Automated macular segmentation can distinguish glaucomatous from compressive optic neuropathy.

PURPOSE: To compare macular damage in glaucomatous optic neuropathy (GON) and compressive optic neuropathy (CON) and assess its diagnostic accuracy in distinguishing between diseases. METHODS: Observational, cross-sectional, single-center study. Patients with GON, CON, and healthy controls were included according to the eligibility criteria. An automated spectral-domain optical coherence tomography (SD-OCT) algorithm was used to segment the circumpapilary retinal nerve fiber layer (cpRNFL) and macula. The layer thickness was measured in each sector according to the Early Treatment Diabetic Retinopathy Study and the 6-sector Garway-Heath-based grids. Data was compared across all study groups, and the significance level was set at 0.05. RESULTS: Seventy-five eyes of 75 participants, 25 with GON, 25 with CON, and 25 healthy controls (CG), were included. Macular thickness was diminished in the ganglion cell complex of GON and CON patients compared to CG (p<0.05). The best Garway-Heath-based grid parameters for distinguishing GON and CON were the nasal-inferior (NI) and nasal-superior sectors and the NI/temporal inferior (TI) damage ratios in the macular ganglion cell (mGCL) and inner plexiform (IPL) layers. Moreover, the combination of the NI sector and NI/TI damage ratios in both layers had higher discriminative power (AUC 0.909; 95% CI 0.830-0.988; p<0.001) than combining parameters in each layer separately. CONCLUSION: Our findings suggest that the evaluation of macular segmented layers damage by SD-OCT may be a helpful add-on tool in the differential diagnosis between GON and CON.

Comparison of the retinal microvasculature between compressive and glaucomatous optic neuropathy.

PURPOSE: To compare the patterns of retinal microvasculature change in the peripapillary and macular region between compressive optic neuropathy (CON) and glaucomatous optic neuropathy (GON), and to assess the ability of optical coherence tomography angiography (OCTA) in differentiating the two conditions. METHODS: This cross-sectional study included 108 participants (108 eyes), 36 with CON, 36 with GON, and 36 healthy controls. The CON and GON eyes were matched by the average peripapillary retinal nerve fiber layer (pRNFL) thickness (1:1). Optical coherence tomography (OCT) and OCTA were performed to compare the structural and vascular change of the peripapillary and macular region between groups. RESULTS: Both CON and GON eyes showed more severe structural and vascular damage than the control eyes. The CON eyes had lower pRNFL thickness than the GON eyes in the temporal and nasal quadrants, and thicker pRNFL thickness in the inferior quadrant. The average GCC thickness did not differ between the two groups. The peripapillary vessel density of the CON group was significantly higher in the inferior sectors than that of the GON group. In the macular region, the CON group had significantly higher vessel density in the whole image, the temporal sector in parafovea region, and the temporal, superior, and inferior sectors in perifovea region. CONCLUSION: To a similar degree of structural damage, CON had less retinal vascular impairment than GON, especially in the macular region, and the significance of the finding needs further evaluation.

Compressive optic neuropathy due to posterior ethmoid mucocele.

Mucoceles are cystic formations characterized by the presence of mucus-secreting epithelial cells, which enlarge when the excretory duct becomes obstructed. Posterior ethmoidal mucoceles are rare conditions that can lead to severe ocular complications requiring immediate intervention. The close anatomical proximity of posterior ethmoidal mucoceles to the optic nerve underscores their significance. In this case report, we present a case of rapidly progressing compressive optic neuropathy secondary to a posterior ethmoidal mucocele. A previously healthy forty-six-year-old woman presented with sudden visual loss in her left eye, preceded by left-sided headache and periorbital pain. Clinical examination and imaging studies revealed an oval-shaped mass within the posterior ethmoid cell compressing the left optic nerve. Emergency surgery was performed to alleviate optic nerve compression, which successfully relieved periocular pain. However, the patient's visual acuity and visual field defect remained unchanged postoperatively. Thinning of the ganglion cell layer in the macula region was observed during follow-up examinations. The role of corticosteroids and antibiotics in visual rehabilitation and the impact of delayed surgical decompression on visual outcome remain subjects of debate. Additional cases of mucocele-associated optic neuropathy should be published and analyzed to establish optimal treatment approaches.

