Herpes Simplex Virus Dacryoadenitis Preceding Skin Vesicles.

Herpes simplex virus involvement of the orbit is rare. We report the case of an immunocompetent patient who presented with preseptal edema, erythema, decreased vision, and dacryoadenitis prior to the appearance of vesicular skin lesions and in the absence of corneal dendrites. The patient's clinical picture failed to improve on empiric therapy with intravenous antibiotics. The patient subsequently developed skin vesicles and a nucleic acid amplification swab of the ocular surface was positive for herpes simplex virus-1. The patient demonstrated rapid clinical improvement after initiation of oral antivirals. This is an unusual case of presumed herpes simplex virus dacryoadenitis preceding skin vesicles in an immunocompetent patient.

Isolated Ophthalmoplegia After Periorbital Hyaluronic Acid Filler Injection.

Facial filler injections are the second most commonly performed in-office cosmetic procedure. Vision loss is the most feared complication of hyaluronic acid (HA) filler injection, but isolated ophthalmoplegia can also occur. We report the case of a 45-year-old woman who developed nausea and diplopia following HA filler injection to the bilateral periorbital region. She presented with a left hypertropia and left-sided motility deficit without vision involvement. MRI of the orbits demonstrated mild enhancement and enlargement of the left inferior rectus and inferior oblique muscles. Treatment consisted of hyaluronidase injection and oral steroids. HA filler can cause isolated ocular misalignment and diplopia without associated vision loss. Patients should be counseled on these risks before undergoing soft tissue augmentation of the face with HA filler.

Periocular Infection With Nocardia nova Following Dacryocystorhinostomy With Silicone Stent Intubation.

Nocardia species are aerobic, rod-shaped, Gram-positive bacteria that are ubiquitous throughout the environment. Nocardia nova has been reported as the second most prevalent Nocardia species. However, reports of ocular and periocular infection caused by this organism are rare. The authors present a case of a 58-year-old woman with a medical history of acquired nasolacrimal duct obstruction of the left eye who underwent a revision dacryocystorhinostomy with silicone stent intubation and developed a surgical site infection that was culture-positive for N. nova . Treatment consisted of removal of the silicone stent and a prolonged course of oral antibiotics, resulting in the patient's full recovery. This is the second reported case of postoperative periocular N. nova infection after dacryocystorhinostomy and silicone stent intubation.

A Case of Fabry Disease With Lacrimal Gland Involvement.

Fabry disease is an X-linked lysosomal storage disease resulting from an error in the glycosphingolipid metabolic pathway, which leads to accumulation of globotriaosylceramide in lysosomes of the skin, kidneys, heart, brain, and other organs. There are no existing reports of histologically proven lacrimal gland involvement in Fabry disease. The authors report the case of a 26-year-old male with Fabry disease who presented with bilateral upper eyelid dermatochalasis, steatoblepharon, and prolapsed lacrimal glands. The patient underwent surgical repair of the upper eyelids and biopsy of the lacrimal glands. The pathologic assessment demonstrated lamellated intracytoplasmic inclusions characteristic of Fabry disease. The prevalence of globotriaosylceramide lacrimal gland deposition in Fabry disease and the effect on lacrimal gland morphology and function have yet to be determined.

Assault-related Orbital Trauma at an Urban Level I Trauma Center: Racial Segregation and Other Neighborhood-level Social Determinants.

