Impact of Teprotumumab on Clinical Practice in Thyroid Eye Disease.

BACKGROUND: Following its FDA approval in January 2020, we examined the impact of teprotumumab on thyroid eye disease (TED) clinical practices. METHODS: Across three referral centers from January 1, 2018, to December 30, 2022, we retrospectively analyzed demographics, clinical features, treatment choices, and insurance status of patients with active, moderate to severe TED. RESULTS: Of 74 patients recommended for medical therapy, 53% received collaborative recommendations from endocrinologists and ophthalmologists in a TED clinic. Prior to teprotumumab availability, 19 patients were recommended medical therapy, and all received medical therapy (100%), which consists of corticosteroids (14, 73.7%) or tocilizumab (5, 26.3%). After teprotumumab became available, out of 55 patients that were recommended medical therapy, only 41 (74.6%) received medical therapy, mostly teprotumumab (33, 60%), followed by corticosteroids (5, 9.1%) or tocilizumab (3, 5.4%), while 14 (25.4%) did not receive medical therapy. Discordance between physicians' recommendations and therapy received or lack thereof was explained by patients' refusal (9, 64.3%), mostly due to side effect concerns (8, 88.9%), and insurance denial (5, 35.7%). Teprotumumab use was mostly associated with otic changes (10, 30.3%), weight loss (9, 27.3%), and hyperglycemia (6, 18.2%), but 2 (6.1%) patients developed serious infections. Corticosteroids were associated with insomnia (4, 21.1%), and one patient in the tocilizumab group had an infusion reaction requiring hospitalization. CONCLUSION: Teprotumumab introduction increased TED therapy evaluations, yet not all received recommended treatment due to safety concerns or accessibility issues. Enhancing collaborative care, medication accessibility, and adverse effect management is crucial.

Obstructive Sleep Apnea Associated With Increased Failure Rate of Ptosis Repair.

PURPOSE: This study investigates how Obstructive sleep apnea (OSA) affects the outcomes of ptosis repair. We hypothesized that patients with OSA have an increased rate of reoperation after ptosis repair. METHODS: This retrospective cohort study included patients age >18 from the Mayo Clinic who underwent ptosis repair by levator advancement or Müller muscle-conjunctiva resection between 2018 and 2021. Outcomes were measured at 1 to 3 months of follow-up with surgical failure defined as asymmetry or unsatisfactory eyelid height requiring revision surgery within 1 year. RESULTS: A total of 577 patients met the inclusion criteria. There was a statistically significant difference in surgical failure between patients with OSA and those without (20.5% vs. 13.1%, p = 0.02). Patients with OSA showed a statistically significant difference in risk of revision by a factor of 1.70 (95% CI: 1.06-2.07). Revisions were attributed to unsatisfactory eyelid height in 72.6% of patients and eyelid asymmetry in 21.1%. All patients who had revision surgery had satisfactory outcomes. On logistic regression analysis, when adjusting for age and sex, OSA was significantly associated with ptosis revision ( p = 0.007). CONCLUSIONS: OSA increases risk of surgical failure and need for revision surgery in patients undergoing blepharoptosis repair but is not a sole risk factor.

Mohs Micrographic Surgery With Immunohistochemistry for the Treatment of Periocular Melanoma In Situ.

PURPOSE: Mohs micrographic surgery with immunohistochemistry allows for same-day comprehensive margin assessment of melanoma in situ prior to subspecialty reconstruction. This study describes the oncologic and reconstructive outcomes of eyelid and periorbital melanoma in situ and identifies risk factors for complex reconstructive demands. METHODS: Retrospective case series of all patients treated with Mohs micrographic surgery with immunohistochemistry for melanoma in situ affecting the eyelids or periorbital region from 2008 to 2018 at a single institution. Tumors were assigned to the eyelid group if the clinically visible tumor involved the skin inside the orbital rim. Reconstructive variables were compared between the eyelid and periorbital cohorts. RESULTS: There were 24 eyelid and 141 periorbital tumors included. The initial surgical margin for all tumors was 5.34 ± 1.54 mm and multiple Mohs stages were required in 24.2% of cases. Eyelid tumors included more recurrences (p = 0.003), and the average defect size was larger (14.0 ± 13.3 cm2 vs. 7.7 ± 5.4 cm2, p = 0.03). Risk factors for complex reconstruction included: initial tumor diameter >2 cm (odds ratio [OR]: 3.84, 95% confidence interval [CI]: 1.95-7.57) and eyelid involved by initial tumor (OR: 4.88, 95% CI: 1.94-12.28). At an average follow-up of 4.8 years, there were no melanoma-related deaths and 1 local recurrence (0.6% recurrence rate). CONCLUSIONS: Mohs micrographic surgery with immunohistochemistry achieves excellent local control rates for periocular melanoma in situ. An initial surgical margin of 5 mm is frequently insufficient to achieve clear margins. The resulting defects are large, and the complexity of reconstruction can be predicted by tumor size and clinical involvement of eyelid skin.