Evaluation of visual evoked potentials in dysthyroid optic neuropathy.

PURPOSE: To evaluate the findings of visual evoked potentials (VEP) in patients with dysthyroid optic neuropathy (DON). METHODS: In this observational, cross-sectional study 40 eyes (22 patients) with a diagnosis of DON were included. RESULTS: We discovered that in 16 out of 37 eyes with pattern-VEP (p-VEP), the latency of P100 wave was normal in spite of having a diagnosis of DON. The same pattern was also observed in the measurement of the amplitude of P100 wave: in 28 out of 37 eyes with p-VEP the amplitudes were observed as normal. In 3 eyes of 3 patients p-VEP showed no response, therefore a flash-VEP (f-VEP) was performed. Flash-VEPs of those patients indicated a prolonged P100 latency with a reduced amplitude. The sensitivity of abnormal P100 latency was 56.8% (95%CI 39.5-72.9%); and that of reduced P100 amplitude was 24.3% (95%CI 11.8-41.2%). Also, in 40 eyes color vision test by Arden was performed. In 36 eyes (20 patients) the tritan value was pathological (based on a threshold of ≥8%). CONCLUSION: According our data, VEP seems to have a limited potential especially in patients with a good best-corrected visual acuity (BCVA ≤0.2 LogMAR) for identifying the optic nerve involvement. The fact that P100 latency and amplitude were normal even in cases with an optic nerve swelling makes us question the usefulness of the VEP for diagnosing cases of DON in daily clinical life.

Spontaneous regression of a presumed orbital apex cavernous venous malformation.

A 42-year-old non-menopausal, non-pregnant woman presented with an incidentally noted right orbital apex lesion producing mild compressive optic neuropathy. Imaging revealed an apical, intraconal, inhomogenously enhancing mass consistent with a cavernous venous malformation displacing the optic nerve. The patient was monitored over a 15-year period with serial orbital imaging and clinical examinations and ultimately demonstrated significant regression in the size of the lesion, accompanied by the complete resolution of optic neuropathy. She was non-menopausal when the regression was noted and remained non-pregnant throughout the follow-up period.

Advances in the Treatment of Thyroid Eye Disease Associated Extraocular Muscle Myopathy and Optic Neuropathy.

PURPOSE OF REVIEW: To review emerging treatments for thyroid eye disease (TED) associated extraocular muscle myopathy and dysthyroid optic neuropathy (DON). RECENT FINDINGS: Emerging targeted biologic therapies may alter the disease course in TED. Teprotumumab, a type I insulin-like growth factor receptor inhibitor, is the most recent addition to the treatments available for TED-associated extraocular muscle myopathy causing diplopia. Small studies also suggest a potential therapeutic benefit for DON. Various recent studies have also expanded our knowledge on conventional TED therapies. The therapeutic landscape of TED and its sequelae has evolved in recent years. New targeted therapies have the potential to reduce the extraocular muscle and orbital volume expansion which can lead to diplopia and vision loss from optic nerve compression. Longer term efficacy and durability data is needed to determine the role biologics, such as teprotumumab, should play in the treatment of TED patients compared to the current standard of care.

Bilateral enlargement of all extraocular muscles: a presenting ophthalmic sign of hematologic malignancy.

Hematologic malignancies such as leukemia and lymphoma can frequently present in the orbit; however, involvement of the extraocular muscles is rare. The authors report two cases of systemic hematologic malignancy presenting with bilateral extraocular muscle enlargement and associated compressive optic neuropathy (CON). Both patients experienced clinical and radiographic improvement of ocular and systemic manifestations of disease with prompt initiation of targeted chemotherapy. These cases highlight the importance of including hematologic malignancy in the differential diagnosis of atypical bilateral extraocular muscle enlargement.

