PMC4930228_01
Patient 1.. Notes: (A) OD optic disk image shows vertically oval cup with INF thinning of the NRR. (B) OS optic disk image with media haze and diffuse disk pallor. (C) OD Goldmann perimetry with diffuse constriction, SUP > INF. (D) OS Goldmann perimetry shows remaining island of central and inferotemporal visual field. (E) Stratus OCT RNFL thickness analysis OD shows no RNFL loss. (F) OS RNFL quadrant and analysis show severe losses (<1% of normative data) in the NAS and TEMP quadrants. OS RNFL clock hour analysis corroborates RNFL thickness findings. OD RNFL quadrant and clock hour analyses were both normal.. Abbreviations: INF, inferior; NRR, neuroretinal rim; SUP, superior; OCT, optical coherence tomography; RNFL, retinal nerve fiber layer; NAS, nasal; TEMP, temporal; NA, not applicable; S, superior; N, nasal; T, temporal; I, inferior.
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He also had significant cystoid macular edema (CME) and inferior thinning of the optic disk OD (Figure 1A). He had significant media opacity, due to intraocular fibrosis and tractional retinal detachment, which obscured view of his optic disk OS (Figure 1B). On Goldmann perimetry, the right eye showed diffuse constriction, superior > inferior (Figure 1C). In the left eye, he had extensive visual field losses with only an island of central and inferotemporal visual field remaining (Figure 1D). Stratus optic disk optical coherence tomography (OCT) showed no losses of retinal nerve fiber layer (RNFL) thickness OD, but severe losses were evident OS, especially in the nasal and temporal quadrants (Figure 1E-F).
Patient 2.. Notes: (A) OD disk image showing fibrous membrane over disk with inferior thinning of NRR. There is no NVD. (B) OS optic disk appears hyperemic with dense fibrous membrane. There is no NVD. (C) OD Goldmann perimetry shows superior visual field loss. (D) OS Goldmann perimetry shows mild constriction of the superotemporal visual field.. Abbreviations: NRR, neuroretinal rim; NVD, neovascularization of the disk.
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OD (Figure 2A and B). On Goldmann perimetry, he had significant superior visual field losses OD (Figure 2C) and mild superotemporal visual field constriction OS (Figure 2D).
PMC4930228_02
Patient 3.. Notes: (A) OD optic disk has pink and regular NRR without peripapillary atrophy. (B) OS optic disk was similar with fibrous membrane and vessel sheathing. (C) Initial OD Goldmann perimetry with relatively intact visual field. (D) Initial OS Goldman perimetry with superotemporal constriction. (E) Interval OD Goldmann visual field after 14 years shows marked visual constriction with central scotoma (OS unavailable since the patient's left eye became phthisical). (F) OD stratus OCT shows mild RNFL thinning superiorly, otherwise RNFL thickness is normal. (G) Normal RNFL quadrant and RNFL clock hour analyses OD.. Abbreviations: NRR, neuroretinal rim; OCT, optical coherence tomography; RNFL, retinal nerve fiber layer; TEMP, temporal; SUP, superior; NAS, nasal; INF, inferior; NA, not applicable; S, superior; N, nasal; T, temporal; I, inferior.
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Her posterior segment examination showed 1+ vitreous cells OU, peripheral retinal pigment epithelium changes OU, mild ERM OU, disk pallor OU (Figure 3A and B), CME OU, attenuated vessels OD (Figure 3A), and early neovascularization of the peripheral retina OS. Goldmann perimetry showed an intact visual field OD and mild superotemporal constriction OS (Figure 3C and D). Her interval OD Goldmann perimetry after 14 years showed severe constriction with a central scotoma (Figure 3E). Stratus optic disk OCT RNFL thickness showed mild thinning superiorly but was otherwise normal (Figure 3E, F, and G).
PMC4930228_03
Patient 4.. Notes: (A and B). Bilateral disk pallor with loss of disk capillaries and attenuation of major vessels, suggestive of optic atrophy. (C) Goldmann perimetry with severe visual field loss and preservation of central vision OD. (D) Goldmann perimetry with severe visual field loss and split fixation OS. (E) Stratus OCT RNFL thickness analysis OU showed marked SUP, NAS, and INF thinning (<1% of normal). (F) RNFL quadrant and clock hours were consistent with the RNFL thickness findings.. Abbreviations: OCT, optical coherence tomography; RNFL, retinal nerve fiber layer; SUP, superior; NAS, nasal; INF, inferior; TEMP, temporal; NA, not applicable; S, superior; N, nasal; T, temporal; I, inferior.
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OU and bilateral pseudophakia. On posterior segment examination, she had dense vitreous hemorrhage OS, scattered retinal pigment epithelium clumps OU, markedly attenuated vessels OU, and optic disk pallor OU (Figure 4A and B). Goldmann perimetry showed severe bilateral visual field loss (Figure 4C and D). Stratus OCT RNFL thickness showed marked superior, nasal, and inferior thinning OU (Figure 4E and F).
PMC4930228_04
Patient 5.. Notes: (A) OS optic disk has pink and regular NRR (OD examination was similar to OS examination). (B and C) Humphrey Visual Field 24-2 SITA-Standard protocol with superior and inferior arcuate defects OS > OD, and superior and inferior nasal step defects OU. (D and E) Interval Humphrey Visual Field 24-2 SITA-Standard protocol (obtained 6 years later) demonstrates glaucomatous progression with denser superior arcuate defects OU.. Abbreviation: NRR, neuroretinal rim.
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Her left optic nerve had a pink neuroretinal rim OS (Figure 5A). Her Humphrey visual field (24-2) indicated inferior and superior arcuate defects OU, OS worse than OD (Figure 5B and C). Interval Humphrey visual field indicated progression of glaucomatous changes OU (Figure 5D and E).