Orbital Optic Nerve Research Articles

Radiologic Predictors of Visual Outcome in Myelin Oligodendrocyte Glycoprotein-Related Optic Neuritis

This study aimed to determine whether magnetic resonance imaging (MRI) biomarkers are associated with visual prognosis in myelin oligodendrocyte protein (MOG)-associated optic neuritis (ON). Cross-sectional analysis. Patients meeting 2023 international diagnostic criteria for MOG antibody-associated disease who were seen for first episodes of MOG-associated ON at 3 tertiary neuro-ophthalmology practices between January 2017 and July 2023 were enrolled. Patients who received < 3 months of neuro-ophthalmic follow-up and did not demonstrate visual recovery (visual acuity [VA] ≥ 20/20 and visual field mean deviation [VFMD] > -5.0 dB) during this time were excluded. Patients underwent contrast-enhanced, fat-suppressed MRI of the brain and orbits within 1 month of symptom onset. The associations between radiologic biomarkers and poor VA outcome (<20/40), incomplete VA recovery (< 20/20), and poor VFMD outcome (VFMD < -5.0 dB) were assessed using multivariable logistic regression adjusting for time from symptom onset to treatment and nadir VA or VFMD. Radiologic biomarkers included length of optic nerve enhancement (> 25% vs. < 25%; > 50%vs.<50%; and >75%vs.< 75%); degree of orbital, canalicular, and intracranial or chiasmal optic nerve enhancement (mild vs. moderate to severe compared with the lacrimal gland); and absence versus presence of optic nerve sheath enhancement on baseline T1-weighted MRI. A total of 129 eyes of 92 patients (median age, 37.0 years [interquartile range, 20.8-51.3 years]; 65.2% female) were included. Poor VA outcome was seen in 6.2% of patients, incomplete VA recovery was seen in 19.4% of patients, and poor VFMD outcome was seen in 16.9% of patients. Compared with eyes with moderate to severe enhancement, eyes with mild orbital optic nerve enhancement were more likely to have poor VA outcome (odds ratio [OR], 8.57; 95% confidence interval [CI], 1.85-51.14; P= 0.009), incomplete VA recovery (OR, 7.31, 95% CI, 2.42-25.47; P= 0.001), and poor VFMD outcome (adjusting for time to treatment: OR, 6.81; 95% CI, 1.85-28.98; P= 0.005; adjusting for nadir VFMD: OR, 11.65; 95% CI, 1.60-240.09; P= 0.04). Lack of optic nerve sheath enhancement additionally was associated with incomplete VA recovery (OR, 3.86; 95% CI, 1.19-12.85; P= 0.02) compared with the presence of enhancement. These associations remained consistent in subgroup logistic regression analysis of MRIs performed before initiation of treatment but were not seen in pairwise analysis of MRIs performed after treatment. In eyes with first MOG-associated ON episodes, milder enhancement in the orbital optic nerve was associated with poorer VA and visual field recovery. Prospective and mechanistic studies are needed to confirm the prognostic usefulness of MRI in MOG-associated ON. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Clinical pathological and genetic mutation characteristics of conjunctival lymphoepithelial carcinoma

