Radiologic Features of Well-circumscribed Orbital Tumors With Histopathologic Correlation: A Multi-center Study.

PURPOSE: To delineate specific imaging characteristics of solitary fibrous tumors, schwannomas, cavernous venous malformations, and well-circumscribed orbital lymphoma. METHODS: Patients undergoing excisional biopsy of solitary fibrous tumor, schwannomas, cavernous venous malformations, or well-circumscribed orbital lymphoma with preoperative MRIs available for review were identified at 3 academic centers in the United States and Australia. An exploratory statistical analysis was performed to identify important radiologic features, which were subsequently included in a random forest model. Histopathologic correlates were evaluated in representative cases. RESULTS: A total of 91 cases were included with a mean age of 52.9 ± 17.2 years. Nearly all solitary fibrous tumors were located in the anterior or mid orbit (87.5%) and they more commonly demonstrated intralesional heterogeneity on T2-weighted imaging (45.5%) (p < 0.01). Compared with the other tumors, schwannomas tended to be intraconal (66.7%) and were often in the mid or posterior orbit (83.4%) (p < 0.01). Cavernous venous malformations characteristically demonstrated progressive contrast enhancement (93.9%; p < 0.01). Most lesions in all 4 groups were hypointense on T1-weighted imaging (80%-100%; p = 0.14) and only well-circumscribed orbital lymphoma tended to also be hypointense on T2 (81.8%) (p < 0.01). Finally, cases of lymphoma had significantly lower apparent diffusion coefficient ratios (0.9 ± 0.2) (p < 0.001), while the other 3 groups were not significantly different from one another (cavernous venous malformations: 1.8 ± 0.4; schwannomas: 1.8 ± 0.5; and solitary fibrous tumor: 1.6 ± 0.6) (p = 0.739). CONCLUSIONS: Key features that aid in the differentiation of these 4 tumors from one another include T2 intensity and homogeneity, early contrast-enhancement pattern, and ADC ratio.

Dedifferentiated liposarcoma of the orbit.

Purpose: To present a rare case of dedifferentiated liposarcoma of the orbit. Observations: A 61-year-old male complained of left-sided proptosis, diplopia, and limited ocular motility for two years. Biopsy results at that time were suggestive of an atypical lipomatous neoplasm. Ten years later, he presented with increase in size of the mass and worsening of his symptoms. Imaging showed a multi-lobulated mass in the left orbit involving the intraconal, medial, and anterior orbit. Decompression and orbitotomy with biopsy were performed to debulk the mass. Pathology showed a low-grade well-differentiated liposarcoma and the patient was monitored thereafter annually. Eight years later, he complained of persistent proptosis and mass effect from the tumor resulting in ptosis and diplopia and underwent orbital exenteration. Histopathological analysis of the exenterated orbit revealed a focal area of dedifferentiated liposarcoma. Conclusions and importance: Dedifferentiation of an orbital mass can occur as a late complication years after the diagnosis of well-differentiated liposarcoma. Compared to the previously published cases of orbital liposarcoma, this presentation shows a prolonged timeline prior to dedifferentiation (18 years after initial diagnosis). Symptoms of growth or invasive features could indicate dedifferentiation and should warrant a biopsy.

Visual Outcomes Following Surgical Debulking in Exophytic Optic Nerve Sheath Meningiomas.

