Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence.

Adult onset xanthogranulomatous disease is a rare orbital disease. IgG4-related disease is a systemic disease that can often manifest in the orbit. In this communication, we present the case of a patient with a xanthelasma-like lesion on the upper eyelid, and an enlarged lacrimal gland, which on biopsy was diagnosed as an orbital xanthogranuloma. Detailed serological workup showed that the patient was found to have elevated serum IgG4 levels. The orbital specimen was re-stained and found to be positive for IgG4. The patient was treated with oral steroids with partial resolution of the lesion. This is an interesting case of both histopathological adult onset xanthogranuloma (AOX) and IgG4-related orbital disease (IgG4-ROD). The early evidence suggests that the diagnosis of one of these disorders should point the physician to investigate for the presence of the other, especially if xanthogranulomatous disease is diagnosed first.

Orbital Glial Heterotopia: A Report of 2 Cases and Review of the Literature.

PURPOSE: To review the clinical radiographic and histopathologic findings associated with orbital glial heterotopia. METHODS: A literature search in PubMed and Scopus was performed to include all articles published in English between 1980 and January 1, 2019. A case series including 29 case reports of 29 patients, as well as the authors' 2 cases, were considered in the literature review. RESULTS: The majority of the cases had onset of symptoms (86%) and age at presentation (71%) before 5 years of age. The most common presenting symptoms and signs were swelling (45%), strabismus (32%), and proptosis (26%). The most common lesion locations described were inferolateral (19%) or primarily posterior orbital or apical (19%). The most common findings associated with orbital glial heterotopia were microphthalmia (10%) and anophthalmia (6%); however, the majority did not have systemic abnormalities (71%). The most common imaging modality was CT scan (71%). Diagnosis was made with histologic analysis in all cases, and confirmed after subtotal resection (35%), total resection (39%), or incisional biopsy (26%). The majority of the cases report no growth on repeat imaging, with only 3 reports of recurrence. CONCLUSIONS: Glial heterotopia in the orbit is a rare clinical entity most commonly presenting in children. We present 2 cases of orbital glial heterotopia in adults, with a literature on these lesions in both the pediatric and adult populations. Surgeons and pathologists should be aware of this atypical presentation in adulthood. Biopsy is required for diagnosis but is not without risk. Prognosis is generally favorable.Orbital glial heterotopia, commonly considered a rare congenital lesion generally presenting in children, may first become symptomatic in adulthood. Biopsy is required for diagnosis, with symptoms and prognosis dependent on location and growth of the lesion.

Normal Lacrimal Gland Volumes by Magnetic Resonance Imaging and the Relationship of Lacrimal Gland Volume to Orbital Size.

PURPOSE: The aim of this study was to measure volumes of normal lacrimal glands (LGs) using magnetic resonance imaging in African-Americans (AAs) and whites and to evaluate relationships of LG volumes to demographic factors. METHODS: A retrospective chart review was performed searching for "optic neuropathy" and "optic neuritis," cross-referencing patients who had orbital MRI with and without contrast. Cases were excluded with known history of orbital trauma or surgery, previous diagnoses known to affect LG size, and poor image quality. LGs were outlined in consecutive axial and coronal slices, and volumes were generated using OsiriX software; cross-sectional area of the orbit was measured by outlining the bony orbit in largest axial section. RESULTS: One hundred orbits from 50 patients were included (26 AAs, 24 whites). Mean LG volumes as measured in coronal and axial section were 0.714 and 0.671 cm, respectively; mean orbital area was 10.42 cm. Axial orbital size was found to be highly correlated (P < 0.01) with LG volume. No significant correlation was found with LG volume and age, sex, race, or laterality. CONCLUSIONS: This article presents normal values for LG volumes by MRI of AAs and whites. LG volumes were highly correlated with orbital size, a relationship which has not been explored in the recent literature. Such an assessment of LG size relative to patients' orbital dimensions may be more clinically useful than comparison to population-based measures of normal LG volume alone. We found no statistically significant relationship between LG volume and race, sex, age, or laterality.

The Phenylephrine Test Revisited.