Dysthyroid optic neuropathy: evaluation and management.

BACKGROUND: Dysthyroid optic neuropathy (DON) is a serious complication of Graves orbitopathy (GO) from optic nerve dysfunction that may result in permanent loss of vision. PURPOSE: This paper reviews the current knowledge of DON, including its pathogenesis and epidemiology, clinical and radiologic features, and management choices and outcomes. METHODS: Literature review and author retrospective case series. RESULTS: Over 90% of DON cases are related to nerve compression by enlarged extraocular muscles (EOM) while the remainder are caused by stretching of the optic nerve without compression. DON's incidence is 5-8% of GO cases. Risk factors include advancing age, male gender, smoking and diabetes mellitus, and these cases should be referred promptly to an ophthalmologist or GO clinic to rule out DON and for ongoing care. Clinical features of DON may include reduction in central and colour vision (unexplained by other ocular disorders), afferent pupil defect and/or optic disc edema. Since most cases are associated with enlarged EOM, restricted motility and soft tissue venous congestion are common. Visual fields and optical coherence tomography (OCT) help confirm the diagnosis while CT or MRI Scans show apical optic nerve compression or proptosis with optic nerve stretch. Standard therapy includes iv/oral corticosteroids (CS) with partial response in most cases, but often relapse with tapering. Radiotherapy may delay or avoid surgery and may prevent the onset of DON when combined with CS in high-risk individuals. The benefits of newer biologic targeted therapy are not clear. Orbital decompression surgery often has positive outcomes, even in cases of severe vision loss or delayed surgery. The most common surgical complication is worsening strabismus, which may worsen visual function and quality of life. In rare cases, permanent vision loss from DON may occur despite full therapy. CONCLUSIONS: Although DON may cause vision loss, most cases are reversible if recognized and managed in a timely manner.

Spontaneous orbital decompression in thyroid eye disease: new measurement methods and its influential factors.

PURPOSE: To evaluate spontaneous decompression of the medial orbital wall and orbital floor in thyroid eye disease using new measurement methods and to analyze the influential factors. METHODS: This retrospective study included 86 patients (172 sides). Regarding evaluation of spontaneous medial orbital decompression, an anteroposterior line was drawn between the posterior lacrimal crest and the junction between the ethmoid bone and corpus ossis sphenoidalis. The bulged and/or dented areas from that line were measured. Regarding evaluation of spontaneous orbital floor decompression, the length of the perpendicular distance from a line that was drawn between the inferior orbital rim and the orbital process of palatal bone to the tip of the superior bulge of the orbital floor was measured. RESULTS: Multivariate linear regression analysis revealed that the maximum cross-sectional areas of the superior rectus/levator palpebrae superioris complex (P = 0.020) and medial rectus muscle (P = 0.028) were influential factors for spontaneous decompression of medial orbital wall (adjusted r2 = 0.090; P < 0.001), whereas the number of cycles of steroid pulse therapy (P = 0.002) and the maximum cross-sectional area of the inferior rectus muscle (P = 0.007) were the ones for that of the orbital floor (adjusted r2 = 0.096; P < 0.001). CONCLUSION: We believe that the identification of multiple influential factors of spontaneous decompression of the medial orbital wall and orbital floor will be helpful for better understanding and planned management of thyroid eye disease patients undergoing orbital decompression surgery.

Segmented retinal layer analysis of chiasmal compressive optic neuropathy in pituitary adenoma patients.

