Mitochondrial A3243G mutation results in corneal endothelial polymegathism.

PURPOSE: The mitochondrial DNA point mutation A3243G leads to a spectrum of syndromes ranging from MIDD to MELAS. Ocular manifestations include pattern macular dystrophy and concentric perifoveal atrophy. Given the high metabolic demand of corneal endothelial cells, we performed specular biomicroscopy analysis in patients harboring the mitochondrial DNA point mutation A3243G to assess for the associated presence of corneal endothelial abnormalities. METHODS: We present a case series with participants from two institutions. Patients diagnosed with macular dystrophy associated with MIDD or MELAS, and the mitochondrial DNA point mutation A3243G were recruited. Exclusion criteria included a prior diagnosis, or a positive family history, of endothelial corneal dystrophy. Slit-lamp corneal examination and specular biomicroscopy were performed. Corneal endothelial cell count, cell size and polymegathism, and central corneal thickness were assessed. Patients diagnosed with MIDD or MELAS based on clinical history and examination were genetically tested for the mitochondrial DNA point mutation A3243G using pyrosequencing. RESULTS: Five patients (two male and three female participants) from five different families, and with different ethnic backgrounds, met the inclusion criteria. Their ages ranged from 41 to 60 years. Corneal endothelial changes observed using slit-lamp examination were primarily mild to rare guttata. Specular biomicroscopy displayed mainly polymegathism associated with guttata. The average endothelial cell count was 2358 ± 456 cells per mm2, the average endothelial cell size was 442 ± 103 µm2 and the average central corneal thickness (CCT) was 551 ± 33 µm. These values were similar to that of the average population. The average coefficient of variation (COV), an index of heterogeneity in cell size, was 42.0 ± 4.1%. When compared to the average population, the average COV was significantly higher than predicted for the patients' age. None of the patients had signs of corneal edema. One patient had a pre-Descemet's opacity. CONCLUSIONS: In patients with the mitochondrial DNA point mutation A3243G, corneal endothelial polymegathism is present. This is mainly associated with mild guttata. The findings of corneal endothelial cell polymegathism may be a biomarker of mitochondrial disease, specifically in patients with the mitochondrial DNA A3243G mutation.

Effect of loteprednol etabonate 0.5% on initiation of dry eye treatment with topical cyclosporine 0.05%.

OBJECTIVE: The purpose of this work was to evaluate the effect of loteprednol etabonate (LE) before the initiation of topical cyclosporine A (tCsA) therapy in patients with mild-to-moderate dry eye disease. Prospective, multicenter randomized double-masked parallel group clinical study (NCT00407043). METHODS: Hundred and eighteen patients with dry eye disease were randomized to receive either LE and tCsA (n=61) or artificial tears (AT) and tCsA (n=57). Hundred and twelve patients completed the study (LE: n=57, AT: n=55) and are included in the data analysis. Patients self-administered either LE or AT for 2 weeks 4 times per day, followed by tCsA twice per day accompanied by either LE twice per day or AT twice per day for an additional 6 weeks of treatment. Primary outcome measures included the Ocular Surface Disease Index (OSDI) questionnaire, the Likert scale using standardized facial expressions, lissamine green staining, fluorescein staining, and the Schirmer test. Additional measures included global self-assessment, and safety outcomes included slitlamp examination, intraocular pressure, and assessment of visual acuity. RESULTS: Loteprednol etabonate pretreatment significantly reduced tCsA stinging (P<0.05). Both groups showed significantly improved OSDI scores at the 14-, 30-, and 60-day visits. Loteprednol etabonate showed significantly more OSDI improvement than AT. Both pretreatment strategies improved global self-assessment scores, Schirmer test, fluorescein staining, lissamine staining, and adjunctive AT use. Loteprednol etabonate showed superior improvement in Schirmer test, fluorescein staining, and lissamine staining. Intraocular pressure did not increase in either group. CONCLUSIONS: Loteprednol etabonate induction therapy 2 weeks before the initiation of long-term tCsA treatment for chronic dry eye disease provides more rapid relief of dry eye signs and symptoms with greater efficacy than tCsA and AT alone.

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.

Evaluation of a novel treatment, selenium disulfide, in killing Demodex folliculorum in vitro.

