Choroidal Findings in Bietti's Crystalline Dystrophy

Zerbib, Jennyfer; Orès, Raphaëlle; Querques, Giuseppe; Bouzitou-Mfoumou, Rislie; Souied, Eric H.

doi:10.1097/icb.0000000000000022

Cited by 9 publications

(7 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[ 5 6 ] The crystals were better evident on en face OCT at the level of RPE. Crystals at the level of the choroid have been reported in patients with BCD using en face OCT,[ 7 ] though the crystals were not seen at the level of choroid in our case.…”

Section: Discussioncontrasting

confidence: 65%

See 1 more Smart Citation

Multimodal imaging of Bietti's crystalline dystrophy

Kumar

Gadkar

2018

Indian J Ophthalmol

View full text Add to dashboard Cite

Bietti's crystalline dystrophy (BCD) is a rare autosomal recessive retinal dystrophy characterized by deposition of crystals in the retina. The purpose of this article is to describe retinal abnormalities in BCD using multimodal imaging. An 18-year-old girl presented with decrease of vision and nyctalopia. She was assessed with color fundus picture, red-free photographs, short-wave autofluorescence, spectral-domain optical coherence tomography (OCT) and en face OCT and was diagnosed to have BCD based on typical presentation. Retinal crystals were better visualized on en face OCT as compared to conventional B scan OCT.

show abstract

Section: Discussioncontrasting

confidence: 65%

“…[ 6 ] The affected areas on OCT initially have loss of the outer retinal layers, which is followed by retinal and choroidal thinning. [ 7 ] Reports have documented crystals as hyperreflective dots on conventional SD-OCT B scans. Crystals were however only faintly visible on SD-OCT B scans in our case.…”

Section: Discussionmentioning

confidence: 99%

Multimodal imaging of Bietti's crystalline dystrophy

Kumar

Gadkar

2018

Indian J Ophthalmol

View full text Add to dashboard Cite

show abstract

“…However, that crystal deposits were seen in all the different retinal layers in SD-OCT studies [9,10]. Another study demonstrated that retinal crystals were observed as tiny hyperreflective lesions localized in the choroid [11].…”

Section: Discussionmentioning

confidence: 99%

Bietti Crystalline Retinal Dystrophy: A Case Report

Şahin

Yıldırım²,

Türkçü³

et al. 2016

J Clin Exp Invest

View full text Add to dashboard Cite

Bietti' crystalline retinal dystrophy (BCD) is a rare, autosomal, recessively inherited disorder, characterized by the deposition of yellow crystals in the corneal limbus and retina. In this paper we aimed to present a pediatric case with BCD, with clinical, electrophysiological and spectral domain optical coherence tomography (SD-OCT) findings and discuss BCD with the light of the literature. J Clin Exp Invest 2016; 7 (1): 94-97

show abstract

“…There were no choroidal hyperreflective spots in four eyes with advanced disease stage and severe chorioretinal thinning. Zerbib et al 61 also mentioned that choroidal crystals could be observed also in the choroid and added that SD-OCT might have allowed the clinicians to detect the hyperreflective foci better due to existing retinal atrophy. 61 …”

Section: Clinical Characteristics On Various Ancillary Testsmentioning

confidence: 99%

Diagnostic and Management Strategies of Bietti Crystalline Dystrophy: Current Perspectives

Saatçi¹,

Atas²,

Çetin³

et al. 2023

OPTH

View full text Add to dashboard Cite

Bietti crystalline dystrophy (BCD) is a rare, genetically determined chorioretinal dystrophy presenting with intraretinal crystalline deposits and varying degrees of progressive chorioretinal atrophy commencing at the posterior pole. In some cases, there can be concomitant corneal crystals noted first in the superior or inferior limbus. CYP4V2 gene, a member of the cytochrome P450 family is responsible for the disease and more than 100 mutations have been defined thus far. However, a genotype–phenotype correlation has not been established yet. Visual impairment commonly occurs between the second and third decades of life. By the fifth or sixth decade of life, vision loss can become so severe that the patient may potentially become legally blind. Multitudes of multimodal imaging modalities can be utilized to demonstrate the clinical features, course, and complications of the disease. This present review aims to reiterate the clinical features of BCD, update the clinical perspectives with the help of multimodal imaging techniques, and overview its genetic background with future therapeutic approaches.

show abstract

Choroidal Findings in Bietti's Crystalline Dystrophy

Abstract: Our study gives new information on the aspect and the location of crystal deposits in the choroid and the abnormalities of the outer retina associated with the disease.

Cited by 9 publications

References 4 publications

Multimodal imaging of Bietti's crystalline dystrophy

Multimodal imaging of Bietti's crystalline dystrophy

Bietti Crystalline Retinal Dystrophy: A Case Report

Diagnostic and Management Strategies of Bietti Crystalline Dystrophy: Current Perspectives

Contact Info

Product

Resources

About