Prevalence and clinical features of orbital vascular anomalies in children.

PURPOSE: To report the prevalence, clinical characteristics, and natural history of orbital vascular anomalies diagnosed among children over a 50-year period. METHODS: The medical records of all patients <19 years diagnosed with any form of an orbital vascular anomaly (OVA) at Mayo Clinic, Rochester, Minnesota from January 1 1966, through December 31 2015, were retrospectively reviewed. RESULTS: A total of 109 children were diagnosed with OVA during the 50-year period, of which 24 were from Olmsted County, MN, yielding a birth prevalence of 1 in 4,305 live births. The median age at diagnosis for the 109 patients was 1.2 years (range, 0-17.9 years) and 67 (61.5%) were female. Common presenting symptoms included proptosis in 80 (73.4%) patients, abnormalities in skin color in 45 (41.3%) patients, and pain in 18 (16.5%) patients. There were 55 (50.5%) vascular malformations [50 (91%) low-flow lymphatic malformations, 3 (5.5%) high-flow arteriovenous malformations, and 2 (3.5%) low-flow venous malformations] and 54 (49.5%) vascular tumors [53 (98%) capillary hemangiomas and 1 (2%) kaposiform hemangioendothelioma]. During a mean follow-up of 5.95 years (range 0-27.7 years), amblyopia and/or strabismus were diagnosed in 46 (43.4%) patients. CONCLUSIONS: Capillary hemangiomas and low-flow lymphatic malformations comprise most of the orbital vascular anomalies in this cohort of children. Amblyopia and strabismus are common sequelae, highlighting the importance of early diagnosis and appropriate management.

Psychosocial and mental health disorders among a population-based, case-control cohort of patients with congenital upper eyelid ptosis.

BACKGROUND/AIM: Recent studies have demonstrated adverse psychosocial and mental health disorders among children with ocular disorders. The mental health burden of children with simple congenital ptosis, however, is unknown. The purpose of this study was to compare the psychosocial and mental health findings of children with simple congenital ptosis with controls. METHODS: The medical records of all children (<19 years) diagnosed with simple congenital ptosis from 1 January 1965 through 31 December 2004 while residing in Olmsted County, Minnesota were retrospectively reviewed for psychosocial and mental health morbidity. One-to-one randomly selected age-matched and gender-matched controls from the same population were similarly reviewed. RESULTS: 81 children with ptosis were diagnosed at a mean age of 3.2 years (range, 1 month-16 years), 35 (43.2%) of whom were girls. An adverse psychosocial development was diagnosed in 41 (50.6%) patients with simple congenital ptosis monitored to a mean age of 21.4 years, compared with 26 (32.5%) controls (p=0.02). A mental illness was diagnosed in 31 (38.3%) patients with ptosis compared with 16 (20%) controls (p=0.01). Children with ptosis were 2.5 times more likely than controls to develop a mental illness and 2.1 times more likely to develop a psychosocial maladjustment. Patients with ptosis were also significantly more likely to have more mental health disorders (p=0.02) and a longer duration of psychotropic medication use (p=0.005). CONCLUSIONS: Children diagnosed with simple congenital ptosis in this population had significantly greater psychosocial and mental health morbidity compared with controls. Children with ptosis may benefit from early psychosocial intervention.

A Case of Chronic Intranasal Cocaine Abuse Masquerading and Exacerbating Underlying Orbital IgG4-Related Disease.

Chronic cocaine use may lead to widespread intranasal inflammation and necrosis. Cases of cocaine use affecting the orbit have been reported in the literature with a clinical spectrum ranging from inflammation-induced p-anti-cytoplasmic neutrophil autoantibodies positive vasculitis to severe midline destructive lesions resulting in orbital apex syndrome. Here, we present a case of chronic intranasal cocaine abuse with midline destruction that initially obscured diagnosis of, and is hypothesized to have exacerbated, underlying IgG4-Related Disease (IgG4-RD) of the orbit over a 2-year period.

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.

Frequency and Clinical Course of Residual Orbital Masses After Treatment of Orbital Rhabdomyosarcoma.