Objective: To analyze the clinical pathological and genetic mutation characteristics of conjunctival lymphoepithelial carcinoma. Methods: A retrospective case series study was conducted. Data from three patients diagnosed with conjunctival lymphoepithelial carcinoma and treated with tumor resection surgery at Tianjin Eye Hospital from January 2006 to December 2022 were collected. Four paraffin specimens (including one patient undergoing two surgeries) were subjected to immunohistochemical staining for epithelial antigen and lymphocytic antigen. Epstein-Barr virus (EBV)-encoded RNA (EBER) was detected using in situ hybridization, and whole-exome sequencing was performed on three specimens from two patients using next-generation sequencing methods. Results: All three patients were males aged over 65, with a disease duration ranging from 3 to 44 months. The tumors were unilateral, located on the bulbar or limbal conjunctiva, appearing red, with a maximum diameter of 4-20 mm. Imaging examinations revealed anterior location of the tumors with no involvement of the orbital bone, extraocular muscles, optic nerve, or paranasal sinuses. No local lymph node metastasis was observed in any patient. Pathological findings included undifferentiated carcinoma nests with significant reactive lymphocytic and plasma cell infiltration. Tumor cells were positive for pan-cytokeratin (CK-pan), epithelial membrane antigen (EMA), tumor protein 40 (p40), and tumor protein 63 (p63), with a cell proliferation index (Ki67) exceeding 80%. Cluster of differentiation 20 (CD20), CD3, and CD8 were positive for lymphocytes. In situ hybridization showed partial tumor cell expression of EBER in two specimens of one patient. Whole-exome sequencing revealed 58, 50, and 36 mutated genes in the three specimens, with enriched signaling pathways including melanoma signaling pathway, Notch1 signaling pathway, and RHOQ GTP cycle; enriched biochemical processes included amino acid starvation response, programmed cell death, regulation of lipid synthesis, sodium ion transport, and chromosome segregation. The common mutated gene in all three specimens was SZT2, and SZT2 was involved in the amino acid starvation response. One patient underwent a second complete resection surgery 40 months after partial excision, while the other two underwent complete resection surgery without recurrence. Two patients did not undergo radiation or chemotherapy, and one was lost to follow-up. Conclusions: Conjunctival lymphoepithelial carcinoma is associated with prominent lymphocytic and plasma cell infiltration, some cases are associated with EBV infection, and SZT2 mutations are present in conjunctival lymphoepithelial carcinoma.

Echographic Optic Nerve Evaluation: A Novel Diagnostic Modality in Glaucoma

Introduction: Primary Open-Angle Glaucoma (POAG) is considered a leading cause of blindness among all others. Different technologies such as Scanning Laser Polarimetry (SLP) and Optical Coherence Tomography (OCT) closely correlate in measuring structural parameters of the Retinal Nerve Fiber Layer (RNFL) and Optic Nerve Head (ONH). Visual impairment related to glaucomatous damage is attributed to the RNFL. Earlier studies have shown that retro-bulbar optic nerve thickness is reduced in glaucoma and have suggested that this is also the result of RNFL destruction. Aim: To investigate the correlation between the orbital and intraocular portions of the optic nerve among POAG patients. Materials and Methods: This was a hospital-based crosssectional study done in a tertiary care ophthalmic institute from October 2019 to February 2021. One eye of 32 volunteers with newly diagnosed POAG underwent optic disc analysis using OCT and echographic measurements of the retrobulbar optic nerve. For statistical calculations, Statistical Package for Social Sciences (SPSS) Statistics version 20.0 software (IBM Corp., Armonk, NY, USA) was used. Spearman’s rho (rs) was used as the index of correlation between retrobulbar optic nerve dimensions and ONH topographical data. A correlation between OCT-based RNFL and optic disc parameters was compared with retrobulbar optic nerve dimensions measured with the help of Ultrasonography- Brightness (USG B) Scan. Results: Orbital Optic Nerve Diameter (OND) and Optic Nerve Cross-sectional Area (ONCSA) significantly and positively correlated with Neuro-retinal Rim (NR) area (OND: p-value=0.00001; ONCSA: p-value=0.00001) and average nerve fiber layer thickness (OND: p-value=0.0001; ONCSA: p-value=0.00002). The Retrobulbar ONCSA-to-disc area ratio (ONCSA/D) was found to have a statistically demonstrable positive correlation with Neuro-retinal Rim Area/Disc area ratio (NR/D) (p=0.00003). Conclusion: This study showed that retrobulbar optic nerve dimensions correlate well with SD-OCT-based ONH parameters. Echographic measurements of the retrobulbar optic nerve add a new biomarker in the diagnosis of glaucoma.

