High diagnostic accuracy of T2FLAIR at 3 T in the detection of optic nerve head edema in acute optic neuritis.

OBJECTIVES: Optic nerve head edema (ONHE) detected by fundoscopy is observed in one-third of patients presenting optic neuritis (ON). While ONHE is an important semiological feature, the correlation between ONHE and optic nerve head MRI abnormalities (ONHMA), sometimes called "optic nerve head swelling," remains unknown. Our study aimed to assess the diagnostic accuracy of T2 fluid-attenuated inversion recovery (FLAIR) MRI sequence in detecting ONHE in patients with acute ON. METHODS: In the present single-center study, data were extracted from two prospective cohort studies that consecutively included adults with a first episode of acute ON treated between 2015 and 2020. Two experienced readers blinded to study data independently analyzed imaging. A senior neuroradiologist resolved any discrepancies. The primary judgment criterion of ONHMA was assessed as optic nerve head high signal intensity on gadolinium-enhanced T2FLAIR MRI sequence. Its diagnostic accuracy was evaluated with both the gold standard of ONHE on fundus photography (FP) and peripapillary retinal nerve fiber layer thickening on optic coherence tomography (OCT). RESULTS: A total of 102 patients were included, providing 110 affected and 94 unaffected optic nerves. Agreement was high between the different modalities: 92% between MRI and FP (k = 0.77, 95% CI: 0.67-0.88) and 93% between MRI and OCT (k = 0.77, 95% CI: 0.67-0.87). MRI sensitivity was 0.84 (95% CI: 0.70-0.93) and specificity was 0.94 (95% CI: 0.89-0.97) when compared with the FP. CONCLUSION: Optic nerve head high T2FLAIR signal intensity corresponds indeed to the optic nerve head edema diagnosed by the ophthalmologists. MRI is a sensitive tool for detecting ONHE in patients presenting acute ON. CLINICAL RELEVANCE STATEMENT: In patients with optic neuritis the high T2FLAIR (fluid-attenuated inversion recovery) signal intensity of the optic nerve head corresponds indeed to optic nerve head edema, which is a useful feature in optic neuritis etiological evaluation and treatment. KEY POINTS: Optic nerve head edema is a prominent clinical feature of acute optic neuritis and is usually diagnosed during dilated or non-dilated eye fundus examination. Agreement was high between magnetic resonance imaging, fundus photography, and optical coherence tomography. Optic nerve head high T2 fluid attenuation inversion recovery signal intensity is a promising detection tool for optic nerve head edema in patients presenting acute optic neuritis.

Diagnosis of idiopathic intracranial hypertension: A proposal for evidence-based diagnostic criteria.

BACKGROUND: Based on expert opinion, abducens nerve palsy and a neuroimaging criterion (≥3 neuroimaging signs suggestive of elevated intracranial pressure) were added to the diagnostic criteria for idiopathic intracranial hypertension. Our objective was to validate this. METHODS: This prospective study included patients with new-onset idiopathic intracranial hypertension for a standardized work-up: interview, neuro-ophthalmological exam, lumbar puncture, neuroimaging. Neuroimaging was evaluated by a blinded neuroradiologist. RESULTS: We included 157 patients classified as idiopathic intracranial hypertension (56.7%), probable idiopathic intracranial hypertension (1.9%), idiopathic intracranial hypertension without papilledema (idiopathic intracranial hypertension-without papill edema; 0%), suggested idiopathic intracranial hypertension-without papill edema (4.5%), or non-idiopathic intracranial hypertension (36.9%). Moderate suprasellar herniation was more common in idiopathic intracranial hypertension than non-idiopathic intracranial hypertension (71.4% versus 47.4%, p < 0.01), as was perioptic nerve sheath distension (69.8% versus 29.3%, p < 0.001), flattening of the globe (67.1% versus 11.1%, p < 0.001) and transverse sinus stenosis (60.2% versus 18.9%, p < 0.001). Abducens nerve palsy was of no diagnostic significance. Sensitivity of ≥3 neuroimaging signs was 59.5% and specificity was 93.5%. CONCLUSION: Moderate suprasellar herniation, distension of the perioptic nerve sheath, flattening of the globe and transverse sinus stenosis were associated with idiopathic intracranial hypertension. We propose that idiopathic intracranial hypertension can be defined by two out of three objective findings (papilledema, opening pressure ≥25 cm cerebrospinal fluid and ≥3 neuroimaging signs).

