Faculty Bibliography

PURPOSE/OBJECTIVE:To evaluate the association between risk of obstructive sleep apnea (OSA) and severity of thyroid eye disease (TED) using a validated OSA screening tool. METHODS:A prospective, observational cohort study was performed. New adult TED patients were offered OSA screening with the Snoring Tired Observed Pressure (STOP)-Bang survey during their initial evaluation. Clinical examination and treatment for TED were standard of care and utilized the International Thyroid Eye Disease Society Vision Inflammation Strabismus Appearance system. At the conclusion of the study period, analysis was performed correlating maximum severity of TED signs and symptoms between high- and low-risk OSA groups. Multivariate logistic and linear regression analyses were also performed to analyze the association between the numerical STOP-Bang score and maximum severity of the potentially actionable clinical features of TED (compressive optic neuropathy, vertical prism deviation, horizontal prism deviation, exophthalmos, vertical fissure height). RESULTS:Eighty-five patients met inclusion criteria. Twenty-eight percent were at high risk of OSA (STOP-Bang score of 3 or higher). When comparing the low- and high-risk cohorts, increased risk of OSA was significantly associated with the development of TED compressive optic neuropathy (p = 0.014), conjunctival injection (p = 0.027), chemosis (p = 0.013), upper eyelid edema (p = 0.024), lower eyelid edema (p = 0.003), eyelid erythema (p = 0.037), and vertical strabismus (p = 0.047). In the multivariate regression analyses, higher STOP-Bang scores correlated with increased risk of TED compressive optic neuropathy (p = 0.006), vertical strabismus (p = 0.019), and higher subjective diplopia scores (p = 0.045). CONCLUSIONS:Increased risk of OSA, as determined by the STOP-Bang survey, is associated with increased severity of multiple clinical features of TED, including TED compressive optic neuropathy and strabismus.

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.

PURPOSE/OBJECTIVE:The reliability of 3 volume measurement strategies was investigated using MRI and a simple method for creating phantom orbit tumors. METHODS:Water-based starch was molded into orbital "tumors" of 3 shapes (sphere, ovoid, diffuse); water displacement was used to calculate volume. "Tumors" were placed into 3D-printed orbit phantoms, MRIs were obtained and volume analysis was performed. Observers measured tumor volume using ellipsoid volume (EV), manual segmentation, and semi-automated segmentation strategies. Intraclass correlation coefficients were calculated comparing observer measurements to true volumes. The coefficient of repeatability determined the percentage of tumor volume change required for each method to detect tumor growth. RESULTS:Intraclass correlation coefficients comparing measured volumes to true volumes using EV, manual segmentation, and semi-automated segmentation were 0.61, 0.98, and 0.99 for spherical, 0.64, 0.97, and 0.98 for ovoid, and 0.18, 0.82, and 0.87 for diffuse tumors. Semi-automated segmentation followed by manual segmentation had the highest correlation between measured and true tumor volume for all 3 tumor geometries. EV had low correlation with true volume for all tumor geometries. Diffuse tumors had high variability and low correlation for all 3 measurement techniques. CONCLUSIONS:This study shows the reliability of 3 strategies to measure orbital tumor volume with MRI based on tumor geometry, using a simple phantom model. EV, the most commonly employed strategy in clinical practice, had low correlation and high variability across tumor shapes. Using manual segmentation and semi-automated segmentation, a measured change in volume greater than 25% may be considered true growth, while the EV strategy required a 40%-400% change in volume to reliably measure tumor growth.

PURPOSE/OBJECTIVE:Well-differentiated neuroendocrine or carcinoid tumors are found most commonly in the gastrointestinal tract. When metastatic to the orbit, they tend to have a propensity for the extraocular muscles. The purpose of this study was to better understand the diversity in presentation of orbital carcinoid disease and to determine predictors for survival. METHODS:In this observational cross-sectional cohort study, data from 8 tertiary orbital practices were compiled. Demographic, clinical, pathologic, American Joint Committee on Cancer stage and grade, imaging, and management data were extracted for all the patients. Descriptive statistics were calculated. Subgroups were compared utilizing analysis of variance analyses and Kaplan-Meier curves. Time to progression and disease-specific and overall mortality were calculated. Comparisons were performed for the following a priori pairs: unknown versus known primary tumor, single versus multiple extraocular muscle involvement, unilateral versus bilateral orbital disease, extraocular muscle versus other orbital involvement, and excisional versus incisional surgery. RESULTS:A total of 28 patients with carcinoid tumors of the orbit were identified. Of these, 57.1% of patients were female, the mean age at diagnosis of the primary tumor was 58.8 years and the mean age at diagnosis of orbital disease was 62.6 years. At primary presentation, all patients were American Joint Committee on Cancer stage III or IV and 21.4% demonstrated carcinoid syndrome. Muscle involvement was noted in 78.6% of patients, and of these, 72% were noted to have single muscle disease. Eight patients had no primary tumor identified; 3 of these 8 demonstrated disseminated disease at the time of diagnosis. The overall 5-year survival rate was 81.8% from diagnosis of primary tumor and 50% from diagnosis of orbital disease. Subgroup analysis revealed that patients with unilateral orbital disease when compared with bilateral orbital disease had a longer progression-free survival and time to death from all causes (p = 0.025). Patients with disease localized to the orbit at presentation had longer time to death than those with disseminated disease. Treatment with surgery, radiation, or octreotide did not appear to affect survival. Patients managed with systemic chemotherapy had a shorter time of survival than the rest of the group. All other subgroup comparisons were not found to be statistically significant. CONCLUSIONS:Neuroendocrine tumors of the orbit represent a wide spectrum of disease, with some cases being part of disseminated disease, while others being localized presentations. This heterogeneity may be responsible for the slightly higher overall survival in these patients than others with metastatic carcinoid tumors in other locations.