PURPOSE: To characterize the phenylephrine test in ptotic patients to help clinicians perform the test more efficiently. METHODS: Adults with involutional ptosis (n = 24, 30 eyes) were assessed with digital photographs for response to topical 2.5% phenylephrine drop instillation. Patient characteristics (age, gender, iris color, dermatochalasis, brow ptosis, and baseline marginal reflex distance-1 [MRD-1] height) were recorded. From the photographs, change in (MRD-1), presence of conjunctival blanching, pupillary dilation, and Hering effect were recorded at specified time intervals, 1 minute to 1 hour after drop placement. Correlations between patient characteristics and measured outcomes were evaluated using analysis of variance, Pearson coefficient, or chi-square tests. RESULTS: The authors found that 73% of eyes had eyelid elevation with phenylephrine. Of these, 50% reached maximal eyelid elevation by 5 minutes, and 86% by 10 minutes after drop placement, but 14% did not reach maximal MRD-1 until 30 minutes. There is a negative correlation between the maximum MRD-1 and the baseline MRD-1 eyelid height (r = -0.5330, p < 0.01). There is no significant relationship between time to pupillary dilation with either time to max eyelid elevation or max eyelid elevation. No patient characteristic studied affected the likelihood of eyelid response to phenylephrine or presence of Hering effect. CONCLUSIONS: Although most ptotic eyelids demonstrate a response to 2.5% phenylephrine within 10 minutes, there is a subset of patients that respond much later. More ptotic eyelids had greater eyelid elevation with phenylephrine. Pupillary dilation and conjunctival blanching are neither predictive of nor temporally associated with eyelid height elevation. The authors did not identify any patient factors (e.g., dermatochalasis, brow ptosis) that can predict the likelihood of response to phenylephrine.

Immunohistochemical profiling including beta-catenin in conjunctival melanocytic lesions.

Conjunctival melanocytic lesions encompass a group of clinically diverse, benign to malignant, neoplasms that may contain overlapping histopathological features, making definitive diagnosis challenging in some cases. In this series, we compared multiple immunohistochemical (IHC) markers in 11 conjunctival nevi, 10 primary acquired melanosis (PAM) lesions, and 11 conjunctival melanomas. Immunostains included the melanocytic markers HMB-45 and Melan-A, as well as the proliferative marker Ki-67. Loss of beta-catenin expression has been associated with more aggressive clinical disease in cutaneous melanoma, but its status in conjunctival melanocytic lesions is not known, therefore we incorporated beta-catenin immunohistochemical staining in our study. In this series, conjunctival melanomas had a higher Ki-67 proliferative index and HMB-45 immunoreactivity than did PAM lesions and conjunctival nevi (P<0.001). Melan-A was highly expressed in all 3 groups. Beta-catenin was more strongly expressed in melanomas and nevi than in PAM (P<0.001). There was high inter-grader reliability (Kappa=0.53). Overall, IHC labeling of HMB-45 and Ki-67 is increased in conjunctival melanomas compared to PAM or conjunctival nevi. Beta-catenin, an IHC marker previously unstudied in conjunctival melanocytic lesions, is not preferentially expressed in benign lesions and may play a different role in conjunctival atypia than it does in cutaneous melanoma.

Extramedullary plasmacytoma of the lateral rectus muscle.

Plasmacytoma is an uncommon presentation of plasma cell malignancy, especially in the absence of multiple myeloma. Orbital plasmacytomas generally originate from bone, although few cases in the literature report orbital extramedullary plasmacytomas. We present the case of a 68-year-old man found to have a solitary extramedullary plasmacytoma in the lateral rectus muscle without further evidence of multiple myeloma. This case demonstrates a rare presentation for such a malignancy, and a review of the literature highlights the importance of proper workup and close monitoring to rule out multiple myeloma to guide management.

A Novel Video-Based Skills Curriculum for Incoming Ophthalmology Residents.