Transorbital sonography: A non-invasive bedside screening tool for detection of pseudotumor cerebri syndrome.

BACKGROUND: Our objective was to assess optic nerve sheath diameter (a marker of elevated intracranial pressure) and optic disc elevation (a marker of papilledema) in pseudotumor cerebri syndrome using transorbital sonography. METHODS: The study was a prospective case-control study. We included patients with new-onset pseudotumor cerebri syndrome and matched healthy controls. All had fundoscopy, lumbar puncture with opening pressure and transorbital sonography. Sonography was assessed by a blinded observer. RESULTS: We evaluated 45 patients and included 23 cases. We recruited 35 controls. Optic nerve sheath diameter was larger in pseudotumor cerebri syndrome compared to controls (6.3 ± 0.9 mm versus 5.0 ± 0.5 mm, p < 0.001) and so was optic disc elevation (0.9 ± 0.4 mm versus 0.4 ± 0.1 mm, p < 0.001). The optimal cut-off point for optic nerve sheath diameter was 6 mm with a sensitivity of 74% for prediction of pseudotumor cerebri syndrome and 68% for prediction of elevated opening pressure. Specificity was 94%. The optimal cut-off point for optic disc elevation was 0.6 mm. Sensitivity was 100% and specificity 83% for prediction of pseudotumor cerebri syndrome. CONCLUSION: Optic disc elevation and optic nerve sheath diameter are increased in new-onset pseudotumor cerebri syndrome. Optic disc elevation achieved high specificity and excellent sensitivity for diagnosis of pseudotumor cerebri syndrome. Transorbital sonography (TOS) is a potential, non-invasive screening tool for pseudotumor cerebri syndrome in headache clinics.

Juxtapapillary Choroidal Neovascularization in a Young Woman with Tubulointerstitial Nephritis and Uveitis (TINU) Syndrome with Onset in Pediatric Age.

We describe the unusual case of a young woman with tubulointerstitial nephritis and uveitis (TINU) with bilateral diffuse uveitis and optic nerve inflammatory involvement since she was a child in the 1990s. Imaging diagnostic tools such as fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), and OCT angiography revealed inactive juxtapapillary choroidal neovascularization (CNV) after 25 years of follow-up. After treatment, uveitis went into remission with BCVA 20/20 in both eyes and CNV lesions became inactive. Although anterior uveitis is more frequently reported in TINU, posterior uveitis with inflammatory involvement of the optic nerve should be accurately investigated to rule out juxtapapillary CNV, both at the time of active uveitis and during follow-up, since TINU may be complicated by CNV even at the later stages of the inflammatory process.

Microvascular assessment of macula, choroid, and optic disk in children with unilateral amblyopia using OCT angiography.

PURPOSE: To investigate the microvascular changes of macula, choroid, and optic disk in children with unilateral amblyopia. METHODS: This prospective cross-sectional study involved 39 unilateral amblyopic children and 39 age- and sex-matched heathy participants who served as control. Vessel densities of the superficial and deep capillary plexuses (SCP and DCP), foveal avascular zone (FAZ) area, macular thickness, optic disk vessel density, retinal nerve fiber layer (RNFL) thickness, choriocapillaris vessel density, and subfoveal choroidal thickness were evaluated by OCT angiography (OCTA). Meanwhile, the correlations of microvascular perfusion and structural changes of macula, choroid, and optic disk were analyzed. RESULTS: The vessel density of SCP and DCP in the whole macula in the amblyopic group was significantly lower than that in the control group after adjusting for age, axial length, and spherical equivalents (all P < 0.05). FAZ area, macular thickness, RNFL thickness, and the optic disk vessel density were not statistically different between the amblyopic group and the control group (all P > 0.05). Subfoveal choroidal thickness of amblyopic eyes was significantly higher than that of control eyes(P = 0.032). Choriocapillaris flow void (FV) in the amblyopic group was greater than that in the control group (P = 0.013). Significant differences were observed between the fellow eyes and the control eyes in choriocapillaris FV and subfoveal choroidal thickness (P = 0.011 and P = 0.042, respectively). Foveal SCP and DCP vessel density in all studied eyes were positively correlated with the whole macular thickness, respectively (r = 0.556 and r = 0.627, respectively, both P < 0.001). Whole SCP and DCP vessel density in the amblyopic eyes were negatively correlated with choriocapillaris FV (r = -0.723, P < 0.001; r = -0.512, P = 0.001, respectively). CONCLUSION: Children with amblyopic eyes have attenuated macular and choriocapillaris perfusion. There is a need for future studies that will investigate the pathophysiology of amblyopia in children by OCTA.

