HOW TO MEASURE THE LARGEST BASAL DIMENSION OF CHOROIDAL MELANOMA: A MATHEMATICAL STUDY.

PURPOSE: To identify the most accurate ultrasonographic technique to measure the largest basal dimension (LBD) of choroidal melanoma. METHODS: B-scan ultrasound images were retrospectively reviewed in 99 eyes of 99 choroidal melanoma patients. The LBD was measured using one, two, and three straight lines along the inner and outer sclera. Theoretical arc length, calculated using trigonometry formulas based on the spherical model with the axial length as the sphere diameter, was used for comparisons with the actual measurements using straight lines. RESULTS: For straight-line measurements in the inner sclera, the lowest error was found when using two straight-line measurements (P = 0.118). Differences in measurement using one-segment or three-segment measurements as compared with the theoretical arc length were found to be statistically significant (P < 0.001 in both cases). For tumors with LBD smaller than 12 mm, the absolute error, compared with the theoretical arc length, was smaller than 1 mm. In the outer sclera, the smallest errors were also found for measurements using two straight-line segments; however, it was statistically different than the theoretical inner arc length (P < 0.001). CONCLUSION: When using ultrasound to estimate LBD of ocular tumors, 2 straight-line measurements should be used when LBD is larger than 12 mm. For tumors with LBD smaller than 12 mm, measurements using 1 straight-line segment can provide accurate estimates.

Cavitary Choroidal Metastasis from Clear Cell Renal Cell Carcinoma.

PURPOSE: To report the clinical, funduscopic, and echographic features of a choroidal metastatic tumor from a clear cell renal cell carcinoma presenting with intralesional cavitation. CASE REPORT: An 81-year-old man with history of clear cell renal cell carcinoma presented with blurred vision. A clinical examination including standardized ultrasound was performed, which revealed a choroidal mass with cystic-like cavitation. The patient underwent brachytherapy with regression of the tumor. CONCLUSIONS: We report the features of a cavitary choroidal metastasis from clear cell renal cell carcinoma. Occurrence of features usually associated with uveal melanoma in a patient with a metastatic choroidal tumor may lead to misdiagnosis.

Iris nodules secondary to retained PFO in the anterior chamber following macula-off rhegmatogenous retinal detachment repair.

PURPOSE: To describe a case of PFO retention, migration into the anterior chamber, and the subsequent formation of iris nodules following the repair of a giant retinal tear. METHODS: Patient data was collected via manual chart review. All patient information was deidentified. RESULTS: Slit lamp photos demonstrate nodules on the iris surface and retained PFO in the anterior chamber. Ultrasound biomicroscopy shows multifocal hyperreflective signals on the iris surface, corresponding to the retained PFO within the iris. CONCLUSION: Retention and migration of PFO to the anterior chamber following retinal detachment repair can lead to formation of nodules on the surface of the iris.

Endophthalmitis in Advanced Microbial Keratitis: Risk Factors and Examination Findings.

PURPOSE: To evaluate clinical and ultrasonographic findings associated with advanced microbial keratitis with concurrent endophthalmitis. METHODS: We performed a retrospective analytical study of patients with severe microbial keratitis who underwent ocular ultrasonography for possible endophthalmitis at our institution over a 5-year period. Ocular and medical history, ocular examination findings, microbiology results, and ultrasonographic features were collected. RESULTS: Of the 81 included eyes, 15 were diagnosed with endophthalmitis based on clinical findings and evidence of vitritis on ultrasound. Historical factors and physical examination findings more common in the endophthalmitis group than the keratitis-only group include presenting vision of light perception or no light perception (73.3% vs. 31.8%, P = 0.003), history of cataract surgery (80.0% vs. 45.5%, P = 0.02), and full thickness ulcer or perforation (33.3% vs. 9.1%, P = 0.03). Gram-positive organisms were more common in the endophthalmitis group (60.0% vs. 30.3%, P = 0.04). Ultrasound findings of choroidal thickening and retinal detachment were more common in the endophthalmitis group (26.7% vs. 3.4%, P = 0.01 and 13.3% vs. 8.4%, P = 0.04) in addition to the expected preponderance of membranous and dispersed vitreous opacities in that group. Final visual acuity was worse in the endophthalmitis group (2.3 vs. 3.5 LogMAR, P = 0.02). CONCLUSIONS: Diagnosing endophthalmitis can be difficult in eyes with advanced microbial keratitis, and the visual outcomes are often extremely poor. Clinicians should have a particularly high index of suspicion in eyes presenting with light perception or no light perception vision, history of cataract extraction, full thickness ulcer or perforation, or choroidal thickening or retinal detachment on ultrasound.

