Orbital masses as a rare presentation of Rosai-Dorfman disease: Clinicopathologic characterization of five cases.

Rosai-Dorfman disease (RDD) is a rare, non-Langerhans cell histiocytosis. Most cases present with marked, non-tender lymphadenopathy due to the proliferation of atypical histiocytes. A minority of cases involves extranodal sites and can present as bone lesions, skin rashes, pulmonary nodules, and rarely orbital masses. Orbital involvement in RDD is rare and may infrequently present as an isolated tumor mass without lymphadenopathy. This study aims to better characterize this uncommon presentation of this rare disease. Five cases of orbital RDD were identified from the last 18 years and the clinical characteristics of each case were compared with histopathological findings. Three men and two women ages 12-36 presented with complaints of eye swelling and/or vision changes. One patient had a history of neurofibromatosis type I and inflammatory pseudotumors while the other four had no signs of systemic disease or other sites of extranodal involvement at the time of presentation. Masses ranged in size from 1.0 cm to 3.5 cm and primarily involved the superior orbit. Resected lesions all displayed characteristic findings of admixed atypical histiocytes, lymphocytes, and plasma cells with a fibrotic background. Emperipolesis was seen in all cases. Immunostaining for S100 and CD68 was diffusely positive in the histiocyte population. Clinical follow-up was obtained for 4 of 5 patients: all four were disease-free at 1 to 15 years after resection. RDD should be considered in the differential for patients with orbital masses, even in the absence of lymphadenopathy or signs of systemic disease.

Systemic Outcomes in Adults Undergoing Emergent Repair of Orbital Blowout Fractures.

Purpose: To analyze the association between emergent surgery status and systemic adverse outcomes in patients undergoing open orbital floor blowout fracture repair. Methods: This retrospective cohort analysis utilized the 2005-2018 National Surgery Quality Improvement Program (NSQIP) database. Current Procedural Terminology (CPT) codes were used to identify cases with open treatment of orbital floor blowout fractures (21385, 21386, 21387, 21390, 21395). Demographics, comorbidities, and complication incidences were compared between patients undergoing emergent surgery and those undergoing non-emergent orbital blowout fracture repair using chi-square analyses. The independent effect of preoperative emergent status on adverse outcomes was analyzed using binary logistic regression. Results: 1,146 (96.0%) non-emergent and 48 (4.0%) emergent orbital blowout fracture repairs were identified from 2005 to 2018. Chi-square analysis indicated patients undergoing emergent repairs had higher incidences of preoperative wound infection (8.3% vs. 2.3%; p = 0.029) and systemic sepsis (8.3% vs. 0.6%; p = 0.001). The emergent cohort had a higher proportion of patients with Hispanic ethnicity (p = 0.011). Unadjusted chi-square analysis indicated the emergent cohort had a higher incidence of prolonged length of stay (50.1% vs. 10.1%; p < 0.001). After adjusting for confounders, logistic regression analysis indicated emergent status was an independent risk factor for prolonged length of stay (OR 13.05; 95% CI 5.26-32.37; p < 0.001). Conclusion: Emergent surgery status is an important factor associated with increased odds of prolonged length of stay in patients undergoing open orbital blowout fracture repair. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-024-04681-0.

Hybrid Schwannoma-Perineurioma of the Orbit.

A 52-year-old woman presented with a 6-month history of progressive right proptosis associated with intermittent right retrobulbar and facial pain. MRI revealed a heterogeneously enhancing, well-circumscribed, ovoid, soft tissue mass in the intraconal space near the right orbital apex displacing the optic nerve medially. Excisional biopsy established the diagnosis of a schwannoma-perineurioma hybrid peripheral nerve sheath tumor (HPNST). This case represents only the second reported occurrence, to our knowledge, of an orbital schwannoma-perineurioma HPNST.

Epidemiology of United States tennis-related ocular injuries from 2000 to 2019.

