Pseudofilariasis, a Presenting Sign of Alkaptonuria.

PURPOSE: To report the association of Pseudofilariasis as a presenting sign of Alkaptonuria. METHOD: Case Report. RESULTS: A 49-year-old Indian man was referred because of wormlike objects in his left conjunctiva. Ocular and family history was non-contributory. He had not been to India in 15 years. Past medical history revealed hypertension, hypercholesterolemia, arthritis, and a myocardial infarct. He had undergone two stents, bilateral Achilles tendon repairs and bilateral knee replacements. ROS showed longstanding back stiffness and pain. On ocular examination the vision was 20/25 in each eye and positive findings were in the left eye bulbar conjunctival which showed stationary black vermiform (filarial in appearance) foreign bodies along with 2 small corneal limbal pigmented deposits. Conjunctival biopsy showed dilated lymph channels with interstitial proteinaceous material of a light brown color consistent with Ochronotic pigment; hence diagnostic of Alkaptonuria. CONCLUSIONS: Pseudofilariasis may be a presenting sign of Alkaptonuria and occur years before a clinical diagnosis is made. Filariasis is always involves white worms and never black. Knowing the ophthalmic signs of this rare disease may lead to an accurate diagnosis earlier thusly avoiding unnecessary tests and examinations.

Effects of Toll-like receptor 1 and 2 agonist Pam3CSK4 on uveal melanocytes and relevant experimental mouse model.

Pam3CSK4 activates Toll-like receptors 2 and 1 (TLR1/2), which recognize mainly molecules from gram-positive pathogens. The effect of Pam3CSK4 on various cytokine and chemokine expression in cultured human uveal melanocytes (UM) has not been studied systematically. The purpose of this study was to investigate the mechanistic expressions of seven cytokines and chemokines of interleukin- (IL-) 6, IL-10, MCP-1 (CCL-2), CXCL-1 (GRO-α), CXCL-8 (IL-8), interferon-gamma (IFN-γ), and tumor necrosis factor-alpha (TNF-α) in UM. These cytokines are reported to be increased in intraocular fluids or tissues of the patients with endophthalmitis and non-infectious uveitis, as well as in various experimental animal uveitic models in the literature. Flow cytometry was used to measure the effects of Pam3CSK4 on the expression of TLR1/2 in UM. ELISA and Real-time PCR analysis were used to estimate the ability of Pam3CSK4 to elevate these cytokines and chemokines levels in conditioned media and cell lysates of UM, respectively. Flow cytometry measured and compared the phosphorylated MAPK pathway and activated NF-κB signals pathway in UM, treated with and without Pam3CSK4. ELISA analysis tested the effect of various signal inhibitors (ERK1/2, JNK1/2, p38 and NF-κB) on Pam3CSK4-induced IL-6 levels in cultured UM. The role of TLR2 in Pam3CSK4-induced acute anterior uveitis in experimental mouse model was tested in TLR2 knockout (TLR2 KO) mice and their wild-type C57Bl/6 controls. Pam3CSK4 increased the expression of TLR1/2 proteins in cultured UM. Pam3CSK4 significantly elevated the IL-6, MCP-1, CXCL-1, CXCL-8 protein, and mRNA levels in cultured UM, but not IL-10, TNF-α, or IFN-γ. Pam3CSK4 activated NF-κB, ERK, JNK, and p38 expression. Pam3CSK4-induced expression of IL-6 was decreased by NF-κB, ERK, INK, and p38 inhibitors; especially the NF-κB inhibitor, which can completely block the IL-6 stimulation. Intravitreal injection of Pam3CSK4 induced acute anterior uveitis in C57Bl/6 mice, this effect was significantly reduced in TLR2 KO mice. TLR1/2 plays an important role against invading pathogens, especially gram-positive bacteria; but an excessive reaction to molecules from gram-positive bacteria may promote non-infectious uveitis. UM can produce IL-6, MCP-1, CXCL-1, and CXCL-8, and are one of the target cells of TNF-α and IFN-γ. TLR-2 inhibitors might have a beneficial effect in the treatment of certain types of uveitis and other ocular inflammatory-related diseases and warrant further investigation.

Sympathetic Ophthalmia in Patients with Enucleation or Evisceration: Pathology Laboratory and IRIS® Registry Experience.

