Optical coherence tomography angiography of choroidal neovascularization in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD)
Issued Date
2023-12-01
Resource Type
ISSN
24519936
Scopus ID
2-s2.0-85178180796
Journal Title
American Journal of Ophthalmology Case Reports
Volume
32
Rights Holder(s)
SCOPUS
Bibliographic Citation
American Journal of Ophthalmology Case Reports Vol.32 (2023)
Suggested Citation
Wongchaisuwat N., Wang J., Yang P., Everett L., Gregor A., Sahel J.A., Nischal K.K., Pennesi M.E., Gillingham M.B., Jia Y. Optical coherence tomography angiography of choroidal neovascularization in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). American Journal of Ophthalmology Case Reports Vol.32 (2023). doi:10.1016/j.ajoc.2023.101958 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/91474
Title
Optical coherence tomography angiography of choroidal neovascularization in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD)
Other Contributor(s)
Abstract
Purpose: To report the clinical utility of optical coherence tomography angiography (OCTA) for demonstrating choroidal neovascularization (CNV) associated with Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHADD) retinopathy. Methods: Thirty-three participants with LCHADD (age 7–36 years; median 17) were imaged with OCTA and the Center for Ophthalmic Optics & Lasers Angiography Reading Toolkit (COOL-ART) software was implemented to process OCTA scans. Results: Seven participants (21 %; age 17–36 years; median 25) with LCHADD retinopathy demonstrated evidence of CNV by retinal examination or presence of CNV within outer retinal tissue on OCTA scans covering 3 × 3 and/or 6 × 6-mm. These sub-clinical CNVs are adjacent to hyperpigmented areas in the posterior pole. CNV presented at stage 2 or later of LCHADD retinopathy prior to the disappearance of RPE pigment in the macula. Conclusion: OCTA can be applied as a non-invasive method to evaluate the retinal and choroidal microvasculature. OCTA can reveal CNV in LCHADD even when the clinical exam is inconclusive. These data suggest that the incidence of CNV is greater than expected and can occur even in the early stages of LCHADD retinopathy.
