Electrical burn of the eye and orbit with severe delayed sequelae from an arc welding accident.

A 28-year-old male presented to the emergency room suffering an ocular burn injury from a welding rod. Given the mechanism of injury, severe delayed injury of the ocular adnexa occurred, requiring enucleation, partial exenteration of the superior orbit, and extensive reconstruction. Histopathology of the affected tissue was analyzed. This is the first report that details the clinical course of a patient with delayed high amperage and low voltage electrical burn injury.

Cyclin D1 Expression and Molecular Genetic Findings in Periocular Histiocytoses and Neoplasms of Macrophage-Dendritic Cell Lineage.

PURPOSE: Frequent activating mutations in the mitogen-activated protein kinase (MAPK) pathway genes have been identified in histiocytoses. MAPK signaling consistently upregulates cyclin D1. The goal of this study was to determine whether cyclin D1 expression by immunohistochemistry is a useful diagnostic marker for periocular histiocytoses and to further characterize their genetic basis. DESIGN: Retrospective observational case series. METHODS: Pathology records were searched for all patients with histiocytoses diagnosed between 1995 and 2020. Eleven histiocyte-rich inflammatory lesions and 10 xanthelasma served as controls. Cyclin D1 immunohistochemistry was performed on all tissues. A subset of histiocytoses was evaluated by next-generation sequencing (NGS) and droplet digital PCR (ddPCR). RESULTS: There were 36 patients, 15 males (42%) and 21 females (58%), with histiocytoses: 9 juvenile xanthogranuloma (25%), 8 adult-onset asthma and periocular xanthogranuloma (22%), 7 Langerhans cell histiocytosis (19%), 5 Rosai-Dorfman disease (14%), 5 xanthogranuloma-not otherwise specified (14%), 1 Erdheim-Chester disease (3%), and 1 histiocytic sarcoma (3%). Moderate to strong nuclear cyclin D1 expression was present in ≥50% of lesional cells in histiocytoses (23/36, 64%), significantly more when compared to histiocyte-rich inflammatory lesions (0/11, 0%, P<.001) and xanthelasma (0/10, 0%, P<.001). Cyclin D1 was expressed in <10% of lesional cells in all 11 histiocyte-rich inflammatory lesions (P<.001) and all 10 xanthelasma lesions (P<.001). MAPK pathway gene mutations were detected in 12 of 14 (86%) histiocytoses successfully assayed by NGS and/or ddPCR. CONCLUSIONS: Our study confirms that the cyclin D1 immunohistochemical stain is a useful diagnostic marker for periocular histiocytoses, correlating with underlying mutations in MAPK pathway genes.

Mucoepidermoid Carcinoma of the Lacrimal Sac: Clinical-Pathologic Analysis, Including Molecular Genetics.

PURPOSE: The aim of this study was to assess whether mucoepidermoid carcinoma of the lacrimal sac is a counterpart of CRTC1/3-MAML2 gene fusion-related salivary gland mucoepidermoid carcinoma. METHODS: In this retrospective observational case series, pathology records were searched for all cases of lacrimal sac mucoepidermoid carcinoma diagnosed between 1990 and 2018. Data collected included demographics, clinical findings, management, and follow-up. Pathologic parameters assessed included tumor morphology, immunohistochemistry, and MAML2 and EGFR fluorescence in situ hybridization (FISH) studies. RESULTS: Six patients with mucoepidermoid carcinoma of the lacrimal sac, 5 males and 1 female, with a median age of 63 years (range 24-66) were identified. Five tumors were managed with radical resection and 1 patient underwent orbital exenteration. None of the patients developed recurrence or metastases with an average follow-up of 18 months (range 13-23). All tumors had morphologic and immunohistochemical features of mucoepidermoid carcinoma and overexpressed EGFR. MAML2 FISH was negative for MAML2 rearrangement in all tumors. EGFR FISH demonstrated EGFR amplification in 1 tumor. CONCLUSIONS: Mucoepidermoid carcinoma of the lacrimal sac is not a lacrimal sac counterpart of CRTC1/3-MAML2 gene fusion-related salivary gland mucoepidermoid carcinoma. EGFR pathway activation and EGFR amplification in a subset of these neoplasms suggest the potential role for anti-EGFR agents.

