Optic nerve head analysis of superior segmental optic hypoplasia using Heidelberg retina tomography

Abstract

PurposeTo evaluate the optic disc characteristics of eyes with superior segmental optic hypoplasia (SSOH) using the Heidelberg retina tomograph (HRT).Patients and methodsThirteen eyes of 13 Japanese patients with SSOH were studied with the HRT (software version: 3.0). The group included six males and seven females, with a mean age of 34.7 years. Six optic disc parameters in the six sectors derived from the patients with SSOH were compared with those of 13 eyes of 13 normal controls. In addition, the diagnostic classification performance of the Frederick S Mikelberg (FSM) discriminant function, glaucoma probability score (GPS), and Moorfields regression analysis (MRA) were assessed.ResultsWhen compared with normal subjects, many of the optic disc parameters were significantly altered in SSOH in the superior sectors. The area under the curve (AUC) for the receiver operating characteristic was 0.932 for the rim area, 0.926 for the cup-to-disc area ratio, and 0.882 for the cup shape measure. Among the HRT parameters, the largest AUC (0.988) was found for the cup shape measure in the nasal superior segment. The proportion classified as outside normal limits by the FSM discriminant function was 92.3% (12 eyes). For GPS, six eyes (46.2%) were classified as outside normal limits. For MRA, when borderline cases were considered test-negative or test-positive, 10 eyes (76.9%) or 11 eyes (84.6%) were classified as outside normal limits, respectively. The AUCs were 0.976 for the FSM discriminant function, 0.914 for the MRA overall classification, and 0.710 for the GPS overall classification.ConclusionsIn eyes with SSOH, there is a significant thinning of the rim, especially in the nasal superior sector. Approximately half of the eyes with SSOH were classified as abnormal using indices developed for detecting glaucoma, but the sectorial analysis revealed that the affected sectors were different from those of glaucoma. Optic nerve head measurements using the HRT may be useful in evaluating the optic disc characteristics in eyes with SSOH.

Comparison of Heidelberg Retina Tomograph with disc‐macula distance to disc diameter ratio in diagnosing optic nerve hypoplasia

“…Recently, newer techniques including HRT and optical coherence tomography (OCT) have been utilised to study the optic disc. A few studies have reported that HRT and/or OCT aid in the diagnosis of segmental CONH . Several case reports have revealed that HRT and/or OCT are valuable tests for diagnosing unilateral CONH, including two cases reported from our group .…”

“…Heidelberg Retinal Tomograph (HRT), as an in vivo , non‐invasive, valid imaging method that provides accurate, quantitative, and reproducible measurement of the optic disc, can be useful for diagnosing optic nerve anomalies . HRT has been reported by a few studies to assist in diagnosis of segmental CONH and to assist in diagnosis of unilateral CONH by a few case reports . However, to the best of our knowledge, no study has been conducted to evaluate HRT in unilateral and/or bilateral CONH that is not segmental.…”

Comparing Structure-Function Correlations in Superior Segmental Optic Nerve Hypoplasia and Juvenile Open Angle Glaucoma Using Spectral-Domain Optical Coherence Tomography

“…Imaging indices developed for detecting glaucoma will also identify approximately half of the optic discs with SSOH, but the affected sectors may differ. Whereas a notch in the inferotemporal or superotemporal rim is commonly found in glaucoma, it is more commonly noted in the superonasal segment in SSOH, creating a "single hump" pattern [4][5], as seen in cases 1 and 2. Also, the optic nerve head rim and RNFL thickness in children with glaucoma has been found to be reduced concentrically, with particular losses in the superior and inferior quadrants, which would also differ significantly from SSOH [6].…”