Inferior Oblique Flap: An Approach for Improved Vascular Supply in Complex Ocular Surface Reconstruction.

This case report describes a novel surgical technique using an inferior oblique muscle flap for a patient requiring complex ocular surface reconstruction. A 63-year-old female with Stevens-Johnson syndrome and complex ocular history including multiple penetrating keratoplasties and keratoprostheses presented with recurrent keratoprosthesis implant plate necrosis. In conjunction with revision keratoplasty, a custom inferior oblique muscle flap was designed to provide improved support and vascularity to the corneal tissue bed; at 12 months postoperation, the graft remained well attached and free of graft failure. Here, we present this technique along with operative photos, describing how this technique may provide surgeons an additional option for complex ocular surface reconstruction.

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.

Orbital Liposarcoma: A Surveillance, Epidemiology and End Results Database Study.

PURPOSE: Orbital liposarcoma is a challenging tumor to treat due to its rarity and high rate of local recurrence, and the role of radiotherapy and chemotherapy remain unclear. Analysis of big data may improve our overall understanding of orbital disease and role of adjuvant therapies. METHODS: Data were extracted from the Surveillance, Epidemiology and End Results (SEER) Research Plus database from 1975 to 2017. All patients with a diagnosis of liposarcoma (ICD-O3 codes 8850-8858, 8869-8862, 8870, 8880, 8881) were included. Cases were divided into 4 groups by primary site: orbit, retroperitoneum, soft tissue, and other. RESULTS: A total of 16,958 patients were included. Patients with orbital involvement were younger and more likely to be female ( p < 0.05). Among orbital lesions, myxoid liposarcoma was the most common histologic subtype (6/19; 31.6%) followed by well differentiated (5/19; 26.3%). This differed from the distribution of histologic subtypes encountered elsewhere, for which well-differentiated liposarcoma was the most common (retroperitoneum 979/3,136; 31%, soft tissue 3,493/11,671; 30%, and other sites 497/2,132; 23%, p < 0.05). Dedifferentiated histologic subtype was the second most common subtype found in the retroperitoneum (946/3,136; 30%), whereas it was less common in the orbit (2/19; 11%) and soft tissue (1,396/11,671; 12%) ( p < 0.001). Patients with orbital liposarcoma had similar disease-specific mortality compared with soft-tissue location ( p = 0.825) and lower disease-specific mortality compared with retroperitoneal location ( p < 0.001). When all locations were combined, patients with well-differentiated liposarcoma had the lowest disease-specific mortality. CONCLUSIONS: Patients with orbital liposarcoma tend to be younger, female, and have a better prognosis than those with retroperitoneal disease, likely due to the lower incidence of dedifferentiated histologic subtype.

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.

Periorbital Intravenous Lobular Pyogenic Granulomas: A Case Report and Literature Review of This Peculiar Pathology.

Intravenous lobular pyogenic granuloma (ILPG) is a seldom-documented pathology in oculoplastic and orbital literature. This study aims to elucidate the clinical presentation and histopathologic findings surrounding periorbital ILPG through a case presentation and literature review. We describe a 42-year-old male with a palpable periorbital subcutaneous nodule that was subsequently diagnosed as ILPG on immunohistochemistry. A literature review was performed by searching articles in the PubMed/MEDLINE database using the keywords "periorbital intravenous lobular pyogenic granuloma," "periorbital intravenous pyogenic granuloma," or "periorbital intravenous capillary hemangioma." The literature review identified 6 patients presenting with similar subcutaneous nodules that were diagnosed as periorbital ILPGs. All patients, including the one in this study, were treated with local excision. Only 1 patient (1/7; 14.3%) noted pain while 5 experienced swelling or fluctuance (5/7; 71.4%). In patients with documented pathology reports, all ILPGs involved the angular vein (6/6; 100%). Of the 3 patients who had follow-ups at 2 to 7 years postexcision, none had recurrence. Histopathologic findings demonstrate an intravascular lobular tumor composed of capillaries with endothelial cells and pericytes. Marked reactivity to anti-Wilms tumor type 1 (WT-1) and anti-Smooth muscle actin was noted in the capillary structure. ILPG can be included on the differential for well-circumscribed, subcutaneous periocular masses. While the etiology of periorbital ILPG is unknown, most cases are managed with surgical excision, and recurrence appears to be uncommon. In sharing these cases and histopathologic underpinnings of periorbital ILPG, we endeavor to describe this peculiar pathology for oculoplastic and reconstructive surgeons.

