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Retina
Pachyvitelliform maculopathy: an optical coherence tomography analysis of a novel entity
  1. Assaf Hilely1,
  2. Adrian Au2,
  3. Won Ki Lee3,
  4. Miri Fogel Levin4,
  5. Dinah Zur1,
  6. Veronica Romero-Morales2,
  7. Ahmad Santina2,
  8. Jong Suk Lee3,
  9. Anat Loewenstein1,
  10. David Sarraf2,5
  1. 1 Division of Ophthalmology, Tel Aviv Sourasky Medical Center affiliated to Sackler Faculty of Medicine Tel Aviv University, Tel Aviv, Israel
  2. 2 Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  3. 3 Department of Ophthalmology, Nune Eye Hospital, Seoul, Republic of Korea
  4. 4 The Goldschleger Eye Insitute, The Chaim Sheba Medical Center, Tel Hashomer, Israel
  5. 5 Greater Los Angeles VA Healthcare Center, Los Angeles, California, USA
  1. Correspondence to Dr David Sarraf, UCLA Jules Stein Eye Institute, Los Angeles, California 90095, USA; dsarraf{at}ucla.edu

Abstract

Purpose To describe the optical coherence tomography features of pachyvitelliform maculopathy (PVM), an acquired vitelliform lesion (AVL) associated with pachychoroid disease.

Methods This study was a retrospective, multicentre, observational analysis.

Medical records and multimodal imaging were reviewed in all patients with pachychoroid disease and AVL. Visual acuity, central choroidal thickness (CCT), AVL dimensions, total choroidal area, luminal choroidal area, stromal choroidal area and choroidal vascular index were measured in all eyes with PVM and compared with normal age-matched control eyes.

Results Mean age of the PVM group (17 eyes of 17 patients) was 71.41 years. Average follow-up was 33.15 months. Baseline VA was 20/40 in the PVM group and declined to 20/100 (p=0.006). AVLs were all detected overlying pachyvessels with optical coherence tomography and were all hyperautofluorescent with fundus autofluorescent imaging. Mean CCT in the PVM group was significantly greater (352.35 µm) than the CCT in the control group (226.88 µm, p<0.001). Retinal pigment epithelium (RPE) disruption was present in 64.71% of eyes with PVM at baseline and 41.18% developed macular atrophy at the end of follow-up.

Conclusions PVM, defined by the presence of AVL associated with pachychoroid features, is a distinct novel entity of the pachychoroid disease spectrum. This study suggests a possible pathogenesis of RPE dysfunction secondary to a thick choroid, leading to accumulation of undigested photoreceptor outer segments and AVL. Clinicians should be aware of this common cause of vitelliform lesions and the poor visual prognosis due to the high risk of atrophy development.

  • Retina
  • Macula
  • Imaging
  • Degeneration
  • Choroid

Data availability statement

Data are available upon reasonable request.

https://doi.org/10.1136/bjo-2022-322553

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    Data availability statement

    Data are available upon reasonable request.

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    Footnotes

    • Contributors AH and DZ: collected data, data interpretation served as scientific advisors and critically reviewed the study proposal. AA: collected data, analysis, data interpretation served as scientific advisors and critically reviewed the study proposal. WKL and AL: collected data and served as scientific advisors. MF, VAR-M, AS and J-SL: collected data and drafting the work. DS: collected data and data interpretation served as scientific advisors. The corresponding author, DS: accepts full responsibility for the finished work and/or the conduct of the study, had access to the data and controlled the decision to publish.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Disclaimer AH, AA, MF, VAR-M, AS and J-SL: no financial disclosures. WKL: advisory board member of Bayer, Novartis and Roche. DZ: consultant for Abbvie, Alnylam, Bayer, Novartis and Roche. AL: consultant to Roche, Novartis, Abbvie, Beyeonics, NotalVision, WebMD and Bayer. DS: consultant to Amgen, Bayer, Genentech, Iveric Bio, Novartis and Optovue/Visionix, and received research grants from Amgen, Boehringer, Genentech, Heidelberg, Optovue/Visionix, Regeneron and Topcon.

    • 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.