Qualitative use of ICG angiography and lymphography in periorbital surgery.

PURPOSE: Open imaging fluorescence devices have been utilized in surgical oncology, vascular and plastic surgery; however, the role of indocyanine green (ICG) in periorbital surgery and lymphatics has not been explored. METHODS: A prospective, single-center diagnostic study was conducted from 2021 to 2022 utilizing ICG to assess both the periorbital vasculature and lymphatics. Fluorescence was captured with open-imaging fluorescent devices. For ICG angiography, a total of 5-10 mg of ICG was given intravenously at various time points to visualize intraoperative blood flow to eyelid flaps, vascular tumors, or extraocular muscles. For ICG lymphography, 0.03-0.06 mg of ICG was injected subcutaneously to visualize the periorbital and facial lymphatic drainage. RESULTS: Twenty-two patients underwent ICG angiography. Periorbital vascular supply was seen in eyelid reconstructions (n = 8), anophthalmic reconstructions (n = 2), lacrimal gland tumors (n = 2), orbital venous malformations (n = 2), tumor metastasis (n = 1) and benign tumors (n = 1). The anterior ciliary arteries were visualized to the extraocular muscles in fracture repairs (n = 3) and muscle biopsies (n = 2). Ten patients underwent ICG lymphangiography highlighting the global periorbital lymphatic system. CONCLUSION: ICG allows for visualization of the vasculature of extraocular muscles and tumors, assessing perfusion of flaps during reconstruction and the global periorbital lymphatic drainage pathways.

Sebaceous Carcinoma of the Eyelid: Proposed Nomenclature for Multifocal and Multicentric Disease.

PURPOSE: To review clinical presentations of periocular sebaceous carcinoma (SC) and introduce standardized nomenclature for multicentric and multifocal disease presentation. METHODS: A comprehensive PubMed/Medline search was conducted to identify all articles reporting periocular multicentric or multifocal SC presentations. The authors also highlight an additional case of SC presenting with 2 clinically distinct tumor foci and complete secondary invasion of the lacrimal gland. RESULTS: This review summarizes clinical presentations of periocular SC exhibiting discrete foci of microinvasion reported in the literature. Discrete microinvasion was associated with high rates of misdiagnosis (80%), simultaneous involvement of both upper and lower eyelids (80%), pagetoid spread (80%), multinodular growth (33%), local tumor spread (60%), previous eyelid manipulation (40%), and local recurrence (40%). Eyelid multifocality with clinically discrete nodules (42%) was associated with more advanced disease including orbital extension and regional invasion (80%). CONCLUSIONS: Despite previous reported associations with poorer outcomes, there is no consensus in the definition or nomenclature for discrete microinvasive or clinical disease presentations in periocular SC. The authors recommend defining multicentric disease as discrete foci of microinvasive tumor with basement membrane disruption and multifocal disease as discrete clinically evident nodules involving both the upper and lower eyelids. Differentiating between discrete microinvasive (multicentric) and clinically nodular (multifocal) disease may improve risk stratification to most accurately identify patients who require more aggressive management and surveillance.

Spheno-Orbital Meningioma - Treatment Outcomes and Factors Influencing Recurrence.

