Orbitocranial Penetration of Ball Bearing Gun Trauma: A Prospective Human Cadaveric Study.

Objective We completed a prospective human cadaveric study to determine the ability of a ball bearing (BB) pellet to penetrate the orbit and/or surrounding structures. Methods A single trained sergeant officer discharged an alloy steel air rifle to eight cadaver orbits from four adult human cadaver heads. Five BB pellets each were aimed at three locations (caruncle, upper eyelid, or lower eyelid) at 10 cm and 1 m, and then less specifically, at the orbital region for 3- and 5-m distances. Computed tomography (CT) of the cadaver heads was performed. Final locations of BB pellets are divided into three categories: intracranial, surrounding orbital structures including the pterygopalatine fossa and infratemporal fossa, and orbit. Results Of 40 BB pellets, 37 penetrated soft tissue and were visualized on CT: 19 (51%) rested in the intracranial space, 17 (46%) in surrounding orbital structures, and 1 (3%) within the orbit. The deepest position of a pellet was in the parietal lobe, and most superficial location anterior to the frontal bone. Pellets discharged from 1 m were more likely to rest in the intracranial space compared with those from 10 cm ( p < 0.001), 3 m ( p = 0.011), and 5 m ( p = 0.004). The distance of discharge was associated with final pellet location ( p = 0.001). Conclusion BB guns should be considered dangerous and potentially deadly when aimed at the orbit. Although the thick calvarium can protect the intracranial space from BB penetration, the orbit may be a vulnerable entry point with relatively low resistance, allowing penetration of the intracranial and periorbital spaces.

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.

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.

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

INTRODUCTION: 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.

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.

A Minimally Invasive Endoscopic Transnasal and Transorbital Approach to Sinonasal Tumor Resection.

A 35-year-old female with an extensive biphenotypic sinonasal sarcoma (BSNS) eroding through the left frontal sinus into the orbit required gross total resection of the tumor. We wanted to forgo an open approach and provide a less invasive alternative via combined endonasal and transorbital endoscopic techniques to allow for tumor removal through small, well-disguised incisions. We utilized three portals-nasal, transorbital, and anterior table window-to create interconnected orbit-sinonasal corridors, thus generating alternate pathways for visualization and manipulation of this extensive tumor. Laryngoscope, 134:1308-1312, 2024.

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.

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.

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.

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.

Retinoblastoma and vision.

The assessment of vision has a growing importance in the management of retinoblastoma in the era of globe-conserving therapy, both prior to and after treatment. As survival rates approach 98-99% and globe salvage rates reach ever-higher levels, it is important to provide families with information regarding the visual outcomes of different treatments. We present an overview of the role of vision in determining the treatment given and the impact of complications of treatment. We also discuss screening and treatment strategies that can be used to maximise vision.

Long-term visual outcomes after ruthenium plaque brachytherapy for posterior choroidal melanoma.

BACKGROUND: To assess the long-term visual outcomes in patients with posteriorly located choroidal melanoma treated with ruthenium plaque brachytherapy between January 2013 and December 2015. METHODS: A retrospective review was conducted on consecutive patients treated with ruthenium plaque brachytherapy for post-equatorial choroidal melanoma with available Snellen visual acuity before and after treatment, and the development and treatment of radiation complications. RESULTS: There were 219 patients with posterior choroidal melanoma treated with ruthenium plaque brachytherapy. Median follow up was 56.5 months, range 12-81 months. Final visual acuity was ≥6/12 in 97 (44.3%) patients, 6/12 to 6/60 in 57 (26.0%), <6/60 in 55 (25.1%) and 10 (4.6%) eyes were enucleated. Radiation maculopathy was the most common radiation complication encountered, occurring in 53 (24.2%) patients. Of these, final visual acuity was 6/12 in 10 patients (18.9%), 6/12 to 6/60 in 26 (49.1%), <6/60 in 16 (30.2%) and 1 eye (1.9%) was enucleated. Twenty-five (47%) with radiation maculopathy were treated with intravitreal anti-angiogenic therapy, 27 (51%) were monitored and one (2%) was treated with scatter photocoagulation. Eyes treated with intravitreal anti-angiogenic therapy had better final vision than those observed or treated with retinal laser (chi-square, p = 0.04). On multivariate analysis, close proximity to the optic nerve and fovea, and large or notched plaque type was associated with final vision worse than 6/12. CONCLUSION: Most patients treated with ruthenium plaque brachytherapy for posterior choroidal melanoma retain 6/60 vision, with almost half retaining 6/12 vision at long term follow up.

