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Bevacizumab for choroidal neovascularisation in enhanced S-cone syndrome

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Abstract

Introduction

We present a case of enhanced S-cone syndrome (ESCS)-associated choroidal neovascularisation (CNV) treated successfully with intravitreal bevacizumab therapy.

Methods/case report

A 14-year-old with a known retinal dystrophy presented with acute visual deterioration. Fluorescein angiography demonstrated CNV, and treatment was initiated with an anti-vascular endothelial growth factor (anti-VEGF) agent, with significant improvement in vision. Subsequent electroretinogram examination of the patient and her younger sister showed severely reduced rod responses with accentuated fast cone (S-cone only) response, confirming the diagnosis of ESCS as the underlying dystrophy.

Conclusion

CNV is a rare complication of ESCS that is responsive to anti-VEGF therapy. Although cystic retinal lesions may develop in patients with retinal dystrophies due to multiple possible aetiologies, CNV is a known cause of macula oedema in these patients that requires treatment with different agents, namely anti-VEGF therapy. Rapid visual loss in patients with inherited retinal disorders should prompt immediate clinical assessment to exclude CNV, and if CNV is detected, anti-VEGF therapy can preserve vision.

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Authors and Affiliations

  1. Save Sight Institute, The University of Sydney, Sydney, NSW, Australia

    G. K. Broadhead, J. R. Grigg, P. McCluskey, M. Korsakova & A. A. Chang

  2. Sydney Institute for Vision Science, Sydney, NSW, Australia

    G. K. Broadhead & A. A. Chang

  3. Royal Victorian Eye and Ear Hospital, East Melbourne, VIC, Australia

    G. K. Broadhead

Authors
  1. G. K. Broadhead
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  2. J. R. Grigg
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  3. P. McCluskey
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  4. M. Korsakova
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  5. A. A. Chang
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Corresponding author

Correspondence to A. A. Chang.

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Conflict of interest

All authors certify that they have no affiliations with or involvement in any organisation or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Additional informed consent was obtained from all individual participants included in the study.

Statement on the welfare of animals

This article does not contain any studies with animals performed by any of the authors.

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Broadhead, G.K., Grigg, J.R., McCluskey, P. et al. Bevacizumab for choroidal neovascularisation in enhanced S-cone syndrome. Doc Ophthalmol 133, 139–143 (2016). https://doi.org/10.1007/s10633-016-9555-9

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  • DOI: https://doi.org/10.1007/s10633-016-9555-9

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