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Multimodal imaging including optical coherence tomography angiography in patients with type B Niemann–Pick disease

  • Selim BolukbasiEmail author
  • Cezmi Dogan
  • Ertugrul Kiykim
  • Akin Cakir
  • Burak Erden
  • Alper Halil Bayat
  • Mustafa Nuri Elcioglu
  • Ayse Cigdem Aktuglu Zeybek
Original Paper

Abstract

Purpose

To evaluate accumulation patterns of deposits in retinal layers of type B Niemann–Pick patients by multimodal imaging.

Methods

Seven patients with type B Niemann–Pick disease were included in this study. All participants underwent a complete ophthalmologic evaluation, high-resolution digital colour imaging, spectral-domain optical coherence tomography, blue light fundus autofluorescence and optical coherence tomography angiography (OCTA).

Results

We demonstrated different accumulation patterns in the retinal ganglion cell layer, the retinal nerve fibre layer and the subfoveolar region by multimodal imaging. Local retinal capillary nonflow areas in the superficial plexus, increased vascular tortuosity and deformed foveal avascular areas were shown in OCTA scans.

Conclusion

Multimodal imaging including OCTA is a useful technique for the identification of different types of accumulation patterns, diagnosis and follow-up of type B Niemann–Pick patients.

Keywords

Niemann–Pick Multimodal imaging Optical coherence tomography Optical coherence tomography angiography 

Notes

Compliance with ethical standards

Conflict of interest

None of the authors has any financial or conflicting interests to disclose.

Ethical standard

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

Informed consent

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

Human and animal rights

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

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Copyright information

© Springer Nature B.V. 2019

Authors and Affiliations

  • Selim Bolukbasi
    • 1
    Email author
  • Cezmi Dogan
    • 2
  • Ertugrul Kiykim
    • 3
  • Akin Cakir
    • 1
  • Burak Erden
    • 1
  • Alper Halil Bayat
    • 1
  • Mustafa Nuri Elcioglu
    • 1
  • Ayse Cigdem Aktuglu Zeybek
    • 3
  1. 1.Department of Ophthalmology, Okmeydani Training and Research HospitalUniversity of Health SciencesIstanbulTurkey
  2. 2.Department of OphthalmologyIstanbul University Cerrahpasa Medical FacultyIstanbulTurkey
  3. 3.Department of Pediatrics, Division of Nutrition and MetabolismIstanbul University Cerrahpasa Medical FacultyIstanbulTurkey

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