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Quantitative analysis of choriocapillaris flow deficits and choroidal thickness in children with Marfan syndrome
  1. Kityee Ng,
  2. Pusheng Xu,
  3. Guangming Jin,
  4. Weijing Cheng,
  5. Xiaoling Luo,
  6. Xiaoyan Ding,
  7. Danying Zheng,
  8. Yizhi Liu
  1. State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangzhou, Guangdong, China
  1. Correspondence to Dr Danying Zheng, Sun Yat-Sen University Zhongshan Ophthalmic Center State Key Laboratory of Ophthalmology, Guangzhou 510060, China; zhengdyy{at}163.com; Dr Yizhi Liu; yzliu62{at}yahoo.com

Abstract

Purpose To evaluate the characteristics and associated factors of choroidal thickness (ChT) and choriocapillaris flow deficit percentage (CC FD%) in children with Marfan syndrome (MFS).

Methods This is a cross-sectional study. The ChT of the subfoveal area and other areas 0.5, 1.5, and 2.5 mm away from the fovea were assessed. The CC FD% of quadrant and circular regions with diameters of 0.5, 1.5 and 2.5 mm were assessed with 3×3 mm macular choriocapillaris images. Best-corrected visual acuity (BCVA) and cardiac function factor such as Z score were measured to analyse the associations with ChT and CC FD%.

Results 51 MFS children and 50 healthy controls were enrolled in this study. Compared with the healthy controls, the ChT in the MFS group was thinner in the subfoveal area, temporal 0.5, 1.5 and 2.5 mm (all p<0.001). The CC FD% was higher in circle 0.5, 1.5 and 2.5 mm (all p<0.001). Multivariate regression analysis showed that CC FD% in the circle 2.5 mm when the Z score ≥2 was associated with BCVA (β=9.08 (95% CI 3.96 to 14.20); p=0.005) and Z score (β=4.19 (95% CI 1.28 to 12.00); p=0.012).

Conclusions Thinner ChT and a higher CC FD% were observed in children with MFS, and an increased CC FD% in circle 2.5 mm was significantly associated with worse BCVA and cardiac function. These findings may help identify future visual impairment and early cardiac events in MFS children.

  • Choroid
  • Imaging
  • Lens and zonules

Data availability statement

Data are available on reasonable request. The data that support the results of this study are available from the corresponding author on reasonable request.

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Data availability statement

Data are available on reasonable request. The data that support the results of this study are available from the corresponding author on reasonable request.

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Footnotes

  • KN and PX are joint first authors.

  • Contributors KN, PX, GJ, DZ and YL designed the study; KN, PX and XL collected the data; KN, PX and WC analysed and interpreted the data; KN and PX drafted the manuscript; GJ, XD, DZ and YL revised the manuscript. DZ and YL are responsible for the overall content as the guarantor.

  • Funding This work was supported by the National Natural Science Foundation of China (81873673), and the Basic and Applied Basic Research Foundation of Guangdong Province (2021A1515011673).

  • Disclaimer None of the authors has a financial or proprietary interest in any material or method mentioned.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.