Optical coherence tomography angiography in healthy children: normative data and age-related changes in microvascular structure of the optic disk and macula.

PURPOSE: To determine normative data and reference ranges according to age groups by measuring the foveal avascular zone (FAZ), superficial capillary plexus vascular density (SCP-VD), deep capillary plexus vascular density (DVP-VD), radial peripapillary capillary plexus vessel density (RPC-VD), and peripapillary retinal nerve fiber layer (ppRNFL) in healthy children and to determine the age and sex-related changes of these values. METHODS: This prospective study included data from 370 eyes of 370 healthy children (202 girls, 168 boys) aged 7-18 years. Participants were divided into four groups according to their age. Optical coherence tomography angiography (OCTA) measurements were taken using AngioVue (Avanti; Optivue). RESULTS: No statistically significant difference was observed in terms of FAZ, SCP-VD, DCP-VD, RPC-VD, and ppRNFL thickness values according to the age groups (except the RPC-VD superior) (p > 0.05 for all). VDs in all deep parafoveal regions in groups 1 and 2 were higher in girls. While FAZ values were higher in girls in all age groups (statistically significant in groups 1, 3, and 4), ad SPD and DPD values were higher in boys in all age groups (statistically significant in group 1 and 2 for SPD, and group 1 and 3 for DPD). CONCLUSIONS: We report normal reference ranges for macula and disk vessel density and ppRNFL parameters in healthy children aged 7-18 years using OCTA. These normative values could be useful in diagnosing retina and optic disk disease early in childhood.

Microvascular alterations in children with optic disk drusen evaluated by optical coherence tomography angiography.

PURPOSE: To asses changes in vessel density (VD) in children with optic disk drusen (ODD) using swept source optical coherence tomography angiography (OCTA). METHODS: Cross-sectional study of 27 eyes with ODD compared with age-matched controls. Peripapillary and macular VD were measured in the superficial retinal capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC). The correlation between VD changes with alterations in retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), and visual field (VF) was analyzed. RESULTS: Mean participant age was 12.5 ± 3.3 years (range, 7-18 years); 63% was females. In the patients vs. controls, median central peripapillary VD was 52.9% vs. 50.6% (p = 0.63) for SCP; 48.1% vs. 53.8% (p = 0.017) for DCP; and 17.0% vs. 28.2% (p = 0.0037) for CC, respectively. VD in the superior and nasal CC layers was significantly lower in the patients (36.3% vs. 56.2%; p < 0.001) and (60.4% vs. 70.3%, p < 0.001), respectively. No significant differences were observed for VD in the macular region. The RNFL was thinner in eyes with superficial drusen versus controls (87 vs. 111 µm; p < 0.001). No significant differences between were observed in GCL thickness (p = 0.13). Nasal SCP and nasal RNFL VD were moderately correlated (r = 0.54, p < 0.01), while mean VF deviation was strongly correlated with median SCP VD in patients with superficial drusen (r = 0.9, p = 0.03). CONCLUSION: Impaired VD was observed in the peripapillary nasal CC in patients with ODD; this impairment was associated with a decreased RNFL thickness. Nasal SCP VD and RNFL thickness were moderately correlated in patients with ODD.

Anti-tumor necrosis factor-alpha therapy for refractory uveitic optic disk neovascularization in active non-infectious posterior and panuveitis.

