Orbital and Optic Nerve Sheath Involvement and Pupil-Involving Cranial Nerve 3 Palsy in Giant Cell Arteritis.

Giant cell arteritis (GCA) can present with atypical presentations that are diagnostically challenging. An 84-year-old woman with a history of breast adenocarcinoma treated with lumpectomy and radiation 2 years prior presented with a seizure-like episode.

Quantitative Assessment of Subjective Symptoms and Corneal Sensitivity in Chronic Orbital Pain Patients.

PURPOSE: To objectively evaluate the subjective symptoms and characteristics of chronic orbital pain as well as to quantify sensitization of peripheral trigeminal nerves. METHODS: In this prospective cohort study, patients who previously showed a response to peripheral trigeminal nerve blocks for unilateral, idiopathic chronic orbital pain and healthy subjects completed validated questionnaires assessing headaches, neuropathic signs and symptoms, photophobia, and pain qualities. Corneal sensitivity was measured in both eyes for all subjects with a Cochet-Bonnet aesthesiometer. For pain patients, the full assessment protocol was repeated 2-4 weeks after the study injection, and corneal sensitivity was also measured 30 minutes postinjection. Outcomes assessed were headache, neuropathic pain, and photophobia scores; pain qualities; and corneal sensitivity. RESULTS: Six female chronic orbital pain patients (mean age 48.2 years) and 11 female controls (mean age 47.5) were included. The mean headache, neuropathic pain, and photophobia questionnaire scores were significantly higher for pain patients than for controls (p < 0.001). On sensory testing, 5 pain patients (83.3%) endorsed allodynia, and all 6 (100%) had hyperalgesia in the ipsilateral frontal nerve dermatome. No controls had allodynia or hyperalgesia. Corneal sensitivity was similar between eyes in pain patients and between groups. Questionnaire scores and corneal sensitivity did not change significantly after the injection. CONCLUSIONS: Chronic orbital pain patients have a measurable reduction in quality of life due to headaches and photophobia. The supraorbital and supratrochlear nerves are sensitized, resulting in cutaneous hypersensitivity in the corresponding dermatome, but corneal nerves have normal sensitivity.

Elevated Intraocular Pressure in Periorbital Sweet's Syndrome.

A 66-year-old immunocompromised man presented with cellulitis around the left eye that was initially concerning for necrotizing fasciitis. Exam findings were remarkable for exquisite periocular tenderness with rigid, immobile eyelids resulting from severe erythema, edema, and induration. Given the concern for orbital compartment syndrome and a necrotizing infection, the patient was taken urgently to the operating room for debridement of the eyelid skin as well as an urgent lateral canthotomy and cantholysis. His eye exam revealed 360° of hemorrhagic chemosis, no relative afferent pupillary defect, and an ipsilateral elevated intraocular pressure of 35 mm Hg. No visual acuity measurement could be obtained secondary to the patient's altered mental status. His intraocular pressure normalized after treatment with antihypertensive drops and further extension of the canthotomy. Histopathological analysis showed extensive neutrophilic infiltrate of the dermis which was compatible with a diagnosis of Sweet's syndrome.

Trends in horizontal periocular asymmetry.

OBJECTIVE: To determine whether there is laterality predominance in the horizontal dimensions of the periocular region. DESIGN: Retrospective study. PARTICIPANTS: Patients >18 years of age who presented to a single academic ophthalmology department. Exclusion criteria included history of facial trauma or surgery, aesthetic injections, or other periocular-altering processes. METHODS: Standardized digital photographs were obtained, and periocular structures were measured with Image J software. The midline was defined as the midpoint between the medial canthi, and the distances measured include midline to medial canthus, pupil centre, lateral canthus, and lateral zygoma. The palpebral fissure width was calculated as the distance between the lateral canthus and medial canthus. Data analysis was done for the full cohort and subsequently according to patient-identified gender. RESULTS: Periocular structures were measured in 83 patients (50 female and 33 male) with a mean age of 57.0 ± 16.2 years (range, 22-84 years). Right-sided predominance was found to be increasingly significant for the following variables: midline to pupil centre (31.34 mm vs 31.08 mm, p < 0.01), midline to lateral canthus (42.57 mm vs 42.23 mm, p < 0.005), and midline to lateral zygoma (65.70 mm vs 64.01 mm, p < 0.001). CONCLUSIONS: Photographic analysis of adults with no periocular-altering history demonstrates that there is a right-sided predominance in the horizontal dimension of the midline to the pupil, lateral canthus, and zygoma with increasing significance. Asymmetry of horizontal periocular measurements was more prevalent in males.