PURPOSE: To report a case of a globular primary optic nerve sheath meningioma managed surgically with improvement in vision and review the literature for outcomes of purely intraorbital exophytic-globular primary optic nerve sheath meningiomas managed surgically. METHODS: A literature review was conducted using Google Scholar and PubMed with the search terms "primary optic nerve sheath meningioma," "surgery," "exophytic," and "globular." Articles were included if they were available in English. Individual cases from the reviewed articles were included if the tumor was purely intraorbital with a globular or exophytic morphology, was managed with total or subtotal surgical excision, and visual outcomes were reported. Cases were excluded if the tumor extended intracanalicularly or intracranially, tumor morphology was unknown, or surgical management consisted of biopsy, optic nerve sheath decompression, or optic canal decompression rather than tumor debulking. RESULTS: A total of 28 patients with intraorbital globular-exophytic primary optic nerve sheath meningiomas managed surgically have been reported in the literature. Vision improved in 29% (n = 8/28) and remained stable in 43% (n = 12/28) of patients. Furthermore, patients with good (Snellen notation ≥ 0.5) vision (n = 10) typically retained good vision postoperatively and at follow-up, with 1 patient experiencing a decline to poor (Snellen ≤0.1) vision at the last follow-up (92 months postoperatively). Similarly, patients with fair (Snellen notation >0.1 and <0.5) vision (n = 5) often improved to good vision (n = 3) or stayed at fair vision (n = 1), with 1 declining to poor vision at postoperative hospital discharge. CONCLUSIONS: Surgical management of exophytic or globular optic nerve meningiomas does not universally lead to vision loss and may be appropriate in select patients.

Transorbital Endoscopic Approach to the Pterygopalatine Fossa: A Less Invasive Alternative to Traditional Access.

PURPOSE: The purpose of this article and accompanying video is to demonstrate a transorbital endoscopic approach for accessing the pterygopalatine fossa (PPF). This technique does not require a skin incision, avoids dissection of critical neurovascular structures, and utilizes a comparatively small osteotomy. The 2 cases presented in this article highlight the utility of a transorbital endoscopic approach for accessing an anatomic region that has traditionally required more invasive techniques to reach. METHODS: Description of surgical technique with 2 illustrative clinical cases and accompanying surgical video. RESULTS: Surgical technique: A trans-conjunctival approach is taken to the inferior orbital rim, and a subperiosteal dissection is propagated posteriorly. The bone of the posterior orbital floor is then deroofed, and the superior portion of the posterior wall of the maxillary sinus is removed, allowing access to the PPF for an incisional biopsy. CASE: A 76-year-old male with a history of left cheek squamous cell carcinoma presented with progressive V2 paresthesia and an abnormally enhancing lesion in the left PPF on MRI. CASE: A 58-year-old male with no significant medical history presented with left facial numbness (V1-V3), ptosis, an abduction deficit, and decreased hearing. Contrast-enhanced MRI demonstrated an abnormally enhanced lesion in the left PPF extending to Meckel's cave.The transorbital approach described was used to successfully obtain a diagnostic biopsy in both cases. CONCLUSIONS: These cases highlight the utility of a transorbital endoscopic approach to the PPF as a less morbid alternative to traditional access. Patient selection is key to identifying appropriate cases.

Orbital Decompression: Conceptual Approach for Orbital Volume Expansion.

PURPOSE: To review the advances in the surgical techniques of orbital decompression. METHODS: A literature review of orbital decompression surgery and experience-based consideration of bony areas for decompression. RESULTS: In the 100-year-plus span of orbital decompression literature, multiple incisions and multiple bone and fat removal strategies have been described. In general, bone removal has been conceptualized as consisting of 4 walls of the orbit. However, the orbital bony anatomy is more subtle than a simple box, and considering 6 areas of potential bony removal allows a more nuanced paradigm for clinical decision-making and for understanding various technique descriptions. Historical and current techniques, and surgical planning and decision-making, are described from the perspective of a 6 area paradigm. Potential complications are reviewed. CONCLUSION: A conceptual framework for orbital decompression focusing on 6 potential areas of bone removal provides a nuanced and flexible paradigm for understanding and describing surgical techniques, and for designing individualized surgery. Orbital decompression surgery should be customized to the patient's anatomy and symptoms.

Teprotumumab and Orbital Decompression for the Management of Proptosis in Patients With Thyroid Eye Disease.

