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

Int Ophthalmol 2019 Apr 11. Epub 2019 Apr 11.

Department of Pediatrics, Division of Nutrition and Metabolism, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey.

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.

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http://dx.doi.org/10.1007/s10792-019-01102-yDOI Listing
April 2019
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References

(Supplied by CrossRef)
Article in BMJ Case Rep
RG Simões et al.
BMJ Case Rep 2014
Article in Int J Mol Sci
MD Manshadi et al.
Int J Mol Sci 2015
Article in Mol Genet Metab
EH Schuchman et al.
Mol Genet Metab 2017
Article in Sci Rep
JP Campbell et al.
Sci Rep 2017
Article in Am J Ophthalmol
J Libert et al.
Am J Ophthalmol 1975
Article in Invest Ophthalmol
MR Robb et al.
Invest Ophthalmol 1973
Article in JAMA Ophthalmol
DS Rudich et al.
JAMA Ophthalmol 2013
Article in Arch d’Anat Microsc
A Rochon-Duvigneaud et al.
Arch d’Anat Microsc 1907
Article in PeerJ
AV Tschulakow et al.
PeerJ 2018
Article in BMC Neurosci
X Yan et al.
BMC Neurosci 2014
Article in BMC Ophthalmol
C Shen et al.
BMC Ophthalmol 2018

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