Basaloid adenocarcinoma of the lacrimal sac: A rare entity and approach to management.

Presented is a rare case of basaloid adenocarcinoma of the lacrimal sac. Low grade features of this malignancy permitted a conservative surgical approach to maximize functional and cosmetic outcomes. This is retrospective case description of a single 77-year-old-female patient who underwent a dacryocystorhinostomy with silicone tubes at an outside hospital for epiphora, but was referred to the Wills Eye Hospital Oculoplastics Department for a lacrimal sac biopsy showing low-grade carcinoma with basaloid features. She was treated with complete excision of the mass, common canaliculus and distal canaliculi, and the nasolacrimal duct. The associated maxillary, lacrimal, and ethmoidal bones were similarly excised. Frozen sections were sent of the surrounding periosteum, orbital fat, nasal and sinus mucosa, and soft tissues, and all were negative. Reconstruction of the lacrimal system was performed with silicone tube intubation and the defect was closed with large glabellar and mid-facial flaps. One year post-operatively she maintained a normal ocular examination, and her eyelids and silicone tubes were well positioned. Systemic evaluation remained unrevealing. Basaloid adenocarcinoma of the lacrimal sac is relatively rare, and we highlight the role of a more conservative surgical intervention given the high rates of survival and low incidence of progression to metastatic disease when treating this rare malignancy.

Unilateral Prostaglandin-Associated Periorbitopathy: A Syndrome Involving Upper Eyelid Retraction Distinguishable From the Aging Sunken Eyelid.

PURPOSE: To study the effects of prostaglandin analogue drops on the eyelids and adnexa in unilaterally treated subjects with the intention of qualifying, quantifying, and categorizing the characteristics of prostaglandin-associated periorbitopathy (PAP). METHODS: Patients using prostaglandin analogue drops in only 1 eye for at least 1 year were evaluated by masked examiners. Orbital and eyelid measurements were obtained for each patient, and adnexal photographs were taken. PAP was divided into 3 grades based on the presence and severity of fat atrophy and the existence and depth of superior sulcus deformity. Statistical analysis was performed comparing data between treated and untreated eyes. RESULTS: Thirty-three patients meeting eligibility criteria were enrolled, with equal numbers of subjects using latanoprost, travoprost, and bimatoprost. Treated eyes had a statistically significant increase in lagophthalmos (0.62 mm, p < 0.001), superior sulcus deformity/PAP grade (0.72, p < 0.001), and eyelid redness (1.08, p < 0.001). Treated eyes had significantly greater marginal reflex distance 1 measurements (0.89 mm, p = 0.02), highest with bimatoprost and moderate PAP. Treated eyes had relatively greater enophthalmos than untreated eyes. Very few patients noticed or complained about eyelid changes. CONCLUSION: Prostaglandin analogue drops cause adnexal changes and orbital fat atrophy leading to eyelid redness, superior sulcus deformity, higher eyelid crease, and enophthalmos. In contrast to previous studies showing ptosis in PAP, relative upper eyelid retraction was seen in most of our treated eyes. Our novel PAP grading scale may help objectify and categorize this syndrome. Awareness of these signs is critical, as the eyelids and eyes may be affected even in the absence of patient recognition.

Odontogenic orbital cellulitis.

PURPOSE: To describe a small series of patients with odontogenic orbital cellulitis and review the visual outcomes of such patients reported in the scientific literature. METHODS: Review of medical records and the scientific literature. Measured parameters included gender, age, days to presentation and surgery, the need for surgical intervention(s), sinus and orbital involvement on CT imaging, and pathogens involved. RESULTS: Current cases and review of the scientific literature resulted in 24 patients with odontogenic orbital cellulitis and adequate examinations for statistical analysis. Twelve of 24 patients had preserved vision (final vision better than 20/70), whereas 11 of 24 patients had vision loss (final vision of light perception or no light perception). Analysis of data showed no statistical correlation between visual loss and age (p = 0.81), days to clinical presentation (p = 0.45), days to surgical exploration (p = 0.96), sinus radiographic involvement (p = 0.50), orbital radiographic findings (p = 0.19), or type of bacterial infection (Gram-positive aerobic vs. anaerobic organisms), (p = 0.31 and p = 0.50 respectively). Male gender, the need for surgical drainage, and multiple surgical procedures performed correlated statistically with loss of vision (p = 0.05, p = 0.03, and p = 0.02, respectively). CONCLUSIONS: Multiple case reports have demonstrated that orbital cellulitis from an odontogenic source can result in devastating visual loss. Male gender, the need for surgical drainage, and multiple surgical procedures correlated statistically with severity of visual loss.