PURPOSE: To compare outcomes of patients with thyroid eye disease treated with teprotumumab or orbital decompression, or both in sequence. METHODS: Patients with thyroid eye disease and treated with decompression, teprotumumab, or both were included. Four groups were defined: decompression only, teprotumumab only, teprotumumab first with decompression later, and decompression first with teprotumumab later. The primary outcome was change in exophthalmometry. Secondary outcomes included change in extraocular muscle motility, strabismus, diplopia, and side effects. RESULTS: One hundred and thirty-nine patients were included. The mean duration for early follow-up was 1.2 months for both decompression and teprotumumab groups. The mean late follow-up was 14.4 and 8.2 months for the decompression and teprotumumab groups respectively. Mean change in exophthalmometry was significantly greater for the decompression group (3.5 mm) compared with teprotumumab (2.0 mm) at late follow-up. Improvement in total extraocular muscle restriction was significantly greater in the teprotumumab group (14.7 degrees) than in the decompression group (2.6 degrees). The teprotumumab group had a significantly higher percentage of patients with diplopia score >1 at baseline and late follow-up (p < 0.01) compared with the decompression group. Additional treatment with teprotumumab or decompression when previously treated with the opposite had similar proptosis reduction effect as that therapy alone. CONCLUSIONS: Surgical decompression has a greater proptosis reduction effect than teprotumumab, whereas teprotumumab better improves extraocular muscle motility. The addition of teprotumumab or decompression to a previous course of the opposite adds a similar effect to the supplemental treatment alone.

Orbital Involvement in Acute Adult Leukemias: Case Series and Review of Literature.

Orbital involvement in acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL) is well-described in children but is uncommon in adults. This series reports 2 adult patients with orbital leukemic involvement and summarizes the existing literature. A 37-year-old male with recently diagnosed AML underwent induction therapy and subsequently developed a tan-pink colored sub-conjunctival lesion in the left eye. Incisional biopsy confirmed AML. A 35-year-old male with history of ALL presented with left-sided orbital mass. Fine needle aspiration biopsy confirmed ALL. Literature review of adult-onset orbital leukemia yielded 29 cases of AML and 3 cases of ALL. Orbital involvement of acute adult-onset leukemia tends to be unilateral, presents in the extraconal space and can occur at any point during systemic leukemic disease. Chemotherapy is the mainstay of treatment, often in combination with radiation and/or hematopoietic stem cell transplant.

Banking Autologous Tarsus: A Simple Technique for Maximizing Preservation of Healthy Tissue.

A 28-year-old female with congenital left-sided ptosis presented after 3 prior surgeries elsewhere. Although centrally her margin to reflex distance1 was 3 mm, persistent ptosis was noted laterally. In an effort to improve the symmetry of her eyelid contour, a lateral tarsectomy was performed. As the authors worried this might worsen her dryness, the decision was made to bank the excised tarso-conjunctival tissue in case additional revision surgery is required at a later date. To accomplish this, a conjunctival incision was created at the inferior tarsal margin in the ipsilateral lower lateral eyelid and the excised upper eyelid tarso-conjunctival tissue was secured in this pocket. Four months postoperatively, the banked tissue appeared healthy and the upper eyelid contour was improved. This technique may be most helpful in multi-operated situations, where the chance of future revision is not insignificant.

External carotid artery to ophthalmic artery flow associated with internal carotid artery stenosis.

PURPOSE: One of the most devastating complications of facial filler injection is sudden ischemic blindness. However, its mechanisms and predisposing factors are poorly understood. The purpose of this study was to investigate the prevalence of external carotid artery (ECA) to ophthalmic artery (OA) anterograde flow in patients with internal carotid artery (ICA) stenosis and in a control population without carotid disease. METHODS: In this cross-sectional cohort study, two groups of patients who underwent catheter cerebral angiography over a 5-year period were identified: patients with symptomatic ICA stenosis and a control group of patients with refractory epistaxis undergoing embolization. Angiograms were reviewed by an interventional neuroradiologist. The primary outcome measure was the presence of ECA to OA flow, defined as choroidal blush before filling of the circle of Willis. Secondary outcome measures included the percentage and location of ICA stenosis and ECA anastomotic branches involved. RESULTS: The study included 149 patients with ICA stenosis and 69 control patients. ECA to OA flow was more prevalent in patients with ICA stenosis (34.9%) compared to controls (2.9%) (p < .001). Logistic regression demonstrated that for each 10% increase in ICA stenosis over 70%, there was 2.8 times increased risk of ECA to OA flow (p < .001). CONCLUSIONS: ECA to ICA anterograde flow can be demonstrated in approximately 3% of control patients and in over 1/3 of patients with symptomatic carotid stenosis. This provides a plausible pathway for small filler particles to pass with blood flow from the facial to the ophthalmic circulation.