Misdiagnosis of fungal infections of the orbit.

OBJECTIVE: To evaluate the accuracy of the initial diagnosis in the case of fungal infections of the orbit and identify factors that may influence patient outcomes. METHODS: An institutional review board-approved retrospective chart review was conducted across 2 large academic centres to identify cases of fungal infections involving the orbit from January 1, 1998, to November 15, 2019. Data collected included patient demographics, past medical history, examination findings, diagnosis, treatment, imaging, and outcomes. RESULTS: Fifty cases of fungal infection involving the orbit were identified. Of these, 33 (66.0%) were initially misdiagnosed as nonfungal diagnoses. Sixteen patients (32.0%) received multiple initial diagnoses. The most common diagnoses on presentation were bacterial cellulitis (n = 12 of 50; 24.0%) and bacterial sinusitis (n = 12 of 50; 24.0%). These were followed by vascular and orbital inflammatory conditions (n = 9 of 50; 18.0%): 5 patients (10.0%) were clinically diagnosed with giant cell arteritis, 3 (6.0%) with nonspecific orbital inflammation, and 1 (2.0%) with optic neuritis. In this subset of patients, 77.8% (n = 7 of 9) were treated initially with systemic steroids. Additional initial diagnoses included neoplastic mass lesions, mucocele, dacryocystitis, cavernous sinus thrombosis, hemorrhage, tick-borne illness, allergic rhinitis, and allergic conjunctivitis. Misdiagnosis was significantly correlated with involvement of the masticator space on imaging (p = 0.04). CONCLUSION: Fungal infections of the orbit are misdiagnosed in 2 of 3 cases. Nearly 15% of patients who are later diagnosed with fungal disease of the orbit were initially treated with systemic steroids. Misdiagnosis is more frequent when the masticator space is involved.

Update on thyroid eye disease: Regional variations in prevalence, diagnosis, and management.

Thyroid eye disease (TED) is a rare disease that can lead to decreased quality of life, permanent disfigurement, and vision loss. Clinically, TED presents with exophthalmos, periorbital edema, extraocular muscle dysfunction, and eyelid retraction, and can lead to vision-threatening complications such as exposure to keratopathy and dysthyroid optic neuropathy (DON). Over the last several years, significant advancements have been made in the understanding of its pathophysiology as well as optimal management. Ethnic variations in the prevalence, clinical presentation, and risk of vision-threatening complications of TED are summarized, and risk factors associated with TED are discussed. Additionally, significant advances have been made in the management of TED. The management of TED traditionally included anti-inflammatory medications, orbital radiation therapy, orbital surgical decompression, and biologic therapies. Most recently, targeted therapies such as teprotumumab, an insulin-like growth factor-1 receptor antagonist, have been studied in the context of TED, with promising initial data. In this review, updates in the understanding and management of TED are presented with a focus on the international variations in presentation and management.

The Effect of Teprotumumab on Eyelid Position in Patients with Thyroid Eye Disease.

