Abstract:This is the first case report of a superior segmental optic hypoplasia (SSOH) combined with normal-tension glaucoma accompanied by a progressive glaucomatous visual field defect. A 40-year-old man, incidentally diagnosed as having bilateral SSOH, had disc hemorrhage associated with expansion of the width of a retinal nerve fiber layer defect and deterioration of a visual field defect in the right eye during the follow-up period. His left eye showed a stable visual field. The diurnal variation in intraocular pr… Show more
“…SSOH is non-progressive, and no treatment is necessary. There have been case reports of SSOH that is also accompanied by NTG with progressive glaucomatous visual field defect [7].…”
“…SSOH is non-progressive, and no treatment is necessary. There have been case reports of SSOH that is also accompanied by NTG with progressive glaucomatous visual field defect [7].…”
“…Myopia is one of the risk factors of glaucoma [ 20 , 21 ], but no previous reports suggested that high myopia may be associated with glaucomatous progression in SSOH eyes. In one previous case report of progressive SSOH, the patient had a high myopia of −8.0 D [ 8 ], so it might not be coincidence that glaucoma with SSOH had a high myopia, and it seems necessary to evaluate whether coexistence of SSOH and high myopia can lead to glaucomatous changes or progression.…”
Section: Discussionmentioning
confidence: 99%
“…However, it is not easy to differentiate the two diseases because SSOH and glaucoma have similar features, such as localized RNFL thinning and neuroretinal rim thinning [ 6 ]. Furthermore, the two diseases sometimes exist simultaneously [ 7 , 8 ].…”
Purpose. To evaluate the different characteristics in superior segmental optic hypoplasia (SSOH) and normal tension glaucoma (NTG) with superior retinal nerve fiber layer (RNFL) defect (NTG-SRD) compared to normal control using cirrus optical coherence tomography (OCT). Methods. SSOH eyes and NTG-SRD eyes were reviewed. The peripapillary RNFL (pRNFL) and ganglion cell inner plexiform layer (GCIPL) of the two groups were compared to age-matched normal controls using cirrus OCT. Results. Included in this study were 31 SSOH eyes, 33 NTG patients, and 49 healthy normal controls. Compared to normal controls, pRNFL thickness in SSOH eyes was thinner except in the inferotemporal to the temporal segment. NTG-SRD eyes had thinner pRNFL except in the nasal to inferonasal segment. Meanwhile, GCIPL thickness in SSOH eyes was thinner in the global and sectoral segment, but not in the superonasal and inferonasal sectors compared to normal controls. NTG-SRD eyes showed thinner GCIPL in all sectors compared to normal controls. In case of comparison between SSOH and NTG-SRD, superonasal sector was thinner in NTG-SRD than in SSOH (P = 0.03). Conclusions. The different distributions of nerve fiber layer were shown in pRNFL and GCIPL between SSOH eyes and NTG-SRD eyes.
“…While SSOH is nonprogressive in nature and is an important entity in the differential diagnosis of childhood glaucoma, there is an isolated case report of SSOH accompanied by NTG in an adult after five years of annual follow up, necessitating long-term monitoring even in low-risk patients [7]. Sequential SD-OCT surveillance and visual field testing are useful tools in detecting progressive glaucomatous changes.…”
Superior segmental optic nerve hypoplasia (SSOH) is a congenital anomaly of the optic nerve that is commonly misdiagnosed as normal tension glaucoma (NTG) and/or juvenile open angle glaucoma (JOAG). We demonstrate the utility of SD-OCT in assessing the structure-function correlation when differentiating between SSOH and JOAG.Keywords Superior segmental optic nerve hypoplasia; Glaucoma; Imaging; Retinal nerve fiber layer optical coherence tomography
Case Report Case 1A 13 year-old male with a history of an increased cup-to-disc ratio of the left eye was referred for evaluation of JOAG. He had no family history of glaucoma. On examination, his visual acuity was 20/25 in both eyes. Applanation tonometry revealed an intraocular pressure (IOP) of 14 mmHg on the right without treatment and 12 mmHg on the left with topical beta-blocker treatment. Records of the IOP prior to presentation and treatment were unavailable.Gonioscopy showed open angles in both eyes. Fundus examination revealed a normal optic disc with a superior peripapillary scleral halo in the right eye and superior disc pallor with thinning of the superior rim in the left eye (Figure 1a). A Humphrey visual field 24-2 (Carl Zeiss Meditec, Dublin, CA) showed an inferior field defect in left eye (Figure 1b), while SD-OCT showed RNFL thinning superiorly ( Figure 1c). The patient was diagnosed with SSOH of the left eye and was instructed to discontinue the topical beta-blocker. In twenty months of follow up, the untreated IOP has remained within physiologic range and serial visual fields and SD-OCT RNFL analyses have remained stable on two subsequent tests.
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