Novel findings in enhanced S-cone syndrome: a case with macular retinal neovascularization and severe retinal vasculitis

  • Fatemeh Bazvand
  • Hasan Khojasteh
  • Mohammad ZareiEmail author
Clinical Case Report



To describe a novel association of enhanced S-cone syndrome (ESCS) with macular retinal neovascularization and severe retinal vasculitis.


Clinical examination, spectral domain optical coherence tomography, fluorescein angiography, fundus autofluorescence, infrared reflectance and electroretinography were used to study a 25-year-old male with a history of night blindness from early childhood and recent accelerated visual loss in right eye.


Pigmented lesions were observed along the arcades without peripheral retinal involvement. Intraretinal cystoid spaces, retinal neovascularization of posterior pole and severe peripheral and posterior retinal vasculitis were found on clinical examination and multimodal imaging. Based on characteristic clinical and electroretinographic findings, a diagnosis of ESCS was made.


This case highlights novel associations of retinal neovascularization and vasculitis with ESCS.


Enhanced S-cone syndrome Electroretinography Neovascularization Retinal vasculitis 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Statement of human rights

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 Declaration of Helsinki and its later amendments or comparable ethical standards.

Statement on the welfare of animals

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

Informed consent

Informed consent was obtained from all participants included in the study.

Patient consent

The patient has consented to the submission of the case report for submission to the journal.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Fatemeh Bazvand
    • 1
  • Hasan Khojasteh
    • 1
  • Mohammad Zarei
    • 1
    Email author
  1. 1.Retina Service, Farabi Eye HospitalTehran University of Medical SciencesTehranIran

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