Conjunctival epithelial involvement by eyelid sebaceous carcinoma. The 2003 J. Howard Stokes lecture.

PURPOSE: To determine incidence and distribution of conjunctival epithelial involvement by eyelid sebaceous carcinoma and to make recommendations regarding its management. METHODS: The medical records were reviewed retrospectively on patients with histopathologically confirmed sebaceous carcinoma of the eyelids managed at the Oncology Service at Wills Eye Hospital. Those tumors with involvement of the conjunctival epithelium were identified and selected for further study. The incidence and distribution of epithelial involvement was determined, based on histopathology of resected tumors and map biopsies. The incidence of metastasis and tumor-related mortality was determined. Based on these findings and personal surgical experience, recommendations are made regarding management of eyelid sebaceous carcinoma with involvement of the conjunctival epithelium. RESULTS: Of 60 patients with sebaceous carcinoma, epithelial involvement of the conjunctiva was identified in 28 (47%). Of the 28 cases, the neoplasm affected the following sites: superior tarsal and fornical conjunctiva in 28 (100%), inferior tarsal conjunctiva in 19 (68%), inferior fornical conjunctiva in 18 (64%), superior bulbar conjunctiva in 19 (68%), and inferior bulbar conjunctiva in 16 (57%). The caruncle was involved in 15 (54%) and the cornea in 11 (39%). Metastasis occurred in 3 of the 28 cases (11%). Map biopsies, combined with cryotherapy, topical chemotherapy, local surgical resection, and orbital exenteration, were used to achieve local control. CONCLUSIONS: Eyelid sebaceous carcinoma was found to exhibit epithelial involvement of the conjunctiva in 47% of cases, predominantly in the superior tarsal and fornical conjunctiva and less often in the inferior tarsal conjunctiva, caruncle, and cornea. Treatment of this condition is challenging, and map biopsy, cryotherapy, topical chemotherapy, and newer surgical methods are being used more often by our group.

Incidence of postblepharoplasty orbital hemorrhage and associated visual loss.

PURPOSE: Orbital hemorrhage, especially when associated with visual loss, is a significant complication of cosmetic eyelid surgery. We investigated the incidence of this complication among cases handled by members of the American Society of Ophthalmic Plastic and Reconstructive Surgery. We also reviewed the length of time between surgery and the onset of bleeding and sought any factors that may contribute to these cases. METHODS: A 2-page questionnaire was sent to all American Society of Ophthalmic Plastic and Reconstructive Surgery members. Responses were tabulated as to total numbers of cases performed, the incidence of postoperative orbital hemorrhage, treatments, and incidence of permanent visual loss. Comorbidities were also reviewed. RESULTS: Two hundred thirty-seven responses were received from American Society of Ophthalmic Plastic and Reconstructive Surgery members, collectively representing 269,433 cases. Among these cases, there were 149 orbital hemorrhages, 48 cases associated with temporary visual loss, and 12 cases associated with permanent visual loss. The majority of these cases occurred within the first 3 hours of surgery, and the risk decreased significantly after 24 hours after surgery. Common comorbidities were hypertension, perioperative aspirin use, postoperative vomiting, and increased physical activity. Treatment commonly consisted of wound drainage with cauterization of active bleeders, lateral canthotomy, intravenous steroids, and intraocular pressure-lowering drugs. Orbital decompression was infrequently used; anterior chamber paracentesis was not used. This study is limited by recall biases regarding the number of cases performed by each responding surgeon and the number of relevant cases of orbital hemorrhage. CONCLUSIONS: The incidence of orbital hemorrhage associated with cosmetic eyelid surgery is 0.055% (1:2,000), and orbital hemorrhage with permanent visual loss is 0.0045% (1:22,000) [corrected] Development of orbital hemorrhage is most common within the first 24 hours after surgery, especially within the first 0 to 3 hours, but can occur as late as several days after surgery.