PURPOSE: Neovascularization of the optic disk (NVD) is mainly a complication of ischemic retinal disorders and of uveitis with vascular occlusion. Rarely, NVD may develop in patients with uveitis in the absence of retinal ischemia. This report aims to present our long-term experience of treating refractory uveitic NVD (uNVD) with adalimumab in three patients with active non-infectious posterior or panuveitis. METHODS: Observational case series was collected from institutional tertiary referral center. Patients with chronic refractory uNVD who completed 24 months of follow-up were included. RESULTS: uNVD was diagnosed on first presentation in all patients (3 eyes). Mean age at presentation was 29 years (median 20, range 18-49). Mean duration of complaints before presentation was 18.7 weeks (median 24, range 4-28). Uveitis was idiopathic in two patients and secondary to Behçet disease in one. All eyes had concomitant cystoid macular edema. Additional posterior segment signs included optic disk hemorrhage, preretinal hemorrhage and vitreous hemorrhage. All eyes showed retinal vascular leakage and macular leakage with no evidence of capillary non-perfusion. All patients were treated with systemic steroids and steroid-sparing agent. Because of NVD refractoriness, anti-TNF-α therapy was introduced at a mean of 24.7 weeks after first presentation (median 20, range 14-40). Complete regression of NVD was observed at a mean of 34.7 weeks (median 32, range 8-64) following adalimumab institution. Mean follow-up time after starting anti-TNF-α agents was 31.3 months. CONCLUSIONS: Our results suggest that targeting TNF-α achieves long-term control of uveitic NVD refractory to conventional treatments.

[Evaluation the risk of optic nerve invasion associated with optic nerve obscuration in advanced retinoblastoma].

Objective: To evaluate the potential association between optic nerve invasion and optic nerve obscuration during treatment of advanced retinoblastoma. Methods: Retrospective case series study. Medical records of 77 patients (77 eyes) with advanced retinoblastoma (Group D/E) who were treated with primary or secondary enucleation in the Ophthalmology Department of Peking University People's Hospital from January 1st 2012 to December 31th 2015 were retrospectively reviewed. RetCam photographs under general anesthesia at diagnosis and each subsequent follow-up were evaluated for complete obscuration of the optic nerve. The primary endpoints included prelaminar invasion, postlaminar invasion and optic nerve transection invasion. Group difference was calculated with chi-square. Results: There were 46 boys and 31 girls in the study. The mean age at the first diagnosis was (27.1±22.1) months. The optic nerve was obscured in 62 eyes (80.5%) at the first diagnosis and 61 eyes (79.2%) at the last ocular examination prior to enucleation. Twenty-nine eyes (37.7%) underwent primary enucleation. Forty-eight eyes (62.3%) were treated with eye-preserving therapy, followed by enucleation. Fourteen eyes (18.2%) were in Group D and 63 eyes (81.8%) were in Group E. Histopathologic analysis of enucleated eyes without optic nerve obscuration (16 eyes) showed prelaminar invasion in 7 eyes, postlaminar invasion in 2 eyes and optic nerve transection invasion in 0 eyes. Histopathologic analysis of enucleated eyes with optic nerve obscuration (61 eyes) showed prelaminar invasion in 26 eyes, postlaminar invasion in 9 eyes and optic nerve transection invasion in 4 eyes. The difference between two groups did not achieve statistical significance (P=0.935, 1.000, 0.296). Histopathologic analysis of enucleated eyes with persistent complete obscuration of the optic nerve showed a high risk factor in 10 eyes (10/40), while in 1 eye (1/8) the optic nerve was visible at the initial presentation and obscured before secondary enucleation (P=0.529). Conclusion: Optic nerve obscuration at the last examination prior to enucleation may not be associated with postlaminar optic nerve invasion in advanced retinoblastoma. (Chin J Ophthalmol, 2020, 56: 681-687).

[Lamina cribrosa defect and progress of glaucoma].