PURPOSE: To determine treatment outcomes, recurrence rates, and predictors of recurrence, to inform future therapeutic approaches for spheno-orbital meningiomas (SOM). METHODS: A retrospective single-center study of SOM treated from 1990 to 2021 was conducted with comprehensive neuro-ophthalmologic follow-up at Columbia University Medical Center (CUMC). Recurrence requiring reintervention was defined clinically as worsening of visual acuity, visual field defect, or ocular motility after an initial period of stabilization or 6 months of improvement following treatment, or radiologically as either a regrowth with an increase in tumor size by 20% at the site of previous growth or a new region of tumor growth. RESULTS: In total 46 patients met the inclusion criteria. The mean follow-up was 106 months (range 1-303). Dictated by the phenotype of the disease, patients underwent either gross- (50%), near- (17%), or subtotal resection (26%). Removal of the anterior clinoid process (ACP) was performed in 52% of patients. Nine patients (20%) required an enucleation or exenteration. Radiotherapy was employed at some point of treatment in 50% of cases. Inherited cases (24%) were referred to CUMC for treatment following 1 or more recurrences. The total recurrence rate, including inherited cases, was 54%, occurring at a mean interval of 43 months. The recurrence rate of patients treated solely at CUMC was 40%, occurring at a mean interval of 41 months. A subset of patients (32%) had 2 or more recurrences. Histopathology at the first surgery was WHO grade I (87%) and II (13%) and at the final surgery was WHO grade I (74%), II (21%), and III (4%). A subset of grade I tumors that received radiotherapy (35%) evolved to a higher grade or developed multiple recurrences without a change in histologic grade I. Grade II tumors and treatment with radiotherapy increased the odds of recurrence. Removal of the ACP and gross total resection decreased the odds of recurrence. CONCLUSION: Due to the routinely long interval to tumor recurrence, lifelong surveillance of patients with SOM is prudent. ACP resection and gross total resection, where possible, reduce tumor recurrence and the need for further treatment. Radiotherapy should be reserved for higher-grade meningiomas and select grade I tumors.

Alemtuzumab-Induced Thyroid Eye Disease: A Comprehensive Case Series and Review of the Literature.

PURPOSE: To present 5 cases of alemtuzumab-induced thyroid eye disease (AI-TED) and review the literature to highlight the natural history, severity, and outcomes as compared with conventional thyroid eye disease (TED). METHODS: A multi-institutional retrospective case series of patients with AI-TED was compiled. Chart review evaluated for clinical characteristics, imaging findings, and treatment for AI-TED. Additionally, a comprehensive review of the literature identified all previously published cases of AI-TED. RESULTS: Five new patients with AI-TED were included in this series. The average clinical activity score on presentation was 2.8 (range 1-4) and reached an average peak of 5.0 during the active phase of the disease (4-7). Patients were treated medically with selenium (40%) or monoclonal antibodies including teprotumumab or tocilizumab (40%). Surgical treatment with orbital decompression for compressive optic neuropathy was performed on 2 (40%) patients. Combined with 11 previously reported cases, these 16 patients with AI-TED had an average clinical activity score on presentation of 3.3. The average length of the AI-TED phase was 14.0 months, and all patients were treated with medical and/or surgical interventions for their disease. CONCLUSIONS: Clinical and imaging findings in AI-TED mirror that of conventional TED, however, AI-TED may present with greater severity. AI-TED may develop many months after Graves' disease; therefore, providers should be aware of this association and monitor patients for the development of severe TED.

Predictors of Surgical Versus Medical Management of Eyes in the Setting of Orbital Fracture at an Academic Level I Trauma Center.

We present clinical and imaging predictors of ocular injuries that required medical management versus surgical intervention in cases of orbital fractures. From 2014 to 2020, a retrospective review of patients with orbital fractures who received ophthalmologic consultation and computed scan (CT) analysis at a level I trauma center was performed. Inclusion criteria were patients with confirmed orbital fracture on CT and ophthalmology consultation. Patient demographics, associated injuries, comorbidities, management, and outcomes were collected. Two hundred and one patients and 224 eyes (11.4% bilateral orbital fractures) were included. Overall, 21.9% of orbital fractures presented with a significant concomitant ocular injury. Associated facial fractures were present in 68.8% of eyes. Management included surgical treatment in 33.5% of eyes and ophthalmology-directed medical treatment in 17.4%. On multivariate analysis, clinical predictors of surgical intervention were retinal hemorrhage (OR=4.7 (1.0-21.0), P =0.0437), motor vehicle accident injury (OR=2.7 (1.4-5.1), P =0.0030) and diplopia (OR=2.8 (1.5-5.3), P =0.0011). Imaging predictors of surgical intervention were herniation of orbital contents (OR=2.1 (1.1-4.0), P =0.0281) and multiple wall fractures (OR=1.9 (1.01-3.6), P =0.0450). Predictors of medical management were corneal abrasion (OR=7.7 (1.9-31.4), P =0.0041), periorbital laceration (OR=5.7 (2.1-15.6), P =0.0006), and traumatic iritis (OR=4.7 (1.1-20.3), P =0.0444). We demonstrated a 22% incidence of concomitant ocular trauma in orbital fracture patients at our level I trauma center. Predictors of the surgical intervention included multiple wall fractures, herniation of orbital contents, retinal hemorrhage, diplopia, and motor vehicle accident injury. These findings emphasize the importance of a multidisciplinary team in managing ocular and facial trauma.