Prevalence, Clinical and Imaging Characteristics of Superior Ophthalmic Vein Periphlebitis in Thyroid Eye Disease.

PURPOSE: To determine the prevalence, clinical features, and radiographic findings of superior ophthalmic vein periphlebitis (SOVP) in thyroid eye disease (TED). METHODS: Patients with a clinical diagnosis of thyroid eye disease and contrast-enhanced imaging were included. Imaging was reviewed for the presence of SOVP, and patients with SOVP were compared to those without. A random eye was determined to be the affected eye in patients without SOVP. RESULTS: A total of 212 patients met the inclusion criteria. Unilateral SOVP was identified in 4.7% of cases. There was no significant difference in age ( p = 0.22), gender ( p = 0.09), or disease duration ( p = 0.14) between patients with and without SOVP. There was a significant ( p < 0.05) difference in stage classification and clinical activity core between the groups. The affected eye in patients with SOVP had significantly ( p < 0.05) greater margin reflex distance 1, degree of relative proptosis, horizontal motility restriction, and vertical motility restriction than in patients without SOVP. There was no significant difference in horizontal strabismus ( p = 1.0), vertical strabismus ( p = 0.87), or relative intraocular pressure ( p = 0.77). On imaging, the maximal diameter of the SR and IR were found to be significantly ( p < 0.05) larger in the affected eye of patients with periphlebitis; however, there was no difference in measured diameter of the medial rectus and ( p = 0.30) or lateral rectus ( p = 0.78). CONCLUSIONS: SOVP is an under-reported imaging finding of thyroid eye disease. It is associated with significantly greater margin reflex distance 1, relative proptosis, and motility restriction on exam as well as larger superior rectus and inferior rectus diameter on imaging. These patients tend to present in the active stage of disease with greater clinical activity score.

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.

Orbital indeterminate cell histiocytosis.

An 8-year-old female presented to the oculoplastics clinic with 3 months of left upper eyelid fullness and edema. Examination showed a mass in the left anterior superior orbit with erythema. Imaging demonstrated a well-circumscribed superolateral orbital mass that was T1 hypointense and T2 hypo-to-iso intense with contrast enhancement. An incisional biopsy was performed via an upper lid crease incision. Histopathology showed aggregates of histiocytic cells with fibrosis and infiltration of eosinophils. Immunohistochemistry revealed positive CD68 and CD163 staining and negative langerin staining, confirming the diagnosis of indeterminate cell histiocytosis. There was no systemic involvement or associated dermatologic findings. Repeat exam 3 months later showed no change in the size of the lesion and the patient was referred to hematology-oncology for treatment. On most recent exam, the patient had no new symptoms or side effects following 3 months of oral hydroxyurea (25 mg/kg/day). Repeat orbital imaging showed no progression of the lesion and the patient will be monitored closely. Here, we report a rare case of isolated orbital indeterminate cell histiocytosis in a young child.

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.

Orbital Vascular Malformations: Relationship Between Enophthalmos and Clinically Apparent Distensibility with Valsalva.

PURPOSE: Determining the hemodynamic characteristics of an orbital vascular malformation is a critical step in management. The purpose of this study is to assess the relationship between enophthalmos and clinically apparent distensibility of orbital vascular malformations, to optimize imaging and treatment. METHODS: In this cross-sectional cohort study consecutive patients at a single institution were screened for study entry. Data extracted included age, sex, Hertel measurements, presence or absence of distensibility during the Valsalva maneuver, whether lesions were primarily venous or lymphatic based on imaging, and location of the lesion relative to the globe. Enophthalmos was defined as ≥ 2 mm difference from the opposite side. Parametric and nonparametric statistics were used, and linear regression was performed to examine factors predictive of Hertel measurement. RESULTS: Twenty-nine patients met the inclusion criteria. Relative enophthalmos ≥2 mm was significantly associated with distensibility ( p = 0.03; odds ratio = 5.33). Distensibility and venous dominant morphology were the 2 most important factors associated with enophthalmos on regression analysis. The relative position of the lesion anterior or posterior to the globe did not have a significant bearing on baseline enophthalmos. CONCLUSIONS: The presence of enophthalmos increases the likelihood that an orbital vascular malformation is distensible. This group of patients was also more likely to be characterized by venous dominant malformations. Baseline clinical enophthalmos may serve as a useful surrogate marker for distensibility and venous dominance, which may be useful in guiding the selection of appropriate imaging.