Teprotumumab has been shown to improve proptosis and clinical activity scores (CAS) in patients with thyroid eye disease, but little has been published regarding its effects on eyelid retraction. The purpose of this work was to evaluate changes in eyelid position in thyroid eye disease patients after teprotumumab. Eight patients completed eight cycles of teprotumumab. Data collected included exophthalmometry; clinical activity scores; margin reflex distance (MRD) 1; MRD2; and pre-, during, and posttreatment photographs. ImageJ analysis was also used to evaluate eyelid position in photographs. Proptosis significantly improved in 15 of 16 orbits [mean 4.75 ± 2.07 mm reduction (P = 0.0001) in study orbits and mean 3.00 ± 2.14 mm reduction (P = 0.0048) in nonstudy orbits]. CAS was significantly reduced (pretreatment mean 4.88 mm and posttreatment mean 1.88 mm, P = 0.006). MRD1 decreased in 11 of 16 orbits and increased in five orbits (P = 0.18 in study orbits and P = 0.22 in nonstudy orbits). MRD2 decreased in six of 16 orbits and increased in eight orbits (P = 0.49 in study orbits and P = 0.43 in nonstudy orbits). Patients exhibited variable changes in eyelid position with teprotumumab. There was a statistically insignificant decrease in MRD1 after teprotumumab. Proptosis reduction led to unpredictable changes in MRD1 and MRD2. Severity of eyelid retraction did not correlate with clinical activity score response to teprotumumab. There are inherent difficulties in evaluating eyelid position in thyroid eye disease, which may necessitate a paradigm shift in how patients are examined, measured, and photographed.

Peripheral Trigeminal Nerve Blocks for Chronic Orbital Pain: Clinical Features and Outcomes.

PURPOSE: To characterize chronic orbital pain in patients who benefitted from peripheral trigeminal nerve blocks and to explore the relationship between pain etiologies and phenotypes, injection attributes, and positive response to treatment. METHODS: In this single-center retrospective descriptive study, patients who underwent peripheral trigeminal nerve blocks for chronic orbital pain from November 2016 to May 2021 were selected. Data reviewed included inciting factors, neuropathic symptoms of orbital pain, injection composition (anesthetic alone versus anesthetic + dexamethasone), and corneal epitheliopathy grades. Primary outcomes assessed were response to injection, duration of injection effectiveness, and overall treatment efficacy. Associations between subgroups of chronic orbital pain, injection attributes, and treatment outcomes were examined. RESULTS: Nineteen patients who underwent a total of 94 peripheral trigeminal nerve blocks for chronic orbital pain were included. During a mean follow-up period of 2.4 years after initial injection (range 7 days-4.6 years), 16 (84.2%) patients achieved either partial or complete improvement. Ocular versus nonocular origin of orbital pain or the presence of neuropathic sensory characteristics was not associated with a treatment outcome. Injections containing dexamethasone had a lower positive efficacy (relative risk, 0.88; 95% CI, 0.81-0.97) and no statistically significant association with prolonged effect. Twenty-nine (50.9%) of the 57 injections for which effect duration was recorded produced a response lasting greater than 6 weeks. CONCLUSIONS: Modulation of trigeminal afferent nerve activity with peripheral trigeminal nerve blocks containing anesthetic with or without dexamethasone may be a promising treatment strategy for chronic orbital pain of diverse etiologies and phenotypes.

Echographic Assessment of Extraocular Muscle Response to Teprotumumab.

PURPOSE: To evaluate extraocular muscle response to teprotumumab using orbital echography in thyroid eye disease. METHODS: This retrospective study included adult thyroid eye disease patients with pre- and post-teprotumumab orbital echography. Data collected included: age, Hertel measurements, clinical activity score, Gorman diplopia scores, ocular motility, and recti muscle diameters measured by echography. The patient's more proptotic eye before treatment initiation was designated as the study orbit. Ocular motility was assessed by totaling the ductions in all 4 cardinal directions. Orbital echography was obtained pre- and post-treatment to assess response of extraocular muscle diameters. RESULTS: Six patients with a mean age of 67 years were included. There was a mean improvement in proptosis of 4.3 mm in the study eye with 11/12 orbits showing improvement in globe position ( p < 0.05). All patients had a decrease in clinical activity score with a mean reduction of 2.5. Four patients had an improvement in Gorman diplopia score. Ocular motility in the study orbits improved by a total mean of 26.9° ( p < 0.05). Mean total extraocular muscle diameter was reduced from 27.4 to 23.4 mm ( p < 0.001). On average, superior recti were largest pre- and post-treatment, followed by inferior, medial, then lateral recti. However, inferior recti showed the greatest reduction of 23% ( p < 0.02). CONCLUSIONS: Orbital echography demonstrated extraocular muscle reduction in all patients after teprotumumab, correlating with improved clinical activity score, ocular motility, and proptosis. Orbital echography is a safe and cost-effective imaging alternative to monitor therapeutic response to teprotumumab.

