Aims To characterise the optic nerve head (ONH) configurations of superior segmental optic hypoplasia (SSOH) using a spectral-domain optical coherence tomography (SD-OCT).
Methods Horizontal cross-sectional images of the ONH were obtained by a SD-OCT in 20 eyes of 14 patients with clinically typical SSOH and 20 age- and refraction-matched normal eyes.
Results Extension of retinal pigment epithelium (RPE) over the disc margin was observed in 20 eyes (100%) with SSOH and eight (40%) control eyes (p<0.001). The maximum length of the overhanging RPE measured at the nasal disc margin was significantly longer in eyes with SSOH than in controls (295.9±112.6 vs 24.3±33.9 μm; p<0.0001). In eyes with SSOH, the retinal nerve fibre layer (RNFL) was significantly thinner in the temporal-superior to nasal sectors (p<0.01).
Conclusions The extension of RPE over the nasal disc margin was more common and longer in eyes with SSOH than in normal eyes, suggesting that the SD-OCT findings might facilitate the diagnosis of SSOH. Substantial thinning of the RNFL was present in wider areas than previously expected in SSOH.
- Optic nerve, spectral-domain optical coherence tomography
- superior segmental optic hypoplasia
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Funding Grant-in-Aid for Scientific Research by the Ministry of Health, Labour and Welfare of Japan (H18-Sensory-General-001).
Competing interests The authors have financial interests in Topcon Corporation as consultant advisors.
Patient consent Obtained.
Ethics approval This study was conducted with the approval of the Institutional Ethics Committee of the University of Tokyo Hospital, Tokyo, Japan, and adhered to the tenets of the Declaration of Helsinki.
Provenance and peer review Not commissioned; externally peer reviewed.