Lamina cribrosa (LC) is considered as the original site of glaucomatous damage of axons of retinal ganglion cells, and therefore understanding the morphological changes in the LC will help to uncover the pathogenesis of glaucoma. Previous studies have indicated that the progress of glaucomatous optic neuropathy may be associated with the LC defects. Based on imaging by swept source optical coherence tomography B-Scan of the optic discs of patients with glaucoma, for the first time the spontaneous local LC defects have been found to balance the gradient between intraocular and cerebrospinal fluid pressures, which in turn can slow down the progress of glaucomatous optic neuropathy. This article provides the direct evidence supporting the role of intraocular and cerebrospinal fluid pressure gradient in the pathogenesis of glaucoma. This finding will increase our understanding of the mechanisms underlying glaucoma and help to develop novel strategies for its treatment and prognosis analysis. (Chin J Ophthalmol, 2020, 56: 17-20).

[Peripapillary and macular vessel density in eyes with different phases of thyroid-associated ophthalmopathy].

Objective: To analyze the characteristics of vessel density in the optic disc and macular area of patients with different phases of thyroid-associated ophthalmopathy (TAO) and their correlation with visual function. Methods: This case-control study was conducted at the Department of Ophthalmology of Peking Union Medical College Hospital between June 2019 and September 2019. TAO patients and healthy volunteers were included in the study. Patients with a clinical activity score greater than or equal to 3 points were categorized as active TAO. Dysthyroid optic neuropathy (DON) patients with a course less than 6 months were categorized as acute phase of DON, and those more than 6 months were in the chronic group. Healthy volunteers were in the control group. Each group included 12 subjects, with right eyes for analysis. There were 6 males and 6 females in each group. All participants underwent comprehensive ophthalmic examination including best corrected visual acuity and visual field examination for the mean defect (MD). Best corrected visual acuity was subsequently converted to logarithm of minimum angle of resolution (logMAR). Optical coherence tomography was used to measure the thickness of the retinal nerve fiber layer (RNFL) and retinal ganglion cell complex (GCC). Optical coherence tomography angiography was used to the peripapillary and macular vessel density. The differences in the vessel densities in the optic disc and macular area between groups and their correlation with different factors were analyzed. Analysis of variance, non-parametric Mann-Whitney U test and Spearman coefficient were conducted for statistical analysis. Results: There was no significant difference in age among the four groups (P>0.05). The logMAR of the acute DON group was 0.1 (0.0, 0.2), worse than the control group, which was 0.0 (0.0, 0.0) (U=114.000, P<0.05). The overall vessel densities of the optic disc in acute DON and chronic DON were significantly lower than the control group (54.70%±2.31% and 54.31%±3.65% vs. 57.54%±2.17%; t=3.104, 2.636; both P<0.05). The overall superficial vessel densities of the macular area in active TAO, acute DON and chronic DON were significantly lower than the control group (46.07%±3.06% and 42.26%±5.05% and 45.63%±3.87% vs. 49.34%±3.08%), and the differences were statistically significant (t=2.614, 4.147, 2.603; all P<0.05). There was no statistically significant difference in the size of the foveal avascular zone or the density of deep blood vessels in the macular area among the four groups (all P>0.05). In the active TAO period, there was no correlation between the MD value, RNFL thickness, GCC thickness and the vessel densities of the optic disc and macular area (all P>0.05). The vascular density of the whole layer of the optic disc in acute DON was negatively correlated with the MD value (r=-0.591, P<0.05) and positively correlated with the RNFL thickness and GCC thickness (r=0.595, 0.693; both P<0.05). In chronic DON, the overall capillary density of the optic disc was negatively correlated with the MD value (r=-0.673, P<0.05); the superficial overall blood vessel density of the macular area was positively correlated with the thickness of RNFL and GCC (r=0.732, 0.712;both P<0.01). Conclusions: In active TAO, only the blood supply to the superficial layer of the macular area is decreased. In the acute and chronic phases of DON, the blood supply to the superficial layer of the macular area and the optic disc is both reduced; the smaller the blood vessel density, the more severe the visual field defect, and the thinner the RNFL and GCC. (Chin J Ophthalmol, 2020, 56:824-831).

Involvement of circulatory disturbance in optic disk melanocytoma with visual dysfunction.