BACKGROUND: Given limited pre-residency ophthalmology exposure, skill training for PGY-2 ophthalmology residents is essential. However, orientation experiences vary, and skills acquisition is often not measured. OBJECTIVE: A novel video-based orientation curriculum was developed and implemented to standardize and effectively teach ophthalmic examination skills to incoming ophthalmology residents. METHODS: An instructional video library (VL) on ophthalmic exam skills was created in 2020. Prior to any instruction, PGY2s were recorded performing basic ophthalmic exams (BOE) using slit-lamp recording smartphone adapters. After a 2-week orientation involving live teaching, practice, and self-directed library review, ophthalmic exams were again recorded. A 36-point ophthalmic exam skills checklist expanding upon the Ophthalmic Clinical Evaluation Exercise (OCEX) was developed for scoring videos. Residents also completed pre- and post- surveys assessing their comfort with the ophthalmic exam. RESULT: 7 of 11 incoming PGY-2 residents (63.7%) ophthalmology residents participated. Average recorded OCEX score improved from 16.5 ± 5.8 to (p = .0002) to 30.9 ± 2.7. Surveyed resident comfort with the exam increased from 2.4 ± 0.6 to 4.2 ± 0.5 on a 5-point Likert scale (p < .0001). CONCLUSION: Our video library orientation curriculum was effective in rapidly increasing resident comfort and BOE skills. With the launch of the integrated internship model, the VL curriculum may be effective for training ophthalmology PGY1s, medical students and non-ophthalmology providers.

Tarsoconjunctival flap for scleromalacia related to treatments for conjunctival melanoma.

Purpose: To describe the use of a tarsoconjunctival pedicle flap for the repair of scleral melt secondary to treatment of conjunctival melanoma. Observation: A 67-year-old woman developed progressive scleromalacia after multiple treatments for an American Joint Committee on Cancer cT2d category conjunctival melanoma. Prior to referral, she underwent synchronous topical chemotherapy (interferon, 5-fluorouracil, mitomycin). Then, incomplete tumor regression led to excision with adjuvant cryotherapy. Lastly, systemic metastasis treated with systemic immunotherapy provided durable remission. However, her multiple treatments (e.g., topical chemotherapy, resection, cryotherapy) were associated with progressive nasal bulbar scleromalacia treated by conjunctival advancement and amniotic membrane grafts. Sclera reinforcement was achieved after a tarsoconjunctival flap was affixed to the eye to cover, and thus vascularize the scleral defect. The tarsoconjunctival flap provided 5 years of tectonic support. Conclusions and Importance: Tarsoconjunctival pedicle flaps can provide scleral integrity for a patient with progressive scleral melting.

Retinal Surface Macrophage Changes in Thyroid Eye Disease before and after Treatment with Teprotumumab.

Retinal surface macrophages play key roles in the regulation of immune response, maintenance of vitreous clarity, and tissue repair. We examined the variation of parafoveal surface macrophages in a thyroid eye disease (TED) patient before and after treatment with teprotumumab (Tepezza, Horizon therapeutics). Pre- and posttreatment parafoveal surface macrophages were imaged using clinical en face OCT, and their density was assessed using a novel cell density mapping technique. Pretreatment, surface macrophage cell density was high. Macrophages had a nonuniform spatial distribution, and their appearance was round with few protrusions, consistent with an "activated" state. Posttreatment, cell density decreased. The macrophages were regularly spaced and had a ramified appearance and filopodia-like processes, consistent with a "quiescent" state. Surface macrophage density decreased as the Clinical Activity Score (CAS) decreased with teprotumumab treatment, suggesting a potential association of these cells with an underlying intraocular and retinal inflammatory process previously not described in TED.

A Pilot Study of Subclinical Non-Capillary Peripapillary Perfusion Changes in Thyroid-Related Orbitopathy Detected Using Optical Coherence Tomography Angiography.

