Proliferative Retinopathy in Sickle Cell Trait

Nagpal, Krishan C.; Asdourian, George K.; Patrianakos, Dimitrios; Goldberg, Morton F.; Rabb, Maurice F.; Goldbaum, Michael H.; Raichand, Motilal

doi:10.1001/archinte.1977.03630150035011

Cited by 45 publications

(9 citation statements)

References 25 publications

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“…In previous cases the above complications occurred in patients with concomitant systemic disease (e.g. hypertension, TB, diabetes, sarcoidosis, syphilis, rheumatoid arthritis) (Nagpal et al 1977). However, four cases have been reported in association with severe blunt ocular trauma in the absence of systemic disease (Kabakow et al 1955; Michelson & Pfaffenbach 1972; Sorr & Goldberg 1975; Jackson et al 1995).…”

Section: Discussionmentioning

confidence: 95%

“…Documented retinal pathology associated with Hb AS includes retinal haemorrhages and exudates (Nagpal et al 1977), angioid streaks (Lieb et al 1959), acute chorioretinal infarctions (Lieb et al 1959), chorioretinitis, vitreous hemorrhage (Kabakow et al 1955), retinal vasculature abnormalities – retinal vein tortuosity/dilation (Lieb et al 1959), microaneurysms (Lieb et al 1959), central retinal artery occlusion (Kabakow et al 1955; Michelson & Pfaffenbach 1972; Sorr & Goldberg 1975) and retinitis proliferans (Lieb et al 1959).…”

Section: Discussionmentioning

confidence: 99%

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Latent proliferative sickle cell retinopathy in sickle cell trait

Mehta

Whittaker

Tsaloumas

2001

Acta Ophthalmologica Scandinavica

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ABSTRACT.Purpose: To describe a patient with sickle cell trait who developed latent proliferative sickle cell retinopathy after mild blunt trauma. P roliferative sickle cell retinopathy is characterized by vascular proliferation and is most commonly observed in patients with Hb SC, Hb S-thalassemia and Hb SS. We report a case of a patient with sickle cell trait (Hb AS) who developed latent, uniocular, proliferative sickle cell retinopathy after an episode of mild blunt ocular trauma. Case ReportA 20-year-old Ghanaian student presented complaining of flashing lights and floaters in his left eye associated with a periorbital aching pain. He was elbowed in the left eye playing football two days earlier, resulting in 'tunnel vision' which settled within one hour. He had no past medical history of note.He had sustained a similar injury to the left eye, playing football three years previously. An ophthalmic assessment then revealed moderate anterior chamber inflammation, intraocular pressure (IOP) 30 mmHg and normal dilated fundoscopy. He was discharged from follow-up after the IOP returned to normal.Current examination showed unaided visual acuity 6 / 5 RE 6 / 6 LE. Pupillary reactions, anterior segments and intraocular pressures were normal. There was no aniscoria. Dilated fundal examination showed no vitreous abnormalities and a normal right fundus. The left fundus showed five areas (150 ae) of peripheral sea-fan neovascularization (Stage 3) with an ischaemic ridge from 9-4 o'clock delineating avascular from vascular retina. He was normotensive and systemic examination was unremarkable.Full blood count, chest X-ray, urea & electrolytes and blood glucose were normal. Haemoglobin electrophoresis revealed the genotype Hb AS (HbA 60%, HbS 40%) with normal mean cell haemoglobin/mean cell volume.Fundus fluorescein angiography delineated the areas of neovascularization and also showed areas of capillary drop out in the peripheral fundus of the left eye (Fig. 1). There was no abnormality in the vasculature in the right eye.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Latent proliferative sickle cell retinopathy in sickle cell trait

Mehta

Whittaker

Tsaloumas

2001

Acta Ophthalmologica Scandinavica

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“…Nagpal et al suggest that the combination of systemic disease and SCT may be more pathogenic for retinopathy than any one of the conditions by itself. The presence of systemic disease creates unfavorable conditions that exacerbate the sickling process, resulting in ocular signs similar to sickle cell disease [ 13 ]. Therefore, the occurrence of retinopathy in sickle cell trait patients requires comprehensive medical assessments to rule out systemic diseases [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…There are a few other reports of proliferative reti-nopathy in SCT cases. In most of these studies, the SCT was accompanied by other systemic or ocular diseases such as diabetes, hypertension, sarcoidosis, tuberculosis, gestational diabetes, and ocular trauma [13][14][15][16]. Nagpal et al suggest that the combination of systemic disease and SCT may be more pathogenic for retinopathy than any one of the conditions by itself.…”

Section: Discussionmentioning

confidence: 99%

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

Ghods

Pour

Faghihi

et al. 2021

Case Reports in Ophthalmological Medicine

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Background. To report a case of a pregnant woman with sickle cell trait (SCT) who presented with unilateral proliferative sickle cell retinopathy. Case Presentation. A 26-year-old otherwise healthy pregnant woman presented with the complaint of visual loss in her left eye. The funduscopic examination showed vitreous hemorrhage, sea fan neovascularization, and pale optic disc. Optical coherence tomography revealed macular inner retinal layer thinning and foveal splaying (saucerization of the foveal pit). The investigations, including hemoglobin electrophoresis, verified the diagnosis of sickle cell trait. Blood pressure, fasting blood glucose tests, and tuberculin skin tests were normal. We treated the patient by peripheral retinal photocoagulation over the area of nonperfusion. Conclusion. Even though the sickle cell trait is generally considered as a milder form of sickle cell disease without severe retinal manifestations, pregnancy should be considered as a trigger that can induce proliferative changes and foveal splaying in this group of patients.

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“…Sickling cells are red blood cells with abnormal shape and lower deformability which can cause them to undergo haemolysis (haemolytic anaemia) [1]. The haemolytic anaemia and the vaso-occlusive processes lead to retinal hypoxia, ischemia, infarction and neovascularization [9, 11]. This is due to their or be removed by macrophages in the spleen.…”

Section: Discussionmentioning

confidence: 99%

A retinopathy in young patient with co-inheritance of heterozygous alpha + −thalassemia and sickle trait: a case report

Ouzzif¹,

Maataoui

Traore³

et al. 2017

BMC Ophthalmol

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BackgroundThe retinopathy is an uncommon complication in individuals with sickle cell trait except for the cases of sickle cell trait associated with systemic arterial hypertension, diabetes mellitus, syphilis, tuberculosis and sarcoidosis.Case PresentationA retinopathy in a 16 year-old child with no history of consanguinity in the parents revealed a sickle S trait associated to heterozygous alpha thalassemia. His mother has Sickle cell anaemia (Hb SS) and his father is a carrier of heterozygous alpha-thalassemia status that it was unknown before.ConclusionThis case report describes a proliferative retinopathy in a 16 year-old patient with co-inheritance of heterozygous alpha + −thalassemia and sickle trait.

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Proliferative Retinopathy in Sickle Cell Trait

Cited by 45 publications

References 25 publications

Latent proliferative sickle cell retinopathy in sickle cell trait

Latent proliferative sickle cell retinopathy in sickle cell trait

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

A retinopathy in young patient with co-inheritance of heterozygous alpha + −thalassemia and sickle trait: a case report

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