PURPOSE: To report the demographics of assault-related orbital fractures over a 7-year period treated at a level I urban trauma center, as well as describe and analyze the variation in assault rates across different racial/ethnic neighborhoods for patients residing in Milwaukee County. METHODS: A retrospective chart review was conducted for patients who sustained assault-related orbital fractures from January 1, 2013, through December 31, 2019, at the Froedtert & Medical College of Wisconsin, in Milwaukee, Wisconsin. A series of negative binomial regression models evaluating the association of neighborhood (i.e., US census tract) racial/ethnic composition, poverty, unemployment, percentage female head-of-household, and education level with neighborhood rate of orbital trauma was conducted. RESULTS: A total of 410 adult patients with orbital fractures attributed to assault were identified during the seven-year period, of whom 326 (80%) resided in Milwaukee County. Among these patients, 242 (74%) were male, 260 (81%) were single, and 206 (63%) were non-Hispanic Black. Majority non-Hispanic Black, Hispanic, and Other-type minority neighborhoods have 5.30, 3.35, and 3.94 times higher incidence rates of orbital assault, respectively, compared with the majority of non-Hispanic White neighborhoods. The elevated incidence rates were significantly attenuated across all minority neighborhoods after accounting for neighborhood factors of poverty, unemployment, and low education level. Low education had the strongest association with the incidence of assault-related orbital fractures, followed by unemployment. CONCLUSIONS: Results indicate that minority neighborhoods suffer from compounded burdens of both social and economic disadvantage as well as violent assaults. Additional resources allocated to poor minority communities are needed.

Inverted Papilloma of the Orbit and Nasolacrimal System.

PURPOSE: Periocular inverted papilloma (IP) is a rare, locally aggressive tumor with a propensity for recurrence and malignant transformation. Historically treated via wide excision, this study examines the characteristics and management of periocular IP, comparing those confined to the nasolacrimal system with those invading the orbit. METHODS: An Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant retrospective, comparative case series was conducted in patients with IP of the orbit or nasolacrimal system across 15 clinical sites. RESULTS: Of 25 patients, 22 met inclusion criteria with 9 limited to the nasolacrimal system and 13 invading the orbit. Mean age was 60.4 years, 55% were women, all were unilateral. Mean follow-up was 48 months. Rates of smoking, dust and/or aerosol exposure, human papillomavirus (HPV) status, and inflammatory polyps were elevated compared to rates in the general population (45%, 18%, 18%, and 14%, respectively). Bony erosion on computed tomography scans was statistically significantly associated with orbit-invading IP (p = 0.002). Treatment involved all confined IP undergoing surgery alone while 39% of orbit-invading IP also received radiation therapy and/or chemotherapy (p = 0.054). Orbit-invading IP was more likely to be excised with wide margins than IP confined to the nasolacrimal system (85% vs. 22%, p = 0.007). Overall rates of malignancy, recurrence, and patient mortality from IP were found to be 27%, 23%, and 9%, respectively. CONCLUSIONS: IP invading the orbit typically requires aggressive therapy, while IP confined to the nasolacrimal system may be treated more conservatively. Using risk factors, characteristics, and outcomes, a treatment algorithm was created to guide management.

Giant Dermatofibrosarcoma Protuberans With Bilateral Orbital Involvement.

Dermatofibroma sarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue sarcoma with a tendency for recurrence after excision. Although reports of unilateral orbital and bilateral eyelid disease exist, there have been no prior reports of DFSP with bilateral orbital involvement and no previously described cases of DFSP associated with transient optic neuropathy. The authors present a case report of a 34-year-old woman with a giant scalp DFSP involving the bilateral orbits. Despite radical resection with 5 cm margins where possible, multiple positive margins remained including deep positive margins at the bilateral superomedial retroseptal soft tissue. The patient completed adjuvant radiation for surgically unresectable disease. This case highlights the challenge of achieving local control given the disease extent and infiltration of the bilateral eyelids and orbits. This is the first reported case of DFSP with bilateral orbital involvement and associated transient optic neuropathy.

Langerhans Cell Histiocytosis of the Orbit: Spectrum of Disease and Risk of Central Nervous System Sequelae in Unifocal Cases.