Introduction: Sympathetic ophthalmia (SO) is a rare bilateral granulomatous panuveitis that can follow surgical or nonsurgical ocular trauma in one eye. Because its diagnosis requires clinical-pathologic correlation, the true incidence of SO is unknown, and there is a need to understand the recent trends in risk factors and frequency of this condition. Methods: Pathology records of all enucleated or eviscerated (ENEV) eyes at three pathology laboratories were reviewed. Data collected included patient demographics, procedure indication, pathology diagnosis, and clinical history of trauma and uveitis. IRIS® Registry (Intelligent Research in Sight) was searched for all patients with SO, acquired absence of eye (AAE), and/or ENEV. Data obtained included patient demographics, ocular procedures, and preoperative diagnoses within 30 days of AAE/ENEV. Results: In the pathology laboratory setting, the incidence of SO over a 36-year period in patients who underwent ENEV was 0.2% (20/9,092); the 5-year incidence ranged from 0.0 to 0.3%. Among the 20 eyes with SO, the inciting event was surgical trauma in 50% (10/20), nonsurgical trauma in 45% (9/20), and missing/undetermined in 5% (1/20). SO was suspected preoperatively in 7/20 (35%) patients. Clinical concern for SO and ruptured globe were indications for ENEV in 50/9,092 (0.5%) and 872/9,092 (10%) patients, respectively. In the IRIS Registry, 0.7% (199/27,830) of patients with AAE/ENEV had diagnosis of SO. The frequency of SO between 2015 and 2020 was 0.01% (7,371/62,318,249); of these 7,371 cases, 199 (3%) had AAE/ENEV. In 25,975 patients with available data, injury and SO were listed as diagnoses less than 30 days prior to AAE/ENEV in 909 (4%) and 63 (0.2%) cases, respectively. Conclusion: The frequency of SO in recent decades has been low. Most cases of SO are not managed with eye removal. In histopathology-confirmed SO, surgical trauma is as frequent as nonsurgical trauma as an inciting etiology of disease.

Occult anterior uveal melanomas presenting as extrascleral extension.

OBJECTIVE: To describe the management of patients with occult anterior uveal melanomas presenting with extrascleral extension. METHODS AND ANALYSIS: Retrospective case series including five patients with small pigmented nodular mass on the episclera. Each lesion was documented by slit-lamp photography and measured with high-frequency ultrasound imaging (ultrasound biomicroscopy). Diagnosis of uveal melanoma was confirmed by biopsy with lamellar sclerectomy. Immediate scleral patch graft repair was performed. Later, each tumour was treated with palladium-103 ophthalmic plaque brachytherapy. The mean plaque diameter was 12 mm (median, 12; range, 10-14). A mean apex prescription dose of 87 Gy (median, 84.5; range, 82.3-99.2) to a tumour depth of 2 mm from the inner sclera delivered over 7 continuous days. The main outcome measures were best-corrected visual acuity, changes in tumour and scleral characteristics and complications. RESULTS: During each surgery, residual tumour was visualised within an emissary passageway at the deep plane of scleral resection. At a mean of 80 months (median, 57; range, 24-159) follow-up, no patients experienced graft infection, scleromalacia or rejection. Biopsy was required to establish the diagnosis, transillumination failed, and therefore ultrasound measurements were used to determine the plaque size required to treat the relatively occult intraocular component. Despite these challenges, there were no cases of local tumour recurrence, secondary enucleation or metastatic disease. Attributed to cataract surgery, visual acuities improved in three patients and two were stable. CONCLUSION: Extrascleral uveal melanoma extension can occur with undetectable, occult intraocular tumours. In these cases, plaque radiation effectively induced local tumour control, preserved vision and prevented metastasis.

Ligneous Conjunctivitis Secondary to Viral Conjunctivitis.

ABSTRACT: Ligneous conjunctivitis is a rare cause of chronic conjunctivitis that may be triggered by ocular insults such as trauma or infections. We present an interesting case of ligneous conjunctivitis caused by a viral infection that responded well to conservative management. Topical cyclosporine and heparin are a good treatment regimen that caused resolution of lesions and prevented recurrences.

Papillary conjunctivitis presenting months after permanent eyebrow tattooing.