PURPOSE: This study aims to examine the relationship between ocular circulation changes and visual field defects in optic disk melanocytoma (ODM). METHODS: Five eyes of five patients were enrolled in this study. All patients were diagnosed with ODM in the Department of Ophthalmology, Hokkaido University Hospital from March 2009 to November 2017. Ophthalmological data including optical coherence tomography angiography (OCTA) and laser speckle flowgraphy (LSFG) findings were retrospectively analyzed. RESULTS: The five ODM cases consisted of two females and three males. Ages of the patients ranged from 47 to 82 years (mean 54 years). Follow-up periods were from 4 to 105 months. Fluorescein angiography showed hypo-fluorescence throughout the examination in all four eyes examined with this modality. OCTA detected dense blood vessel networks in the tumor in two out of the five eyes. Nasal visual field defects were found in two other eyes, which were correlated with locations of tumors free of vessel networks. One ODM eye without marked visual field defects and pigmentations showed lower mean blur rates determined by LSFG in optic disk vessels and tissue circulations than those in the contralateral eye. During follow-up, there was no tumor enlargement in any case. CONCLUSIONS: This study showed the relationship between the deficit of blood vessel networks and visual field defects in ODM patients. LSFG demonstrated reduced blood flow in the tumor, suggesting that circulatory disorder caused by the optic disk tumor might be correlated with visual field defect.

[Clinical analysis of juxtapapillary retinal capillary hemangioma].

Objective: To discuss the clinical features and treatment of juxtapapillary retinal capillary hemangioma (JRCH). Methods: Retrospective study of the clinical data of 6 patients (7 eyes) who were diagnosed with JRCH, among which 2 eyes were treated by laser therapy (thermotherapy TTT or photodynamic therapy PDT), 2 eyes were treated by intravitreal anti-VEGF injection, 2 eyes with vitreous hemorrhage were treated with vitrectomy (PPV)+ anti-VEGF, and 1 eye was untreated. Results: In the 6 cases, the gender ratio of male to female is 2∶1 with average age of 46 years. Four eyes were associated with macular edema(57.1%), vitreous hemorrhage(n=2, 28.6%), and epiretinal membrane(n=1, 14.2%) in the initial examination. Three patients were associated with von Hippel-Lindau(VHL). During the follow-up period, the visual acuity of the 2 patients treated by TTT decreased. Among the 2 eyes treated by anti-VEGF, the visual acuity of 1 eye associated with macular edema increased, and the visual acuity of 1 eye with macular epiretinal membrane did not change significantly, the visual acuity of both 2 eyes treated by PPV+anti-VEGF improved, the vision of the 1 eye untreated appeared to be stable. Conclusions: Juxtapapillary retinal capillary hemangioma is the orange or red vascular hamartomas that occur on or adjacent to the optic nerve head. It is often associated with macular edema, vitreous hemorrhage, and local subretinal effusion. Symptomatic treatment of complications can effectively improve the visual acuity of patients, while long-term follow-up observation should be organized for patients without complications, laser treatment appears to be ineffective. (Chin J Ophthalmol, 2019, 55:609-615).

[Optic nerve morphology and vessel density in eyes with different phases of non-arteritic anterior ischemic optic neuropathy].