A 15-year-old girl presented with a mobile lesion with yellowish hue on the posterior lamella of the right lower eyelid adjacent to the punctum. Four years prior, a lesion thought to be a chalazion was excised from the same location. There was subsequent progressive painless enlargement. The patient ultimately underwent an internal excision of the mass. Histopathology demonstrated infiltrative nests and cords of epithelioid and plasmacytoid cells with abundant eosinophilic myxoid-collagenous stroma along with focal infiltration of skeletal muscle with immunohistochemical staining consistent with an invasive myoepithelioma of mixed-cell type. Given concern for invasive disease, the patient underwent subsequent Mohs resection resulting in a 25% full thickness eyelid defect, which was repaired with direct closure of the wedge defect. There has been no recurrence of the disease for 7 months since the Mohs resection. This case illustrates the atypical presentation of an invasive myoepithelioma of the eyelid in a pediatric patient.

PURPOSE/OBJECTIVE:Prior color-flow Doppler ultrasound studies of the eye have been performed with systems that exceed US Food and Drug Administration permissible ophthalmic ultrasonic energy limits. The authors report a study of orbital vascular malformations using a novel, Food and Drug Administration compliant, ultrafast compound coherent plane-wave ultrasound device to produce power Doppler images. METHODS:Using a Verasonics Vantage 128 ultrasound engine and a user-developed MATLAB program with a 5-MHz linear-array probe, compound coherent plane-wave ultrasound data were collected on patients with orbital vascular malformations. Real-time color-flow Doppler visualized orbital blood flow. Power Doppler images were produced by post-processing compound coherent plane-wave ultrasound data acquired continuously for 2 seconds. RESULTS:Compound coherent plane-wave ultrasound was performed on 3 orbital vascular malformations (1 venolymphatic malformation, 1 infantile hemangioma, and 1 arteriovenous malformation). Compound coherent plane-wave ultrasound produced a high-resolution depiction of orbital blood flow for orbital vascular malformations with high sensitivity to slow flow. CONCLUSIONS:Analysis of blood flow within orbital lesions informs treatment planning. Compound coherent plane-wave ultrasound is an emerging ultrasound modality that falls within the Food and Drug Administration guidelines for use in the orbit and provides information to characterize orbital vascular malformations.

PURPOSE/OBJECTIVE:The purpose of this study was to describe the clinical characteristics, management, and outcomes of orbital osteomas in the pediatric age group, and compare these to osteomas managed in the adult population. METHODS:A retrospective review of 3 cases of rapidly progressive orbital osteomas in young patients was performed. In addition, a systematic review of the literature following Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines was conducted to describe the clinical characteristics, management, and outcomes of orbital osteomas in the pediatric age group. The PubMed/MEDLINE database was searched in December 2019 for studies reporting data on patients with orbital osteomas. RESULTS:Fifty-nine patients were included in this pediatric review, 39 male patients (68%), 18 female patients (32%), and 2 cases with unreported genders. Average age was 15.9 years with a range of 4-21 years. Average osteoma size measured 30.7 mm with a range of 9-60 mm. Adult osteoma size for comparison was smaller than 10 mm in majority of cases. The most common location of pediatric osteomas was the ethmoid sinus. The frontal sinus was the most common location in adult patients. Ninety-seven percent of reported pediatric cases required surgical intervention, compared with only 6.5% in adults. CONCLUSIONS:Orbital osteomas in younger patients are larger when identified, grow faster, are more likely to be symptomatic, and more likely to require surgical intervention compared with those identified in older patients. We recommend close monitoring of osteomas identified in young patients as well as counseling regarding the potential need for future resection.

Pediatric orbital and skull base surgery comprises a wide array of tumors. An understanding of the location of the lesion, nature of the disease, and surrounding anatomy is paramount to surgical planning in these small spaces. The goals of pediatric skull base surgery are to avoid injury to the surrounding structures, minimize cosmetic deformities, and remove some or all of the tumors based on anticipated pathology and biologic cost of removal. Safe surgery on many of these tumors requires an understanding of the location of the lesion relative to the optic nerve or orbit. This is particularly challenging because the dimensions of the orbital confines change continuously as one navigates from rostral to caudal. Management of these tumors may require a multidisciplinary approach including orbital surgery, neurosurgery, otolaryngology, oral maxillofacial surgery, plastic surgery, and interventional neuroradiology.