Ectopic orbital brain tissue: a case report with radiographic and clinical review.

Orbital heterotopic brain tissue is a rare entity with heterogenous clinical features requiring a multi-faceted diagnostic approach. The authors present a case of ectopic orbital brain tissue in an infant with a comprehensive literature review to highlight the radiographic findings of these lesions. Imaging findings are variable but describe well-circumscribed homogenous lesions with variable enhancement, without communication intracranially. The combination of computed tomography and magnetic resonance imaging can identify associated bony abnormalities, lesion-specific features, and effects on surrounding structures, which in combination with the clinical exam can be a valuable diagnostic and surveillance tool. Although ectopic orbital brain tumors are benign with excellent outcomes following complete resection, conservative management with observation and serial imaging may be an alternative method of management in patients with mild, non-vision threatening, non-distorting tumors.

Evolution of asymmetric proptosis during the active phase of thyroid eye disease.

PURPOSE: To assess the evolution of proptosis asymmetry during the active phase of bilateral thyroid eye disease (TED). METHODS: A retrospective study was conducted on patients with bilateral, active TED. Patients were measured by a single observer, using Hertel exophthalmometry from the time of initial presentation, during the active phase of TED, to the stable phase, 24-months later. Asymmetric proptosis was defined as a >2 mm intra-orbital difference in Hertel measurements. RESULTS: Fifty-one patients were enrolled. Patients presented at a mean time of 1.1 ± 2.9 months following the onset of TED symptoms. Stability of TED was established at 15.7 ± 12.3 months. At initial presentation, 41% of patients demonstrated asymmetric proptosis. Upon reaching the stable phase, asymmetric proptosis persisted in only 22% of patients. A decline in the rate asymmetric proptosis was greatest within the first 3 months of the active phase. CONCLUSIONS: Asymmetric proptosis is common in the setting of early active TED and decreases by 50% when the stable phase is reached. Therefore, diagnostic imaging is not routinely required to exclude alternative pathology in the cases of asymmetric TED. Perhaps more importantly, this finding supports the surgical paradigm of stable phase, graded orbital decompression, performed when the ultimate globe positions are achieved to avoid late postoperative asymmetry, resulting from the unanticipated evolution of proptosis when surgery is performed during the active phase of TED.

Genetic Alterations in Melanocytoma Associated with Oculodermal Melanocytosis: Molecular Characteristics.

Melanocytoma is a benign tumor with histologic similarity to oculodermal melanocytosis, which can undergo malignant transformation in rare cases. Melanocytoma more commonly involves the optic disc, and few cases of orbital melanocytoma have been reported. Primary orbital melanoma is a rare malignancy known to arise from pigmentary conditions, although there is little information on this tumor arising from melanocytoma. The authors present a case of malignant transformation of orbital melanocytoma in the setting of ipsilateral oculodermal melanocytosis. This case illustrates histopathologic features associated with malignant transformation and highlights the significance of GNAQ, BAP1, and specific intrachromosomal alterations occurring in oculodermal melanocytosis and melanocytoma. The molecular markers observed in this case are of interest as they have overlap with those present in uveal melanoma. This case demonstrates a timeline of genetic and molecular alterations occurring in the malignant transformation of orbital melanocytoma.

The Arched Rainbow Brow in Thyroid Eye Disease.

PURPOSE: To morphologically describe and mathematically quantify a novel clinical feature of thyroid eye disease (TED). METHODS: A retrospective study was conducted of TED patients and age-sex-matched normal controls. The arched Rainbow Brow appearance in TED patients was determined by unanimous agreement of 3 oculoplastic surgeons. Eyebrow curvature was assessed by plotting 15 points along the eyebrow in ImageJ. The fourth-degree polynomial ( y = ax4 + bx3 + cx2 + dx + e ) was fitted to each eyebrow. RESULTS: Two hundred seventy-one eyes were analyzed (200 TED and 71 age-sex-matched normal controls). A Rainbow Brow was identified in 42% of TED patients. A unilateral Rainbow Brow was seen in 15% of patients. The fourth-degree polynomial coefficients yielded significant differences between Rainbow Brow patients and age-sex-matched normal controls for the coefficients a, b, c , and d . Similar analysis of TED patients with and without a Rainbow Brow showed differences in coefficients a and b . Age >50 years ( p = 0.009) and the presence of brow fat expansion ( p < 0.001) were associated with the presence of a Rainbow Brow. Proptosis >24 mm showed a trend toward association with the presence of a Rainbow Brow ( p = 0.057). When considering the contribution of these features in a multivariable analysis, only brow fat expansion was a significant contributing factor ( p = 0.009). CONCLUSIONS: The Rainbow Brow is a distinct entity in TED and is likely consequent to brow fat pad expansion. Patients with a Rainbow Brow have different eyebrow curvature as compared to both normal age-sex-matched controls and TED patients without a Rainbow Brow.