AIMS: To evaluate changes in the segmented retinal layers of pituitary adenoma (PA) patients and to identify the relationship between these changes and visual function. METHODS: A total of 47 (PA patients) and 22 (healthy subjects) eyes were reviewed from the medical records. The PA patients performed a visual field (VF) test before surgery and 1 month after surgery. By optical coherence tomography scanning, eight retinal layers were measured: retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer (INL), outer plexiform layer, outer nuclear layer, retinal pigment epithelium, and photoreceptor layer. RESULTS: The PA group showed reduced RNFL, GCL, and IPL thicknesses (p = 0.004,< 0.001,< 0.001) and thicker INL thickness (p = 0.012) than did the controls. The mean deviation of preoperative VF in the PA group was positively correlated with RNFL, GCL, and IPL thicknesses (R = 0.664, 0.720, 0.664; p < 0.001,< 0.001,< 0.001) and negatively correlated with the INL thickness (R = -0.400; p = 0.010). Among the 47 eyes, 32 eyes (68%) were included for subgroup analysis. Preoperative RNFL, GCL, and IPL thicknesses were thicker in the postoperatively improved VF group (p = 0.019, 0.009, 0.005). The preoperative cutoff values for visual recovery were 23.6 µm for RNFL thickness, 30.6 µm for GCL thickness, and 28.9 µm for IPL thickness. CONCLUSION: During chiasmal compression, the thickening of the INL has presented in addition to thinning of the inner retinal layers. Also, changes in retinal anatomical structures are related to the extent of VF defect and can be used as a predictor of postoperative visual recovery.

Visual field and orbital computed tomography correlation in dysthyroid optic neuropathy due to thyroid eye disease.

Purpose: The pathogenesis of dysthyroid optic neuropathy (DON) in thyroid eye disease (TED) is thought to be compression of the apical optic nerve by hypertrophied extraocular muscles. We correlated worsening DON to the area occupied by extraocular muscles.Methods: Records of adults with TED DON evaluated from 1/1/2013 to 1/1/2018 were retrospectively reviewed. Each patient's visual field with the worst mean deviation (MD) was selected. Orbit CT scans were reviewed. Reformatted oblique coronal images were created perpendicular to the optic nerve. The cross-sectional area (CSA) of the orbit and each muscle group was measured and expressed as ratios of the CSA of the orbital apex. Univariate and multivariate analysis was performed for predictors of HVF MD.Results: 34 orbits with TED DON were analyzed. On orbital CT, the superior muscle complex occupied 15% of the apex (range 6-26%), inferior 18% (range 6-33%), lateral 10% (range 4-18%), medial 17% (range 8-27%), and all combined 61% (range 28-80%). Increasing total muscle area and superior complex area correlated with worsening MD. In multivariate linear regression, the superior muscle complex remained a significant predictor of MD (p = 0.01) over total muscle area (p = 0.25).Conclusions: Enlargement of extraocular muscles is common in TED, but DON occurs in only 6%. Our findings demonstrate that as DON worsens, as quantified by visual field MD, the superior muscle complex crowds the apex. This is consistent with the typical inferior visual field findings seen in TED DON. Hypertrophy of the superior rectus and levator palpabrae superioris complex may be predictive of worsening DON.

Compressive Optic Neuropathy with a Concurrent Mutation of Leber's Hereditary Optic Neuropathy: A Case Report.

Leber's hereditary optic neuropathy (LHON) is a mitochondrial genetic disease that leads to acute or subacute, painless, bilateral loss of vision, caused by degeneration of retinal ganglion cells that most affects men in their second or third decade of life. We describe a woman with compressive optic neuropathy with a concurrent LHON-associated mitochondrial mutation. Temporal hemifield defect connected to central scotoma and concurrent abduction limitation are diagnostic clues in identifying chiasmal compression with craniopharyngioma. This case emphasizes an awareness of the possible coexistence of compressive and hereditary optic neuropathy.

Bruch's Membrane Opening Minimum Rim Width in the Differential Diagnosis of Optic Neuropathies.