OBJECTIVE: To evaluate the effectiveness of varying concentrations of selenium sulfide, an active ingredient in antidandruff shampoo, in killing Demodex folliculorum. METHODS: Sixty-five eyelashes with live Demodex from 29 patients seen at the Nassau University Medical Center (17 patients) and Ophthalmic Consultants of Long Island (12 patients) were observed under light microscopy for 90 minutes in 0.1%, 0.5%, 1.0%, and 4% selenium sulfide with either carboxymethyl cellulose (CMC) solution or petroleum jelly ointment (Vaseline, Unilever, London) as excipients. Positive and negative controls also were evaluated as separate solutions of CMC, petroleum jelly, basic saline solution, 50% tea tree oil, and 100% tea tree oil. The number of Demodex deaths and time of death were recorded. RESULTS: Demodex deaths in the selenium sulfide reagents were observed in the 4% selenium sulfide solution with CMC (36.4% kill rate, 4 of 11 Demodex, average time of death 17.5 minutes, p = 0.038) and 4% selenium sulfide with petroleum jelly ointment (12.5% kill rate, 1 of 8 Demodex, time of death 75 minutes, p = 0.351). No Demodex deaths were witnessed in the other selenium sulfide treatment groups. All Demodex died in the 50% and 100% tea tree oil positive control group. CONCLUSIONS: Selenium sulfide has shown efficacy in killing Demodex at a 4% concentration with the CMC solution and mild activity with 4% petroleum jelly. More research on selenium sulfide solutions at higher concentrations is indicated.

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.

Randomized Prospective Evaluation of Microblepharoexfoliation BlephEx as Adjunctive Therapy in the Treatment of Chalazia.

PURPOSE: Chalazia are benign eyelid lesions caused by the obstruction and inflammatory reaction of the meibomian glands. Demodex mites are one potential cause of chalazia leading to mechanical obstruction of the meibomian gland. In this prospective randomized study, we examine a novel approach to treating chalazia with the use of microblepharoexfoliation (MBE), an in-office lid hygiene technique that exfoliates the eyelid margins. METHODS: Fifty patients with clinical evidence of acute chalazion were enrolled in this study. Subjects were randomly assigned to a MBE plus lid hygiene group (23 patients, mean age 66.6 ± 16.6 years) or a lid hygiene alone group (27 patients, mean age 62.1 ± 14.4). The MBE plus lid hygiene group received MBE treatment and were evaluated 1 month after the baseline visit. The main outcome measured was the resolution of the chalazion at the 1-month follow-up visit. RESULTS: The lid hygiene plus MBE treatment group demonstrated a statistically significant resolution of the chalazion compared with the lid hygiene group alone ( P = 0.007; chi-square test). Among the MBE plus hygiene group, 87% of the patients had resolution of their chalazion as opposed to the lid hygiene alone group, which had 44% resolution. CONCLUSIONS: This is the first prospective, randomized clinical trial that demonstrated efficacy of MBE as a noninvasive adjunctive treatment method for chalazion resolution.

Argon laser iridoplasty to improve visual function following multifocal intraocular lens implantation.

PURPOSE: To describe the use of argon laser iridoplasty following implantation of a multifocal intraocular lens (IOL) to improve visual function. METHODS: Argon laser spots of 500-mW power, 500-µm spot diameter, and 500-ms duration were placed in the midperipheral iris in the area in which the iris was encroaching on the IOL. RESULTS: Argon laser iridoplasty provided statistically significant improvement in visual function including corrected distance visual acuity (CDVA) and subjective quality of vision in 14 eyes from 11 patients. Mean CDVA improved from 0.24 (20/35 Snellen) to 0.10 (20/25 Snellen) logMAR (P<.0001), and mean subjective quality of vision improved from 2.9 to 7.5 (P<.0001). CONCLUSIONS: Argon laser iridoplasty should be considered in correcting visual problems associated with decentered multifocal IOLs.

Update on the Management of Demodex Blepharitis.

