High-speed, ultrahigh resolution optical coherence tomography of the retina in Hunter syndrome.
A 42-year-old man with Hunter syndrome developed bilateral visual field loss. Visual field testing demon-strated bilateral ring scotomata that corresponded to areas of thinning seen on standard resolution optical coherence tomography. High-speed, ultrahigh resolution optical coherence tomography, capable of 3.5-micron axial resolution, showed a loss of photoreceptors outside the fovea and cystoid spaces within the inner nuclear, ganglion cell, and outer nuclear layers. These results were consistent with histopathologic features that have been reported previously in patients with Hunter syndrome. Optical coherence tomography could be used as a diagnostic modality to monitor patients with Hunter syndrome and to detect subclinical forms of disease.
Yoon
Michael K
MK
New England Eye Center, Tufts-New England Medical Center, Tufts University, Boston, Massachusetts 02111, USA.
Chen
Royce W
RW
Hedges
Thomas R
TR
Srinivasan
Vivek J
VJ
Gorczynska
Iwona
I
Fujimoto
James G
JG
Wojtkowski
Maciej
M
Schuman
Joel S
JS
Duker
Jay S
JS
D000328:Adult; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D016532:Mucopolysaccharidosis II / Q000175:diagnosis* / Q000503:physiopathology; D009898:Optic Disk / Q000473:pathology; D041623:Tomography, Optical Coherence / Q000379:methods*; D058609:Visual Field Tests; D014794:Visual Fields