Observing optic disc pallor during a patient's first visit frequently raises a diagnostic challenge, particularly in regards to whether the cause is due to glaucoma or another form of optic neuropathy. Bruch's membrane opening (BMO) was recently discovered as the anatomical border of the optic disc. BMO minimum rim width (BMO-MRW) seems to be a reliable representation of the neuroretinal rim. In our study, we demonstrate the ability of BMO-MRWs to differentiate between glaucomatous and non-glaucomatous. Additionally, we propose an MRW ratio which may allow discrimination of open angle glaucoma from either non-arteritic anterior ischaemic optic neuropathy or compressive optic neuropathy.

A Case of Compressive Optic Neuropathy Secondary to Lymphoma of the Extraocular Muscles.

Compressive optic neuropathy is an urgent condition that may lead to blindness. We report a rare case of compressive optic neuropathy secondary to lymphoma of the extraocular muscles in which the vision was successfully rescued. A 67-year-old man presented with visual acuity of hand motion in the right eye for 1 month. Magnetic resonance imaging demonstrated enlargement of the medial and inferior rectus muscles with their tendons. Tendon enlargement, which is uncommon as Graves' orbitopathy, motivated us to perform a biopsy. Intravenous steroids following biopsy and chemotherapy after definitive diagnosis allowed for recovery of vision. Clinicians should be aware that enlarged extraocular muscles due to lymphoma can cause compressive optic neuropathy.

Pain in optic neuropathies.

Pain occurs with optic neuropathies associated with inflammatory central nervous system diseases (MS and NMO), idiopathic intracranial hypertension and spontaneous hypotension, giant cell arteritis, immunomediated systemic diseases, compressive lesions, or infective disorders. Pain can precede the onset of visual loss in acute optic neuritis, it can be irradiated to the orbital region in giant cell arteritis and parasellar compressive optic neuropathies, or it may be located to the back of the eye with posterior scleritis. History of symptoms together with complete neuro-ophthalmological examination must guide the differential diagnosis and neuroimaging. Painful visual loss due to different pathophysiological mechanisms requires specific treatment and prognosis.

The Case of IgG4-related Ophthalmic Disease Accompanied by Compressive Optic Neuropathy.

A 76-year-old woman was diagnosed with thyroid-associated orbitopathy (TAO) associated with hypothyroidism at another clinic. Magnetic resonance imaging showed enlargements of the extraocular muscles. Because her best-corrected visual acuity (BCVA) was 0.01OD, she was referred to the Chiba University Hospital. She had an elevation of serum IgG4. She was diagnosed with possible IgG4-related ophthalmic disease (IgG4-ROD) accompanied by optic nerve compression with the mass. She underwent methylprednisolone pulse therapy, and, two months later, her BCVA and visual field defect were greatly improved. Our findings indicate that ophthalmologists should be aware that IgG4-ROD can be masked by lesions associated with TAO.

[Etiology and diagnostics of compressive optic neuropathies]. / Analiz prichin i vozmozhnosti diagnostiki kompressionnykh opticheskikh neiropatii.

PURPOSE: To determine the causes and study the clinical manifestations of compressive optic neuropathy (CON). MATERIAL AND METHODS: The study included 24 male and 36 female patients with CON (in total 60 patients, 97 eyes). All patients underwent standard ophthalmic examination, as well as computer perimetry, magnetic resonance imaging (MRI), and/or computed tomography (CT) of the brain and orbit and magnetic resonance angiography (MRA). RESULTS: Tumor or infiltrative lesions of the anterior optic pathway were detected in 60 patients (97 eyes). In 2/3 of cases, the brain and orbit diseases were established for the first time as the cause of optic neuropathy. Adenoma of the pituitary gland and meningioma as the most common cause of CON was diagnosed in 66%. According to the results of computed perimetry, in 65.9% of cases patients with CON are diagnosed with hemianopsia, while in other cases, other forms of scotomas characteristic for the defeat of any part of the optic nerve may be observed. CONCLUSION: Computed perimetry is the most important study for detection of optic neuropathy. An optic nerve disease of unknown origin is an absolute indication for radiological examination of the brain and eye orbits.

