Macular Lesions Associated With Retinitis Pigmentosa

Fishman, Gerald A.; Fishman, Marlene; Maggiano, John

doi:10.1001/archopht.1977.04450050076006

Cited by 115 publications

(57 citation statements)

References 11 publications

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“…[1][2][3][4] The prevalence of 11-20% macular oedema in RP patients has most commonly been reported using fluorescein angiography (FA) or optical coherence tomography (OCT). 2,[5][6][7] In future treatment trials where central visual acuity is used as an outcome measure, the presence of cystic macular lesions could represent a confounding variable for monitoring a therapeutic intervention aimed at primarily improving macular photoreceptor cell function.…”

Section: Introductionmentioning

confidence: 99%

The prevalence of cystoid macular oedema on optical coherence tomography in retinitis pigmentosa patients without cystic changes on fundus examination

Hajali

Fishman

2008

Eye

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Purpose To determine the prevalence of cystoid macular oedema (CME) by optical coherence tomography (OCT) in retinitis pigmentosa (RP) patients with no evidence of cystic macular lesions on fundus examination. Methods We included 63 RP patients with no evidence of cystic-appearing macular changes on fundus examination. All patients underwent a complete ocular examination including best-corrected visual acuity using an ETDRS (Early Treatment Diabetic Retinopathy Study) chart, intraocular pressure measurement, anterior segment examination, and a detailed fundus examination. On 50 of the 63 patients, Fourier-domain OCT was performed using the radial slicer protocol. An additional 13 of the 63 patients were scanned using the macular thickness protocol on a time-domain OCT unit. The diagnosis of CME was defined by the presence of hyporeflective lacunae with well-defined boundaries on at least two of the scans. Results The mean age of patients included in the study was 36 years (range 9-71 years). Out of the 63 patients examined, 20 showed CME in at least one eye (32%), whereas 11 patients showed CME in both eyes (18%). Conclusions Our findings demonstrate that a substantial number of RP patients with CME, as determined by OCT, may not show cystic changes by direct ophthalmoscopy or contact lens biomicroscopy. Knowledge of the high frequency for CME in such patients can serve to identify those who may be amenable to current or future treatment strategies of their macular oedema and can potentially impact on future therapeutic trials where visual acuity is used as an outcome measure.

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Section: Introductionmentioning

confidence: 99%

The prevalence of cystoid macular oedema on optical coherence tomography in retinitis pigmentosa patients without cystic changes on fundus examination

Hajali

Fishman

2008

Eye

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“…A gradual decrease in visual The gradual decrease in visual acuity in retinitis pigmentosa is usually attributed to three types of macular changes [2]. The most prevalent macular change is RPE atrophy, which was reported in 58% of a series of 31 patients who had macular involvement.…”

Section: Discussionmentioning

confidence: 99%

“…Other macular changes are cystoid macular edema, which is found in 23% of cases, and macular cysts or lamellar holes, in 19%. Cystoid macular edema has been attributed to retinal traction by condensed posterior vitreous or preretinal membranes, while retinal cysts and holes have been attributed to macular traction or macular atrophy [2]. Such atrophy may result when the photoreceptor and RPE layers are lost.…”

Section: Discussionmentioning

confidence: 99%

Central serous chorioretinopathy associated with retinitis pigmentosa

Dorenboim

Rehany

Rumelt

2004

Graefe's Arch Clin Exp Ophthalmol

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Macular changes may appear in retinitis pigmentosa patients and include macular atrophy, cystoid macular edema, retinal cysts, and holes. However, other primary macular diseases have not been described in patients with retinitis pigmentosa, probably because of atrophy of the retinal pigment epithelium (RPE) and the overlying retina. We present a 35-year-old patient whose first symptom was an acute decrease in visual acuity due to central serous chorioretinopathy (CSCR). Retinitis pigmentosa was subsequently diagnosed. We assume that the macular RPE changes may be attributed to both cone and RPE atrophy or other macular pathophysiologic processes, one of which may be CSCR.

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“…Instead clear cystoid spaces were seen covering the atrophic area in the centre of the macula. Cystoid changes of the macula are fairly common in other hereditary dystrophies like retinitis pigmentosa (4,5,6) or dominant cystoid macular oedema (3). In these diseases macular oedema is usually due to capillary leakage, although dye leakage on fluorescein angiogram has not always been detected in retinitis pigmentosa with cystoid macular changes (4,5).…”

Section: Discussionmentioning

confidence: 99%

“…Cystoid changes of the macula are fairly common in other hereditary dystrophies like retinitis pigmentosa (4,5,6) or dominant cystoid macular oedema (3). In these diseases macular oedema is usually due to capillary leakage, although dye leakage on fluorescein angiogram has not always been detected in retinitis pigmentosa with cystoid macular changes (4,5). Capillary leakage has been thought to be secondary to toxic effects of the degenerative process of the photoreceptors or pigment epithelium (1,9) or shrinkage of the preretinal membrane (4).…”

Section: Discussionmentioning

confidence: 99%

Presumed vitelliform dystrophy with perimacular flecks and retinal detachment

Laatikainen¹

1979

Int Ophthalmol

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Seven of eight siblings of asymptomatic non-consanguineous parents were investigated. Two of them had atrophic cystoid macular degeneration and flat or subnormal electro-oculograms suggesting the diagnosis of vitelliform dystrophy. In one eye the central cystoid lesion was surrounded by atypical small whitish hyperfluorescent flecks resembling fundus flavimaculatus. In the other eye of this patient cystoid macular degeneration progressed to shallow non-rhegmatogenous detachment of the retina. One of the asymptopmatic siblings had a mild colour vision defect of tritan-type and some fleckish hyperfluorescence around the macula and another sister showed abnormal EOG responses. These patients are probably carriers of the pathological gene responsible for the disease.

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Macular Lesions Associated With Retinitis Pigmentosa

Cited by 115 publications

References 11 publications

The prevalence of cystoid macular oedema on optical coherence tomography in retinitis pigmentosa patients without cystic changes on fundus examination

The prevalence of cystoid macular oedema on optical coherence tomography in retinitis pigmentosa patients without cystic changes on fundus examination

Central serous chorioretinopathy associated with retinitis pigmentosa

Presumed vitelliform dystrophy with perimacular flecks and retinal detachment

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