Atypical orbital primary optic nerve sheath meningioma with severe disfiguring proptosis: an alternative surgical approach

Primary optic nerve sheath meningioma is generally a benign tumor. In rare instances, however, the growth rate and intraocular and intracranial extensions can be highly aggressive, especially in children, leading to poor prognosis. Here, we reported a case of a 24-year-old woman who presented with left eye swelling for 3 years. This was associated with blurred vision, retrobulbar pain, and redness. On examination, the left eye was severely proptosed with complete ophthalmoplegia. Magnetic resonance imaging showed an extensive tumor occupying the whole left orbital cavity with a disfigured eyeball. However, no intracranial extension was observed. Interestingly, complete surgical excision was feasible via transconjunctival anterior orbitotomy without bone removal. The histopathological examination confirmed the diagnosis of optic nerve sheath meningioma. Adjunct radiotherapy was given. On a follow-up after 2 years, left enophthalmos with esotropia was observed.

Alternative Path for Optic Nerve Decompression in Pseudotumor Cerebri With Full Endoscopic Lateral Transorbital Approach.

Endonasal endoscopic approaches are the most preferred surgical methods in patients with pseudotumor cerebri because of easy access to the optic nerve, but the choice of this technique may not apply to all endoscopic endonasal cases. Moreover, there may be difficulties in practice in some cases, including the coronavirus disease 2019 pandemic. This study aimed to suggest an alternative endoscopic approach by lateral orbitotomy for optic nerve decompression in patients with pseudotumor cerebri. The study was performed using 5 fresh-frozen cadaver heads (bilaterally, total of 10 sides) injected intravenously with colored silicone preserved in the cold chain. An average of 2.5 cm skin incision was made to fit the lateral orbitotomy. The lengths of the recurrent meningeal artery (mm), the meningo-orbital band (mm), and the optic nerve (mm) to the orbital margin were measured. After these morphometric measurements, optic nerve decompression was performed endoscopically, and the length of the decompression was measured (mm). The average length (mm) between the orbital rim and meningeal recurrent artery (or meningolacrimal branch) was 16.2mm, between the orbital rim and the meningo-orbital band was 18.5mm, and between the orbital rim and optic nerve was 44.1mm. The average optic nerve decompression length was 4.4mm. The endoscopic lateral orbitotomy approach provides easy access to the optic nerve by anatomically following the recurrent meningeal artery and the meningo-orbital band. It can be a safe second-line approach after endonasal approaches for optic nerve decompression in pseudotumor cerebri.

Elevated perioptic lipocalin-type prostaglandin D synthase concentration in patients with idiopathic intracranial hypertension.

The pathophysiology of vision loss and loss of visual field in patients with idiopathic intracranial hypertension with papilloedema is not fully understood. Although elevated CSF pressure induces damage to the optic nerve due to stasis of axoplasmic flow, there is no clear relationship between the severity of papilloedema and CSF pressure. Furthermore, there are cases of purely unilateral papilloedema and cases without papilloedema despite significantly elevated intracranial pressure as well as papilloedema that can persist despite a successfully lowered intracranial pressure. We hypothesize that at least in some of such cases, in addition to purely pressure-induced damage to the optic nerve, the biochemical composition of the CSF in the subarachnoid space surrounding the orbital optic nerve may play a role in the pathogenesis of vision loss. In this retrospective study, we report on lipocalin-type prostaglandin D synthase concentrations in the CSF within the perioptic and lumbar subarachnoid space in 14 patients with idiopathic intracranial hypertension (13 females, mean age 45 ± 13 years) with chronic persistent papilloedema resistant to maximum-tolerated medical therapy and visual impairment. CSF was collected from the subarachnoid space of the optic nerve during optic nerve sheath fenestration and from the lumbar subarachnoid space at the time of lumbar puncture. CSF was analysed for lipocalin-type prostaglandin D synthase and the concentrations compared between the two sites using nephelometry. The mean lipocalin-type prostaglandin D synthase concentration in the perioptic subarachnoid space was significantly higher compared with the concentration in the lumbar subarachnoid space (69 ± 51 mg/l without correction of serum contamination and 89 ± 67 mg/l after correction of serum contamination versus 23 ± 8 mg/l; P < 0.0001, Mann–Whitney U-test). These measurements demonstrate a change and imbalance in the biochemical environment of the optic nerve. Its possible effect is discussed.

