Multimodal imaging of Bietti's crystalline dystrophy

Kumar, Vinod; Gadkar, Amit

doi:10.4103/ijo.ijo_39_18

Cited by 7 publications

(4 citation statements)

References 6 publications

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“…Previous studies have shown that crystalline deposits in BCD are better visualized on the NIR imaging when compared with other modalities. 6,[15][16][17] However, in view of noninferiority of RF imaging in delineating these deposits and its wider availability, it might be prudent to consider it as a primary screening tool before NIR reflectance. Image resolution is important to detect subtle fundus changes and can affect interpretation of data.…”

Section: Discussionmentioning

confidence: 99%

Role of red free imaging, retinal reflectance and fundus autofluorescence in Bietti crystalline dystrophy: case report

Surve

Balaji

Azad

et al. 2020

Therapeutic Advances in Rare Disease

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Bietti crystalline dystrophy (BCD), a rare autosomal recessive hereditary disorder, is identified by its clinical features. It is characterised by crystalline deposits and hence called crystalline retinopathy. The retinopathy progresses with age, showing a decrease in the number of crystalline deposits and increase in the area of chorioretinal degeneration, which spreads in a centrifugal pattern. Thus, BCD can be confused with other disorders with crystalline-like deposits and chorioretinal degenerations. The red-free and near-infrared reflectance allows prominent visualisation of crystalline deposits that may be missed. The non-inferiority of red-free imaging and its wider availability could allow its use as a screening tool. The enhanced depth imaging optical coherence tomography shows crystalline deposits throughout the retina and outer retinal tubulation. Thus, multimodality imaging can act as an adjunct in diagnosis, monitoring and follow up in these cases, acting not only as a teaching tool but also giving an insight into the underlying pathophysiology of the disorder. Plain language summary Use of imaging in diagnosis of Bietti crystalline dystrophy Bietti crystalline dystrophy is a rare familial disorder but is not shown in all family members and may skip generations, as it has an autosomal recessive pattern of inheritance. It shows refractile yellow-white crystalline deposits and degenerative changes in the retina. These crystalline deposits disappear with age while degenerative changes increase and spread from centre to the periphery. This may cause difficulty in early detection, and confusion with similar degenerative diseases of the retina. The use of various imaging modalities can help in diagnosis and follow up of these cases. These modalities also provide understanding of the basic disease process.

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Section: Discussionmentioning

confidence: 99%

Role of red free imaging, retinal reflectance and fundus autofluorescence in Bietti crystalline dystrophy: case report

Surve

Balaji

Azad

et al. 2020

Therapeutic Advances in Rare Disease

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“…Swept-source OCT was also utilized in BCD patients and SS-OCT also revealed the tubulations in the outer nuclear layer, destruction of the ellipsoid and interdigitation zone of the retina, hyperreflective retinal deposits in all retinal layers, primarily in the RPE/Bruch membrane complex, and patchy loss of the outer retina and RPE in the advanced stage. 31 , 62 , 63 …”

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

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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.

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“…In addition, 1/2–1/3 of the patients show bright yellow crystals in the superficial paralimbal cornea. [ 1 2 3 ]…”

Section: Introductionmentioning

confidence: 99%

Bietti crystalline dystrophy complicated by choroidal neovascularization treated with a single dose of aflibercept

Özbek

Pehlivanoğlu²,

Artunay³

2023

Oman J Ophthalmol

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Bietti crystalline dystrophy is a rare hereditary autosomal recessive disease that causes photoreceptor loss secondary to degeneration of retinal pigment epithelium due to intracellular retinal pigment epithelial crystalline deposits and abnormal lipid metabolism. We aimed to present a case of choroidal neovascular membrane secondary to Bietti crystalline dystrophy diagnosed with multimodal imaging and treated with an intravitreal injection of aflibercept. A single dose of aflibercept injection might be effective due to its higher affinity for vascular endothelial growth factor (VEGF) in comparison with other anti-VEGFs. It might be a treatment alternative that can be considered in the choroidal neovascular membrane due to uncommon etiologies.

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Multimodal imaging of Bietti's crystalline dystrophy

Cited by 7 publications

References 6 publications

Role of red free imaging, retinal reflectance and fundus autofluorescence in Bietti crystalline dystrophy: case report

Role of red free imaging, retinal reflectance and fundus autofluorescence in Bietti crystalline dystrophy: case report

Diagnostic and Management Strategies of Bietti Crystalline Dystrophy: Current Perspectives

Bietti crystalline dystrophy complicated by choroidal neovascularization treated with a single dose of aflibercept

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