ABSTRACT: Demodex blepharitis is a common ophthalmologic disease that is often overlooked in the workup of blepharitis. Demodex infestation occurs in both symptomatic and asymptomatic individuals at similar rates; consequently, its role in the development of blepharitis has not been well elucidated. Two species have been confirmed to inhabit the human eyelid- Demodex folliculorum and Demodex brevis. These species differ in their preferred location of infestation, with D. folliculorum occupying the base of the eyelash and D. brevis inhabiting the meibomian glands, contributing to anterior and posterior Demodex blepharitis, respectively. A clinical index of suspicion must be developed from the history when blepharitis, conjunctivitis, or keratitis in adults and blepharoconjunctivitis or chalazia in children are resistant to treatment. The diagnosis can be strongly suspected by the presence of cylindrical dandruff at the base of the eyelash and confirmed by light microscopy of an epilated lash or by in vivo confocal microscopy. No cure is currently available. Management most frequently includes topical tea tree oil and its active ingredient terpinen-4-ol, both of which have produced good clinical outcomes. Topical tea tree oil is typically applied by a professional due to risk of toxicity. Several second-line treatment options have been studied, including ivermectin, metronidazole, selenium sulfide, microblepharoexfoliation, and lid hygiene. Novel, targeted therapies such as TP-03 (Tarsus Pharmaceuticals) are also currently being investigated in phase 2b/3 clinical trials. The purpose of this review purpose is to characterize Demodex blepharitis in detail, including its historical perspective and various classifications, and describe the latest diagnostic and management strategies.

Bell's palsy with Herpes simplex disciform keratitis: A case report.

Purpose: To report a novel case of unilateral peripheral facial nerve palsy manifesting as a complication of Herpes simplex keratitis. Observations: A 51-year-old immunocompetent male presented with severe eye pain for 3 weeks. He was found to have Herpes simplex keratitis in the left eye and started on valacyclovir 3g per day for 10 days. At three weeks his pain and blurred vision resolved. He returned 2.5 months later with worsening of vision and five days of unilateral facial numbness and weakness. Ocular evaluation revealed a visual acuity of 20/25 in the left eye, which showed mid-stromal corneal edema bordered by several keratoprecipitates. Both the keratitis and Bell's palsy markedly improved with valacyclovir, topical prednisolone, and a 10-day course of oral prednisone. Conclusions and Importance: This is the first reported case of Bell's palsy accompanying disciform keratitis. Both entities have been observed to be caused by Herpes simplex, however they have not been described together in one patient. Knowledge that these conditions may present simultaneously allows for earlier identification and treatment, resulting in decreased morbidity.

Corneal Classics: The Most-Cited Article in the 36-Year History of Cornea.

PURPOSE: To describe the most cited article in the 36 year history of Cornea. METHODS: All articles from Cornea in the past 36 years were reviewed to find the the article with the greatest number of citations. RESULTS: Descemet-Stripping Automated Endothelial Keratoplasty by Mark S. Gorovoy, MD, Cornea 2006;25:886-889 was found to have the greatest number of citations. CONCLUSION: This study led to the single greatest change of cornea transplant going from Penetrating Keratoplasty to Descemet Stripping Automated Endothelial Keratoplasty.

Nocardia keratitis mimicking superior limbic keratoconjunctivitis and herpes simplex virus.

PURPOSE: Nocardia keratitis is a rare type of infectious keratitis and may mimic other corneal diseases and lead to delay in diagnosis. This case illustrates how Nocardia often escapes accurate diagnosis due to its insidious onset, variable clinical manifestations, and unusual characteristics on cultures. OBSERVATION: The patient presented with an epithelial defect and superior pannus and scarring, which was misdiagnosed as superior limbic keratoconjunctivitis (SLK) and herpes simplex virus (HSV) keratitis. Repeat corneal scraping cultures, smears, and conjunctival biopsy were necessary to elucidate the diagnosis. It can be effectively treated with the intravenous preparation of trimethoprim-sulfamethoxazole 80 mg/mL (brand name SEPTRA) used topically as eye drops. CONCLUSION: The diagnosis of Nocardia keratitis relies on a high clinical suspicion and a prompt corneal scraping with culture. Due to its potential for rapid resolution with early therapy, it is important to isolate Nocardia early in its disease course. IMPORTANCE: Topical amikacin had been the standard of care for Nocardia keratitis for many years. However, recently there is increasing resistance of Nocardia to amikacin. SEPTRA offers an alternative therapy. Nocardia keratitis mimics other infectious and inflammatory etiologies so rapid diagnosis and treatment is critical in the prevention of long-term complications.