Skull Base Chordoma: The Thumb Sign

A 57 year old woman with no past medical history presented with headaches and progressive left vision loss. Subsequently, a contrast enhanced brain MRI was performed showing a large well defined skull base mass located laterally in the left petro clival fissure, exhibiting high T2 and FLAIR signal intensity (Figure A), with areas of low signal intensity on susceptibility weighted images consistent with calcifications and a heterogenous enhancement after injection. This tumor was responsible for mass effect on the orbital apex and optic nerve anteriorly and the mesio temporal lobe laterally. Most importantly, on the sagittal planes we can see the tumor projection indenting the pons which is displaced posteriorly. This finding is known as the thumb sign (Figure B and C) characteristic of chordomas. Given these MRI features and after a stereotactic biopsy the diagnosis of chordoma of the skull base was confirmed. Because of the major local extension, the patient underwent radiotherapy rather than surgical resection. Chordomas are rare skull base tumors arising from embryonal rest cells [1]. The major differential being chondrosarcomas with similar appearance but higher ADC values. The thumb sign is relatively specific of chordomas [2] although itcan be described in chondromas of the skull base and can also be seen in chondrosarcomas.

Association of optic nerve sheath lesion and brain or meningeal metastasis caused by lung cancer.

BackgroundWith the development of imaging, the etiology of ocular lesions caused by lung cancer was not found only intraocular. Neuro‐ophthalmological imaging has been found to be useful for the diagnosis of meningeal carcinomatosis, although few studies have previously been published. Our study used magnetic resonance imaging (MRI) to determine if there was a the relationship between ocular symptoms and lung cancer metastasis.MethodsWe carried out a retrospective analysis which included patients with ocular lesions and lung cancer on which orbit MRI was performed together with ocular examination during January 2014 to January 2022. Here, we describe the characteristics of intraocular metastasis and optic nerve sheath lesions.ResultsA total of 21 lung cancer patients had ocular lesions, of which eight cases had choroidal metastasis; a further eight patients had optic nerve sheath lesions. There was one case (12.5%) of choroidal metastasis with brain or meningeal metastasis, and eight cases (100%) with optic nerve sheath lesions with brain or meningeal metastasis. A total of 75.0% patients with choroidal metastasis did not have any history of known lung cancer, and 25.0% of optic nerve sheath lesions in patients were found before a meningeal metastasis diagnosis. The features of optic nerve sheath lesions included thickening and strengthening of the long segment of the orbital optic nerve sheath with a clear boundary.ConclusionsThe pathway of choroidal and optic nerve sheath lesions was different. Optic nerve sheath lesions were associated with brain and meningeal metastasis. When lung cancer‐induced meningeal metastasis is suspected, orbital MRI is recommended to assist in the diagnosis.

Differences in Neuropathic Pain and Radiological Features Between AQP4-ON, MOG-ON, and IDON

PurposeThe purpose of this study was to investigate pain and radiological features of different types of first-episode demyelinating optic neuritis (ON).MethodsEighty-three patients presenting with first-episode aquaporin-4 (AQP4) antibody-associated ON (AQP4-ON; n = 28), myelin oligodendrocyte glycoprotein (MOG) antibody-associated ON (MOG-ON; n = 26) and idiopathic demyelinating optic neuritis (IDON, n = 29) were included in this retrospective case-control study. We assessed optic nerve lesions on magnetic resonance imaging (MRI), acute pain associated with onset of optic neuritis and clinical characteristics of those ON patients with different serum autoantibody status.Results24 AQP4-ON patients (85.75%), 23 MOG-ON patients (88.5%) and 24 IDON patients (82.8%) suffered from ON-associated pain. MOG-ON had mostly retro-orbital pain; AQP4-ON and IDON had mostly neuropathic pain. In addition, pain was more severe in AQP4ON patients than in other ON patients. In MRI, bilateral involvement was more common in AQP4-ON than IDON (26.9 and 3.7%); radiological optic nerve head swelling was more common in MOG-ON than in AQP4-ON and IDON (68.0 vs. 23.1 vs. 25.9%). MRI lesion in peri-optic nerve sheath was more common in AQP4-ON (53.8 vs. 16.0 vs. 3.7%). In 70 patients with ON-associated pain, gadolinium enhancement of orbital optic nerve was most common in MOG-ON patients (82.4 vs. 55.0 vs. 33.3%, P = 0.018), and enhancement of optic chiasma was most common in AQP4-ON patients (40.0 vs. 5.9 vs. 6.7%, P = 0.015). Perineural and orbital enhancement was observed only in patients with MOG-ON (P < 0.001). The length of enhancement was longer in AQP4-ON patients than in MOG-ON and IDON patients.ConclusionPain is a common symptom in patients with all types of demyelinating ON. AQP4-ON is frequently associated with severe ON-associated pain and longitudinally extensive optic nerve inflammatory lesions. Intra-orbital and peri-optic inflammation were more frequently observed in patients with MOG-ON, which was closely related to optic disc swelling and retro-orbital pain provoked by eye movements.

