Retinal nerve fiber layer evaluation by optical coherence tomography in Leber's hereditary optic neuropathy.
OBJECTIVE
To study retinal nerve fiber layer (RNFL) thickness by optical coherence tomography (StratusOCT) in patients with Leber's hereditary optic neuropathy (LHON).


METHODS
Cross-sectional study.


METHODS
Thirty-eight patients with LHON were analyzed and compared with an age-matched control group of 75 patients. Patients with LHON were classified as having early LHON (E-LHON, n = 8) when the duration of the disease was shorter than 6 months and atrophic LHON (A-LHON, n = 30) when the duration was longer than 6 months.


METHODS
The fast RNFL thickness (3.4) scan acquisition protocol was used.


METHODS
Retinal nerve fiber layer thickness as measured by StratusOCT.


RESULTS
Compared with the control group, eyes with E-LHON showed a thicker RNFL in the 360 degrees average measurement (P<0.01) and in the superior (P<0.01), nasal (P<0.05), and inferior quadrants (P<0.05); no significant changes were detected in the temporal quadrant. Eyes with A-LHON revealed a thinner RNFL in all measurements (P<0.001); the fibers of the nasal quadrant showed the lowest amount of reduction (38% vs. 42%-49.8% in the other quadrants). In cases with A-LHON and visual recovery, RNFL was significantly thicker in all measurements (P<0.001), except the temporal quadrant, with respect to A-LHON without visual recovery.


CONCLUSIONS
On the basis of OCT data, the RNFL is thickened in E-LHON and severely thinned in A-LHON. RNFL is likely to be partially preserved in A-LHON with visual recovery. The temporal fibers (papillomacular bundle) are the first and most severely affected; the nasal fibers seem to be partially spared in the late stage of the disease.
Barboni
Piero
P
Centro di Oftalmologia Salus, Bologna, Italy. p.barboni@eyeproject.com <p.barboni@eyeproject.com>
Savini
Giacomo
G
Valentino
Maria Lucia
ML
Montagna
Pasquale
P
Cortelli
Pietro
P
De Negri
Anna Maria
AM
Sadun
Federico
F
Bianchi
Stefania
S
Longanesi
Lora
L
Zanini
Maurizio
M
de Vivo
Antonello
A
Carelli
Valerio
V
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003430:Cross-Sectional Studies; D003941:Diagnostic Techniques, Ophthalmological; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009412:Nerve Fibers / Q000473:pathology*; D029242:Optic Atrophy, Hereditary, Leber / Q000175:diagnosis* / Q000235:genetics; D012165:Retinal Ganglion Cells / Q000473:pathology*; D041623:Tomography, Optical Coherence