Kaveh Vahdani

Purpose: To evaluate the clinical characteristics, management, and outcomes for histopathologically confirmed primary orbital melanoma (POM). Methods: A systematic review was conducted in accordance with PRISMA guidelines and registered with PROSPERO. Studies reporting 1 or more patients with histopathologically confirmed POM and extractable data were included; secondary orbital involvement and nonoriginal reports were excluded. Data were extracted for demographics, clinical features, treatment, and outcomes. Tumors were classified as either melanocytosis/nevus-associated (MATs) or de novo melanomas. Outcomes for local recurrence, metastasis, overall survival (OS), and recurrence-free survival (RFS) were analyzed using Kaplan-Meier methods. Results: A total of 112 patients were included, of whom 49 (44%) had POMs that arose de novo, 34 (30%) were MATs, and the remainder were unclassified. Patients with MATs were slightly younger (40.1 vs. 47.9 y; P =0.078) and had shorter symptom duration (5.3 vs. 13.1 mo; P =0.158). Globe-sparing surgery was performed in 47% of cases, exenteration in 53%, and adjunctive radiotherapy in 37%. At a mean follow-up of 26.9 months, local recurrence occurred in 21% and metastasis in 39%. Estimates of 5-year OS showed a nonsignificant trend that favored MATs (74% vs. 44%; P =0.058), whereas recurrence-free survival was longer with melanocytosis-associated tumors ( P =0.048). Adjuvant radiotherapy was associated with improved survival (HR=0.23; 95% CI: 0.08-0.66; P =0.006). Conclusions: POM is a rare malignancy. MATs may represent a distinct subset with more favorable outcomes, although interpretation is limited by the many small and heterogeneous data sets. Globe-sparing management appears to be valuable in some cases.

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The historical evolution of globe-preserving management provides important context for interpreting contemporary outcomes in lacrimal gland carcinoma. Since the early 1990s, evidence has challenged the assumption that orbit-sacrificing surgery, including cranio-orbital resection and orbital exenteration, confers a survival advantage. This was subsequently reinforced by more contemporary long-term comparative outcome data demonstrating no significant survival benefit over globe-preserving surgery with radiotherapy. Recent findings in lacrimal gland adenoid cystic carcinoma are broadly consistent with this established evidence base, although comparisons with exenteration remain susceptible to treatment-selection bias.

To describe a technique for treating extraocular muscle adherence after failed fracture repair(s). Retrospective review of patients referred with persistent diplopia after prior release of inferior rectus from an orbital fracture. The muscle was freed from all adhesions, intraconal fat mobilised on either side of the affected muscle and the two pedicled fat pads sutured together in the extraconal space. 17 patients had markedly restricted motility after fracture repair, with 13 having had 2-4 prior interventions. After 'fat-wrapping', 10/17 patients showed a major improvement and 3/17 moderate improvement in ocular ductions, binocular single vision (BSV) fields and ocular deviations-together with reduced contralateral overactions. To improve late BSV position, a single strabismus procedure was performed in 3 of these 13 patients (23%). Tissues from seven patients showed muscle infiltrated by gross fibrosis extending from the capsule around the underlying implant. Four patients had poor outcomes due to major parabulbar adhesions, with two out of four being referred with longstanding surgical fistulae in the lower fornix and/or lacrimal sac. After release from adhesions at the site of prior fracture repair(s), fat-wrapping of the outer surface of the inferior rectus muscle can markedly improve BSV fields, ocular ductions and deviations. Restoration of a viable extraconal fat layer appears to provide a 'fluid' medium between freed muscle and the orbital wall and block further adhesion between the two raw surfaces but the technique has limited results where prior surgery has caused adhesions involving the lower fornix or pre-equatorial tissues.