Optimizing Management of Asymmetric Ptosis: A Comparison of Three Posterior Approach Resection Algorithms.

PURPOSE: The purpose of this study was to compare the efficacy of 3 resection algorithms in the management of patients with asymmetric ptosis. METHODS: Patients undergoing bilateral Muller's muscle-conjunctival resection (MMCR) were identified. Standardized preoperative clinical photographs were examined and margin reflex distance 1 (MRD1) was measured using ImageJ. Patients presenting with ≥1 mm of asymmetry in MRD1 were included. Three groups were identified: variable (4:1 ratio, with the lower side receiving a greater resection), fixed (7 mm resection bilaterally), and tarsectomy (7 mm bilaterally + 1 mm of tarsus resected on the lower preoperative side). Postoperative MRD1 was measured from photographs obtained 3 months after surgery. The primary outcome was postoperative asymmetry. RESULTS: A total of 95 patients with a mean age of 71.0 ± 11.0 years were included. There was no significant difference in age ( p = 0.277) or length of follow-up ( p = 0.782) between the groups. Although the fixed tarsectomy group had significantly greater preoperative asymmetry ( p = 0.001), there was no significant difference in postoperative asymmetry ( p = 0.166). On multivariate analysis, preoperative asymmetry was the only significant predictor of postoperative asymmetry ( p < 0.001). Specifically, the surgical group was not a predictor of the primary outcome ( p = 0.723). CONCLUSIONS: Resection amount and technique did not predict postoperative outcomes in cases of asymmetric ptosis. This may support the hypothesis that changes in eyelid position and symmetry following MMCR is due to a dynamic system, rather than as a result of purely mechanical forces.

Extrascleral extension of choroidal melanoma after iodine-125 brachytherapy treatment: a case series.

BACKGROUND/OBJECTIVES: The purpose of this study is to report cases of choroidal melanoma that developed extrascleral tumour recurrence after treatment with iodine-125 brachytherapy. SUBJECTS/METHODS: In this single-institution retrospective observational case series, all instances of biopsy-confirmed orbital melanoma after known intraocular melanoma were reviewed. Tumour characteristics, clinical course, time to recurrence, cytogenetics of initial tumour and recurrence, and presence of intraocular recurrence were documented. RESULTS: Five cases of orbital melanoma following treatment with plaque radiotherapy are described. Tumour staging was Ia (1), IIa (2), and IIb (2). The primary lesion in four of the five appeared to have undergone complete regression for an average of 2 years, with the orbital melanoma developing after this interval. Recurrence of the intraocular tumour was seen in conjunction with an extrascleral component in two cases. Four cases ultimately underwent enucleation or exenteration; three had evidence of direct extension of tumour through the sclera. Four cases in this series had molecular characteristics associated with high metastatic risk (three patients with monosomy 3, one with BAP1 mutation). CONCLUSIONS: High-risk tumour biology may predispose to late appearance of extrascleral melanoma despite optimal treatment and adequate control of the intraocular tumour. Extended follow-up with detailed orbital examination and imaging is recommended for this population.

Endoscopic endonasal approach for resection of a recurrent spheno-orbital meningioma resulting in complete resolution of visual symptoms: A case report and review of literature.