Size Matters for Interplicata Diameter: A Case-Control Study of Plateau Iris.

PURPOSE: Ultrasound biomicroscopy (UBM) has been used to characterize anterior segment dimensions in plateau iris configuration (PIC), but transverse measurements between the recesses of the ciliary sulcus (sulcus-to-sulcus diameter [STSD]) and the ciliary body processes (interplicata diameter [IPD]) have not been reported. We measured STSD and IPD and compared these among eyes with PIC, primary angle closure (PAC), and control eyes with open angles. DESIGN: Retrospective, cross-sectional clinical study. PARTICIPANTS: Sixty-nine participants, 37 PIC, 13 PAC, and 19 controls. METHODS: We searched our clinical UBM database for PAC and PIC cases. Controls were assembled by reviewing images obtained for surveillance of ocular surface lesions. Anterior segment measurements were performed using the UBM digital caliper tool. Robust-fit ANOVA identified among-group differences. Pairwise t tests were used to test the significance of between-group differences. MAIN OUTCOME MEASURES: Anterior chamber depth (ACD), angle opening distance (AOD), ciliary body area and thickness, iris area, horizontal and vertical STSD, and horizontal and vertical IPD. RESULTS: Fifty-five left eyes were analyzed (30 PIC, 10 PAC, and 15 controls). ACD was smaller in PAC than in PIC and control eyes (P < 0.05 for PIC vs. PAC; P < 0.01 for control vs. PAC). Mean AOD was smaller in PIC than controls (P < 0.05) and smaller in PAC than PIC (P < 0.001). Vertical STSD was smaller in both PAC and PIC than controls (P = 0.04 for PIC vs. control; P < 0.01 for PAC vs. control). Horizontal STSD was smaller in PIC than controls (P = 0.02). Vertical IPD was smaller in PIC than controls (P = 0.04) and smaller in PAC than PIC eyes (P = 0.02). Horizontal IPD was smaller in PIC and PAC than controls (P = 0.03 for PIC vs. control; P < 0.01 for PAC vs. control). CONCLUSIONS: STSD and IPD are narrower in PIC and PAC than in healthy eyes. Further studies that examine the ratio of white-to-white cornea diameter to the IPD may provide a mechanism for reported cases of in-the-bag uveitis-glaucoma-hyphema syndrome in PIC.

Novel TMEM98, MFRP, PRSS56 variants in a large United States high hyperopia and nanophthalmos cohort.

Nanophthalmos is a rare condition defined by a small, structurally normal eye with resultant high hyperopia. While six genes have been implicated in this hereditary condition (MFRP, PRSS56, MYRF, TMEM98, CRB1,VMD2/BEST1), the relative contribution of these to nanophthalmos or to less severe high hyperopia (≥ + 5.50 spherical equivalent) has not been fully elucidated. We collected probands and families (n = 56) with high hyperopia or nanophthalmos (≤ 21.0 mm axial length). Of 53 families that passed quality control, plausible genetic diagnoses were identified in 10/53 (18.8%) by high-throughput panel or pooled exome sequencing. These include 1 TMEM98 family (1.9%), 5 MFRP families (9.4%), and 4 PRSS56 families (7.5%), with 4 additional families having single allelic hits in MFRP or PRSS56 (7.5%). A novel deleterious TMEM98 variant (NM_015544.3, c.602G>C, p.(Arg201Pro)) segregated with disease in 4 affected members of a family. Multiple novel missense and frameshift variants in MFRP and PRSS56 were identified. PRSS56 families were more likely to have choroidal folds than other solved families, while MFRP families were more likely to have retinal degeneration. Together, this study defines the prevalence of nanophthalmos gene variants in high hyperopia and nanophthalmos and indicates that a large fraction of cases remain outside of single gene coding sequences.