Purpose: Hemodynamic changes surrounding the optic nerve head are known to occur in thyroid-related orbitopathy (TRO). This pilot study explores the capillary and non-capillary peripapillary perfusion changes of the retina in TRO eyes without dysthyroid optic neuropathy (DON) using optical coherence tomography angiography (OCT-A). Methods: Non-capillary and capillary peripapillary perfusion densities were calculated using single 4.5 × 4.5mm en face "RPC layer" OCT-A scans of 8 TRO patients without DON (8 eyes, mean age 40.6 years, range 23-69 years). Results were compared to a previously published dataset of 133 healthy controls (133 eyes, mean 41.5 years, range 11-83 years). The strength of association was measured between OCT-A perfusion densities and clinical measures of TRO. Results: Non-capillary peripapillary perfusion density in TRO eyes was found to be significantly decreased compared to healthy controls (TRO group 15.4 ± 2.9% vs controls 21.5 ± 3.1%; p < 0.0001). Capillary peripapillary perfusion densities showed no significant difference (TRO group 42.5 ± 1.8% vs controls 42.5 ± 1.5%; p = 1.0). Clinical measures of disease did not correlate well with OCT-A perfusion densities (p>0.05). Conclusion: These findings may represent decreased blood flow and subclinical ischemia to the optic nerve. We discuss possible pathogenic mechanisms of thyroid-related vasculopathy, including vessel wall thickening due to immunologically-induced media enlargement.

Ocular biometry and open-angle glaucoma: the Los Angeles Latino Eye Study.

PURPOSE: To examine the associations among myopic refractive error (MRE), corneal power (CP), axial length (AL), and the prevalence of open-angle glaucoma (OAG) in an adult Latino population. DESIGN: Population-based, cross-sectional epidemiologic study. PARTICIPANTS: A total of 5927 Latinos aged 40 years and older in the Los Angeles Latino Eye Study (LALES). METHODS: Myopic refractive error was defined as a non-cycloplegic sphero-cylindrical refractive error of < or =-1 diopter (D). Axial length was measured by A-scan ultrasound. Corneal power was measured using a Humphrey auto-refractor. Open-angle glaucoma was diagnosed by a combination of optic nerve and visual field changes. Pseudophakic and aphakic eyes were excluded from the analysis. The associations among MRE, AL, CP, and the prevalence of OAG were calculated using a logistic regression model, adjusting for age, gender, intraocular pressure, central corneal thickness, diabetes mellitus, family history of glaucoma, and lens nuclear opacification (NO). MAIN OUTCOME MEASURES: Odds ratios (OR) for the prevalence of OAG. RESULTS: After adjusting for covariates, persons with greater MRE (OR 1.82; confidence interval [CI], 1.20-2.77; P = 0.005), longer AL (OR 1.25; CI, 1.03-1.50; P = 0.02), and flatter corneas (OR 1.21; CI, 1.08-1.35; P = 0.0007) were associated with a higher prevalence of OAG when compared with those with emmetropic refractive error, shorter AL, and steeper corneas. CONCLUSIONS: Persons with an MRE, flatter corneas, and longer ALs should be considered to be at higher risk of having OAG.

Large particle hyaluronic Acid gel for the treatment of lower eyelid retraction associated with radiation-induced lipoatrophy.

A 42-year-old female with a remote history of a left maxillary sinus tumor treated with excision and radiation therapy was referred for dry eye symptoms. Ophthalmic examination revealed left-sided exposure keratopathy, lagophthalmos, lower eyelid retraction, and fat atrophy of inferior periorbital tissue with associated hollowing. Large particle hyaluronic acid gel was injected to expand and reinforce the lower eyelid. After treatment, there was significant improvement in lagophthalmos, inferior scleral show, and periorbital hollowing. Excellent symmetry with the fellow eye was achieved. The patient reported markedly reduced dry eye symptoms. No adverse side effects were observed. The use of large particle hyaluronic acid gel shows promise as a novel nonsurgical therapy in the management of lower eyelid retraction associated with radiation-induced lipoatrophy. Additionally, large particle hyaluronic acid gel may provide better soft tissue expansion and retention than small particle hyaluronic acid gel, increasing the longevity of treatment.

Optical coherence tomography of corneal and scleral melts.

Corneal and scleral melts can be difficult to assess by slit-lamp due to the overlying opacity. The authors demonstrate the role of optical coherence tomography (OCT) in the diagnosis and management of two cases of corneal and scleral melt. A high-speed anterior segment OCT system operating at a 1310-nm wavelength was used. Cross-sectional OCT images showed the depth and extent of the melt. OCT images were obtained through an opaque pannus in one case and through a calcium plaque in the other. OCT images at the follow-up examination revealed a thin fluid space between the amniotic graft and cornea and its subsequent resolution in the first case and the fits of an epicardial graft and a subsequent clear lamellar corneal graft in the second case. OCT images allow physicians to assess melts through opaque media and subsequent graft integration after repair.