Mathematical Modeling of Eyebrow Curvature.

The aim of the study is to describe a mathematical model for analyzing eyebrow curvature that can be applied broadly to curvilinear facial features. A total of 100 digital images (50 men, 50 women) were obtained from standardized headshots of medical professionals. Images were analyzed in ImageJ by plotting either 8 or 15 points along the inferior-most row of contiguous brow cilia. A best-fit curve was automatically fit to these points in Microsoft Excel. The second derivative of the second-degree polynomial and a fourth-degree polynomial were used to evaluate brow curvature. Both techniques were subsequently compared with each other. A second-degree polynomial and fourth-degree polynomial were fit to all eyebrows. Plotting 15 points yielded greater goodness-of-fit than plotting 8 points along the inferior brow and allowed for more sensitive measurement of curvature across all images. A fourth-degree polynomial function provided a closer fit to the eyebrow than a second-degree polynomial function. This method provides a simple and reliable tool for quantitative analysis of eyebrow curvature from images. Fifteen-point plots and a fourth-degree polynomial curve provide a greater goodness-of-fit. The authors believe the described technique can be applied to other curvilinear facial features and will facilitate the analysis of standardized images.

Utilizing a mobile platform to obtain quantitative measurements of exophthalmos.

PURPOSE: This study introduces a novel exophthalmometry method utilizing a mobile platform to obtain calibrated quantitative measurements of globe position and compares its reliability to Hertel exophthalmometry. METHODS: A prospective, comparative study included 50 patients (99 eyes) for a total of 594 mobile measurements. Healthy individuals from the community in Augusta, Georgia, and individuals from a routine oculoplastics clinic at Mayo Clinic in Rochester, Minnesota, were included to represent variety within the population. The main outcome was the inter-modality correlation and agreement between Hertel and mobile exophthalmometry, and the intra-observer and inter-observer reliability with repeated mobile measurements. RESULTS: There was no significant difference in the measurements obtained by Hertel exophthalmometry and mobile exophthalmometry. There was a strong linear correlation between Hertel and mobile exophthalmometry with a Pearson Correlation Coefficient of 0.910 and 0.888 for the right and left eyes, respectively (p < .001, 2-tailed). Bland-Altman plot analysis showed excellent agreement between the two modalities. The mobile platform demonstrated high intra-observer reliability with a Cronbach's alpha of 0.992 and 0.985 for the right and left eyes. An intraclass correlation coefficient of 0.992 (95% CI: 0.987-0.995) for the right eye and 0.986 (95% CI: 0.978-0.991) for the left eye demonstrated excellent reliability between observers. CONCLUSIONS: Mobile exophthalmometry may be a promising tool for obtaining calibrated quantitative measurements of globe position for situations in which Hertel exophthalmometry is not available. The strong correlation and excellent agreement between Hertel and mobile measurement suggest that mobile exophthalmometry can yield reliable and accurate measurements.

Atypical presentation of invasive myoepithelioma in a pediatric patient.

A 15-year-old girl presented with a mobile lesion with yellowish hue on the posterior lamella of the right lower eyelid adjacent to the punctum. Four years prior, a lesion thought to be a chalazion was excised from the same location. There was subsequent progressive painless enlargement. The patient ultimately underwent an internal excision of the mass. Histopathology demonstrated infiltrative nests and cords of epithelioid and plasmacytoid cells with abundant eosinophilic myxoid-collagenous stroma along with focal infiltration of skeletal muscle with immunohistochemical staining consistent with an invasive myoepithelioma of mixed-cell type. Given concern for invasive disease, the patient underwent subsequent Mohs resection resulting in a 25% full thickness eyelid defect, which was repaired with direct closure of the wedge defect. There has been no recurrence of the disease for 7 months since the Mohs resection. This case illustrates the atypical presentation of an invasive myoepithelioma of the eyelid in a pediatric patient.

Natural killer T-cell lymphoma causing bilateral recurrent recalcitrant dacryocystitis.