The Effect of Sleep Position Preference on Eyelid and Eyebrow Symmetry.

PURPOSE: To investigate the relationship between sleep position preference and eyebrow and eyelid position and degree of upper eyelid dermatochalasis. METHODS: A prospective study evaluating the impact of sleep position on facial asymmetry was conducted at an academic ophthalmology department. Eligibility criteria included the absence of periocular-altering trauma or surgery, contact lens use, or other periorbital disease processes. Patients reported their sleep position preference on a questionnaire. Standardized digital photographs of patients were obtained, and Image J software was used for measurements and converted into millimeters based on a standard corneal limbus-to-limbus ratio. Upper and lower eyelid position, upper eyelid dermatochalasis, and eyebrow position were assessed by the following image-derived measurements: marginal reflex distance 1 (iMRD1), marginal reflex distance 2 (iMRD2), tarsal platform show (iTPS), and central brow position (iBP). These results were compared with the patient reported sleep position preference to determine correlation. RESULTS: Seventy-one patients were enrolled and reported the following sleep position preferences: 28 (right), 24 (left), 13 (both), and 6 (supine). Patients with a right- or left-sided preference demonstrated lower iMRD1 measurements for the preferred sleep side (p < 0.0004) with no other significant difference in periorbital measurements. A larger degree of upper eyelid height (iMRD1) asymmetry was observed among patients with a sleep side preference. CONCLUSION: Patients with a predominant sleep side preference demonstrate a significant increase in ipsilateral upper eyelid asymmetry and an inferior upper eyelid position on the sleep side. There were no differences noted in lower eyelid position, central eyebrow position, or amount of upper eyelid dermatochalasis.

Scleral Contact Lens as Initial Management in a Neonate With a Large Upper Eyelid Coloboma.

A neonate presented with a large full-thickness upper eyelid coloboma with near-complete exposure of the cornea. After failing lubrication and a moisture chamber, he was fit with a customized scleral contact lens that protected the ocular surface. It was tolerated well, and ocular surface health was maintained for 13 months to permit the growth of eyelid tissue for future oculoplastic surgery. Delaying reconstruction by using scleral contact lenses as management for large eyelid colobomas has not been previously described. A temporizing measure such as this could be considered for large eyelid colobomas in neonates.

An Eye on Gender Equality: A Review of the Evolving Role and Representation of Women in Ophthalmology.

PURPOSE: In recent decades, women have achieved greater representation in ophthalmology. Globally, women now constitute approximately 25%-30% of ophthalmologists and 35%-45% of trainees. Nevertheless, women remain under-represented in key areas, including positions of professional and academic leadership and ophthalmic surgical subspecialization. Furthermore, there is evidence that women in ophthalmology encounter more bias and discrimination across multiple domains than men, including a gender-pay gap that is wider than in many other surgical subspecialties. Women ophthalmologists and trainees report sharply differing training experiences from male peers, including fewer opportunities to operate, more bullying and harassment, less access to mentorship, and contrasting expectations around contributions to family life. DESIGN: Perspective. METHODS: An extensive literature search was undertaken to compile and review papers published with a focus on gender equity across ophthalmology, surgery, and medicine. RESULTS: We identified 8 broad domains that were widely discussed: leadership, research and academics, income, surgical exposure and subspecialization, harassment, career satisfaction, mentorship, and family and marital differences. We have summarized the current research across each of these areas, and discussed possible solutions to reduce the inequities reported. CONCLUSIONS: This review draws on current research published around representation and experiences of women in ophthalmology and suggests that there are opportunities to improve gender inequity.