Multi-Parametric Diffusion Tensor Imaging of The Optic Nerve for Detection of Dysthyroid Optic Neuropathy in Patients With Thyroid-Associated Ophthalmopathy.

ObjectiveTo evaluate the microstructural changes of the orbital optic nerve in thyroid-associated ophthalmopathy (TAO) patients with or without dysthyroid optic neuropathy (DON) using diffusion tensor imaging (DTI) and investigate whether DTI can be used to detect DON.Materials and Methods59 bilateral TAO patients with (n= 23) and without DON (non-DON, n= 36) who underwent pretreatment DTI were included and 118 orbits were analyzed. The clinical features of all patients were collected. DTI parameters, including mean, axial, and radial diffusivity (MD, AD, and RD, respectively) and fractional anisotropy (FA) of the intra-orbital optic nerve for each orbit were calculated and compared between the DON and non-DON groups. ROC curves were generated to evaluate the diagnostic performance of single or combined DTI parameters. Correlations between DTI parameters and ophthalmological characteristics were analyzed using correlation analysis.ResultsCompared with non-DON, the DON group showed decreased FA and increased MD, RD, and AD (P < 0.01). In the differentiation of DON from non-DON, the MD was optimal individually, and the combination of the four parameters had the best diagnostic performance. There were significant correlations between the optic nerve’s four DTI metrics and the visual acuity and clinical active score (P < 0.05). In addition, optic nerve FA was significantly associated with the amplitude of visual evoked potentials (P = 0.022).ConclusionsDTI is a promising technique in assessing microstructural changes of optic nerve in patients with DON, and it facilitates differentiation of DON from non-DON eyes in patients with TAO.

Severe Self-Inflicted Gunshot Wound of the Face

A 26-year-old male was transported to the hospital with a severe self-inflicted gunshot wound of the middle and lower face (Panel A) due to the suicide attempt. Three-dimensional computed tomography (CT) showed multiple site comminuted fracture of the facial skeleton including maxilla, mandible, right zygomatic, ethmoid, and nasal bones (Panel B). Also, the sockets of multiple avulsed teeth were noted. The patient was transported from the region several days after the injury. Due to the osseous and soft tissue loss, this type of gunshot wounds typically poses a considerable challenge to the oral and maxillofacial and plastic surgeons. Acute management in such cases includes resuscitative and life-saving measures. Timing of each surgical procedure should be chosen according to the patient condition, extent of the injury, and international recommendations.1 Operative principles include: (1) general considerations (all reconstructive options are to be taking into account, moreover, the volume of bone and soft tissue loss, functional and aesthetic purposes should be analyzed and planned very precisely), (2) skeletal fixation, (3) bone grafts, (4) soft tissue coverage, and even (5) a facial transplantation in some cases. In our case, the right eye enucleation (removal of the entire globe with all intraocular contents, while preserving orbital/periorbital structures) was done due to the severely ruptured globe. Typical indications for enucleation are severe eye trauma and blind, intraocular tumors, painful, and cosmetically disfiguring eyes. In case of evisceration the intraocular contents are removed from an intact sclera, extraocular muscle attachments and the orbital adnexa are preserved, with placement of an implant within the scleral shell with a purpose to retain orbital volume. Common indications for enucleation are unresponsive endophthalmitis and for improvement of cosmesis in a blind eye. The most common indication for orbital exenteration (removal of the globe and all orbital contents, including muscles, fat, optic nerve, lacrimal gland, nerves, periorbital bone, upper and lower lid complexes) is malignancy.