A 35-year-old female with a history of chronic extensive rhinosinusitis, previously treated twice with functional endoscopic sinus surgery, presented with recurrent dacryocystitis despite prior dacryocystorhinostomy. Histopathological specimens taken at the most recent sinus surgery demonstrated a lymphocytic inflammatory reaction without evidence of angiodestruction or necrosis. Flow cytometry was normal. Over the following 9 months, the patient developed worsening hypertelorism and bilateral recurrent acute dacryocystitis with a fistula tract to the skin. Neuroimaging revealed a hyperintense enhancing soft tissue expansion into the periorbital regions, invading the nasolacrimal canals, and obstructing the paranasal sinuses. A skin biopsy at the fistula site revealed natural killer T-cell lymphoma. Metastatic work-up disclosed lung, spleen, and bone marrow involvement. The patient underwent chemotherapy with mixed clinical response, and ultimately passed away from metastatic disease. The authors present a rare case of natural killer T-cell lymphoma involving the nasolacrimal sac, presenting as recurrent dacryocystitis and diagnosed by skin biopsy of the fistula site.

Clinical characteristics and outcomes of ocular adnexal mantle cell lymphoma.

PURPOSE: To compare characteristics of initial ocular adnexal (OA) mantle cell lymphoma (MCL) and initial systemic MCL. METHODS: Retrospective, comparative case series. Patients treated for MCL at Mayo Clinic from 1/1/1990 to 11/30/2020. MCL was classified as initial OA if first site was OA or initial systemic if first site was elsewhere with progression or recurrence to the OA region. OUTCOME MEASURES: Features, treatment, and survival. RESULTS: There were 50 patients with MCL, 23 initial OA and 27 initial systemic. Patients with initial OA MCL had more conjunctival (52% vs. 19%, p = .017) involvement and less frequently received chemotherapy plus autologous stem cell transplant (ASCT) (9% vs. 33%, p = .046) as initial treatment. Complete remission was achieved in 41 (91% vs. 74%, p = .152) patients. Five-year disease-specific survival was similar in initial OA and initial systemic MCL (92% vs. 83%, p = .187). Subanalysis of patients with initial OA MCL revealed 9 (39%) patients developed tumor recurrence, with mean time to recurrence of 28 months. Comparison (no recurrence vs. recurrence) of initial OA MCL patients revealed those with no recurrence had shorter mean final follow-up (3.3 vs. 9.8 years, p = .005) and more frequent initial treatment with rituximab-based chemotherapy plus ASCT (43% vs. 0%, p = .048). Recurrence had no effect on the 5-year age-adjusted risk of death from lymphoma (HR 2.17, 95% CI 0.55-9.09, p = .266). CONCLUSIONS: Initial OA and initial systemic MCL patients differ in presentation and management but have similar survival.

An elusive case of malignant optic nerve glioma with a suprasellar mass.

A 67-year-old man with multiple comorbidities presented with 3 weeks of orbital pain, proptosis, and no light perception vision in the left eye. Examination was notable for a left orbital apex syndrome with CN III, VI palsies, an optic neuropathy, and central retinal vein occlusion. Magnetic resonance imaging of the orbits was notable for extensive enhancement, enlargement, and T2 hyperintensity of the optic nerve, with perineural sheath enhancement, and chiasmal hyperintensity. Inflammatory workup and lumbar puncture were unremarkable. No improvement was seen after a 3-day course of intravenous solumedrol. Initial optic nerve biopsy revealed necrotic nerve tissue, macrophage infiltration, increased vascularization, and peripheral gliosis. The volume of tissue was inadequate for genomic analysis. The patient was lost to follow-up but returned 5 months later with right-sided vision loss. Repeat neuroimaging showed a new suprasellar mass and progressive expansion and enhancement of both optic nerves. Biopsies of the suprasellar mass and left nerve at this time were consistent with a high-grade glial neoplasm, WHO grade IV. This is a rare case of glioblastoma involving the optic nerves and suprasellar region. In such cases, molecular profiling can improve diagnosis and may provide for targeted treatments in the future.

Partial Vision Loss After Orbital Decompression in a Patient With Thyroid Eye Disease, Chorioretinal Folds, and Disc Edema.

ABSTRACT: Concomitant chorioretinal folds with disc edema can be seen in cases of thyroid eye disease presenting with compressive optic neuropathy and may portend optic nerve ischemia. We describe an unusual case of a 64-year-old man who developed partial vision loss after orbital decompression.

Atypical Cogan Syndrome Featuring Orbital Myositis and Dacryoadenitis.

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.