Adaptive optics imaging of the outer retinal tubules in Bietti’s crystalline dystrophy

Battu, Rajani; Akkali, Mukunda C.; Bhanushali, Devanshi; Srinivasan, Priya; Shetty, Rohit; Berendschot, Tos T. J. M.; Schouten, Jan S. A. G.; Webers, Carroll A.B.

doi:10.1038/eye.2016.22

Cited by 7 publications

(4 citation statements)

References 20 publications

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“…Adaptive optics (AO) is a new imaging method that enables the direct observation of the individual rod, cone, and retinal pigment epithelial (RPE) cells. 29 , 75 Gocho et al 29 found circular patches and clusters of hyperreflective signals in the AO images that matched the crystals in three individuals with BCD. According to their thinking, the circular spots were the remains of the cone photoreceptors that lay over the crystals.…”

Section: Clinical Characteristics On Various Ancillary Testsmentioning

confidence: 95%

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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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Section: Clinical Characteristics On Various Ancillary Testsmentioning

confidence: 95%

“…According to their thinking, the circular spots were the remains of the cone photoreceptors that lay over the crystals. Battu et al 75 looked for the ORTs in BCD patients with AO and noted that ORTs appeared as serpentine tubules of varying sizes.…”

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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“… 3 , 4 Furthermore, the typical histological degeneration, including the ellipsoid zone disruption, retinal pigment epithelium (RPE) atrophy, and outer nuclear layer degeneration, can be detected much earlier than the onset of clinical symptoms, suggesting a time interval for early interventions before functional impairment. 5 , 6 Presumably, promising advances in gene therapy and other interventions for treating BCD may greatly improve the quality of patients' life in the near future. Given these potential advances, it is necessary to find morphologic indicators for prognosticating the disease course, setting therapeutic schedule, and establishing treatment strategies.…”

mentioning

confidence: 99%

“…Outer retinal tubulation has been reported in series of retinal degenerative diseases, with a significantly higher frequency in BCD. [6][7][8][9] The clinical significance of ORT lies in its prognostic value for assessing the advanced disruption of the outer retina. 7,10 However, there is limited research on elucidating the correlation between the progression of BCD and the formation of ORT.…”

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confidence: 99%

Outer Retinal Tubulation in Bietti Crystalline Dystrophy Associated With the Retinal Pigment Epithelium Atrophy

Wang¹,

Chen²,

Sun³

et al. 2022

Retina

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Supplemental Digital Content is Available in the Text.We conducted a multicenter, retrospective cohort study to determine the prognostic value of outer retinal tubulation in Bietti crystalline dystrophy. We demonstrated the morphologic characteristics of outer retinal tubulation and found that the progression of retinal pigment epithelium atrophy and vision loss was slower in eyes with outer retinal tubulation than eyes without outer retinal tubulation.

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Cross-Sectional Analysis of Outer Retinal Tubulation in Inherited Retinal Diseases: A Multicenter Study

Liu,

Lee,

et al. 2025

American Journal of Ophthalmology

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Adaptive optics imaging of the outer retinal tubules in Bietti’s crystalline dystrophy

Cited by 7 publications

References 20 publications

Diagnostic and Management Strategies of Bietti Crystalline Dystrophy: Current Perspectives

Diagnostic and Management Strategies of Bietti Crystalline Dystrophy: Current Perspectives

Outer Retinal Tubulation in Bietti Crystalline Dystrophy Associated With the Retinal Pigment Epithelium Atrophy

Cross-Sectional Analysis of Outer Retinal Tubulation in Inherited Retinal Diseases: A Multicenter Study

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