Crystalline retinopathy and optical coherence tomography angiography: new insights in pathogenesis

Kovach, Jaclyn L.; García, Diley Pérez

doi:10.21037/aes-20-89

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Few studies investigated the OCTA findings in patients with BCD. 6,[16][17][18] Miyata et al identified a CC flow deficit in 92% of eyes using OCTA, demonstrating that the presence of residual subfoveal CC was significantly associated with visual function. 6 Demir and colleagues confirmed the flow deficit in CC, highlighting also a significant deficit in VD of DCP and SCP in atrophic retina in BCD.…”

Section: Discussionmentioning

confidence: 99%

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Optical coherence tomography angiography in Bietti crystalline dystrophy

Montemagni

Arrigo

Parodi

et al. 2022

European Journal of Ophthalmology

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Purpose Bietti crystalline dystrophy (BCD) is a rare autosomal recessive retinal dystrophy caused by pathogenic variants of CYP4V2 gene and characterized by shiny yellow deposits in the retina and progressive atrophy of the retinal pigment epithelium (RPE) and choriocapillaris. The main aim of the present study is to describe the optical coherence tomography angiography (OCTA) characteristics of a patient affected by BCD. Methods A 59-years-old female with genetically confirmed BCD underwent an ophthalmological examination complete of OCTA performed in the atrophic retina, the junctional zone and the apparently normal retina. The area of choriocapillaris (CC) atrophy was compared to the area of RPE atrophy on fundus autofluorescence (FAF) imaging. Results A severe vessel density (VD) deficit at the level of superficial and deep capillary plexa as well as CC was registered in atrophic areas, which resulted deeper with respect to the junctional area, whereas the apparently preserved retina revealed VD values similar to that of control eyes. The area of RPE atrophy on FAF was larger (55.90 mm2 in right eye and 48.76 mm2 in left eye) than the area of CC atrophy on OCTA imaging (51.86 mm2 and 42.44 mm2 respectively in right and left eye). Conclusions Our findings suggest that VD impairment of retinal plexa and CC follows the degeneration of RPE as demonstrated by the greater size of the area of RPE atrophy compared to CC atrophy. Further investigations based on OCTA imaging are necessary to enhance our knowledge of this rare disease.

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Section: Discussionmentioning

confidence: 99%

“…Few studies investigated the OCTA findings in patients with BCD. 6 , 16 – 18 Miyata et al . identified a CC flow deficit in 92% of eyes using OCTA, demonstrating that the presence of residual subfoveal CC was significantly associated with visual function.…”

Section: Discussionmentioning

confidence: 99%

Optical coherence tomography angiography in Bietti crystalline dystrophy

Montemagni

Arrigo

Parodi

et al. 2022

European Journal of Ophthalmology

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Automated detection of crystalline retinopathy via fundus photography using multistage generative adversarial networks

Young Choi,

Han,

Hee Ryu

et al. 2023

Biocybernetics and Biomedical Engineering

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Sjögren–Larsson syndrome

Karakosta

Paraskevopoulos

Bisoukis

et al. 2023

Indian Journal of Ophthalmology - Case Reports

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A girl was referred to our clinic for alternating esotropia. The angle of deviation was measured at +45 prism diopters (Δ). The esotropia gradually turned to exotropia over the last 9 years (about −15Δ). Fundoscopy revealed crystalline retinopathy and in combination with the additional clinical findings of ichthyosis, spastic diplegia, and genetic testing, the diagnosis of Sjögren–Larsson syndrome (SLS) was made. The esotropia in this case, which gradually turned to exotropia without any intervention, may reflect the change in the muscle tone and the neurological damage in SLS. Thus, early strabismus surgery should be avoided to prevent its unpredictable outcomes.

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Crystalline retinopathy and optical coherence tomography angiography: new insights in pathogenesis

Cited by 3 publications

References 7 publications

Optical coherence tomography angiography in Bietti crystalline dystrophy

Optical coherence tomography angiography in Bietti crystalline dystrophy

Automated detection of crystalline retinopathy via fundus photography using multistage generative adversarial networks

Sjögren–Larsson syndrome

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