Patterns of visual field changes in thyroid eye disease.

This article provides a systematic description of visual field changes in thyroid eye disease-compressive optic neuropathy (TED-CON). A retrospective, non-comparative chart review of patients with TED-CON and documented Humphrey Visual Field 24-2 or 30-2 testing was conducted with IRB approval. Ninety-six visual fields in 68 patients were classified into 7 broad categories (superior, inferior, diffuse, temporal, nasal, central/paracentral, enlarged blind spot) and 17 mutually exclusive patterns from the Ocular Hypertension Treatment Study (OHTS) or "other." Fifty-three of 96 visual fields (55%) showed an inferior defect using the broad categories, with the remaining 6 categories ranging from 2% to 14%. The five most common OHTS patterns were other (28%), partial arcuate (28%), partial peripheral rim (9%), arcuate (8%) and altitudinal (7%). Further sub-classification showed a predominance of inferior visual field defects, ranging from 33% to 93% of each category. Of the 78 visual fields in these five categories combined, 52 (67%) were inferior defects. Inferior defect is the most typical TED-CON-associated visual field change. While the OHTS categories are geared toward classification of glaucomatous patterns, the overall predominance of inferior field defects in TED-CON was clearly demonstrated. These "other" visual field changes showing central inferior defect up to but not crossing the horizontal meridian and not contiguous from blind spot to nasal meridian should be designated as "TED-CON pattern." The high proportion of visual fields falling under the "other" category, however, does demonstrate the need for a more specific and tailored visual field classification system for TED-CON.

Bilateral Compressive Optic Neuropathy from Renal Osteodystrophy Caused by Branchio-oto-renal Syndrome Stabilised After Parathyroidectomy.

Renal osteodystrophy can cause calvarial hypertrophy and narrowing of the neural canals and foramina. Compressive optic neuropathy is extremely rare in renal osteodystrophy and was reported once only. The authors report bilateral, simultaneous compressive optic neuropathy secondary to renal osteodystrophy with features of uremic leontiasis ossea in chronic renal failure caused by branchio-oto-renal syndrome. Because of the extensive calvarial hypertrophy and the surgical difficulties envisaged with optic canal decompression, conservative approach was pursued. The patient's visual acuity and fields improved after partial parathyroidectomy. Visual improvement may be explained by the arrest of renal osteodystrophy and reduced optic nerve compression after parathyroidectomy.

Quantitative comparison of disc rim color in optic nerve atrophy of compressive optic neuropathy and glaucomatous optic neuropathy.

PURPOSE: The purpose was to investigate an objective and quantitative method to estimate the redness of the optic disc neuroretinal rim, and to determine the usefulness of this method to differentiate compressive optic neuropathy (CON) from glaucomatous optic neuropathy (GON). METHODS: In our study there were 126 eyes: 40 with CON, 40 with normal tension glaucoma (NTG), and 46 normal eyes (NOR). Digital color fundus photographs were assessed for the redness of disc rim color using ImageJ software. We separately measured the intensity of red, green, and blue pixels from RGB images. Three disc color indices (DCIs), which indicate the redness intensity, were calculated through existing formulas. RESULTS: All three DCIs of CON were significantly smaller than those of NOR (P < 0.001). In addition, when compared with NTG, DCIs were also significantly smaller in CON (P < 0.05). A comparison of mild CON and mild NTG (mean deviation (MD) > -6 dB), in which the extent of retinal nerve fiber layer thinning is comparable, the DCIs of mild CON were significantly smaller than those of mild NTG (P < 0.05). In contrast, DCIs did not differ between moderate-to-severe stages of CON and NTG (MD ≤ -6 dB), though the retinal nerve fibers of CON were more severely damaged than those of NTG. To differentiate between mild CON and mild NTG, all AUROCs for the three DCIs were above 0.700. CONCLUSIONS: A quantitative and objective assessment of optic disc color was useful in differentiating early-stage CON from GON and NOR.