A Case of Invasive Aspergillosis Involing Orbital Apex and Optic Nerve Sheath.

Spread of fungal infection neurally is a rare phenomenon. Hereby we report a case of fifty year old diabetic female with primary optic atrophy and aspergillosis of orbital apex and optic nerve sheath. In this case fungal debris was found in sphenoid sinus, orbital apex and optic nerve sheath. This patient had mild proptosis on left eye. This is a rare condition of fungal growth in the optic nerve sheath. Radiological investigation revealed bony erosion in posterior wall of sphenoid sinus. Functional endoscopic sinus surgery was done and sample sent for KOH mount, fungal culture and histopathological examination revealing aspergillus fumigatus and post op follow up with antifungal therapy.

Influence of eye movement on lens dose and optic nerve target coverage during craniospinal irradiation

PurposeOptic nerves are part of the craniospinal irradiation (CSI) target volume. Modern radiotherapy techniques achieve highly conformal target doses while avoiding organs-at-risk such as the lens. The magnitude of eye movement and its influence on CSI target- and avoidance volumes are unclear. We aimed to evaluate the movement-range of lenses and optic nerves and its influence on dose distribution of several planning techniques. MethodsTen volunteers underwent MRI scans in various gaze directions (neutral, left, right, cranial, caudal). Lenses, orbital optic nerves, optic discs and CSI target volumes were delineated. 36-Gy cranial irradiation plans were constructed on synthetic CT images in neutral gaze, with Volumetric Modulated Arc Therapy, pencil-beam scanning proton therapy, and 3D-conventional photons. Movement-amplitudes of lenses and optic discs were analyzed, and influence of gaze direction on lens and orbital optic nerve dose distribution. ResultsMean eye structures’ shift from neutral position was greatest in caudal gaze; −5.8±1.2 mm (±SD) for lenses and 7.0±2.0 mm for optic discs. In 3D-conventional plans, caudal gaze decreased Mean Lens Dose (MLD). In VMAT and proton plans, eye movements mainly increased MLD and diminished D98 orbital optic nerve (D98OON) coverage; mean MLD increased up to 5.5 Gy [total ΔMLD range −8.1 to 10.0 Gy], and mean D98OON decreased up to 3.3 Gy [total ΔD98OON range −13.6 to 1.2 Gy]. VMAT plans optimized for optic disc Internal Target Volume and lens Planning organ-at-Risk Volume resulted in higher MLD over gaze directions. D98OON became ≥95% of prescribed dose over 95/100 evaluated gaze directions, while all-gaze bilateral D98OON significantly changed in 1 of 10 volunteers. ConclusionWith modern CSI techniques, eye movements result in higher lens doses and a mean detriment for orbital optic nerve dose coverage of <10% of prescribed dose.

Optic nerve Hemangioblastoma with bilateral frontal lobe Oedema: a case report

BackgroundHemangioblastomas are rare benign tumours that are most commonly detected in the subtentorium or spinal cord. Optic nerve hemangioblastoma is very rare and is most commonly associated with Von Hippel-Lindau (VHL) syndrome.Case presentationHere, we report a case of hemangioblastoma of the optic nerve with bilateral frontal lobe oedema without VHL syndrome, which has not yet been reported. A 51-year-old woman presented with progressive and painless deteriorating vision in the left eye. Magnetic resonance imaging showed a mass at the back of the left orbital optic nerve. Endoscopic-assisted intraorbital tumour resection was performed successfully. The pathological diagnosis was left optic nerve hemangioblastoma.ConclusionsThis is the first reported case of optic nerve hemangioblastoma (HBL) with bilateral frontal lobe oedema.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis in Canada.

