Hypoplasia of the Optic Nerve

Mosier, Marjorie A.; Lieberman, Marc F.; Green, W R; Knox, David L.

doi:10.1001/archopht.1978.03910060185017

Cited by 113 publications

(49 citation statements)

References 38 publications

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“…86,124 Changes may often be very subtle in the less affected optic disc, but may be useful additional clues, but the sign should be used in the context of changes in the disc as a whole; by itself it is not pathognomonic of ONH and, in mild degrees, occurs not uncommonly as an incidental finding in normal eyes ( Figure 13).…”

Section: Eyesupporting

confidence: 41%

“…161 It may be mixed with direct damage to the developing visual system occurring, in the same patient, with transsynaptic degeneration. 162 Pathology: A few pathological studies have been carried out: 86,122,124,154,[163][164][165] most showed an absence of or a reduction in the numbers of ganglion cells and their axons, small optic discs and nerves with abnormal glial tissue. Other parts of the neuroretina are usually normal and, apart from incidental anomalies, the eyes were otherwise normal.…”

mentioning

confidence: 41%

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Optic nerve axons: life and death before birth

Taylor

2005

Eye

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Section: Eyesupporting

confidence: 41%

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confidence: 41%

Optic nerve axons: life and death before birth

Taylor

2005

Eye

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“…12,13,18 To date, the potential of SD OCT to assess optic nerve and macula morphology in patients with ONH in vivo has been largely unexplored. In 2013, Moon and Park 16 confirmed previous histopathology data in 1 patient with ONH compared with the healthy eye using SD OCT.…”

Section: Discussionmentioning

confidence: 44%

“…One hypothesis suggests that failure of GCL differentiation leads to RNFL reduction, one of the features of ONH. 12,13 During normal pregnancy, retinal ganglion cells first develop axons that extend toward the optic stalk at 8 weeks gestational age. These axons later form the RNFL.…”

Section: Optic Nerve Head Changesmentioning

confidence: 50%

High resolution imaging of the optic nerve and retina in optic nerve hypoplasia

Gottlob

Pilat

Sibley

et al. 2015

Journal of the Neurological Sciences

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Purpose: To investigate the optic nerve and macular morphology in patients with optic nerve hypoplasia (ONH) using spectral-domain optical coherence tomography (SD OCT).Design: Prospective, cross-sectional, observational study. Subjects: A total of 16 participants with ONH (10 female and 6 male; mean age, 17.2 years; 6 bilateral involvement) and 32 gender-, age-, ethnicity-, and refraction-matched healthy controls.Methods: High-resolution SD OCT (Copernicus [Optopol Technology S.A., Zawiercie, Poland], 3 mm resolution) and handheld SD OCT (Bioptigen Inc [Research Triangle Park, NC], 2.6 mm resolution) devices were used to acquire horizontal scans through the center of the optic disc and macula.Main Outcome Measures: Horizontal optic disc/cup and rim diameters, cup depth, peripapillary retinal nerve fiber layer (RNFL), and thickness of individual retinal layers in participants with ONH and in controls.Results: Patients with ONH had significantly smaller discs (P < 0.03 and P < 0.001 compared with unaffected eye and healthy controls, respectively), horizontal cup diameter (P < 0.02 for both), and cup depth (P < 0.02 and P < 0.01, respectively). In the macula, significantly thinner RNFL (nasally), ganglion cell layer (GCL) (nasally and temporally), inner plexiform layer (IPL) (nasally), outer nuclear layer (ONL) (nasally), and inner segment (centrally and temporally) were found in patients with ONH compared with the control group (P < 0.05 for all comparisons). Continuation of significantly thicker GCL, IPL, and outer plexiform layer in the central retinal area (i.e., foveal hypoplasia) was found in more than 80% of patients with ONH. Clinically unaffected fellow eyes of patients with ONH showed mild features of underdevelopment. Visual acuity and presence of septo-optic dysplasia were associated with changes in GCL and IPL. Sensitivity and specificity for the detection of ONH based on disc and retinal optical coherence tomography (OCT) parameters were >80%.Conclusions: Our study provides evidence of retinal changes in ONH. In addition to thinning of retina layers mainly involving the RNFL and GCL, signs reminiscent of foveal hypoplasia were observed in patients with ONH. Optic nerve and foveal parameters measured using OCT showed high sensitivity and specificity for detecting ONH, demonstrating their useful for clinical diagnosis. Ophthalmology 2015;-:1e10 ª

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“…The optic disc had the characteristic features of SSOH, i.e., relative superior entrance of the central etinal artery, superior double ring sign (a hallmark of optic hypoplasia) [9][10][11] and thinning of the superior RNFL. Definition of NTG with only inferior visual field defects in this study Patients were diagnosed with NTG with only inferior visual field defects when they met the four criteria listed below.…”

Section: Subjectsmentioning

confidence: 47%

Differentiation by imaging of superior segmental optic hypoplasia and normal-tension glaucoma with inferior visual field defects only

et al. 2012

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Purpose: To differentiate superior segmental optic hypoplasia (SSOH) from normal-tension glaucoma (NTG) with inferior visual field defects only. Methods Eighteen eyes with SSOH (SSOH group) and 19 eyes with NTG (NTG group) were examined by optical coherence tomography (OCT), Heidelberg retina tomography (Heidelberg Retinal Tomograph II, HRT II) and standard automated perimetry.Results: Retinal nerve fiber layer thickness (RNFLT) based on OCT measurements was significantly reduced (thinner) in the superior to superonasal sectors and significantly greater (thicker) in the inferotemporal sector in the SSOH group than in the NTG group. The cup was significantly smaller and the rim significantly larger in the superotemporal and temporal sectors in the SSOH group than in the NTG group based on HRT II measurements. The greatest area under the receiver operating characteristic curve for discrimination of SSOH from NTG by OCT and HRT II was for the RNFLT ratio of 1 +2 o'clock/10 +11 o'clock (0.985) and for the ratio of the superonasal to superotemporal sector of rim to disc area ratio and cup to disc area ratio (0.955), respectively. The frequent location of the inferior visual field defects corresponded to the difference in structural changes in both groups.Conclusions: Comparison of the superonasal to superotemporal sectors by OCT and HRT II were useful in differentiating SSOH from NTG with only inferior visual field defects.

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Hypoplasia of the Optic Nerve

Cited by 113 publications

References 38 publications

Optic nerve axons: life and death before birth

Optic nerve axons: life and death before birth

High resolution imaging of the optic nerve and retina in optic nerve hypoplasia

Differentiation by imaging of superior segmental optic hypoplasia and normal-tension glaucoma with inferior visual field defects only

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