Double-Masked and Unmasked Prospective Study of Terpinen-4-ol Lid Scrubs With Microblepharoexfoliation for the Treatment of Demodex Blepharitis.

PURPOSE: To evaluate terpinen-4-ol lid scrubs (Cliradex) with Microblepharoexfoliation (MBE) (BlephEx) in the treatment of Demodex blepharitis. METHODS: An Institutional Review Board (IRB) approved the randomized prospective double-masked trial of 50 patients with Demodex positive blepharitis. All subjects were given an in-office MBE treatment and randomized to masked lid scrubs (terpinen-4-ol or sham) twice daily for 1 month. All subjects then returned for an additional MBE treatment and received open-label terpinen-4-ol scrubs twice daily for 1 month. OUTCOMES: Demodex levels, Ocular Surface Disease Index, tear osmolarity, MMP-9, Schirmer 1 and grading of the lid margin appearance, meibomian gland dropout, and meibomian gland secretions. RESULTS: Forty-six subjects (23 per group) were included for the analysis; 4 lost to follow up. Total Demodex folliculorum levels in the Cliradex group improved: 4.7 to 3.6 (first month interval P = 0.266) to 2.6 (second month interval P = 0.279), overall P < 0.05 over 2 months. Total levels in the sham group improved: 5.1 to 3.0 (first month interval P < 0.05) to 2.5 (second month interval P = 0.496), overall P < 0.05 over 2 months. No clear statistically meaningful improvements in other dry eye and blepharitis data metrics were observed. CONCLUSIONS: In-office MBE treatments combined with either Cliradex terpinen-4-ol medicated lid scrubs or sham scrubs showed a statistically significant reduction in D. folliculorum infestation levels; however, conclusions on clinical significance could not be made. In-office MBE with terpinen-4-ol lid scrubs showed no significant improvement over sham scrubs.

Influence of bacterial burden on meibomian gland dysfunction and ocular surface disease.

PURPOSE: Bacterial burden on the eyelid margin and within meibomian glands was evaluated for influence on specific ocular surface disease (OSD) markers across the meibomian gland dysfunction (MGD) spectrum. METHODS: In this prospective, observational, single-center study, 40 patients were divided into 4 equal groups of 10 that encompassed increasingly worse MGD/OSD categories. All patients answered the standard Ocular Surface Disease Index questionnaire, and underwent tear osmolarity testing (TOT), Schirmer 1, matrix metalloproteinase 9 (MMP-9) testing, meibography, and lissamine green staining. Cultures of eyelid margins and meibomian gland secretions were directly plated on blood, chocolate, and Sabouraud agar; smears were sent for gram and Papinicolau evaluation. RESULTS: Mean patient age was 55.25±17.22 years; there were 10 males and 30 females. TOT and MMP-9 testing were similar across groups. Culture positivity was 62.5% for right eyes, 70% for left eyes, and was not statistically different across groups (for both eyelid margin and meibomian glands). The majority of cultures were positive for coagulase-negative staphylococcus (CNS). CONCLUSION: This study is in concordance with others, citing the predominance of CNS within the biofilm of both "normal" and clinically significant MGD/OSD patients. Our study exemplifies that symptoms of OSD do not necessarily correlate with degree of clinical exam findings, nor culture positivity. These results argue that bacterial burden should be reconsidered as a direct risk factor and treatment target for MGD/OSD patients.

Differential Bacterial Colonization and Biofilm Formation on Punctal Occluders.

