Abstract

Aims: To describe the clinical characteristics of the patients with superior segmental optic hypoplasia (SSOH) and quantitatively assess the structural abnormalities with optical coherence tomography (OCT) and Heidelberg retina tomography (HRT).

Methods: Twenty-three eyes from 23 patients (11 men and 12 women, mean age 44.3 years) with SSOH were enrolled and 23 control subjects were matched for age and optic disc size. In addition to detailed ophthalmoscope examination and standard automated perimetry, OCT and HRT were performed for the quantitative assessment of the retinal nerve fibre layer (RNFL) and optic nerve head.

Results: The OCT images showed that the eyes with SSOH had significantly thinner RNFL than the control subjects in all segments except for the papillomacular bundle area. The area under the receiver operator characteristic curve (AROC) was greatest for the RNFL thickness of superonasal one o’clock segment (AROC = 0.991, p<0.001) measured by the OCT, and for the rim area of superonasal segment (AROC = 0.837, p = 0.001) measured by the HRT.

Conclusion: More generalised thinning of the RNFL, beyond the superior retina, was identified in the eyes with SSOH. The OCT and HRT were valuable as ancillary diagnostic tests for SSOH, even in mild cases.