Purpose: To present a case of tattoo side effects not limited to the tattoo site and rise an alarm regarding using non-FDA-approved products. Observations: A 30-year-old female presented with bilateral ocular pain, dryness, and itching. The ocular exam showed bilateral injection and edema of the superior palpebral and bulbar conjunctiva. Several 1-2 mm dark pigmented lesions and papillae coursing along the upper palpebral conjunctival lid margin and 5 mm above the margin were found in both eyes. The ocular surface was dry with diffuse superficial punctate keratitis. The biopsy report showed granular foreign material in the dermis. SOX-10 and MART-1 immunostaining highlighted melanocyte distribution and the sample was diagnosed as exogenous pigment consistent with tattoo ink by the pathologist. On further investigation following the pathology report, the patient stated that she got bilateral permanent eyebrow tattoos 4 months before presentation in a country other than the United States, and she was not aware about the standards of the ink used, nor the certification of the person performing the tattoo. The patient denied any type of tattoo or manipulation on the eyes or orbit, including sclera or conjunctivae. Conclusions: Importance: The complications of periorbital tattooing are not limited to the point tattoo location and can potentially spread to the nearby segments. It is notable that there is no FDA approved tattoo ink available, even with a certified tattoo artist performing the tattoo, the risks of inflammation, infection, and other side effects are still present.

Toll-like receptor 2 and 6 agonist fibroblast-stimulating lipopeptide increases expression and secretion of CXCL1 and CXCL2 by uveal melanocytes.

Fibroblast-stimulating lipopeptide (FSL-1) can activate Toll-like receptor 2 and 6 (TLR2/6), which recognize relevant molecules from gram-positive pathogens, fungus, and mycoplasma, and elevates the expression of CXCL1 and CXCL2, neutrophil chemoattractants, in certain types of cells. This effect has not previously been reported in the uveal melanocytes (UM). This study was designed to test the hypothesis that FSL-1 can induce the expression and secretion of CXCL1 and CXCL2 via activation of TLR2/6 in cultured human UM and producing an acute non-infectious uveitis reaction in the mouse. Flow cytometry and fluorescent immunostaining were used to measure the effect of FSL-1 on the expression of TLR2/6 in UM. Real time PCR and ELISA analysis were used to assess the ability of FSL-1 to elevate CXCL1/CXCL2 levels in cell lysates and conditioned media of UM, respectively. Flow cytometry measured phosphorylated MAPK and activated NF-κB signals in UM, with and without FSL-1 treatment. ELISA analysis tested the impact of various signal inhibitors (NF-κB, p38 MAPK, JNK1/2 and ERK1/2) and TLR2/6 antagonists on FSL-1-induced CXCL1/CXCL2 levels in cultured UM. The effects of neutralizing antibodies to TLR2 on FSL-1-induced mouse uveitis were tested in an experimental animal model. FSL-1 induced the expression of TLR2/6 proteins in cultured UM. FSL-1 significantly elevated the CXCL1 and CXCL2 proteins and mRNA levels in cultured UM time- and dose-dependently. FSL-1 mainly activated NF-κB, JNK, and expression of TLR2. FSL-1-induced expression of CXCL1 and CXCL2 was blocked by NF-κB, JNK, ERK inhibitors and TLR2 antagonists. Intravitreal injection of FSL-1 induced acute non-infectious mouse uveitis, which was significantly reduced in severity by a TLR2 antagonist. These results suggest that UM may play a role in the immune reaction, which targets invading pathogens, especially gram-positive bacteria. On the other hand, an excessive reaction to molecules from gram-positive bacteria may promote an inflammatory state of non-infectious uveitis.

Androgen Activity Is Associated With PD-L1 Downregulation in Thyroid Cancer.

Thyroid cancer is the most prevalent endocrine malignancy in the United States with greater than 53,000 new cases in 2020. There is a significant gender disparity in disease incidence as well, with women developing thyroid cancer three times more often than men; however, the underlying cause of this disparity is poorly understood. Using RNA-sequencing, we profiled the immune landscape of papillary thyroid cancer (PTC) and identified a significant inverse correlation between androgen receptor (AR) levels and the immune checkpoint molecule PD-L1. The expression of PD-L1 was then measured in an androgen responsive-thyroid cancer cell line. Dihydrotestosterone (DHT) treatment resulted in significant reduction in surface PD-L1 expression in a time and dose-dependent manner. To determine if androgen-mediated PD-L1 downregulation was AR-dependent, we treated cells with flutamide, a selective AR antagonist, and prior to DHT treatment to pharmacologically inhibit AR-induced signaling. This resulted in a > 90% restoration of cell surface PD-L1 expression, suggesting a potential role for AR activity in PD-L1 regulation. Investigation into the AR binding sites showed AR activation impacts NF-kB signaling by increasing IkBα and by possibly preventing NF-kB translocation into the nucleus, reducing PD-L1 promoter activation. This study provides evidence of sex-hormone mediated regulation of immune checkpoint molecules in vitro with potential ramification for immunotherapies.