Dry eye is a common condition that is treated primarily by topical lubricants, immunomodulation, and a variety of punctal and canalicular plugs (occluders). Biofilm formation has been reported as an ongoing problem with the clinical use of occluders. In order to explore the role of biofilm formation on occluders, we tested the bacteria strain, Staphylococcus aureus, with three different types of occluders, DeltaR, OdysseyR, and AlphamedR. Scanning electron microscopy (SEM) of these occluders revealed a variation in surface appearance, with OdysseyR being the smoothest (but with grooves), followed by DeltaR, and AlphamedR. Exposing each type of occluder to dynamically grown bacterial cultures of S. aureus, a ~3 fold statistically significant difference in bacteria colonization between the OdysseyR and AlphamedR occluder and a ~2 fold higher trend between OdysseyR and DeltaR were detected. These quantitative results were also verified with SEM, showing extensive S. aureus colonization and biofilm formation on the surface of the OdysseyR occluder. The results also indicate that bacterial colonization readily occurs on all three types of occluders. The occluder with the smoothest but grooved surface (OdysseyR), displayed increased biofilm formation when compared to those with rougher surfaces.

Visual and keratometric outcomes of keratoconus patients after sequential corneal crosslinking and topography-guided surface ablation: Early United States experience.

PURPOSE: To evaluate a sequential treatment algorithm for visual and keratometric improvement in keratoconus patients after corneal crosslinking (CXL) followed by topography-guided photorefractive keratectomy (PRK). SETTING: Ophthalmic Consultants of Long Island, Garden City, New York, USA. DESIGN: Retrospective case series. METHODS: This study reviewed patients with keratoconus who had CXL followed by custom topography-guided PRK between April 2016 and December 2016. The following data were collected at baseline, the time of CXL, and 3 months and 6 months after PRK: uncorrected (UDVA) and corrected (CDVA) distance visual acuities, keratometric astigmatism, spherical equivalent, maximum and mean keratometry readings, and corneal thickness at the cone apex. Demographic data, age at time of CXL and PRK, and time elapsed between CXL and PRK were analyzed for significance and a correlation with visual and astigmatic outcomes. RESULTS: The study comprised 56 patients (62 eyes), 34 who had both topographic and refractive treatment and 28 patients who had treatment of topographic irregularities only. The mean age was 38.08 years ± 13.07 (SD) at CXL and 40.33 ± 13.44 years at topography-guided PRK. Six months after PRK, there was a significant improvement in UDVA and CDVA in the refractive group (20/60 and 20/30, respectively) versus the nonrefractive group (20/100 and 20/40, respectively). Ninety-three percent of eyes that had refractive treatment had 20/40 or better CDVA. There were no significant adverse events in any case. CONCLUSIONS: The data support the use of refractive treatment in addition to topographic treatment for visual improvement in patients with keratoconus having CXL and PRK.

Late-Onset Microsporidial Keratitis in Femtosecond Astigmatic Keratotomy After Laser-Assisted Phacoemulsification.

PURPOSE: To present a case of microsporidial keratitis in a femtosecond laser-created astigmatic keratotomy (AK) incision. METHODS: Case report. RESULTS: A 65-year-old Middle Eastern man presented 2 months after uncomplicated femtosecond laser-assisted cataract surgery (FLACS) and AK with mildly decreased vision and corneal edema in the operative eye. Shortly after treatment with topical corticosteroids, a fulminant corneal infiltrate manifested along the temporal arcuate incision. Multiple corneal scrapings sent for laboratory analysis were inconclusive. Two weeks after the initial presentation, a deep tissue sample was obtained using a 27-gauge cannula passed within the arcuate incision. The gram stain was directly observed, revealing intracellular microsporidial spores. The patient was treated with oral albendazole 400 mg once daily over 2 weeks and topical voriconazole 1% and fumagillin 3 mg/mL eye drops over 10 weeks. During this course, visual function steadily recovered as the infiltrate coalesced and ocular inflammation subsided. CONCLUSIONS: This is the first reported case of microsporidial keratitis presenting as a late-onset infection after femtosecond laser-assisted AK.

Long-term resolution of chronic dry eye symptoms and signs after topical cyclosporine treatment.