PURPOSE: To evaluate the frequency and clinical course of residual orbital masses on imaging studies after multimodality treatment for orbital rhabdomyosarcoma. DESIGN: Retrospective case series. METHODS: We reviewed records of patients with primary orbital rhabdomyosarcoma who underwent chemotherapy and radiotherapy after surgical biopsy or debulking at 4 US centers during 1998-2019. Demographics, histologic subtype, tumor response 12 weeks after chemotherapy initiation and after completion of all treatment, and imaging findings were analyzed. RESULTS: Thirty-two patients met inclusion criteria. Twenty-two were male, and 30 were younger than 18 years. Histologic subtype was embryonal in 22 patients, alveolar in 8, and mixed embryonal/alveolar in 2. Median follow-up time was 46 months (range, 4.9-199 months). Two patients died. Twenty-seven patients had reliable end-of-treatment imaging findings, of whom 9 had a residual mass. Three residual masses disappeared spontaneously (by 4, 32, and 53 months), 2 remained at last contact, at 2 and 7 years of follow-up, and 3 were excised; 1 progressed and underwent an exenteration. Complete response at 12 weeks was associated with complete response at the end of treatment (P < .001). Patients with T1 or T2 tumor at presentation were more likely to have complete response at last contact than were those with T3 or T4 tumor (P < .05). Biopsy type (incisional or excisional) was not associated with response to treatment at any time point. CONCLUSION: A residual orbital mass on imaging may be present after multimodality treatment in approximately one-third of patients. Resolution without biopsy or excision varied from months to years.

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.

Swallow-Induced Eyelid Myokymia: A Novel Synkinesis Syndrome.

Herein, we describe a novel manifestation of facial nerve synkinesis, swallow-induced eyelid myokymia, and hypothesise that this phenomenon is due to synkinetic facial nerve innervations of the stylohyoid-posterior digastric complex of suprahyoid muscles and orbicularis oculi muscle. In our patient's case, onabotulinum toxin A treatment provided good therapeutic response. Swallow-induced eyelid myokymia is a unique and previously unreported variety of facial nerve synkinesis.

Implementation of a Novel Near Visual Acuity Chart in an Emergency Department Setting.

PURPOSE: The quality of visual acuity (VA) measurement in emergency department (ED) settings can be affected by patient immobility and lack of standardized testing conditions. We implemented a previously validated, novel VA chart, the Runge Sloan letter near card, in a hospital ED and evaluated its impact on frequency and consistency of VA testing. METHODS: Two hundred seventeen hospital ED ophthalmology consult records from December 1, 2016, to November 15, 2017, were evaluated in an IRB-approved protocol. Frequency of VA measurement and agreement between nonophthalmic ED technicians and ophthalmology physicians-in-training were assessed. RESULTS: Implementation of the Runge card saw missed technician VA evaluations decrease from 36% (43/120) to 21% (20/97) of ophthalmic consults (p = .01), without significant change in agreement of VA measurements. After implementation, the proportion of VA measurements differing between technicians and residents by ≤2 lines was 51%; with pinhole testing, it improved to 64% (p < .05). In patients with good VA of >20/80, pinhole increased agreement from 58% to 73% (p < .05). CONCLUSIONS: Implementation of the Runge card was associated with improved frequency of VA measurement and, when combined with pinhole testing, increased agreement rates. Our findings suggest utility of training in the use of the Runge card in ED settings.

Incidence and clinical characteristics of pediatric eyelid retraction.

PURPOSE: To describe the incidence and clinical characteristics of upper and lower eyelid retraction in children. METHODS: The medical records of all pediatric patients (<19 years of age) diagnosed with upper and/or lower eyelid retraction from January 1, 1976, through December 31, 2010, were retrospectively reviewed. RESULTS: A total of 85 patients were diagnosed during the 35-year period, of whom 15 were residents of Olmsted County, Minnesota, yielding an annual age- and sex-adjusted annual incidence of 1.38 per 100,000 persons (95% CI, 0.70-2.05), or 1 in 72,463. Upper eyelid retraction was documented in 38 patients (45%; 24 unilateral and 14 bilateral); lower, in 25 (29%; 12 unilateral and 13 bilateral); and both upper and lower in 18 (21%; 3 unilateral and 15 bilateral). It was not recorded in 4 patients. The most common causes of eyelid retraction were thyroid eye disease (48 [56%]), primary congenital eyelid retraction (11 [13%]), and trauma (9 [11%]). Although there were no cases of visual impairment secondary to eyelid retraction, tearing, ocular surface irritation, and photophobia were noted in 38 patients (45%). Nineteen patients (22%) underwent surgical eyelid correction. CONCLUSIONS: Pediatric eyelid retraction is relatively rare, occurring in approximately 1 in 72,000. The leading causes of childhood eyelid retraction in this cohort were thyroid eye disease, primary congenital eyelid retraction, and trauma. No visual disturbances due to eyelid retraction were noted, and approximately 1 in 5 patients required corrective eyelid surgery.