Ultrasound features of orbital granular cell tumor.

The authors report the echographic characteristics of a rare orbital granular cell tumor and correlate these findings with histopathology. A 56-year-old woman presented with proptosis. Complete ophthalmic and ultrasound examinations were performed. Ultrasound revealed an oval, well-outlined orbital mass in the intraconal space with low-medium reflectivity and regular internal structure. An orbitotomy with complete excision of the tumor was performed. Histopathologic evaluation showed sheets and nests of cells with abundant eosinophilic and granular cytoplasm in a uniform distribution throughout the lesion. The echographic characteristics correlated well with the morphologic surgical findings and the histologic architecture. This is the first report describing the echographic characteristics of orbital granular cell tumor.

Nanophthalmos: A Review of the Clinical Spectrum and Genetics.

Nanophthalmos is a clinical spectrum of disorders with a phenotypically small but structurally normal eye. These disorders present significant clinical challenges to ophthalmologists due to a high rate of secondary angle-closure glaucoma, spontaneous choroidal effusions, and perioperative complications with cataract and retinal surgeries. Nanophthalmos may present as a sporadic or familial disorder, with autosomal-dominant or recessive inheritance. To date, five genes (i.e., MFRP, TMEM98, PRSS56, BEST1, and CRB1) and two loci have been implicated in familial forms of nanophthalmos. Here, we review the definition of nanophthalmos, the clinical and pathogenic features of the condition, and the genetics of this disorder.

Comparative Study of Clinical, Ultrasonographic, Conventional Imaging, and Ultra-Wide-Field Fundus for Measurements of the Longest Basal Diameter of Choroidal Tumors.

BACKGROUND AND OBJECTIVE: To compare the maximum basal diameter of choroidal tumors measured by clinical estimation and different imaging methods in intraocular tumors for therapeutic planning. PATIENTS AND METHODS: Retrospective, observational study reviewing the measurements of the longest basal diameter of choroidal tumors using the clinical estimation based on the indirect ophthalmoscopy, ophthalmic ultrasonography, 50° fundus imaging, ultra-wide-field imaging color composite, red laser, and green laser. RESULTS: The study included measurements of 74 tumors in 74 eyes. Correlation analysis between imaging measurements and clinical estimates led to following results: ultra-wide-field imaging measurements were 2.7% to 7.1% smaller than estimated clinical measurements by indirect ophthalmoscopy; followed by ultrasound, with 10.9% smaller measurements; and 50° fundus imaging, measuring 25.8% smaller than clinical estimates. When evaluating the precision of tumor measurements, ultrasound resulted in the highest precision, with a standard error of 1.6 mm compared to clinical estimates, followed by other imaging techniques with standard errors ranging from 2.4 mm to 3.0 mm. Determination of tumor margins with the ultrasound technique was better defined when subretinal fluid was present, or when the height of the tumor was greater than 3 mm. CONCLUSION: The authors' results suggest that measurements of the longest basal diameter of choroidal tumors can be accurately performed with ultrasound and ultra-wide-field color composite or red laser images. In the presence of subretinal fluid or tumor height above 3 mm, ultrasound can better delineate tumor margins. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:459-464.].

Clinical, histologic, and immunohistochemical evaluation of iris metastases from small cell lung carcinoma.

CASE REPORT: We report the case of a patient with small cell lung carcinoma who developed multiple metastases in the iris. The eye was enucleated after failure of a conservative approach. The specimen underwent histologic and immunohistochemical evaluation that confirmed the diagnosis. COMMENTS: Metastatic carcinoma is the most common form of intraocular malignancy, and breast is the most prevalent primary location, followed by lung. Small cell lung carcinoma constitutes 23.1% of lung neoplasias. It has a significantly worse prognosis than non-small cell carcinoma due to its propensity for early and widespread dissemination. Uveal metastases involve the choroid more often than the iris and ciliary body, probably because the arterial distribution is more abundant in the posterior than in the anterior uvea. Iris involvement represents only 9% of the metastatic uveal tumours.