Association of Age-adjusted Charlson Comorbidity Index With Orbital Fungal Disease Outcomes.

PURPOSE: To determine whether the age-adjusted Charlson comorbidity index (age-CCI) in sino-orbital fungal disease patients correlates with disease-specific mortality. METHODS: Hospital billing systems at 2 academic institutions were queried for patients with ICD-9, ICD-10, and CPT codes used in fungal disease who also had orbital disease and significant visual loss. Thirty-two patients at Institution A and 18 patients at Institution B met the inclusion criteria of microbiologic or pathologic confirmation of fungal infection and completion of inpatient ophthalmology evaluation. Patients without radiographic abnormality in the sinus or orbit were excluded. Demographic, diagnostic, treatment, and outcome variables were recorded. Our primary outcome was death due to fungal disease. RESULTS: Of the 50 medical records examined, 44 patients met the criteria for fungal-related death outcome on multivariate analysis. The regression coefficient for age-CCI and fungal-related mortality was 0.242 (95% CI, 0.012-0.779) with a p value of 0.038. CONCLUSIONS: Age-CCI is significantly associated with fungal-related mortality. This relationship remains significant when controlling for 5 covariates of fungal organism phylum, presence or absence of CNS disease, exenteration, local treatment use, and presence or absence of an immunosuppressive diagnosis. Age-CCI shows promise as a clinical and research tool in the evaluation of invasive fungal disease involving the orbit.

A direct transcutaneous approach to infraorbital nerve biopsy.

PURPOSE: To describe a novel transcutaneous infraorbital nerve biopsy technique which can be performed to aid in the diagnosis of perineural invasion (PNI) of facial cutaneous squamous cell carcinoma (SCC). METHODS: A single-center retrospective chart review was performed. Patients diagnosed with SCC with PNI via an infraorbital nerve biopsy between February 2019 and February 2020 were included. Data collected consisted of patient demographics, medical history, clinical presentation and exam, histologic and radiographic findings, treatment, and outcomes. RESULTS: Four patients (3 male, 1 female) met inclusion criteria. The mean age at diagnosis was 79.5 years (range 66-85 years). Three of the four patients had a history of facial skin lesions, including actinic keratosis and SCC, involving the nose, cheek, or ear. One patient had no history of cutaneous malignancy. All patients presented with cranial neuropathies, including total V2 hypoesthesia. The most common presenting symptom was facial pain, followed by diplopia, unilateral facial weakness, and hypoesthesia in the V1 and/or V2 distribution. Transcutaneous infraorbital nerve biopsy in all patients revealed squamous cell carcinoma with no biopsy complications. CONCLUSION: Definitive diagnosis of PNI can be challenging but is important to minimize tumor-related morbidity. Infraorbital nerve biopsy can establish this diagnosis, especially in the context of negative or indeterminate imaging findings. This work comprises the first description of a transcutaneous approach to infraorbital nerve biopsy, which is a minimally invasive technique that can be performed in an outpatient procedure suite with limited to no sedation.

Aesthetic Eyelid Measurements of "Beautiful People": Gender Differences and Application for Thyroid Eye Disease Patients.