PURPOSE: Spheno-orbital meningiomas are rare tumors, accounting for up to 9% of all intracranial meningiomas. Patients commonly present with proptosis, and visual deficits. These slow growing tumors are hard to resect due to extension into several anatomical compartments, resulting in recurrence rates as high as 35-50%. Although open surgical approaches have been historically used for resection, a handful of endoscopic approaches have been reported in recent years. We aimed to review the literature and describe a case of spheno-orbital meningioma with severe vision loss which was resected with an endoscopic endonasal approach achieving complete resolution of visual symptoms. METHODS: A systematic review of literature was conducted in accordance with the PRISMA guidelines. PubMed, Cochrane, and Web of Science databases were queried for spheno-orbital meningiomas resected via an endoscopic endonasal approach. Furthermore, the presentation, surgical management, and post-operative outcomes of a 53-year-old female with a recurrent spheno-orbital meningioma are described. RESULTS: The search yielded 26 articles, of which 8 were included, yielding 19 cases. Average age at presentation was 60.5 years (range: 44-82), and 68.4% of patients were female. More than half of the cases achieved subtotal resection. Common complications associated with endoscopic endonasal surgery included CN V2 or CN V2/V3 hypoesthesia. Following surgical intervention, visual acuity and visual field remained stable or improved in the majority of the patients. CONCLUSION: Endoscopic approaches are slowly gaining momentum for treatment of spheno-orbital meningiomas. Further studies on the clinical benefits of this approach on patient outcomes and post-operative complications is warranted.

Hyperostosis associated with orbital vascular malformation.

A previously healthy adult male presented with a slowly enlarging orbital mass associated with 5 mm of non-pulsatile proptosis. On imaging, a soft tissue lesion with avid contrast enhancement and associated bony hyperostosis was noted. The lesion and hyperostotic bone were surgically debulked, and significant arterial bleeding was noted intraoperatively consistent with an arteriovenous malformation. Histopathologic analysis revealed a vascular malformation with enhanced microvasculature infiltrating the periosteum. While vascular lesions elsewhere in the body can be associated with skeletal changes, bony hyperostosis is a rare feature of orbital vascular malformations.

Orbital Aging: A Computed Tomography-Based Study of 240 Orbits.

BACKGROUND: Previous studies have attempted to explain age-related changes to the orbit in isolation, often producing conflicting results. The authors used highly accurate imaging software to analyze computed tomographic scans to characterize changes related to age objectively. METHODS: In this case-control study, patients seen in an ear, nose, and throat clinic were screened for study entry. Male and female participants were divided into two age groups (20 to 30 years and 60 to 75 years). Primary outcomes included measurement of bony orbital dimensions, volume of soft tissues (muscle and fat volume), and anterior globe position. Three-dimensional reconstructions were created of each orbit allowing these measurements. The generalized estimating equation was used so that both orbits from each patient could be included without any bias. RESULTS: The final sample included 240 orbits from 120 patients. There were 30 patients in each age group. Among female participants, the bony orbital volume ( p < 0.05), fat volume ( p < 0.01), and central width ( p < 0.001) of the bony orbit increased with age. The anterior globe position was significantly greater in older female participants ( p < 0.01). For male participants, the fat volume ( p < 0.0001) and central height ( p < 0.03) increased with age; the lateral rim moved posteriorly with age ( p < 0.007). The anterior globe position was not different between the age groups in male participants ( p = 0.56). CONCLUSION: The female bony orbit expands with age and is associated with a more anterior position of the globe; the male bony orbital volume remains the same and the lateral rim moves posteriorly.

Upper Eyelid Contour Changes After Orbital Decompression in Graves Orbitopathy.