Acetazolamide-Induced Bilateral Ciliochoroidal Effusion Syndrome in Plateau Iris Configuration.

PURPOSE: Our purpose is to describe a 60-year-old male, who has plateau iris configuration and developed bilateral ciliochoroidal effusion syndrome after ingestion of acetazolamide. OBSERVATIONS: Our case was a research participant in a multi-center clinical study (ClinicalTrials.gov NCT01677507). During the course of this study, he was treated with a single dose of acetazolamide (500 mg), and seven days later treated with latanoprost one drop daily at bedtime both eyes for seven days, and then was administered another dose of acetazolamide (500 mg). Several hours later he complained of blurred vision in the distance and mild headache. On examination, he had a myopic shift, intraocular pressures of 36 mmHg in right eye and 35 mmHg in left eye, shallow anterior chambers both eyes, and occluded angles by gonioscopy both eyes. An echographic exam confirmed the bilateral ciliochoroidal effusion syndrome. He was treated by no further dosing of acetazolamide and started on timolol, atropine and prednisolone. Two weeks later, the bilateral choroidal effusion and acute angle closure were resolved. Repeat echography showed plateau iris configuration. CONCLUSIONS AND IMPORTANCE: To the best of our knowledge, drug-induced bilateral ciliochoroidal effusion syndrome has not been reported with acetazolamide in plateau iris configuration.

Acute bilateral angle closure glaucoma induced by methazolamide.

PURPOSE: To report a case of bilateral acute myopia and angle closure glaucoma after ingestion of methazolamide. METHODS: An interventional case report of a 70-year-old male who developed bilateral, acute myopia and angle closure glaucoma after ingesting methazolamide tablets for the treatment of normal tension glaucoma. RESULTS: Bilateral anterior chamber shallowing associated with ciliary body edema, supraciliary effusions, and shallow posterior choroidal effusions were documented with slit-lamp photography and high-frequency ultrasonography. Near complete resolution of these signs after discontinuation of methazolamide were also documented. CONCLUSION: Methazolamide may be associated with secondary myopia and angle closure glaucoma. Discontinuation of methazolamide leads to resolution of this process, as documented by slit-lamp photography and high-frequency ultrasonography.

Correlation between baseline echographic features of endophthalmitis, microbiological isolates, and visual outcomes.

BACKGROUND: The purpose of this study was to examine the baseline echographic features in culture-positive and culture-negative endophthalmitis and to correlate these echographic features with final visual outcomes. METHODS: We identified a retrospective noncomparative case series of patients with a clinical diagnosis of endophthalmitis and a baseline echographic examination between 1996 and 2010 at a single institution. Graded echographic features studied included: dense, moderate, and mild vitreous opacities; marked, moderate, and mild vitreous membranes; retinal detachment; and choroidal detachment. These were compared between culture-negative, coagulase-negative staphylococci, and other culture-positive cases of endophthalmitis, and were correlated with final visual outcomes. RESULTS: Of 149 patients reviewed, 60 were culture-negative, 26 grew coagulase-negative staphylococci, and 60 grew other culture-positive species. Three had multiple culture isolates. The presence of dense, moderate, and mild vitreous opacities, marked, moderate, and mild vitreous membranes, retinal detachment, and choroidal detachment was not significantly different between the three groups (P = 0.86, P = 0.56, P = 0.34, P = 0.45, respectively). The presence of advanced echographic features of dense vitreous opacities, marked vitreous membranes, retinal detachment, and choroidal detachment was correlated with poorer visual acuity outcomes (P = 0.005, P = 0.0001, P < 0.0001, and P < 0.0001, respectively). CONCLUSION: No correlation could be made between echographic features and the organism. The presence of advanced echographic features, such as dense vitreous opacities, marked vitreous membranes, retinal detachment, and choroidal detachment, was correlated with worse visual outcomes.

Treatment of late-onset capsular distension syndrome with a neodymium:YAG laser peripheral iridotomy and anterior capsulotomy.