There remains a paucity of studies investigating measurements of periocular structures of people popularly seen as "beautiful." Such measurements may be helpful in establishing postoperative goals and measuring aesthetic outcomes. This study (1) identifies aesthetic measurements of the periocular structures in idealized celebrities, (2) determines gender differences in such measurements, and (3) compares these measurements to patients who underwent surgical repair of upper eyelid retraction associated with thyroid eye disease. METHODS: Digital analysis of 38 celebrity photographs in People's "Most Beautiful People" and "Sexiest Man Alive" was performed to measure image-derived (denoted with an "i") margin reflex distance (iMRD1), tarsal platform show (iTPS), brow fat span (iBFS), and iTPS:iBFS ratio. The same analysis was used for 35 women who underwent surgical repair for thyroid eye disease-related upper eyelid retraction. RESULTS: Significant gender differences (P < 0.05) were observed in celebrity metrics, with women having higher upper eyelids (longer iMRD1) (3.30 mm versus 2.50 mm), longer iTPS measurements (3.90 mm versus 2.50 mm), and larger iTPS:iBFS values (0.31 versus 0.20). Postoperative thyroid eye disease patients had significantly higher upper eyelids (longer iMRD1s) (4.80 mm versus 3.30 mm), longer iTPS (5.10 mm versus 3.90 mm), and larger iTPS:iBFS (0.37 versus 0.31) than celebrities. CONCLUSIONS: There are significant gender differences in the periocular metrics of "beautiful people." Optimal aesthetic outcomes may be more effectively obtained by achieving a preferred range of ratios than by relying on independent measurements. Although aesthetic outcomes are multi-factorial, measurements of "beautiful" people provide helpful guidelines to gauge aesthetic outcomes.

The Effect of Photographic Visual Aids in Preoperative Patient Counseling in Oculoplastic Surgery.

PURPOSE: To investigate the effect of visual supplementation and its mode of delivery in preoperative counseling of patients undergoing oculoplastic surgery. METHODS: A prospective randomized controlled trial of consecutive patients undergoing oculoplastic eyebrow and eyelid surgery was conducted. Patients were randomized to an "oral only" group receiving routine preoperative oral counseling or an "oral and visual" group receiving identical counseling visually supplemented with photographs demonstrating common postoperative physical findings. Patients in the "oral and visual" group were further randomized to receive education from the medical team in person versus prerecorded video. Patient emotions and expectations regarding postoperative healing were assessed preoperatively and at 1 week and 2 months postoperatively. RESULTS: 103 patients were included: 32 received in-person oral education, 33 received in-person oral education with photographs, and 38 received education with photographs via video. There were no significant differences in demographics or preoperative patient fear, anxiety, or preparedness. The "oral and visual" group expected more severe postsurgical discomfort and physical findings at postoperative day 1 and week 1. There were no significant differences between groups in surgery signup, cancellation, or triage call rates; patient expectations of postoperative months 2 and 4; or in anxiety, preparedness, or satisfaction. CONCLUSIONS: Visual supplementation in preoperative counseling increases patient expectations of postoperative physical findings without escalating fear or anxiety, and has no significant impact on patient emotions, triage call rates, and satisfaction throughout their surgical experience. Preoperative education via video is perceived by patients to be equivalent to counseling in person by the surgeon.

Thyroid eye disease with choroidal folds.

Purpose: To determine the clinical course of patients with chorioretinal folds (CRF) in thyroid eye disease (TED).Methods: A multi-center retrospective case series of patients with TED who developed CRF.Results: Ten patients (17 eyes) with CRF related to TED were identified. The mean age of presentation was 59.3 ± 8.3 years old. The majority of patients were male (70%), hyperthyroid (70%), hyperopic (53%), had a history of radioactive iodine (60%), and currently on methimazole treatment (30%). Three patients (3 eyes) had unilateral involvement of CRF with bilateral TED. The average clinical activity score was 3.6 ± 2.1 at the time of presentation. The most commonly enlarged extraocular muscles were medial (76%), inferior (64%), superior (64%) and lateral rectus (35%). Compressive optic neuropathy was seen in 47% of eyes. Treatment included oral prednisone (70%), orbital decompression (59%), thyroidectomy (20%) and tocilizumab (10%). The CRF did not resolve over a follow up period of 24.7 ± 23.7 months in 70% of eyes. There was no significant difference in average axial length (25.7 ± 4.9 mm) and optic nerve to optic strut distance (37.8 ± 3.9 mm) between patients with CRF and the eight age-and sex-matched TED control patients without CRF (p = 0.81 and 0.65 respectively). A univariable and multivariable analysis found an enlarged inferior rectus as a factor in TED patients with persistent CRF.Conclusions: CRF are often an indicator of visually threatening situations and often do not resolve despite treatment of TED.