The MOLES system to guide the management of melanocytic choroidal tumours: can optometrists apply it?

CLINICAL RELEVANCE: Although melanocytic choroidal tumours of the choroid are a common eye pathology, no standardised protocol exists for their management in the community. BACKGROUND: Choroidal naevi are found in approximately 6% of the adult White population, whereas choroidal melanomas are rare, with an annual incidence of 5-10/million/year. Multimodal imaging has advanced the understanding of malignancy imaging biomarkers, but distinguishing between a small melanoma and naevus remains difficult and an algorithm for their management by community practitioners has not been uniformly adopted. One of the authors (BD) devised the MOLES scoring system, which indicates malignancy likelihood according to mushroom shape, orange pigment, large size, enlargement, and subretinal fluid. When applied by ocular oncologists, the system accurately distinguishes choroidal naevi from melanomas. The aim of this study was to evaluate whether community optometrists can appropriately manage patients with melanocytic choroidal tumours using this system. METHODS: Clinical images of 25 melanocytic choroidal tumours were presented in an online survey, including colour fundus photographs, fundus autofluorescence, optical coherence tomography, and B-scan ultrasound images. Using the MOLES system, 39 optometrists diagnosed tumours as naevus or probable melanoma and decided between community monitoring and ophthalmologist referral. Responses were compared to MOLES grading of the same clinical images by ocular oncologists. RESULTS: Using MOLES, optometrists correctly identified 389/406 probable melanomas (95.8% sensitivity) and 331/516 choroidal naevi (64.1% specificity); correctly referred 773/778 tumours to an ophthalmologist (99.4% sensitivity); and correctly managed 80/144 lesions (55.6% specificity) in the community. CONCLUSION: Optometrists safely applied the MOLES scoring system in this survey. Further measures are indicated to reduce choroidal naevi over-referral and evaluate MOLES system usage in clinical optometric practice, where some imaging modalities may not be readily available.

Radiological analysis of orbital lymphoma histological subtypes.

PURPOSE: To characterise the radiological features of orbital lymphoma subtypes. METHODS: This was a multicentre, retrospective study to analyse radiological and clinical characteristics of orbital lymphoma by histological subtype across two sites within Australia and the United States. RESULTS: A total of eighty-eight patients were included. The most common subtypes were extranodal marginal zone lymphoma [EMZL] (48, 54.5%), follicular lymphoma [FL] (16, 18.2%), and diffuse large B-cell lymphoma [DLBCL] (15, 17.0%). Clinically, significant associations were found between DLBCL and vision change (p < .01), pain (p < .01), extraocular movement limitation (p = .01), and optic neuropathy (p = .01). Radiologically, there was no significant difference between the individual histopathological sub-groups with respect to bone destruction (p = .30), optic nerve involvement (p = .30) and diffuse appearance (p = .84). However, if categorised as either aggressive or indolent, aggressive lymphoma subtypes were significantly more likely to demonstrate globe indentation radiologically than indolent subtypes (Odds Ratio = 5.5, 95% CI: 1.3, 23.3; p = .02). CONCLUSIONS: DLBCL was significantly associated with vision change, pain, extraocular movement limitation and optic neuropathy clinically. Aggressive lymphoma subtypes were significantly more likely to demonstrate globe indentation radiologically. Otherwise, there were no significant differences between lymphoma subtypes and radiological findings on MRI and CT.

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.

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.

Low-grade fibromyxoid sarcoma of the orbit.

A 70-year-old male presented with diplopia and painless proptosis of the left eye for 5 months. Examination showed 6 mm of axial proptosis and restriction of supraduction, abduction and adduction, and mild limitation of infraduction of the left eye. Magnetic resonance imaging demonstrated a large, moderately well-circumscribed intraconal mass in the left lateral orbit, and excisional biopsy was performed. Histopathologic features of mixed fibrous and myxoid areas in a whorl-like pattern and immunohistochemical staining for MUC4 confirmed the diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Next-generation sequencing revealed genetic fusion of EWSR1-CREB3L1. LGFMS is an extremely rare neoplasm with only two prior documented cases of orbital involvement. Here, we report the third case of orbital LGFMS.