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.

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.

Orbital manifestations of B-cell acute lymphoblastic leukemia/lymphoma.

A 2-year-old boy presented with left periorbital edema, proptosis, hyperglobus and esotropia. Imaging revealed an inferotemporal orbital mass with adjacent bony erosion. Histological evaluation of an orbital biopsy revealed B-cell acute lymphoblastic leukemia/lymphoma (B-ALL/BLL). The patient was subsequently treated with chemotherapy. Although orbital involvement in acute myelogenous leukemia has been well-described, orbital manifestations of B-ALL/BLL are uncommon, with only a limited number of previous reports in the literature.

Solitary Extramedullary Plasmacytoma of the Lacrimal Sac With Associated Crystal-Storing Histiocytosis.

PURPOSE: To report a rare case of crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma of the lacrimal sac and to review literature on the 2 entities to summarize important diagnostic, management, and prognostic considerations. METHODS: A case report of the ophthalmologic presentation, pathology workup, and oncologic management is presented. Literature search with focus on lesions occurring in ophthalmic sites and management guidelines from expert panels and working groups. RESULTS: Crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma arose within the lacrimal sac of a previously healthy middle-aged woman and presented as a painless nodule with epiphora. The biopsy tissue showed sheets of crystal-filled histiocytes, interspersed with monoclonal plasma cells and rarely demonstrated plasma cell phagocytosis. Imaging and laboratory studies confirmed the localized nature. CONCLUSIONS: Crystal-storing histiocytosis is an uncommon entity in which crystals, most commonly arising from altered immunoglobulins, aggregate within histiocytes and form symptomatic mass lesions. It has been reported in ophthalmic regions in patients with a concurrent lymphoproliferative or plasma cell disorder and can rarely predate a malignancy. The current case is notable because crystal-storing histiocytosis occurs with a localized process, solitary extramedullary plasmacytoma, and presents in an unusual site, the lacrimal sac. Tissue biopsy with multimodal pathological evaluation is necessary to make the diagnosis. Ophthalmologists should recognize that crystal-storing histiocytosis is commonly associated with a hematologic malignancy and, when appropriate, refer the patient for oncologic management. Surveillance may be indicated in cases with no established etiology. Solitary extramedullary plasmacytoma should also be monitored, as a proportion of cases progress to multiple myeloma.

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.

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.

A Predictive Model for Temporal Artery Biopsy in the Setting of Suspected Giant Cell Arteritis: A Validation Study.

PURPOSE: A previously published predictive model based on threshold parameters for erythrocyte sedimentation rate, c-reactive protein, and platelet count demonstrated that 40% of patients who underwent biopsy may not have required it. The current study was performed to evaluate the model's performance on an independent data set. METHODS: This is a retrospective consecutive series of patients undergoing temporal artery biopsy (TAB) in a single health region in Canada. The model was applied to a multicenter cohort of patients undergoing TAB by a variety of surgical services. A centralized pathological database serving multiple institutions and surgical services was used to identify patients undergoing TAB. RESULTS: Over a 7-year period, patients undergoing TAB were identified via a central pathological database. Those who had concurrent illnesses which would likely affect erythrocyte sedimentation rate, c-reactive protein, and platelet count, patients on steroids for >2 weeks by the time of biopsy, and those with missing serum markers were excluded. The previously developed model was applied to the 222 patients enrolled. The model correctly identified 29% of patients with a pretest probability of 0% for a positive biopsy and 9% with a pretest probability of 100%, suggesting that in total, 38% of patients could have avoided TAB. CONCLUSION: The results of this independent data set support the previously published predictive formula. Utilizing a simple, clinically applicable predictive model of the pretest probabilities, approximately 38% of TAB currently being performed may be avoided. The results suggest that evaluation with a prospective multicentre study would be appropriate.

Early Experience With the Clinical Use of Teprotumumab in a Heterogenous Thyroid Eye Disease Population.