Cultured Human Uveal Melanocytes Express/secrete CXCL1 and CXCL2 Constitutively and Increased by Lipopolysaccharide via Activation of Toll-like Receptor 4.

Purpose: Lipopolysaccharide (LPS) can activate Toll-like receptor 4 (TLR4) and increase the expression of CXCL1 and CXCL2, the potent neutrophils chemoattractants, in various cell types. These effects have not been previously reported in the uveal melanocytes. This study was designed to investigate the effects of LPS on the activation of TLR4 and expression of CXCL1/CXCL2 in cultured human uveal melanocytes and the relevant signal pathways.Methods: Effects of LPS on the expression of TLR4 were tested using real-time PCR, flow cytometry and fluorescence immunostaining. Effects of LPS-induced expression/secretion of CXCL1/CXCL2 were studied using real-time PCR in cell lysates and ELISA in conditioned media of cultured uveal melanocytes. Activated NF-κB and phosphorylated MAPK signals were tested in cells with and without LPS treatment using flow cytometry. Effects of various signal inhibitors on p38, ERK1/2, JNK1/2 and NF-κB on the secretion of CXCL1/CXCL2 were tested by ELISA. The effects of neutralized antibodies of CXCL1/CXCL2 on the severity of LPS-induced uveitis were tested in a mouse model.Results: LPS stimulation increased the expression of TLR4 mRNA and protein in culture uveal melanocytes. Constitutive secretion of CXCL1/CXCL2 was detected in uveal melanocytes and was significantly increased dose- and time-dependently by LPS stimulation. LPS mainly increased the activated NF-κB and phosphorylated JNK1/2. LPS-induced expression of CXCL1/CXCL2 was blocked by NF-κB and JNK1/2 inhibitors. The severity of LPS-induced uveitis was significantly inhibited by neutralizing antibody to CXCL1/CXCL2Conclusions: This is the first report on the LPS-induced expression of CXCL1 and CXCL2 by uveal melanocytes via the activation of TLR4. These results suggest that uveal melanocytes may play a role in the immune reaction that eliminates the invading pathogens. Conversely, an excessive LPS-induced inflammatory reaction may also lead to the development of inflammatory ocular disorders, such as non-infectious uveitis.

First report of human infection caused by Colletotrichum chlorophyti occurring in a post-corneal transplant patient with endophthalmitis.

Keratomycosis or mycotic keratitis is recognized as one of the major causes of ophthalmic morbidity worldwide. The most common organisms linked to keratomycosis include Candida spp., Fusarium spp., and Aspergillus spp. However, varieties of saprobic fungi have been reported as causative agents of keratomycosis. Amongst these are members of the genus Colletotrichum. Herein we present the first reported case of C. chlorophyti infection in a post-corneal transplant patient, suggesting an increasing role for Colletotrichum species as emerging human pathogens, particularly in the transplant population.

Androgen receptor activation decreases proliferation in thyroid cancer cells.

The American Cancer Society predicted more than 52 000 new cases of thyroid cancer in 2020, making it the most prevalent endocrine malignancy. Due to the approximately threefold higher incidence of thyroid cancer in women, we hypothesize that androgens and/or androgen receptors play a protective role and that thyroid cancer in men represents an escape from androgen-mediated cell regulation. The analysis of androgen receptor (AR) expression in patient tissue samples identified a 2.7-fold reduction in AR expression (p < 0.005) in papillary thyroid cancer compared with matched, normal tissue. An in vitro cell model was developed by stably transfecting AR into 8505C undifferentiated thyroid cancer cells (resulting in clone 84E7). The addition of DHT to the clone 84E7 resulted in AR translocation into the nucleus and a 70% reduction in proliferation, with a shift in the cell cycle toward G1 arrest. RNASeq analysis revealed significant changes in mRNA levels associated with proliferation, cell cycle, and cell cycle regulation. Furthermore, androgen significantly decreased the levels of the G1-associated cell cycle progression proteins cdc25a CDK6 CDK4 and CDK2 as well as increased the levels of the cell cycle inhibitors, p27 and p21. The data strongly suggest that DHT induces a G1 arrest in androgen-responsive thyroid cancer cells. Together, these data support our hypothesis that AR/androgen may play a protective, antiproliferative role and are consistent with younger men having a lower incidence of thyroid cancer than women.