Pediatric Periocular Dermoid Cysts: Incidence, Clinical Characteristics, and Surgical Outcomes.

PURPOSE: To report the incidence, clinical findings, and surgical outcomes of periocular dermoid cysts diagnosed among children over a 20-year period. METHODS: All patients ≤5 years of age, who were diagnosed with a periocular dermoid cyst in Olmsted County, Minnesota from 1986 to 2005 were reviewed to determine the population incidence, clinical presentation, and management outcomes. RESULTS: A total of 54 children were diagnosed with a periocular dermoid cyst during the 20-year period, yielding a birth incidence of 1 in 638 live births. The mean age at diagnosis was 12 months (range, 1 month-60 months) and 29 (53.7%) were female. A total of 44 cysts (81.5%) occurred in the superotemporal orbital rim, 6 (11.1%) in the superonasal orbital rim, 3 (5.6%) in other periocular areas, and one (1.9%) within the orbit. A total of 34 (63%) children had an ophthalmic exam, all without amblyopia or other ocular sequelae. A total of 48 (88.9%) patients underwent surgical excision with 5 (10.4%) having documented intra-operative cyst rupture, none of whom had post-operative complications. Two (4.2%) other patients were, however, noted to have lesion recurrence following surgical intervention. CONCLUSIONS: Periocular dermoid cysts occur in approximately 1 in 650 live births and most commonly occur in the superotemporal region of children aged 1 year or less. Ocular sequelae are rare and surgical excision often yields excellent results.

Local Disease Control in Ocular Adnexal Lymphoproliferative Disorders: Comparative Outcomes of MALT Versus Non-MALT Histologies.

INTRODUCTION: Ocular adnexal lymphoproliferative disorders (OALDs) are almost exclusively of B-cell origin, with the majority being extra-nodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT). The comparative efficacy of involved field radiation therapy (IFRT) in MALT vs. non-MALT OALDs is not known. MATERIALS AND METHODS: We present a single-center, large cohort, retrospective study of the efficacy of IFRT in OALDs. Failure-free survival (FFS), complete remission, and local, regional, and distant failure were determined for 112 patients with MALT OALDs (n = 71) and non-MALT OALDs (n = 41) cohorts. RESULTS: Fifty-six patients with MALT OALD and 26 patients with non-MALT OALD received IFRT only (without any planned concurrent or sequential systemic chemothereapy or chemo-immunotherapies). Among the OALD cohorts treated with only IFRT, complete remission was achieved in 49 (87.5%) patients in the MALT cohort and 23 (88.4%) in the non-MALT cohort (P = .99). Clinically, resolution of symptoms occurred in 83.3% and 93.3% of the patients in the MALT and non-MALT cohorts, respectively. Local failure occurred in 4 (7.1%) patients in the MALT cohort, compared with 4 (15.3%) patients in the non-MALT cohort (P = .24). Regional failure (or extra-orbital failure) occurred in 5 (8.9%) patients in the MALT cohort and in 3 (11.5%) patients in the non-MALT cohort (P = .71). Distant failure was reported in 1 (1.7%) and 2 patients (7.6%) in the MALT and non-MALT cohorts, respectively (P = .18). The median follow-up of survivors was 5.1 years (range, 0.1-22.5 years) in the MALT cohort and 3.9 years (range, 0.1-22.9 years) in the non-MALT cohort. The 5-year and 10-year FFS was 95% (95% confidence interval [CI], 88%-100%) and 83% (95% CI, 70%-98%) for the ocular MALT and 67% (95% CI, 48%-94%) and 56% (95% CI, 34%-91%) for the non-MALT cohorts, respectively (log rank for P = .025). On multivariate analyses, age (hazard ratio [HR], 1.06; 95% CI, 1.10-1.12; P = .03), presence of non-MALT histology (HR, 13.9; 95% CI, 2.05-94.4; P = .007), and radiation dose < 30.6 Gy (HR, 5.27; 95% CI, 1.14-24.3; P = .03) were associated with worse FFS. The 5-year and 10-year overall survival was 92% (95%, CI 83%-100%) and 80% (95% CI, 66%-96%) for the MALT and 78% (95% CI, 61%-100%) and 62% (95% CI, 38%-100%) for the non-MALT cohorts, respectively (P = .80). CONCLUSION: Our results reveal that IFRT provided excellent disease control with superior FFS in the MALT cohort when compared with the non-MALT group.