PURPOSE: To characterize trends in ocular tennis injuries over the last 20 years. METHODS: The National Electronic Injury Surveillance System was utilized to characterize tennis-related eye injuries in a nationally representative sample of emergency department visits. Data were divided into 5 age groups, and various demographic information was obtained. RESULTS: Approximately 16,000 tennis-related ocular injuries were identified with males being affected nearly 2:1 compared to females. The youngest age group (0-20) had the greatest proportion of injuries, with most injuries in boys 11-15 years old. Injuries occurred most often during the spring season. Most patients were treated and released from the ED. Of those patients who were hospitalized, one-third had an open globe injury. CONCLUSIONS: The overall number of injuries trended downward during the timespan of the study. Although most patients did not experience serious visual consequences, the greatest proportion of ocular tennis injuries occurred in the pediatric age group in whom the risk of amblyopia is high. Primary care providers and tennis regulatory bodies should consider recommending eye safety sports goggles in children to mitigate the potential for significant visual morbidity.

Orbital glomangiopericytoma in a 5-year-old child.

A 5-year-old girl presented with a 6-month history of strabismus and painless left proptosis. Magnetic resonance imaging revealed a homogenously enhancing, anterior, superomedial, left orbital mass. Excisional biopsy established the diagnosis of orbital glomangiopericytoma. Glomangiopericytoma of soft tissue is a perivascular myoid neoplasm with hemangiocytoma-like vascular channels that has overlapping features between glomus tumor and myopericytoma. To the authors' knowledge, glomangiopericytoma of the orbit has not previously been reported.

Enucleation in pediatric open globe injuries: demographics and risk factors.

PURPOSE: The purpose of this study is to report the demographics and risk factors for undergoing primary enucleation in the setting of acute open globe injury (OGI) in the pediatric population in the USA. METHODS: This retrospective, cross-sectional study of pediatric patients with OGIs in the USA between 2002 and 2014 was conducted utilizing data from the National Inpatient Sample Database. Descriptive statistics, chi-square testing, and univariate and multivariable analyses were performed. RESULTS: In the USA, 8944 cases of pediatric OGI were identified between 2002 and 2014 in the NIS Database, of which 344 underwent primary enucleation. Blacks and Asian/Pacific Islanders made up higher proportions of enucleated cases compared to non-enucleated cases. Older age, male sex, being Black or Asian/Pacific Islander, OGI with an intraocular foreign body, rupture type OGI, and concurrent endophthalmitis were identified as risk factors for undergoing enucleation. There was no significant difference in insurance status among enucleated versus non-enucleated cases. Mean length of hospital stay (in days) was almost 3 times higher in enucleated OGIs. By hospital's geographic location, the Midwest hospitals had a greater proportion of enucleated versus non-enucleated cases compared to other regions. CONCLUSION: Significant demographic differences were identified in OGI patients that underwent primary enucleation versus repair with regard to age, sex, race, the geographic location of hospital admission, mean length of hospital stay, type of ocular injury, and other ocular complications. Most pediatric traumatic enucleations between 2002 and 2014 were reported in teenagers (16-20 age group), in males, and in Blacks.

Second Primary Neoplasms in Patients With Sebaceous Carcinoma of the Eyelid: A Population-based Study, 2000 to 2016.

PURPOSE: To investigate the risk of second primary neoplasms (SPNs) after primary sebaceous carcinoma of the eyelid (SCE). METHODS: Data on patients diagnosed with primary SCE as their first malignancy were extracted from the Surveillance, Epidemiology, and End Results database from 2000 to 2016. Observed-to-expected ratios of SPNs were calculated to estimate standardized incidence ratios (SIRs). Patients were compared with a reference population (RP) matched for age, gender, and race. RESULTS: Five hundred fifty-nine patients with primary SCE were identified, 16% of whom developed SPNs. SCE patients displayed a 61% increased risk of developing SPNs compared with the RP (p < 0.001). Overall, the risk of SPNs of the lungs (SIR = 1.82; p < 0.05), pancreas (SIR = 2.94; p < 0.05), salivary glands (SIR = 41.65; p < 0.001), and skin (SIR = 8.33; p < 0.05) was elevated. Only non-Hispanic Whites were at an increased risk (SIR = 1.51; p < 0.05). Patients 40-54 years old at the time of diagnosis were at the highest risk of developing SPNs compared with the RP (SIR = 3.15; p < 0.05). Women with SCE experienced an increased risk of breast cancer (SIR = 3.6; p < 0.05) and chronic lymphocytic leukemia (SIR = 8.8; p < 0.01). CONCLUSION: SCE patients are more likely to develop SPNs of the lungs, pancreas, salivary gland, and skin than the RP. Forty to fifty-four years old Caucasian patients are at the highest risk. Women are at an increased risk of developing breast malignancies and chronic lymphocytic leukemia. Clinicians should be cognizant of these risks when managing SCE patients.