Objective: To compare the blood flow around the optic disc and related factors in patients with acute and chronic non-arteritic anterior ischemic optic neuropathy (NAION) and healthy volunteers with small disc cups under the same anatomical structure. Methods: This was a prospective case-control study. NAION patients with unilateral onset and healthy volunteers of the same phase were included in the study conducted at the Department of Ophthalmology of Peking Union Medical College Hospital between February 2017 and September 2018. Patients with a course of ≤ 3 months were categorized in the acute phase of NAION, and those with a course of >3 months were in the chronic phase of NAION. Healthy volunteers were in the control group. All subjects underwent the examination of best corrected visual acuity converted to logarithm of the minimum angle of resolution (LogMAR), measurement of non-contact intraocular pressure, slit lamp examination, small pupil fundus examination, and axial measurement. Optical coherence tomography was used to measure the thickness of retinal nerve fiber layers (RNFL) and retinal ganglion cell complex (GCC). Optical coherence tomography angiography was used to measure the vessel density around the optic disc. NAION patients underwent the visual field examination. Analysis of variance, non-parametric Mann-Whitney U test and Spearman coefficient was used for statistical analysis. Results: This study included 16 patients with acute phase of NAION, aged (57±9) years, 6 males and 10 females. There were 17 patients with chronic disease, aged (56±10) years, 7 males and 10 females. There were 15 healthy controls, aged (57±10) years old, 6 males and 9 females. There were no significant differences in age and gender between the groups (both P>0.05). The RNFL and the GCC in the NAION chronic phase group were significantly thinner than those in the acute phase group [(78±38) µm vs. (191±99) µm, (75±19) µm vs. (98±28) µm; t=4.389, 2.758; both P<0.05]. The cup/disc area ratio, cup/disc vertical diameter ratio and cup/disc horizontal diameter ratio in the chronic phase group were larger than those in the acute phase group [0.18 (0.11, 0.31) vs. 0.05 (0.01, 0.18), 0.45 (0.39, 0.56) vs. 0.22 (0.11, 0.41), 0.39 (0.28, 0.54) vs. 0.20 (0.07, 0.42)], and the difference was statistically significant (U=212.000, 208.000, 205.000; all P<0.05). Compared with the optic disc vessel density in the control group (53%±6%), there was a significant decrease in the acute phase group and the chronic phase group (45%±7%, 41%±8%; t=3.705, 4.940; both P<0.01). The blood vessel density in the nasal inferior of the chronic phase group was significantly lower than that in the acute phase group (36%±8% vs. 42%±7%, P=0.039), other sections didn't have significant difference (all P>0.05). There were tortuous capillaries in 8/16 of the acute phase cases, with a low blood flow density and visual field defect in relative positions. Correlation analysis showed that the whole density and peripapillary density in the NAION patients were negatively correlated with LogMAR, mean visual field defect, cup/disc area ratio, focal loss of volume of GCC and general loss of volume of GCC (r=-0.510, -0.733, -0.372, -0.532, -0.648; all P<0.01), but positively correlated with GCC and RNFL thickness (r=0.604, 0.508; both P<0.01). Conclusions: The optic disc vessel density in the acute phase and chronic phase of NAION is significantly reduced. The vessel density in the nasal area of the chronic phase is significantly reduced compared with the acute phase. The vessel density is correlated with visual acuity, visual field defect, disc indexes, thickness of RNFL and GCC. (Chin J Ophthalmol, 2019, 55: 677-686).

Association between the Frequency of Optic Disk Hemorrhage and Progression of NTG Related with the Initial Location of RNFL Defect.

PURPOSE: This study aimed to investigate the association of the frequency of optic disk hemorrhage (DH) and progression of normal tension glaucoma (NTG) between each group based on the location of the initial retinal nerve fiber layer (RNFL) defect. METHODS: In this retrospective, observational cohort study, 142 NTG patients who underwent more than 5 reliable visual field tests with initial superior hemifield (group 2, n = 51), inferior hemifield (group 1, n = 44), or both hemifield (group 3, n = 47) defects were included. The number of DHs was inspected in serial optic disk photographs by 2 different ophthalmologists. Progression rates, which are the slope of mean thresholds from the 52 points, were calculated using a linear mixed effect model. RESULTS: The mean follow-up period was 8.19 ± 3.30 years. DHs related with the initial RNFL defect occurred significantly more frequently in group 2 (35 in inferior hemifield) than in group 1 (6 in superior hemifield) (p = 0.009) or group 3 (6 in inferior hemifield) (p = 0.006). The progression rate in group 2 was significantly faster than in group 1 (p = 0.019) or the superior hemifield of group 3 (p = 0.001). The progression rate of subjects showing recurrent DH was significantly faster than those showing single DH from all groups (-0.5460 vs. -0.2867 dB/year, p = 0.0053). CONCLUSIONS: More careful examination and caution are required when NTG patients show recurrent DH in the inferior hemifield related to the initial RNFL defect.

[Clinical observation on the evolution process of macular ganglion cell complex and peripapillary retinal nerve fiber layer of neuritis patients].