PURPOSE: To describe the clinical course in a heterogeneous series of subjects with thyroid eye disease (TED) treated with teprotumumab. METHODS: Cross-sectional cohort study including patients with clinical diagnosis of TED who was treated with teprotumumab. The entire cohort was analyzed together and subsequently in clinical subgroups based on stage and grade of disease. Primary outcome measure was change in proptosis ≥2 mm. Secondary outcome measures included change in clinical activity score (CAS), ductions, strabismic deviation, MRD1, and MRD2. Bivariate and multivariate statistics were performed. RESULTS: The study included 21 patients. Mean ± SD age was 61.5 ± 12.6 years and 71.4% were female. Reduction in proptosis ≥2 mm was achieved in 71.4% of the sample. Stage and grade were not significant predictors of outcome. Treatment with teprotumumab resulted in a 2.5 ± 1.8 mm reduction of proptosis (P < 0.001), 2.2 ± 1.4 reduction in CAS (P < 0.001), and 16.9 ± 19.3 degree improvement in extraocular motility (P < 0.001). There were no significant differences for change in CAS, proptosis, ductions, or MRD2 between different grades and stages of disease. Total strabismus and MRD1 improvement were greater in the active stage of disease (P < 0.05). Three cases of dysthyroid optic neuropathy, refractory to methylprednisolone therapy improved after initiation of teprotumumab. CONCLUSIONS: Treatment of TED with teprotumumab in a heterogeneous patient population is associated with improvement in proptosis, extraocular motility, and CAS. Patients beyond those defined in the clinical trials, including those affected by stable stage, milder grade, and vision-threatening TED may benefit from this therapy. There are, however, limits on the overall efficacy of this medication in the management of certain physical characteristics in TED including eyelid position and strabismus.

Adjuvant External Beam Radiotherapy Following Enucleation of Eyes With Extraocular Extension From Uveal Melanoma.

PURPOSE: To report local disease control and all-cause mortality in patients with extraocular extension (EOE) of uveal melanoma undergoing enucleation followed by observation or external beam radiotherapy (EBRT). METHODS: Charts of patients enucleated between January 1, 1997 and December 31, 2019, with histopathological evidence of EOE of uveal melanoma were reviewed. RESULTS: The cohort comprised 51 patients with a mean age of 67 ± 15 years, 22 (43%) of whom underwent adjuvant postenucleation EBRT. Risk factors for metastasis included presence of epithelioid cells (29/45; 88%), closed loops (20/43; 47%), monosomy 3 (16/25; 64%), and gain of 8q (20/22; 91%). Patients undergoing EBRT had more extensive EOE (median: 5.1 mm vs. 2.6 mm, p = 0.008) and surgical excision was less likely to be histologically complete (2/20; 10% vs. 14/25; 56%, p = 0.002). Local side effects following EBRT were seen in 64% (14/22). At latest follow up, 59% of patients (30/51) were alive, with a median follow up of 1.8 years (interquartile range: 2.9; range: 0.1-6.5]. By Kaplan-Meier survival analysis, the 5- and 10-year overall survival rates were 56% and 12%, respectively. There was no difference in all-cause mortality between those receiving adjuvant EBRT and those who were observed (log rank, p = 0.273). No cases of orbital recurrence were documented. CONCLUSIONS: Orbital EBRT causes significant morbidity. Cases with relatively small EOE undergoing enucleation can be safely observed, without adjuvant EBRT. Multicenter studies are required to better assess the role of EBRT when EOE is more extensive.

Orbital Emphysema: A Case Report and Comprehensive Review of the Literature.

PURPOSE: The objective of this study was to report a case of persistent and likely self-induced orbital emphysema (OE) following functional endoscopic sinus surgery with dislodgement of a previously placed orbital floor implant and to review the literature surrounding etiologies, pathophysiology, and management of OE. METHODS: Case report and review of the literature. RESULTS AND DISCUSSION: While blunt trauma resulting in disruption of the medial orbital wall is the most common cause of OE, there are an additional 25 underlying etiologies reported in the current literature. Pathophysiology of OE is somewhat dependent on underlying etiology but often involves a 1-way ball valve mechanism such that air may enter the orbit but not exit. When sufficient air enters the orbit, complications secondary to increased intraorbital pressure, including central retinal artery occlusion and compressive optic neuropathy, can occur. Mild cases of OE are typically observed, with most resolving within 7 to 10 days. Moderate cases are often managed by lateral canthotomy and cantholysis with possible needle decompression. Severe cases may require urgent surgical decompression. While the majority of cases of OE are benign and self-limited, there have been 4 reports in the literature documenting significant vision loss. CONCLUSIONS: Although there is often a history of trauma in patients presenting with OE, many other underlying etiologies have been reported with several cases occurring spontaneously. As such, OE should be included on the differential for a patient presenting with a sudden onset of orbital signs.