Demographic trends of open globe injuries in a large inpatient sample.

PURPOSE: To evaluate demographic trends of open globe injuries (OGIs) using a large dataset representative of United States population. METHODS: Retrospective cross-sectional observational study using the National Inpatient Sample (NIS) database from 2002 to 2013. Only patients with a primary admitting diagnosis of OGI were included. Data included age (in years), gender, race, type of OGI, death rate and length and cost of stay. RESULTS: During the 12-year period, 27,467 adults (age > 20) with acute OGIs were admitted to the US hospitals. The incidence of OGIs in the adult US population was 10.6 cases per 1,000,000 persons. The mean age was 50.4 years (SD 21.52); the average ages of men and women were 44.34 (SD 17.63) and 65.69 (SD 22.77), respectively. Men accounted for 71% of all cases, with 84% of patients under 60. A decrease in the number of OGIs was seen with advancing age in men, whereas the opposite was true for women. Men, elderly over 80 and Blacks were at the highest risk of sustaining an OGI. The most and least common types of injuries were penetrating injuries (73%) and IOFBs (11%), respectively. Over half of young adults in the 21-40 cohort and 43% of men were uninsured (p < 0.001). The average length of hospital stay increased with age and was significantly much higher in women than men (3.4 vs 2.5 days). CONCLUSIONS: Racial, gender and age disparities are prevalent in patients with OGIs. Although the majority of cases were seen in Whites and young men age 21-40 years, the incidence of OGIs per 1,000,000 persons per year was the highest in Blacks and Hispanics, elderly over 80, and men. One-third of all cases were uninsured. These disparities should be the basis of future public health safety measures.

Predictive factors of enucleation after open globe injuries.

BACKGROUND: Trauma is the leading cause of enucleations in the USA. Current information regarding open globe injuries (OGI) is based mainly on data from individual tertiary care centers across the country which might skew the findings towards the population served by these level-one trauma centers. The aim of this study is to evaluate the demographics, characteristics, and risk factors of traumatic enucleations in a large data sample. METHODS: Descriptive cross-sectional observational study using the National Inpatient Sample (NIS) Database from 2002 to 2013. Inpatients with traumatic enucleations were identified using ICD-9 codes. Chi-square and logistic regression analyses were used to identify differences between the enucleated and non-enucleated cohorts and to evaluate the predictive factors of enucleation in OGIs. RESULTS: Enucleations were performed in 3020 (6.2%) of 48,563 OGIs identified. The average age in the enucleated cohort for males vs. females was 44.7 vs. 62.2 years. In the USA, the highest number of traumatic enucleations occurred in the 21-40 group (41.8%) and the fewest in the 80+ age group (11.8%). The risk of enucleation decreased across the age groups significantly. Compared with the 21-40 age group, the risk of undergoing enucleation was 15% lower in patients 41 to 60 years of age, 35% in patients 61 to 80, and 40% lower in patients over 80. In total, 5.1% OGIs in women and 6.7% of OGIs in men were enucleated. The risk of enucleation was 29% higher in men than in women. The highest absolute number of enucleations was seen in Whites. Compared with Whites, Blacks had a 63% higher risk of enucleation following an OGI. OGIs with rupture-type injury, endophthalmitis, or phthisis were significantly higher odds to be enucleated. CONCLUSIONS: The risk of enucleation following traumatic OGI significantly increased for patients who were in the 21-40 age group, of Black race, or of male gender; the risk also increased if the injury was a rupture-type or associated with endophthalmitis or phthisis. The risk of depression was 75% higher in enucleated patients versus non-enucleated patients.

Myoepithelioma of the Lower Eyelid.

A 59-year-old woman presented with a 4-year history of a progressively growing, subcutaneous, painless, left lower eyelid mass with associated swelling and discomfort. Excisional biopsy established the diagnosis of a myoepithelioma, a tumor which, to the authors' knowledge, has not been previously reported in the lower eyelid.