Strabismus in childhood eyelid ptosis.

PURPOSE: To report the prevalence and causes of strabismus in children with eyelid ptosis diagnosed in a well-defined population over a 40-year period. DESIGN: Retrospective, population-based cohort study. METHODS: We retrospectively reviewed the charts of 107 patients (<19 years) for the prevalence and causes of strabismus in children who were diagnosed with childhood eyelid ptosis as residents of Olmsted County, Minnesota, from January 1, 1965, through December 31, 2004. RESULTS: Strabismus was diagnosed in 20 (18.7%) of the 107 patients with childhood ptosis. Of the 81 patients, 8 (9.9%) were diagnosed with simple congenital ptosis and had strabismus, of which there were 4 (4.9%) cases of exotropia and 4 (4.9%) cases of esotropia. There were no cases of isolated vertical deviation. CONCLUSIONS: Strabismus occurred in 1 of 5 children diagnosed with any form of childhood ptosis in this population-based cohort. Strabismus affected approximately 1 of 10 patients diagnosed with simple congenital ptosis, and a predominance of isolated horizontal deviations was equally divided between esotropia and exotropia.

A clinicopathologic case study of two patients with pediatric orbital IgG4-related disease.

The purpose of this report is to describe the clinical, radiographica, and histopathologic findings in two pediatric patients with orbital IgG4-related disease.

Anatomical position of hyaluronic acid gel following injection to the eyebrow.

PURPOSE: To examine with histology the anatomical location of hyaluronic acid gel injected to the eyebrow of cadaver specimens. METHODS: The authors dissected 5 fresh hemifacial cadaver specimens following preperiosteal injection of hyaluronic acid gel to the eyebrow. Following tissue fixation, full-thickness soft-tissue sections were obtained followed by histologic examination. RESULTS: Histologic examination revealed the location of hyaluronic acid gel at the intended preperiosteal plane in all 5 specimens. Very dense retro-orbicularis oculi fat septa appeared to limit the anterior displacement of filler in each specimen. CONCLUSIONS: This study provides a greater understanding of the anatomical barriers and boundaries that help to determine, in part, the anatomical position of hyaluronic acid gel when injected to the preperiosteal eyebrow. The high degree of histologically confirmed consistency of product location of eyebrow injections noted in this study stands in contrast to the variability of position of gel injected in the infraorbital hollows.

Tumors masquerading in patients with thyroid eye disease.

Thyroid eye disease (TED) is the most common cause of proptosis in adults. The external manifestations of TED are characteristic and the diagnosis is typically made without imaging. Although there are multiple descriptions of primary and secondary orbital tumors initially mistaken for TED in the literature, there are limited reports detailing the findings of patients with long-standing TED whom developed an orbital tumor at a later date. Herein, we present a 6-year retrospective multi-center report of three patients with long-standing TED who developed an initially unsuspected orbital or cavernous sinus tumor.

Modified full-thickness anterior blepharotomy for upper eyelid retraction in children.

Graded full-thickness anterior blepharotomy has been used to treat eyelid retraction in adult patients with thyroid eye disease. We report 2 children diagnosed with upper eyelid retraction who underwent a modified full-thickness anterior blepharotomy. In both cases, symptoms resolved and cosmetically acceptable outcomes were achieved.