PURPOSE: To describe the spectrum of disease extent and clinical response in Langerhans cell histiocytosis (LCH) presenting with orbital involvement and to determine if unifocal orbital disease increases the risk for central nervous system sequelae (CNS-LCH). METHODS: Retrospective chart review of patients with orbital LCH representing a range of severity treated at the Children's Hospital of Wisconsin from 2003 to 2011; analysis of current international treatment protocols; literature review. RESULTS: Six patients presenting with orbital LCH are described: 1 with unifocal orbital disease completely responsive to local measures; 1 with multifocal bone disease completely responsive to local intervention; 1 with unifocal orbital disease incompletely responsive to surgical intervention, and requiring systemic chemotherapy; and 3 with multisystem disease at presentation. Literature review identified 806 cases of CNS-LCH. Orbital involvement could be determined in 11 cases. Of these, 6 had multisystem disease and 3 had multifocal bone disease; 1 presented with unifocal orbital disease but progressed to multifocal bone involvement; 1 had insufficient clinical information to distinguish unifocal from multisite presentation. No cases of CNS-LCH directly resulted from isolated unifocal orbital disease. CONCLUSIONS: Initial treatment of orbital LCH should depend on disease extent at diagnosis. Unifocal cases that completely respond to biopsy, curettage, and/or corticosteroid instillation may be managed with initial oncologic staging and careful long-term observation, with default to chemotherapy for local recurrence or multisite progression. There is currently little evidence that unifocal orbital disease increases the risk for CNS-LCH and therefore warrants prophylactic systemic chemotherapy in all patients.

Attempted auto-enucleation in two incarcerated young men with psychosis.

Auto-enucleation is a sign of untreated psychosis. We describe two patients who presented with attempted auto-enucleation while being incarcerated. This is an observation two-case series of two young men who suffered untreated psychosis while being incarcerated. These young men showed severe self-inflicted ocular trauma during episodes of untreated psychosis. Injuries included orbital bone fracture and dehiscence of the lateral rectus in one patient and severe retinal hemorrhage and partial optic nerve avulsion in the second patient. Auto-enucleation is a severe symptom of untreated psychosis. This urgent finding can occur in a jail setting in which psychiatric care may be minimal.

Pseudotumor cerebri in children with Down syndrome.

PURPOSE: To report 4 pediatric Down syndrome patients with optic nerve elevation and pseudotumor cerebri. DESIGN: Retrospective observational case series. PARTICIPANTS: Four pediatric Down syndrome patients found to have optic nerve elevation on ophthalmologic examination at a single institution. METHODS: Medical record review of 117 pediatric Down syndrome patients aged 0 to 18 years was performed. All patients were seen at the University of Wisconsin pediatric ophthalmology clinic between 1995 and 2005. MAIN OUTCOME MEASURES: Visual acuity, optic nerve appearance, and neurologic work-up. RESULTS: Four (3.4%) of 117 Down syndrome patients seen at the university's pediatric ophthalmology clinic were found to have optic nerve elevation. This included 2 boys and 2 girls, aged 2 to 10 years. None of the patients had a history of headache or transient visual obscuration. One of the patients had high hyperopia at the time of presentation, and all were referred for neurologic evaluation of suspected increased intracranial pressure. Three patients were diagnosed with pseudotumor cerebri (PTC) based on a normal brain magnetic resonance imaging (MRI) scan, an elevated opening cerebrospinal fluid (CSF) pressure on lumbar puncture, and normal CSF analysis results. The fourth patient's elevated optic nerve appearance was likely secondary to PTC after obtaining normal brain MRI results; however, this patient did not undergo a lumbar puncture. One child with PTC demonstrated improvement in visual acuity and optic nerve appearance after acetazolamide and weight loss therapy. The 2 additional patients with PTC were treated with either low-dose acetazolamide or weight loss therapy and experienced optic atrophy. The final patient, who did not undergo lumbar puncture, experienced spontaneous resolution of optic nerve elevation. Mean follow-up duration was 49 months. CONCLUSIONS: Only 3.4% (4/117) of Down syndrome children were found to have optic nerve elevation in a single institution. Pseudotumor cerebri should be considered in asymptomatic Down syndrome patients with elevated optic nerves. Medical therapy in PTC with acetazolamide can lead to improvement in visual acuity and optic nerve appearance, although optic atrophy also was seen.