Purpose: To review and assess published imaging evidence for optic nerve stretch (ONS) in patients with dysthyroid optic neuropathy (DON), evaluating its prevalence, measurement methods, and potential association with disease pathophysiology. Methods: PubMed, EMBASE, Cochrane CENTRAL, and ClinicalTrials.gov were searched using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, for computerized tomographic or magnetic resonance imaging studies of ONS in thyroid eye disease. Data were extracted on imaging modality, ONS definition, and association with DON. Pooled proportions were calculated using random-effects models with logit transformation, and heterogeneity assessed with I ² . Results: Sixteen studies, comprising 1,277 orbits (508 DON and 769 non-DON), met the inclusion criteria. Five noncomparative studies, mostly earlier papers, report ONS in 43% (0%–100%) of 75 DON orbits. Eleven studies (1,202 orbits) compared orbits with and without DON: 6 studies examined optic nerve contour (317 DON and 435 non-DON) and reported ONS in 66% of DON and 37% of non-DON orbits. Only 2 studies reported ONS as being significantly associated with DON (74% and 64% of orbits), and both reported marked apical crowding (70% and 67%). The pooled ONS proportion was 56% (95% confidence interval: 37%–74%) in orbits with DON and 31% (95% confidence interval: 12%–59%) in non-DON orbits, with high heterogeneity (I ² > 80%). Conclusions: While optic-nerve stretch is frequently observed in DON, current evidence does not establish it as an independent cause. ONS may reflect geometric changes due to orbital soft-tissue expansion, and the principal mechanism for DON probably remains apical compression and microvascular ischemia of the optic nerve.

Tumors of the lacrimal drainage system are rare and include benign and malignant variants. Their presentation frequently mimics inflammatory or obstructive lacrimal disease, which often results in delayed diagnosis and diagnostic uncertainty. A high index of suspicion, appropriate imaging, and timely biopsy are required to verify the histology and guide management. Surgical excision remains the cornerstone of treatment for most lesions, with the aim of achieving histologically clear margins. Advances in endoscopic and combined surgical approaches have enabled radical tumor resection whilst reducing morbidity and improving globe preservation. Adjuvant radiotherapy is commonly employed in malignant disease, while systemic chemotherapy, immunotherapy, and targeted therapies may be considered in selected advanced or recurrent cases. Emerging molecular insights and evolving surgical techniques may further refine future treatment strategies. This review summarizes current evidence and clinical principles to provide a practical framework for the diagnosis and management of lacrimal drainage system tumors.

Leiomyosarcomas (LMSs) are malignant smooth-muscle tumors that most commonly arise in the uterus, retroperitoneum, or gastrointestinal tract, and only rarely originate in the adrenal gland. Orbital metastases from LMS are exceptionally rare. Two patients with histologically-confirmed orbital metastatic leiomyosarcoma arising from different primary sites are described: The first had uterine LMS with a destructive sphenoid lesion extending into the orbit, anterior and middle cranial fossae. The second developed bilateral metastases within extra-ocular muscles, several years after resection of an adrenal LMS - a source from which orbital spread has not previously been described. Both patients were managed within multidisciplinary teams: one underwent serial orbital debulking to relieve the mass effect, whilst the other received palliative orbital radiotherapy. These cases highlight the potential for late and atypical metastatic spread in LMS and emphasize the importance of considering metastatic smooth-muscle sarcoma in patients presenting with new orbital lesions and a relevant oncological history.

Introduction: Vacuoles, E1-ligase, X-linked Auto-inflammatory, Somatic (VEXAS) syndrome is a recently described multisystem inflammatory disorder. Ocular features are described, though not well known; we aimed to describe them. Methods: This is a case series of VEXAS patients submitted by ophthalmologists from relevant specialty organisations, including the Australian and New Zealand Society of Ophthalmic Plastic Surgeons, British Oculoplastic Surgical Society and the Orbital Society. Patient consent was received. Results: 14 males with VEXAS syndrome and eye features were included, median age 73 years (range 49–78). The associated UBA1 mutation was most commonly p.Met41Val (n=8, 57%), and most patients were Caucasian (n=11, 79%). All patients reported eyelid swelling (n=14, 100%); the next most frequent symptom was eye pain (n=10, 71%). Eye symptoms showed variable duration at presentation, most commonly 2–7 days (n=6, 43%). Eye involvement was typically bilateral (metachronous n=5, 36%, synchronous n=2, 14%). Overall, visual acuity was normal and did not change. Severe vision loss occurred unilaterally in two patients (14%), due to orbital compartment syndrome and periorbital necrotising fasciitis. Ophthalmologist-reported clinical features included periorbital oedema, present in all cases (n=14, 100%), followed by dacryoadenitis (n=8, 57%) and orbital myositis (n=7, 50%). There were no cases of posterior segment inflammation. Death due to complications of VEXAS occurred in one patient (7%). Conclusion: Orbital inflammation was a feature in all cases of VEXAS with eye involvement, and severe loss of vision occurred unilaterally in 14%. Ocular complaints in VEXAS patients should prompt urgent ophthalmic assessment.