Doppelgänger dilemma: Leiomyoma versus uveal melanoma.

BACKGROUND: Ciliary body tumors can remain undetected and achieve large dimensions. Pigmented ciliary body tumors include: melanoma, leiomyoma and melanocytoma, however correct diagnosis may require tissue diagnosis with immunohistochemical stains. CASE PRESENTATION: Two men presented with identical ciliochoroidal tumors. Both had darkly pigmented dome-shaped anterior uveal masses, exudative retinal detachments and transillumination shadowing. Ocular PET-CT imaging revealed that both were metabolically active consistent with a diagnosis of cancer. However, immunohistochemical examination revealed one a leiomyoma and the other melanoma. CONCLUSION: Uveal leiomyoma can be an indistinguishable doppelgänger to ciliochoroidal melanoma, where the diagnosis can only be established by immunohistopathology.

Micro-incision, trans-iridal aspiration cutter biopsy for ciliary body tumours.

OBJECTIVE: To perform micro-incision, trans-iridal, aspiration-cutter-assisted biopsy for ciliary body tumours. DESIGN: Retrospective, nonrandomized, observational, interventional case series. METHODS: Five consecutive patients undergoing ciliary body tumour biopsy were clinically diagnosed using slit-lamp photography, gonioscopy, high-frequency ultrasound imaging, and systemic radiographic staging. A 1-2 mm clear cornea incision was placed opposite to the central clock hour of the ciliary body tumour. Viscoelastic was infused into the anterior chamber for stabilization and endothelial protection. Then, a 27-gauge aspiration cutter was used to make an iridotomy at the iris root and then extend through the iris into the tumour. Biopsy was performed using mechanical cutting starting at 300 cuts per minute and aspiration at 600 mm Hg. After withdrawal of the cutter from the eye, the effluent tube was flushed into a 3 cc syringe, inspected for specimen under the operating microscope and sent for pathology. Multiple biopsies were performed on each patient. Viscoelastic was removed and Seidel examination of the corneal wound performed. RESULTS: Five eyes were biopsied. A mean 3.6 passes were used to obtain tumour tissue. Tumour cells and tissue were obtained in all cases. Cytologic, histopathologic, and immuno-histochemical analysis were performed (100%, n = 5/5). Diagnoses included melanoma (60%, n = 3/5), melanocytoma (20%, n = 1/5), and leiomyoma (20%, n = 1/5). Transient postoperative hyphemas cleared within 1 week (80%, n = 4/5). No secondary glaucoma, infection, or cataracts were noted. CONCLUSION: Aspiration-cutter biopsy through the iris root provided a minimally invasive, safe method for obtaining ciliary body tissue for cytology, histopathology, and immunohistochemical analysis.

Cost-effective treatment of ocular surface squamous neoplasia for an undocumented and uninsured New York City patient: a case report.

BACKGROUND: New York City has a heterogeneous population with many undocumented and uninsured immigrants from equatorial areas who have a higher incidence of ocular surface squamous neoplasia. To the best of our knowledge, this is the first documented selection of this cost-effective treatment of ocular surface squamous neoplasia (the use of absolute ethanol along the corneal margin, primary excision, double freeze-thaw cryopexy, and primary conjunctival closure) for an undocumented and uninsured New York City patient. CASE PRESENTATION: A 35-year-old man from Ecuador presented to a New York City emergency department due to worsening discomfort of a long-standing left eye pterygium. A slit-lamp examination of the left eye demonstrated a nasally located conjunctival mass measuring 6 × 8 mm extending onto the cornea (3 mm superiorly and 6 mm inferiorly on the cornea). Histological diagnosis confirmed squamous cell carcinoma in situ arising from the pterygium. Surgical excision with adjunctive absolute alcohol with additive double freeze-thaw cryopexy was performed. Our patient has remained free of tumor recurrence at year 2 postoperative visit. CONCLUSIONS: Our case highlights the need to choose a cost-effective treatment for ocular surface squamous neoplasia in an at-risk population among undocumented and uninsured patients. Areas in the world with similar types of populations or treatment challenges may need to consider this approach as a primary treatment option.

Quantitative Study of Human Scleral Melanocytes and Their Topographical Distribution.

