Purpose: To describe a 42-year-old man, a known case of Bietti crystalline dystrophy who underwent surgery for unilateral full thickness macular hole.
Case Report: Clinical features, color fundus photographs, and optical coherence tomography, electroretinography, and electrooculography findings of the patient are reported. His visual acuity improved from counting fingers to 20/50 after pars plana deep vitrectomy with internal limiting membrane (ILM) peeling and gas injection.
Conclusion: Macular hole can occur in Bietti crystalline dystrophy and the post-surgical outcome is good.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5525505 | PMC |
http://dx.doi.org/10.4103/jovr.jovr_154_15 | DOI Listing |
JAMA Ophthalmol
January 2025
Xiamen Eye Center of Xiamen University, Xiamen, Fujian, China.
Importance: Bietti crystalline dystrophy (BCD) is a severe genetic retinopathy caused by variants in the CYP4V2 gene. Currently, there is no approved treatment for BCD.
Objective: To evaluate safety and vision outcomes following gene therapy with adeno-associated virus (AAV) encoding CYP4V2 (rAAV-hCYP4V2, NGGT001 [Next Generation Gene Therapeutics]).
Invest Ophthalmol Vis Sci
November 2024
Southwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Medical University), Chongqing, China.
Purpose: To assess the clinical and genetic characteristics of patients with Bietti crystalline dystrophy (BCD) with a focus on potential of microperimetry in monitoring macular function.
Methods: A total of 208 genetically-confirmed BCD patients were enrolled in this retrospective study. The patients were categorized into subgroups based on their fundus characteristics (fovea sparing and fovea involved), optical coherence tomography (OCT) findings (presence/absence of retinal pigment epithelium [RPE] or ellipsoid zone [EZ] line at the fovea/parafovea), and genetic profiles (Mis/Mis, Tru/Mis, Tru/Tru).
Microvasc Res
January 2025
Beijing Tongren Eye Center, Beijing Key Laboratory of Intraocular Tumor Diagnosis and Treatment, Beijing Ophthalmology&Visual Sciences Key Lab, Medical Artificial Intelligence Research and Verification Key Laboratory of the Ministry of Industry and Information Technology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China. Electronic address:
Background: This study aimed to explore retinal changes in Bietti crystalline dystrophy (BCD) patients, including retinal metabolism, blood flow, vascular remodeling, and pupillary light reflex (PLR) abnormalities.
Methods: This cross-sectional study included 120 eyes from BCD patients and 120 eyes from healthy controls, utilizing a multimodal imaging system (MEFIAS 3200, SYSEYE, Chongqing, China) to evaluate retinal oxygenation, blood flow, vascular structure, and PLR. Measurements included oxygen saturation, blood flow velocity, vessel diameters, and pulsatility metrics.
BMC Ophthalmol
October 2024
Eye Institute and Department of Ophthalmology, Eye & ENT Hospital, Fudan University, NHC Key laboratory of Myopia and Related Eye Diseases; Key Laboratory of Myopia and Related Eye Diseases, Chinese Academy of Medical Sciences, Shanghai, China.
Background: To analyze the difference and agreement between measurements obtained by a new fully automatic optical biometer, the SW-9000 μm Plus, based on optical low-coherence reflectometry (OLCR) and a commonly used optical biometer (Pentacam AXL) based on Scheimpflug imaging with partial coherence interferometry (PCI).
Methods: The central corneal thickness (CCT), anterior chamber depth (ACD, from epithelium to anterior lens surface), lens thickness (LT), mean keratometry (K), corneal astigmatism, corneal diameter (CD), pupil diameter (PD), and axial length (AL) of 74 eyes (from 74 healthy subjects) were measured using the SW-9000 μm Plus and the Pentacam AXL to determine the agreement. Double angle plots were used for astigmatism vector analysis.
J Fr Ophtalmol
October 2024
Service d'ophtalmologie, centre hospitalier Hassan II, hôpital Omar-Drissi, Fès, Maroc; Faculté de médecine et de pharmacie et de médecine dentaire de Fès, université Sidi-Mohammed-Ben-Abdellah, Fès, Maroc.
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