Purpose: This prospective study investigates longitudinal changes in retinal structure in patients with achromatopsia (ACHM) using optical coherence tomography (OCT).
Methods: Seventeen patients (five adults, 12 children) with genetically confirmed CNGA3- or CNGB3-associated ACHM underwent ocular examination and OCT over a follow-up period of between 2 and 9.33 years (mean = 5.7 years). Foveal tomograms were qualitatively graded and were segmented for quantitative analysis: central macular thickness (CMt), outer nuclear layer thickness (ONLt), and size of the foveal hyporeflective zone (vertical HRZ thickness: HRZt and horizontal HRZ width: HRZw) were measured. Data were analyzed using linear mixed regression models. Both age and visit were included into the models, to explore the possibility that the rate of disease progression depends on patient age.
Results: Fifteen of 17 patients (88%) showed longitudinal changes in retinal structure over the follow-up period. The most common patterns of progression was development of ellipsoid zone (EZ) disruption and HRZ. There was a significant increase in HRZt (P = 0.01) and HRZw (P = 0.001) between visits and no significant change in CMt and ONLt. Retinal parameters showed no difference in changes by genetic mutation (CNGA3 (n = 11), CNGB3 (n = 6)).
Conclusions: This study demonstrates clear longitudinal changes in foveal structure mainly in children, but also in adults with ACHM, over a long follow-up period. The longitudinal foveal changes suggest that treatment at an earlier age should be favored.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9363676 | PMC |
| http://dx.doi.org/10.1167/iovs.63.9.6 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Retinal OCT-Derived Texture Features as Potential Biomarkers for Early Diagnosis and Progression of Diabetic Retinopathy.
Invest Ophthalmol Vis Sci
January 2025
University of Coimbra, Coimbra Institute for Clinical and Biomedical Research (iCBR), Faculty of Medicine, Coimbra, Portugal.
Purpose: Diabetic retinopathy (DR) is usually diagnosed many years after diabetes onset. Indeed, an early diagnosis of DR remains a notable challenge, and, thus, developing novel approaches for earlier disease detection is of utmost importance. We aim to explore the potential of texture analysis of optical coherence tomography (OCT) retinal images in detecting retinal changes in streptozotocin (STZ)-induced diabetic animals at "silent" disease stages when early retinal molecular and cellular changes that cannot be clinically detectable are already occurring.
View Article and Find Full Text PDFCase report: Cilioretinal artery occlusion combined with central retinal vein occlusion as the initial presentation of pulmonary arterial hypertension.
Front Med (Lausanne)
December 2024
Department of Ophthalmology, Affiliated Eye Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China.
Background: Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressure and vascular resistance, leading to systemic venous hypertension and potential right heart failure. These elevated pressures can extend to ocular veins, resulting in complications such as central retinal vein occlusion (CRVO). This case report highlights a rare instance of CRVO combined with cilioretinal artery occlusion (CilRAO), an uncommon ocular manifestation associated with PAH.
View Article and Find Full Text PDFPhysiological change in ganglion cell inner plexiform layer and nerve fibre layer thickness over six years.
Clin Exp Ophthalmol
January 2025
Singapore Eye Research Institute, Singapore National Eye Centre, Singapore.
Background: To evaluate the 6-year physiological rates-of-change in ganglion cell inner plexiform layer (GCIPL) and retinal nerve fibre layer (RNFL) thickness measured with optical coherence tomography.
Methods: We included 2202 out of 2661 subjects from the population-based Singapore Chinese Eye Study who returned for follow-up 6 years after baseline examination (follow-up rate 87.7%).
Association of Blood Pressure and Retinal Nerve Fiber Layer Rates of Thinning in Patients with Moderate to Advanced Glaucoma.
Ophthalmol Glaucoma
January 2025
Glaucoma Division, Stein Eye Institute, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California. Electronic address:
Purpose: Investigate the influence of baseline blood pressure (BP) on retinal nerve fiber layer (RNFL) rates of change (RoC) in glaucoma patients with central damage or moderate to severe disease.
Design: Prospective cohort study.
Participants: 110 eyes with ≥4 RNFL optical coherence tomography scans and ≥2 years of follow-up.
General retinal layer segmentation in OCT images via reinforcement constraint.
Comput Med Imaging Graph
December 2024
Beijing Institute of Technology, No. 5, Zhong Guan Cun South Street, Beijing, 100081, China. Electronic address:
The change of layer thickness of retina is closely associated with the development of ocular diseases such as glaucoma and optic disc drusen. Optical coherence tomography (OCT) is a widely used technology to visualize the lamellar structures of retina. Accurate segmentation of retinal lamellar structures is crucial for diagnosis, treatment, and related research of ocular diseases.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!