PURPOSE: To report the resolution of symptoms and signs of chronic dry eye for at least 1 year after completion of a minimum 6-month course of topical 0.05% cyclosporine in a subgroup of patients from 2 practices. DESIGN: Retrospective case series. PARTICIPANTS: Eight patients who had had chronic dry eye disease for 3 to 20 years were treated with topical cyclosporine for 6 to 72 months, and then remained free of symptoms or signs of disease for at least 1 year (range, 16-29 months; mean, 21 months) after stopping treatment. INTERVENTION: Treatment with topical 0.05% cyclosporine with or without topical corticosteroids twice a day along with nonpreserved artificial tears. Tests performed were patient history, slit-lamp examination, rose bengal and fluorescein staining of the ocular surface, tear breakup time, and Schirmer's test with anesthesia. MAIN OUTCOME MEASURES: Resolution of all symptoms and signs of dry eye disease after a minimum 6-month course of topical 0.05% cyclosporine. RESULTS: Eight patients, including approximately 4% of the total patients with chronic dry eye treated with 0.05% cyclosporine in one author's practice, were free of signs or symptoms of dry eye disease a minimum of 1 year after completing a 6- to 72-month course of therapy. There were no parameters noted that distinguished these patients from others who required maintenance topical cyclosporine or who did not appear to respond to topical cyclosporine. CONCLUSIONS: Topical cyclosporine treatment appears to be associated with a cure of symptoms and signs in a subgroup of chronic dry eye patients. In such patients, presumably there is effective elimination of inflammatory processes underlying chronic dry eye disease. Such patients should be monitored long term because a return of disease may be noted. These results suggest that topical cyclosporine treatment halts progression of chronic dry eye in some patients.

Methicillin-resistant Staphylococcus aureus infectious keratitis following refractive surgery.

PURPOSE: To elucidate risk factors, clinical course, visual outcomes, and treatment of culture-proven methicillin-resistant Staphylococcus aureus (MRSA) infectious keratitis following refractive surgery. DESIGN: Interventional case series. METHODS: Multicenter chart review of 13 cases of MRSA keratitis following refractive surgery and literature review. RESULTS: Thirteen eyes of 12 patients, nine of whom were either healthcare workers or exposed to a hospital surgical setting, developed MRSA keratitis following refractive surgery. All patients presented with a decrease in visual acuity and complaints of pain or irritation in the affected eye. Common signs on slit-lamp biomicroscopy were corneal epithelial defects, focal infiltrates with surrounding edema, conjunctival injection, purulent discharge, and hypopyon. All patients were diagnosed with infectious keratitis on presentation and treated with two antibiotics. All eyes were culture-positive for MRSA. CONCLUSIONS: According to a computerized MEDLINE literature search, this is the first case series of MRSA infectious keratitis following refractive surgery, the first reports of MRSA keratitis after refractive surgery in patients with no known exposure to a healthcare facility, the first report of MRSA keratitis after a laser in situ keratomileusis (LASIK) enhancement, and the first reports of MRSA keratitis after prophylaxis with fourth-generation fluoroquinolones. MRSA keratitis is a serious and increasing complication following refractive surgery. Patients with exposure to a healthcare environment should be considered at additional risk for developing MRSA keratitis. However, in addition, surgeons should now be vigilant for community-acquired MRSA. Prompt identification with culturing and appropriate treatment of MRSA keratitis after refractive surgery is important to improve visual rehabilitation.

High-frequency ultrasound of extranodal limbal Rosai-Dorfman disease: affecting the conjunctiva, sclera, and cornea.

PURPOSE: To correlate the clinical, high-frequency ultrasound, and pathology characteristics of an epibulbar Rosai-Dorfman tumor. METHODS: We report a case of a steroid-resistant yellow perilimbal epibulbar tumor referred for ophthalmic oncology evaluation. It was documented by slit-lamp photography and evaluated by high-frequency ultrasound. A hematology-oncology evaluation and excisional biopsy were performed. RESULTS: Ophthalmic examination revealed a solitary yellow perilimbal epibulbar tumor. High-frequency ultrasound imaging revealed low internal reflectivity and partial-thickness scleral and corneal invasion with no extension into the anterior segment. Primary excision was performed. Although histopathology revealed large atypical histiocytes, immunochemistry found them to be both S-100 positive and CD1a negative (diagnostic of Rosai-Dorfman disease). Hematology-oncology evaluation revealed no systemic disease or links to human herpesvirus. Local control required cryotherapy and sub-Tenon steroid injection. CONCLUSIONS: Epibulbar Rosai-Dorfman tumors can invade the sclera and are often treated by surgical excision. High-frequency ultrasound imaging should be used to determine the presence or extent of invasion before surgery.