To describe clinical characteristics of Canadian patients with myelin-oligodendrocyte glycoprotein immunoglobulin-G optic neuritis (MOG-IgG ON). Retrospective observational case series of MOG-IgG seropositive patients with ON referred to tertiary neuro-ophthalmology practices. Outcome measures included clinical characteristics, radiologic findings, and visual outcomes. Forty-six eyes of 30 patients were included. Twenty-three (76.7%) were women, mean onset age was 40.7 years (range 16-77), and most were Caucasian. Seventeen (56.7%) presented with their first ON episode. Sixteen (53.3%) had bilateral eye involvement. Isolated ON without associated neurological symptoms occurred in 90.0%. In 22 patients with acute ON (seen within 1 month of onset), presenting mean visual acuity (VA) was 20/258 (logMAR 1.11), mean deviation (MD) on Humphrey visual fields was -16.90 ± 10.83 dB, and peripapillary retinal nerve fiber layer (RNFL) thickness on ocular coherence tomography (OCT) was 164.23 ± 46.53 um. Orbital magnetic resonance imaging (MRI) within 1 month of symptom onset for 19 patients demonstrated orbital optic nerve enhancement in 11 (57.9%) and perineural enhancement in 11 (57.9%). Brain MRI was normal in 28 (93.3%) patients. Twenty out of 22 patients with acute presentation were treated with high-dose glucocorticoids and 5 with plasma exchange in addition to corticosteroids. Long-term immunosuppression was utilized in 9 (30%) out of all 30 patients. Final VA was 20/30 (logMAR 0.18), MD was -7.17 ± 8.85 dB, and RNFL thickness was 72.15 ± 20.16 um. MOG-IgG ON in Canada has a variable presentation with most patients having substantial initial vision loss with good recovery. This is the largest characterization of the disease in Canada to date.

En-Bloc Resection Versus Resection After Evacuation and Suction of the Content for Orbital Optic Nerve Glioma Causing Visual Loss and Disfiguring Proptosis.

To evaluate the surgical outcomes of two different techniques of resection for optic nerve gliomas confined to the intra-orbital segment. This prospective, comparable, clinical interventional case series was conducted at the orbital clinic of Assiut University Hospital, the referral centre of Upper Egypt in the period between 2006 and 2018. The study included 10 children with optic nerve gliomas confined to the intra-orbital part without intracanalicular or intracranial extension and causing severe visual loss and disfiguring proptosis. In all cases, lateral orbitotomy was performed to expose the mass. In 5 cases (group A), the glioma was resected en-bloc. In 5 cases (group B), a new technique of resection was introduced. The wall of the glioma was incised, the content was evacuated and suctioned and followed by resection under good visualization of the markedly reduced mass in size. In the 2 groups, no tumour regrowth was reported during the follow-up period of 3-12 years. In group A, the 5 cases developed postoperative third nerve damage with paralytic ptosis and one case had severe neurotrophic keratitis ended by dense corneal opacity. In group B, a healthy ipsilateral eye was preserved in all cases and no one case developed postoperative paralytic ptosis. Reduction of size of an intra-orbital optic nerve glioma by evacuation and suction of the content before resection is highly recommended. This makes the surgical field during resection more visible and minimizes the possibility of third or other nerves damage.

Bilateral Posterior Canal Benign Paroxysmal Positional Vertigo Co-existing with Cranial Nerves Schwannoma and Meniere Disease: Case Report

Benign Paroxysmal Positional Vertigo (BPPV) is characterized by intense positional provoked vertigo with a brief duration and is one of the most common types of peripheral vestibular problems. The most commonly affected semi-circular canal is the posterior canal with a greater prevalence seen in females between the ages of 71-80. BPPV has a 60% right ear predisposition with canalithiasis as the predominant type. Most cases are unilateral, although bilateral BPPV is not atypical when associated with falls with head trauma, ototoxicity or co-existing vestibulopathy. To our knowledge, reports on bilateral BPPV coexisting with bilateral progressive vestibulopathy are scant. We describe a case of bilateral posterior canal BPPV with definite Menieres Disease (MD), Vestibular Schwannoma (VS) secondary to a schwannoma arising from the Jugular Foramen (JF) with mass effect and an incidental schwannoma of the right orbital optic nerve. Underlying BPPV may mask other clinical presentations, making it challenging to diagnose co-existing vesitbulopathy, especially those of a progressive episodic nature. In making management decisions, this serves as a reminder for clinicians to have in-depth conceptual and procedural knowledge to recognise and focus on the insidious progressive pathologies and not be distracted by the easily treatable BPPV.

