Spectral OCT analysis in Bietti crystalline dystrophy.
OBJECTIVE
To report the analysis of the cornea and the macular retina using both time domain (TD-OCT) and Fourier domain optical coherence tomography (FD-OCT) in a case of Bietti crystalline dystrophy (BCD).


METHODS
This is a case study of a 32-year-old woman who presented with moderate visual loss in both eyes with nyctalopia. She had a complete ophthalmologic examination including fundus examination. She also underwent fluoroangiography, Goldmann perimetry, electroretinography/electrooculography, and both TD-OCT and FD-OCT examinations.


RESULTS
Large atrophy of retinal pigment epithelium and choroidal sclerosis were observed on both funduscopy and angiography. Microcrystals deposits were observed in the cornea and in the retina. Visual fields were constricted and electrophysiology was greatly impaired. BCD was diagnosed. TD-OCT examination was normal whereas FD-OCT revealed modifications of the outer retina and the presence of numerous lesions corresponding to microcrystals deposits in all retinal layers. The diameter of the deposits was comprised between 18 and 100 microm.


CONCLUSIONS
FD-OCT was superior to TD-OCT in detecting and localizing microcrystals in BCD. FD-OCT might be useful in the understanding and the follow-up of this rare disease.
Gaucher
David
D
Service d'Ophtalmologie du Nouvel Hôpital Civil, Hôpitaux Universitaires de Strasbourg, Strasbourg Cedex - France. david.gaucher@chru-strasbourg.fr
Saleh
Maher
M
Sauer
Arnaud
A
Bourcier
Tristan
T
Speeg-Schatz
Claude
C
D000328:Adult; D001284:Atrophy; D002829:Choroid / Q000473:pathology; D003316:Corneal Diseases / Q000175:diagnosis*; D003460:Crystallization; D004585:Electrooculography; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D012162:Retinal Degeneration / Q000175:diagnosis*; D055213:Retinal Pigment Epithelium / Q000473:pathology; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000175:diagnosis; D058609:Visual Field Tests; D014794:Visual Fields