = 0.003); but stage of PSCR, genotype and treatment used had no correlation (P > 0.05). Conclusion Several options effectively treat PSCR. Visual outcome improved or remained same in 90.7% of treated eyes. Randomized controlled trials will determine the optimum treatment for each distinct presentation of PSCR. Treatment guidelines and a disease classification system of prognostic value are unmet needs.

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Section: Discussionmentioning

confidence: 99%

Visual outcome of treating proliferative sickle cell retinopathy in 108 eyes

Okonkwo,

Hassan,

Oyekunle

et al. 2023

European Journal of Ophthalmology

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“…Symptoms suggestive of retinopathy include abrupt vision loss or visualization of floaters [ 10 , 11 ]. In most cases, patients had a concomitant uncontrolled systemic disease that increased the progression of the disease [ 11 , 12 - 14 ]. However, our case did not have any such systemic illnesses.…”

Section: Discussionmentioning

confidence: 99%

“…However, our case did not have any such systemic illnesses. The diagnosis was usually confirmed by fundoscopic examination [ 11 , 12 - 14 ].…”

Section: Discussionmentioning

confidence: 99%

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Rare Cause of Acute Loss of Vision in a Patient With Sickle Cell Trait

Bojja,

Javed,

Allena

et al. 2023

Cureus

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Sickle cell disease (SCD) is a prevalent inherited blood disorder with various ocular manifestations, including sickle cell retinopathy (SCR), characterized by retinal microcirculation impairment and ischemic complications. We present the case of a 21-year-old male with sickle cell trait who experienced a sudden, painless loss of vision in his left eye. Ophthalmologic examination revealed vitreous hemorrhage and neovascularization, indicating SCR. Initial treatment with hydroxyurea and exchange transfusions led to partial improvement. However, due to persistent vitreous hemorrhage, the patient underwent a vitrectomy. The sickle cell trait affects a large global population, and its retinopathy is a rare but severe complication. The pathogenesis and risk factors for SCR are similar to those for SCD. The diagnosis of SCR is established through fundoscopic examination and graded based on Goldberg's classification. Management involves a multidisciplinary approach targeting systemic illness and visual defects, including hydroxyurea, photocoagulation, anti-vascular endothelial growth factors, and vitrectomy. Awareness, early diagnosis, and timely intervention are essential to preventing vision-threatening complications in sickle cell trait patients with SCR.

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Pachychoroid neovascular membrane in a patient with sickle cell disease trait

Córdoba-Ortega,

Arias Aristizabal,

Suta Figueroa

et al. 2024

European Journal of Ophthalmology

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Purpose To describe a rare presentation of pachychoroid neovascular membrane in a patient with sickle cell trait and the accuracy of ruling out hemoglobinopathies in the presentation of pachychoroid spectrum. Methods The patient was subjected to physical examinations, multimodal images (fluorescein angiography, optical coherence tomography), hemoglobin electrophoresis, and peripheral blood smear, documenting sickle cell trait. The management included laser treatment to target non-perfusion areas, along with a single dose of anti-VEGF. Results A 45-year-old male patient with diagnosis of pachychoroid neovascularization treated for 6 years with multiple anti VEGF injections in the left eye. A detailed clinical evaluation included hypochromic conjunctiva, peripheral vascular occlusion with non-perfusion areas led us to suspect sickle cell disease retinopathy. The images of fluorescein angiography showed peripheral arteriovenous anastomosis with non-perfusion areas; the optical coherence tomography revealed a thinner neuroepithelium with a thicker choroid; also, hemoglobin electrophoresis and peripheral blood smear documenting sickle cell trait. The chosen management was photocoagulation of the peripheral retina on the non-perfusion areas and anti VEGF without neovascular activity recurrence in the follow up period. Conclusion Although sickle cell trait is considered a mild form of this pathology without serious retinal manifestations, it has to be noted that in the context of pachychoroid spectrum diseases is a trigger that could perpetuate retinal ischemia and neovascular activity.

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Sickle Cell Trait Presenting as Unilateral Proliferative Retinopathy and Macular Thinning in a Pregnant Woman

Cited by 3 publications

References 29 publications

Visual outcome of treating proliferative sickle cell retinopathy in 108 eyes

Visual outcome of treating proliferative sickle cell retinopathy in 108 eyes

Rare Cause of Acute Loss of Vision in a Patient With Sickle Cell Trait

Pachychoroid neovascular membrane in a patient with sickle cell disease trait

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