PURPOSE: While fibroblasts constitute the main cell component of the sclera, the purpose of the present study was to investigate the cell densities of melanocytes at different regions of the sclera, and to compare them with associated scleral fibroblast densities in human donor eye sections. METHODS: . Paraffin-embedded sections of sclera from 21 human eyes were stained with hematoxylin-eosin (H&E) and immunohistochemical staining (S-100/AEC). Scleral melanocyte and fibroblast numbers were counted in different regions of the sclera. The relationship between the melanocyte density and iris pigmentation was also analyzed. RESULTS: . Melanocytes were found in the posterior region of the sclera, especially around the vessels and nerves in emmissarial canals, whereas no or rare melanocytes were found in equatorial and anterior regions. In H&E sections, melanocyte densities in eyes with light-colored irides were significantly less than in eyes with medium or dark-colored irides (P < .05). In S-100-stained sections, more melanocytes could be detected than those in the H&E sections in light-colored eyes (P < .05), but not in medium or dark-colored eyes (P > .05). The numbers of scleral fibroblasts were relatively stable in different regions. In the posterior scleral region, the numbers of fibroblasts were slightly higher than the number of melanocytes, however, this differences were not statistically significant (P > .05). CONCLUSION: . Notable numbers of melanocytes were present in the posterior sclera suggesting that these cells may play a role in ocular physiology and in the pathogenesis of various disorders of the sclera.

Candida auris and endogenous panophthalmitis: clinical and histopathological features.

PURPOSE: To report an unusual case of endogenous panophthalmitis involving Candida auris and describe its clinical and histopathological features. FINDINGS: A 30 year-old man with history of human immunodeficiency virus, polysubstance abuse, syphilis, and recently treated pneumonia presented with polymicrobial endogenous panophthalmitis. Two separate ocular specimens confirmed simultaneous Pseudomonas aeruginosa and Candida auris involvement. Histopathological analysis demonstrated fulminant polymorphonuclear infiltration of all ocular tissue layers. Despite aggressive management including two intravitreal injections and enucleation, the patient died, ultimately after receiving care at four neighboring urban medical centers. CONCLUSIONS AND IMPORTANCE: Candida auris has been a recently and increasingly described pathogen leading to mortality in metropolitan hospitals worldwide. To the authors' knowledge, Candida auris has not previously been reported with endophthalmitis or panophthalmitis. Future cases may be expected with the reported rise in Candida auris. A high suspicion of its contribution to panophthalmitis could be warranted early in the evaluation and management of profoundly immunocompromised patients, particularly those who have had sequential care at multiple neighboring metropolitan hospitals.

Genomic profiling proves metastasis of cutaneous melanoma to vitreal fluid.

Cutaneous melanoma metastases can contribute to visual disturbances through a variety of factors, including metastasis to the vitreal fluid. The optimum management of metastatic cutaneous melanoma to the vitreal fluid is unknown, but can include radiation therapy or systemic therapy including immunotherapy. A high degree of suspicion is necessary to consider this complication while working with patients with cutaneous melanoma.

Intraocular leiomyoma: Current concepts.

Intraocular leiomyoma is a benign smooth muscle tumor. First recognized before the era of immunohistochemistry, uveal leiomyomas have been described in case reports and small case series. We add 3 new cases, for a total of 80. Of these, there were 29 men and 51 women. The mean and median ages were 35.8 and 30.5 years respectively, with a range of 8 to 80 years. Curiously, ciliary body tumors were more common in females, whereas iris and posterior choroidal leiomyomas were more prevalent in males. Infrequently associated with systemic fibroids, nuclear expression of sex steroid receptors was inconsistent. Iris and posterior choroidal leiomyoma were predominantly amelanotic, while 40% of ciliary body leiomyomas were brown. Two-thirds of the leiomyomas blocked transillumination partially or completely, a feature shared by uveal melanoma. In general, low-frequency ultrasound imaging reveals low to moderate internal reflectivity; however, high-frequency anterior uveal ultrasound was used to localize a leiomyoma as resident in the suprachoroidal space with an overlying layer of intact choroid. In the few cases examined by physiologic imaging, increased metabolic activity (typically associated with malignancy and inflammation) has been noted. We found that pigmented uveal leiomyomas can be clinically identical to melanoma. Therefore, histopathology with immunohistochemical staining for smooth muscle actin was the most reliable diagnostic method to differentiate pigmented uveal leiomyoma from melanoma. Treatment is governed by the clinical diagnosis, tumor size and location, as well as prognosis for vision and globe preservation.