May 2008
Volume 49, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2008
Characteristics of Fundusautofluorescence in Pseudoxanthoma Elasticum and Correlation With High-Resolution Spectral Domain Optical Coherence Tomography
Author Affiliations & Notes
  • R. P. Finger
    Department of Ophthalmology, Bonn Univ, Bonn, Germany
  • P. Charbel Issa
    Department of Ophthalmology, Bonn Univ, Bonn, Germany
  • F. G. Holz
    Department of Ophthalmology, Bonn Univ, Bonn, Germany
  • H. P. N. Scholl
    Department of Ophthalmology, Bonn Univ, Bonn, Germany
  • Footnotes
    Commercial Relationships  R.P. Finger, None; P. Charbel Issa, None; F.G. Holz, Novartis; Acucela; Pfizer; Sirion Therapeutics; Heidelberg Engineering; Zeiss Meditec, C; Novartis; Genentech; Allergan, R; H.P.N. Scholl, None.
  • Footnotes
    Support  EU FP6, Integrated Project "EVIGENORET" (LSHG-CT-2005-512036); DFG Heisenberg Fellowship SCHO 734/2-1; DFG WE 1259/14-3; PXE Patient Association Germany
Investigative Ophthalmology & Visual Science May 2008, Vol.49, 2173. doi:https://doi.org/
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      R. P. Finger, P. Charbel Issa, F. G. Holz, H. P. N. Scholl; Characteristics of Fundusautofluorescence in Pseudoxanthoma Elasticum and Correlation With High-Resolution Spectral Domain Optical Coherence Tomography. Invest. Ophthalmol. Vis. Sci. 2008;49(13):2173. doi: https://doi.org/.

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

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Abstract

Purpose: : Pseudoxanthoma elasticum (PXE) is a systemic disease which frequently leads to a loss of vision due to ocular involvement. Typical findings include angioid streaks, peau d’orange and salmon spots. The retinal pigment epithelium (RPE) has been suggested to be of importance in the progression of disease and the occurrence of visual loss. To assess the integrity of the RPE-photoreceptor complex fundus autofluorescence (FAF) imaging and spectral domain optical coherence tomography (SD-OCT) were performed in a large cohort of PXE patients.

Methods: : The diagnosis of PXE was confirmed by clinical examination, ABCC6 gene mutation screening and skin biopsies. Retinal imaging techniques included digital fundus photography, fluorescein angiography (FAG), FAF imaging and SD-OCT (HRA SPECTRALIS, Heidelberg Engineering). Findings were described and classified into several categories related to the disease stage (early changes, presence of CNV or fibrovascular scar) and appearance on FAF.

Results: : Mean age of the 46 patients (92 eyes) analyzed was 50 years (SD 13.8, range 13 - 74) and mean best-corrected distance visual acuity (BCVA) 20/125 (LogMAR mean 0.8, SD 0.69). PXE-typical fundus changes were present in all eyes. Angioid streaks were visible in all eyes in all imaging modalities and showed abnormal layering of the RPE-photoreceptor complex in SD-OCT. Focal spots of increased FAF alongside angioid streaks, consisting of pigmentations visible on fundus photography, constitute the parastreak phenomenon. Peau d’orange was - contrary to salmon spots - not visible on FAF and SD-OCT but detectable by infrared reflectance imaging. Further findings such as drusen of the optic nerve head (5 eyes) and pattern dystrophies (8 eyes) were observed in a subset of patients.

Keywords: retinal degenerations: hereditary • imaging/image analysis: clinical • retinal neovascularization 
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