We describe a new approach to treat late-onset capsular distension syndrome in which the fluid in the capsular bag is cloudy and prevents a posterior neodymium:YAG (Nd:YAG) laser capsulotomy. A peripheral laser iridotomy is created through which the anterior lens capsule peripheral to the IOL optic is accessed. This opening in the iris provides an access point through which an anterior Nd:YAG laser capsulotomy can be performed. Following disruption of the anterior lens capsule, the capsular fluid is released into the anterior chamber and absorbed through the inherent drainage system of the eye. This approach avoids the need for a more invasive surgical intervention.

Mushroom-shaped secondary vasoproliferative tumor.

The authors report a clinical and echographic correlation of a retinal vasoproliferative tumor that assumed a mushroom shape. A 64-year-old man with a history of retinal detachment repair presented with vitreous hemorrhage. A clinical examination including standardized ultrasound was performed, which revealed a mushroom-shaped tumor with high reflectivity. The patient underwent pars plana vitrectomy with fine needle aspiration biopsy. Histopathology of the biopsy specimen demonstrated vascular proliferation. The lesion was diagnosed to be a retinal vasoproliferative tumor. Clinicians should be aware that a mushroom-shaped configuration can be seen in such cases, although it is common for uveal melanoma.

Epibulbar foreign body granuloma masquerading as extrascleral extension following brachytherapy for choroidal melanoma.

PURPOSE: To describe a patient with epibulbar foreign body granuloma following brachytherapy for choroidal melanoma and to illustrate the echographic features that aided in the differential diagnosis. METHODS: Observational case report. Patient had multiple clinical exams over time to evaluate a treated choroidal melanoma. PATIENTS: A 61-year-old man presented for echographic follow-up examination following treatment for choroidal melanoma. An epibulbar lesion was noted adjacent to the site of the previously treated choroidal melanoma. Incisional biopsy and histologic evaluation were required. RESULTS: Ultrasonography revealed regression of the intraocular tumor; however, an oval epibulbar mass with irregular internal structure, irregular reflectivity, and no vascularity was noted. The echographic features were not typical of extrascleral extension of choroidal melanoma. Histopathologic evaluation showed fibrous connective tissue with an intense infiltrate of histiocytes with occasional giant cells and rare birefringent foreign body materials. CONCLUSION: Foreign body granuloma should be considered in the echographic differential diagnosis of an atypical extrascleral lesion following uveal melanoma brachytherapy.

Presumed eccrine carcinoma metastatic to the choroid.

The purpose of this article is to describe a patient with presumed choroidal metastasis from an eccrine adenocarcinoma of the scalp. A 45-year-old Caucasian woman presented with decreased visual acuity. Ophthalmologic examination was unremarkable. Her past medical history was significant for eccrine carcinoma of the scalp. The patient developed bilateral cervical lymph node metastases, and received chemotherapy and radiotherapy. She had recurrence of the scalp lesion and developed bone metastasis. The patient was again referred to an ophthalmologist owing to reduced visual acuity. Multiple choroidal metastases were detected in right eye, and one metastatic lesion in left eye. The patient passed away 2 months after choroidal metastases. This is the first report of choroidal metastases from an eccrine carcinoma. This is a rare aggressive neoplasm with poor outcome in most cases of metastatic disease, and in this case report, uveal metastasis was indicative of poor prognosis.

Ciliary body medulloepithelioma: clinical, ultrasound biomicroscopic and histopathologic correlation.

A 3-year-old girl presented with a distorted pupil and decrease of visual acuity. A ciliary body mass at the superior-temporal quadrant could be seen at slit-lamp examination. Ultrasound biomicroscopy revealed a lesion with lobulated surface, arising at the ciliary body, composed of compact and cystic areas. An iridocyclectomy was performed but the tumour recurred after a few months. The eye was then enucleated. Histopathologic evaluation showed sheets and cords of proliferated medullary epithelium. Cystic spaces and Flexner-Wintersteiner rosettes were also seen. Those findings correlated well with ultrasound biomicroscopic images. The knowledge of echographic characteristics can significantly assist in the diagnosis of medulloepithelioma of the ciliary body.