PURPOSE: To evaluate the effect of orbital decompression on the upper eyelid contour. METHODS: A paired cross-sectional analysis of the upper eyelid contour was performed for 103 eyes of 66 patients who underwent orbital decompression. A control group of 26 normal subjects was also included. The eyelid contour of all participants were measured with Bézier lines adjusted to the eyelid contour and 9 midpupil eyelid margin (MPD) distances from a horizontal line bisecting the pupil. One central, corresponding to the margin reflex distance (MRD 1), and 8 equally distributed medially and laterally at 20% of the interval between the lines. Patients were classified as with flare if the height of the most lateral MPD relative to the MRD 1 was above the upper limit of the controls. RESULTS: Preoperatively 63 of the 103 contours were classified as flare + (F+). After decompression MRD1 showed a mean decrease of 0.4 mm and the location of the contour shifted 0.8 mm medially. These changes were not correlated with proptosis reduction. Orbital decompression decreased the lateral curvature of the contours especially for the F+ lids. In 40% of the F+ eyelids the flare sign disappeared after decompression. CONCLUSIONS: Orbital decompression affects the lateral eyelid contour and diminishes the amount of lateral eyelid retraction surgery necessary to correct the flare sign. In 40% of the patients, the eyelid contour is normalized with proptosis reduction only.

The Effect of Lighting and Photograph Exposure on Perceived Attractiveness.

PURPOSE: To assess the effect of various lighting conditions and photograph exposures on perceived attractiveness. METHODS: In the first experiment, 5 variably exposed photographs were taken of 10 subjects using a consistent lighting condition (45° superior box light). In the second experiment, 10 subjects were photographed under variable lighting conditions with consistent exposure: 1) 90° overhead box light, 2) ring light, 3) 45° superior box light, 4) built-in camera flash, 5) 2 straight on box lights, each 45° from midline, and 6) natural light. Participants were instructed to maintain a neutral expression, were placed in front of a standardized blue-gray background, and were photographed during a single session. Photographs were imported into an online survey platform (Qualtrics 2020) and displayed in random order. Volunteer survey respondents were instructed to rate the subject's attractiveness on a scale of 0 to 10. Between the two experiments, a total of 22,000 scored photographs were included in the analysis. Mixed ANOVA and pairwise comparisons with Bonferroni correction were used to compare between- and within-subject ratings. RESULTS: Lighting condition had a significant impact on perceived attractiveness ( p < 0.001), with the 90° overhead box light achieving lower scores and the 45° superior box light yielding greater scores of attractiveness relative to the other conditions. Photograph exposure did not have a significant impact on subjective attractiveness ( p = 1.000). CONCLUSIONS: Our findings suggest that perceived attractiveness is enhanced when a 45° superior box light is used for illumination, and attractiveness is reduced when 90° overhead exposure is utilized. Exposure did not play a prominent role in perceived attractiveness.

Recurrence of Distensible Orbital Venous-dominant Venolymphatic Malformations After Sclerotherapy Versus Embolization With Excision.

PURPOSE: Treatment for orbital venolymphatic malformations (VLMs) commonly includes 3 major options: sclerotherapy, surgery, and embolization followed by surgical excision. Each has certain advantages, although it is not clear whether all are effective. The authors characterize the clinical course for a series of patients with distensible orbital venous-dominant VLM treated with sclerotherapy and/or embolization with excision. METHODS: In this cross-sectional cohort study, patients affected by distensible orbital venous-dominant VLM presenting to the orbital and ophthalmic plastic surgery service from 2014 to 2020 were identified. Patients were included if they presented with a moderate-flow, distensible venous-dominant malformation associated with Valsalva-related symptoms (e.g., pain, proptosis, and diplopia). RESULTS: Six cases were treated with sclerotherapy. Four underwent multiple treatments, with a mean ± SD of 3.5 ± 2.3 (range 1-7). All patients in this group failed to improve or experienced recurrence of symptoms after sclerotherapy. Twelve cases were treated with embolization and excision. Resolution of symptoms in all 12 cases was noted and maintained for a mean of 3.4 ± 2.1 years. There have been no cases of recurrence. Patients treated with sclerotherapy were more likely to experience recurrence of symptoms compared to those treated with embolization and excision (p < 0.001). CONCLUSIONS: Treatment of distensible venous-dominant moderate-flow orbital VLM with sclerotherapy may provide temporary improvement in some cases. However, in the medium to long term, recurrence was universal in this series. Embolization with excision appears to provide more definitive management, avoiding recurrence in all cases for a mean follow-up of 3 years.