June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
Long-term Visual Outcomes of juxta-papillary retinal hemangioblastoma in Von Hippel-Lindau Disease
Author Affiliations & Notes
  • Eleni Konstantinou
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Sanjeeb Bhandari
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Elvira Agron
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Tiarnan D L Keenan
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Catherine A Cukras
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Alisa Thavikulwat
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Henry Wiley
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Emily Y Chew
    Retina, National Eye Institute, Bethesda, Maryland, United States
  • Footnotes
    Commercial Relationships   Eleni Konstantinou None; Sanjeeb Bhandari None; Elvira Agron None; Tiarnan Keenan None; Catherine Cukras None; Alisa Thavikulwat None; Henry Wiley None; Emily Chew None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 3140 – A0035. doi:
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    • Get Citation

      Eleni Konstantinou, Sanjeeb Bhandari, Elvira Agron, Tiarnan D L Keenan, Catherine A Cukras, Alisa Thavikulwat, Henry Wiley, Emily Y Chew; Long-term Visual Outcomes of juxta-papillary retinal hemangioblastoma in Von Hippel-Lindau Disease. Invest. Ophthalmol. Vis. Sci. 2022;63(7):3140 – A0035.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To evaluate the features and the visual outcomes of retinal hemangioblastomas arising in proximity to the optic disc in Von Hippel Lindau Disease (VHL) at the National Institutes of Health Clinical Center

Methods : Retrospective longitudinal analysis of patients with ONRH associated with VHL. Data were collected on the duration of follow-up, visual acuities (VA), characteristics of RH on multimodal imaging including color fundus photography, ultra-widefield photography, optical coherence tomography, and autofluorescence imaging. Ocular and lesion characteristics at last follow up were compared with baseline, and response to any treatment was assessed.

Results : A total of 59 patients (53% females) with VHL first examined between 2003 - 2020 had ONRH in at least 1 eye. Of these, 10 (17%) patients were examined only once and exclude from further analysis. The total cohort’s mean (SD) age at baseline was 37 (16) years. Twenty-six (44%) patients had ONRH in the right eye while bilateral ONRH was found in 5 (8%) patients. The presence or absence of ONRH could not be confirmed in 7 prosthetic eyes (1 right and 6 left). The mean (SD) VA at baseline in eyes with ONRH was 62.9 (27.9) letters (Snellen equivalent 20/60) and 80.4 (18.5) letters (20/25) in eyes that did not have ONRH. Patients were followed for a median (Q1, Q3) of 6.05 (2.09, 10.3) years and the mean VA in the eye with ONRH dropped to 52.85 (31.7) letters (Snellen equivalent of 20/100) at the time of last follow-up.

Conclusions : This longitudinal case series demonstrates progressive loss of visual acuity in this subset of VHL-associated retinal hemangioblastomas, for which treatment options are limited. New treatments, such as systemic hypoxia inducible factor (HIF)-2α inhibitors, hold promise for management of these vision-threatening tumors.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.

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