Objective: To discuss the dynamic changes and correlation of macular ganglion cell (mGCC) and peripapillary retinal nerve fiber layer (pRNFL) of neuritis patients through optical coherence tomography (OCT). Methods: A retrospective case series study. Eleven eyes from 11 patients who have been diagnosed with neuritis and received regular follow-up during June 2013 through June 2015 were included. The dynamic characteristic changes of mGCC and pRNFL over the course of disease, as well as their correlations between best visual acuity and visual field have been analyzed based on OCT measurements. Results: According to the observation on the 11 eyes of the 11 patients, patients showed symptoms of decreased vision, abnormal visual field, swollen mGCC and pRNFL with normal or increased thickness during the subnormal period. During the advance-separation period (around 3 weeks), the thickness of mGCC decreased resulting from atrophy while pRNFL was still swollen. Druing the late period (usually 6-8 weeks after onset of the disease), both mGCC and pRNFL were getting thinner due to atrophy. Vision acuity and visual field of the patients improved after treatment, however, they were still not comparable with the normal level before the disease. Conclusions: The thickness changes of mGCC and pRNFL differs in neuritis patients over time. OCT can help us in detecting these changes, thus provide a foundation for us to further explore the treatment and anatomical changes of neuritis patients. (Chin J Ophthalmol, 2018, 54: 62-68).

[A novel method of optic disk segmentation based on visual saliency and rotary scanning].

Fast optic disk localization and boundary segmentation is an important research topic in computer aided diagnosis. This paper proposes a novel method to effectively segment optic disk by using human visual characteristics in analyzing and processing fundus image. After a general analysis of optic disk features in fundus images, the target of interest could be located quickly, and intensity, color and spatial distribution of the disc are used to generate saliency map based on pixel distance. Then the adaptive threshold is used to segment optic disk. Moreover, to reduce the influence of vascular, a rotary scanning method is devised to achieve complete and continuous contour of optic disk boundary. Tests in the public fundus images database Drishti-GS have good performances, which mean that the proposed method is simple and rapid, and it meets the standard of the eye specialists. It is hoped that the method could be conducive to the computer aided diagnosis of eye diseases in the future.

Cataract and optic disk drusen in a patient with glycogenosis and di George syndrome: clinical and molecular report.

BACKGROUND: We report the ophthalmic findings of a patient with type Ia glycogen storage disease (GSD Ia), DiGeorge syndrome (DGS), cataract and optic nerve head drusen (ONHD). CASE PRESENTATION: A 26-year-old white woman, born at term by natural delivery presented with a post-natal diagnosis of GSD Ia. Genetic testing by array-comparative genomic hybridization (CGH) for DGS was required because of her low levels of serum calcium. The patient has been followed from birth, attending the day-hospital every six months at the San Paolo Hospital, Milan, outpatient clinic for metabolic diseases and previously at another eye center. During the last day-hospital visit, a complete eye examination showed ONHD and cataract in both eyes. Next Generation Sequencing (NGS) was subsequently done to check for any association between the eye problems and metabolic aspects. CONCLUSIONS: This is the first description of ocular changes in a patient with GSD Ia and DGS. Mutations explaining GSD Ia and DGS were found but no specific causative mutation for cataract and ONHD. The metabolic etiology of her lens changes is known, whereas the pathogenesis of ONHD is not clear. Although the presence of cataract and ONHD could be a coincidence; the case reported could suggest that hypocalcemia due to DGS could be the common biochemical pathway.

[The vessel and primary glaucoma].

Glaucoma, as the first leading cause of irreversible blindness in the world, is a chronic progressive optic neuropathy. The pathogenesis of glaucoma is still not fully understood till now. A lot of studies about vascular diameter, tortuosity, location, disc perfusion, vascular regulation and systemic vascular factors had been conducted to investigate the relationship between the vascular states and glaucoma since vascular hypothesis proposed. However, direct and convincing evidence for primary mechanisms of glaucoma is still lacking. The development of OCT, especially the Angio-OCT makes the real time visualization and measurement of ocular perfusion in vivo possible, gives some new evidences of vascular dysfunction of optic nerve head associated with glaucoma, which enhancing thinking of the pathogenesis of glaucoma. This review summarizes the literatures on vascular factors associated with glaucoma to provide the references for clinical researches. (Chin J Ophthalmol, 2017, 53:791-796).