Improving Resident Performance in Oculoplastic Surgery: A New Curriculum Using Surgical Wet Laboratory Videos.

OBJECTIVE: To develop a new oculoplastic curriculum that incorporates learning theory of skill acquisition. To develop and evaluate the effectiveness of instructional videos for an oculoplastic surgical wet laboratory. DESIGN: Proof of concept, randomized controlled trial. SETTING: New York Eye and Ear Infirmary of Mount Sinai-tertiary care academic institution. PARTICIPANTS AND METHODS: In total, 16 ophthalmology residents were randomly assigned to 1 of 2 groups and given either video and text or text instructions alone for the following 2 procedures: blepharoplasty and eyelid laceration repair. Operating time and esthetic result were measured, and the groups were statistically compared. A brief survey was administered. RESULTS: We developed a new 6 component oculoplastics curriculum that incorporates concepts of the Fitts and Posner skill acquisition model and mental imagery. In the wet laboratory pilot study, the group that watched the video of the laceration repair showed better esthetic grades than the group that received text alone (p = 0.038). This difference was not found for the blepharoplasty (p = 0.492). There was no difference between groups in operating time for the laceration repair (p = 0.722), but the group that watched the blepharoplasty video required more time to complete the task than those that reviewed text only (p = 0.023). In total, 100% of residents reported the videos augmented their learning. CONCLUSIONS: Methods to optimize surgical education are important given limited operating room time in oculoplastics, a subspecialty in which the number of surgeries performed during residency is relatively low. We developed a curriculum based on learning theory and sought to formally test one important aspect, surgical video for wet laboratories. Our pilot study, despite its limitations, showed that wet laboratory surgical videos can be effective tools in improving motor skill acquisition for oculoplastic surgery.

Eyelid trichilemmal carcinoma.

Trichilemmal carcinomas are rare malignant adnexal tumors which can involve the head and, rarely, the eyelid. Accurate diagnosis and differentiation from basal cell carcinoma or trichoepithelioma are dependent on histopathological and immunohistochemical analysis. We report a case of an eyelid mass, initially diagnosed by incisional biopsy as a trichoepithelioma, later found on excisional biopsy to be a trichilemmal carcinoma and present a review of the literature of the histopathology, differential diagnosis and management. Consideration of these entities will be useful to the histopathologist and clinician to better identify and treat these challenging lesions.

Syphilis: reemergence of an old adversary.

OBJECTIVE: To describe the clinical findings in 4 patients with ocular syphilis and to provide a review of the recent rise in syphilis cases in the United States, along with a brief description of current diagnosis and treatment guidelines. DESIGN: Retrospective, observational case series. PARTICIPANTS: Four consecutive patients who presented to our facility during a 6-month interval in 2005 with ocular syphilis. METHODS: Review of clinical, laboratory, photographic, and angiographic records of patients with neurosyphilis who underwent intravenous penicillin G treatment. MAIN OUTCOME MEASURES: Resolution of signs and symptoms of ocular syphilis, including changes in visual acuity. RESULTS: Four patients (3 male: mean age, 46 [range, 39-60 years]; 1 female: age 46 years) demonstrated cerebrospinal fluid findings consistent with neurosyphilis, as evidenced by increased leukocyte counts (>5 white blood cells/mm3), and positive Venereal Disease Research Laboratory or serum fluorescent treponemal antibody absorbed tests. All 4 patients presented with a variety of clinical findings that led to a delay in diagnosis and treatment. Two patients presented with discrete placoid lesions consistent with acute syphilitic posterior placoid chorioretinitis, a manifestation typically observed among the immunocompromised. CONCLUSIONS: Despite a decade of steady decline, syphilis has reemerged in the United States with outbreaks throughout the country in the past few years. Ocular findings, including posterior placoid chorioretinitis, are important diagnostic features in the early treatment of tertiary syphilis and neurosyphilis. Ophthalmologists have the opportunity to play a key role in the early diagnosis and management of this potentially fatal disease.