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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.

A 47 year-old female with lymphangioleiomyomatosis developed right periorbital pain worsened while chewing, six months prior. Neuroimaging demonstrated a heterogenous inferotemporal right orbital mass extending through the inferior orbital fissure into the temporalis fossa, with flow voids. Given the patient's past medical history, the lesion was presumed to be a perivascular epithelioid cell tumor, a manifestation of lymphangioleiomyomatosis. A lateral orbitotomy revealed a well-circumscribed bluish-red lesion with areas of hemorrhage that was resected in total. Histopathology, however, was consistent with a thrombosed orbital arteriovenous malformation likely arising from the zygomaticotemporal neurovascular bundle. Lymphangioleiomyomatosis is a rare progressive disease that causes cystic destruction of the lungs and is frequently associated with extrapulmonary tumor infiltration, typically of the kidney and liver. Lymphangiomyoleiomyomatosis cell pathogenesis includes a pro-angiogenic micro-environment, classically expressing vascular endothelial factor-C and -D, which we postulate may have contributed to the development of the orbital arteriovenous malformation.

PURPOSE/OBJECTIVE:To measure orbital cavernous hemangioma size using 3 segmentation methods requiring different degrees of subjective judgment, and to evaluate interobserver agreement using these methods. METHODS:Fourteen patients with orbital cavernous hemangiomas were included in the study. Pretreatment T2-weighted MRIs were analyzed by 2 observers using 3 methods, including 1 user-dependent image segmentation method that required high degrees of subjective judgment (ellipsoid) and 2 parameter-dependent methods that required low degree of subjective judgment (GrowCut and k-means clustering segmentation). Interobserver agreement was assessed using Lin's concordance correlation coefficients. RESULTS:Using the ellipsoid method, the average tumor sizes calculated by the 2 observers were 1.68 ml (standard deviation [SD] 1.45 ml) and 1.48 ml (SD 1.19 ml). Using the GrowCut method, the average tumor sizes calculated by the 2 observers were 3.00 ml (SD 2.46 ml) and 6.34 ml (SD 3.78 ml). Using k-means clustering segmentation, the average tumor sizes calculated by the 2 observers were 2.31 ml (SD 1.83 ml) and 2.12 ml (SD 1.87 ml). The concordance correlation coefficient for the ellipsoid, GrowCut, and k-means clustering methods were 0.92 (95% CI, 0.83-0.99), 0.12 (95% CI, -0.21 to 0.44), and 0.95 (95% CI, 0.90-0.99), respectively. CONCLUSIONS:k-means clustering, a parameter-dependent method with low degree of subjective judgment, showed better interobserver agreement compared with the ellipsoid and GrowCut methods. k-means clustering clearly delineated tumor boundaries and outlined components of the tumor with different signal intensities.

Purpose/UNASSIGNED:We report a case a pediatric patient with an eyelid lesion found to be a basaloid follicular hamartoma. Observations/UNASSIGNED:A six-year-old female with juvenile diabetes who presented with a benign eyelid lesion harboring an aberrant eyelash. Conclusions and Importance/UNASSIGNED:Basaloid follicular hamartoma is a rare benign neoplasm arising from hair follicles. These lesions can resemble basal cell carcinomas and require complete excision.