Normal measurements of the optic nerve, optic nerve sheath and optic chiasm in the adult population.

BackgroundImaging assessment of the anterior visual pathway structures, particularly the optic nerves (ON), requires knowledge of normal dimensions. Several studies suggesting techniques and normal ranges have been performed, but most suffer from various methodological flaws. This study is the first to be performed in a South African population.ObjectivesThe aim of this study was to establish normal measurements of the ON, optic nerve sheath (ONS) and optic chiasm (OC) on magnetic resonance imaging (MRI).MethodEighty normal adults between ages of 12 and 65 years were included in this prospective, quantitative, observational, descriptive study to establish normal measurement of the ON, ONS and OC using a T2W 3D MRI sequence. Measurements (width and height) were undertaken by two observers independently.ResultsA total of 80 participants with a mean age of 35 years were studied: 49 females (61.25%) and 31 males (38.75%). There were no statistical differences in the measurements between gender and age correlation. Interobserver agreement was best for larger structures, that is, OC width and intracranial ON width, respectively. The overall mean of OC width was 13.63 mm (range: 11.13 mm–16.92 mm, standard deviation [s.d.] 1.21); intraorbital ON height at 5 mm behind the globe 2.29 mm (range: 1.63 mm–3.33 mm, s.d. 0.43), and intracranial ON width 4.27 mm (range: 2.46 mm–5.19 mm, s.d. 0.53).ConclusionNormal measurements of the anterior visual pathway structures on MRI are best reflected in the larger structures. Interobserver variability was poor for the orbital ON, ONS, intracranial ON height and OC height. We recommend that measurements be obtained for the OC width and intracranial ON width. The overall mean for the OC width is 13.63 mm and intracranial ON width 4.27 mm.

Magnetic Resonance Imaging of Idiopathic Intracranial Hypertension: Before and After Treatment.

This study aimed to identify the reversibility of MRI findings indicative of increased intracranial hypertension in idiopathic intracranial hypertension (IIH) patients after treatment. This retrospective, observational study included demographic and clinical data from 10 patients with IIH and 10 controls. Brain MRI findings in IIH patients were recorded twice: once when patients had papilledema and again after resolution of papilledema. Neuroradiologists graded MRI findings in both groups based on an imaging grading scale. After resolution of papilledema, all patients showed improvement in 2 or more of the MRI characteristics of IIH. This was especially the case for the height of the midsagittal pituitary gland and optic nerve sheath thickness (ONST), which were significantly different in all pairwise group comparisons. Sellar configuration, globe configuration, and horizontal orbital optic nerve tortuosity were different between the IIH pre-treatment group and controls, but not between controls and the IIH post-treatment group. We found no difference in optic nerve head hyperintensity or optic nerve thickness among the 3 groups. We demonstrated that several morphometric MRI characteristics in IIH are reversible to a certain extent after treatment. Enlarged subarachnoid spaces filled with cerebrospinal fluid seem to remain reduced, and the ONST and height of the pituitary gland are not fully normalized after treatment.

Transnasal endoscopic resection of orbital cavernous hemangiomas: our experience with 23 cases.

The aim of this work was to present our single-center experience on orbital cavernous hemangiomas managed using an endonasal endoscopic approach. The study design used was one-institutional retrospective review. Twenty-three cases of orbital cavernous hemangioma were analyzed and followed up. There were 10 males and 13 females, with an average age of 46.1 years, included in our study. Seven cases were extraconal, 16 cases were intraconal, and in 10 of the patients the optic nerve was involved and compressed. Total resection of the tumor was achieved in 16 cases; of the remaining 7 cases, partial tumor resection and orbital decompression were conducted, orbital decompression was conducted in 2 cases, and orbital decompression and optic nerve decompression were conducted in 3 cases. Visual acuity in 9 patients improved significantly, as 16 of the 20 patients with a preoperative visual field defect had a smaller defect after surgery. This study adds to the literature showing that endoscopic transnasal surgery is likely a reasonable option for patients with the orbital cavernous hemangiomas medial of the optic nerve. With greater experience, skilled surgical technique, and careful operation, better clinical efficacy can be achieved.