Plateau iris in children.

Narrow iridocorneal angles, a very rare condition in the pediatric population, can lead to visual loss through angle closure glaucoma. In the workup for patients with narrow iridocorneal angles, plateau iris must be considered in the differential diagnosis. We describe 5 children with plateau iris, the youngest 5 years of age. All were confirmed using ultrasound biomicroscopy and were offered iridotomy for treatment.

Role of optical coherence tomography in verifying the specificity of ultrasonography in detecting subtle subretinal fluid associated with small choroidal melanocytic tumors.

PURPOSE: To report the use of optical coherence tomography (OCT) in verifying the specificity of the ultrasonographic "apical double echo" sign in detecting subtle subretinal fluid (SRF) associated with small choroidal melanocytic tumors. METHODS: Retrospective review of consecutive patients demonstrating ultrasonographic "apical double echo," indicative of subtle SRF, who concurrently underwent OCT on initial evaluation of untreated small choroidal melanocytic tumors. Ultrasonography was performed with eyecubed version 3, ophthalmic ultrasound system (Ellex/Innovative Imaging, Inc, Adelaide, Australia) and OCT with spectral-domain OCT, Cirrus HD-OCT, model 4000 (Carl Zeiss; Meditec, Dublin, CA). Optical coherence tomographic images were examined to verify the presence of SRF and to identify other retinal pathology producing apical double echo in the absence of SRF. RESULTS: The study included 36 patients. With OCT, subtle SRF was present at tumor apex and/or margins in 20 patients (55%) and absent in 16 patients (45%). When SRF was present, it was detected at tumor apex only in 3 of 20 patients (14%), gravitated at the inferior margin only in 13 of 20 patients (64%), and at apex and inferior margin in 4 of 20 patients (22%). When SRF was absent, the retina over the tumor showed degenerative cystic changes in 10 of 16 patients (65%), retinal thickening in 4 of 16 patients (25%), and retinal pigment epithelium detachment in 2 of 16 patients (10%), accounting for the ultrasonographic apical double echo. CONCLUSION: Ultrasonography showed limited specificity for detecting subtle SRF related to small choroidal melanocytic tumors, with 45% of patients showing ultrasonographic apical double echo in the absence of SRF with OCT. The retinal changes that yielded false-positive results included retinal cystic degenerations, retinal thickening, and retinal pigment epithelium detachment. Optical coherence tomography is preferred to verify the presence of subtle SRF over small choroidal melanocytic tumors, provided that the inferior tumor margin is included in imaging.

Spectral-domain optical coherence tomography versus ultrasound biomicroscopy for imaging of nonpigmented iris tumors.

PURPOSE: To evaluate the use of spectral-domain optical coherence tomography (SDOCT) for imaging of nonpigmented iris tumors, through its comparison with ultrasound biomicroscopy (UBM). DESIGN: Retrospective observational case series. METHODS: Consecutive patients with non-pigmented iris tumors, not extending to the ciliary body, who were concurrently evaluated with SD-OCT and UBM were included. Demographics, anterior segment clinical photographs, images obtained with SD-OCT (Cirrus HD-OCT, Zeiss Meditec, Dublin, California, USA) with 5.1.1 anterior segment software upgrade, and UBM (Humphrey Instruments, San Leandro, California, USA) were reviewed. The images produced were compared regarding the degree of anterior and posterior tumor surface resolution, internal structures, tumor thickness measurement, image artefacts, and overall tumor visualization. RESULTS: Thirty-seven patients with nonpigmented iris tumors were included. Comparing SDOCT to UBM, the image definitions of anterior tumor surface and internal tumor heterogeneity were equivalent. Posterior tumor surface was well defined in 54% of SDOCT vs 100% in UBM images. Full tumor thickness measurement was possible in 86% of SDOCT vs 100% with UBM. The maximum measurable tumor thickness with SDOCT was 1.34 mm. SDOCT images showed optical aberrations such as shadowing and ghost images in 22 tumors (59%), which encroached on the tumor image in 8 patients (22%). The overall tumor visualization with SDOCT was possible in 65% of the iris tumors. CONCLUSIONS: UBM generally provides superior imaging quality and reproducible measurements of nonpigmented iris tumors. Nevertheless, SDOCT, being a noncontact technique, can be a reliable alternative in imaging and following some selected nonpigmented iris tumors.

Bilateral multifocal choroidal melanosis: a report of two cases.

Flat choroidal pigmented lesions have few differential diagnoses but encompass a wide range of clinical presentations. The authors report two cases of bilateral multifocal choroidal hyper-pigmented patches discovered in asymptomatic patients. Fluorescein angiography showed a normal pattern without blockage in the hyperpigmented areas with an otherwise normal choroidal vascular architecture. Optical coherence tomography with enhanced depth imaging demonstrated normal choroidal thickness and structure in the hyperpigmented areas. These cases may represent an atypical presentation of previously described bilateral isolated choroidal melanocytosis or a different clinical entity. Histopathological evidence and longer follow-up of these patients and patients with isolated choroidal melanocytosis may elucidate the best descriptive term for this unusual condition.

Ultrasound biomicroscopy of the ciliary body in ocular/oculodermal melanocytosis.

PURPOSE: To describe the ultrasound biomicroscopy (UBM) findings of the ciliary body in patients with ocular/oculodermal melanocytosis. DESIGN: Retrospective observational case series study. METHODS: A retrospective chart and imaging database review was conducted for patients with unilateral ocular/oculodermal melanocytosis who underwent UBM examination at the Ocular Oncology Clinic of Princess Margaret Hospital. Radial images of the ciliary body at the 3-, 6-, 9-, and 12-o'clock positions were obtained in both eyes. UBM characteristics included ciliary body thickness and reflectivity. The eye with ocular/oculodermal melanocytosis was compared with the contralateral unaffected eye as a control. Statistical significance was analyzed with Student t test. RESULTS: Twelve patients were included. All patients showed unilateral diffuse pigmentation involving episclera and anterior chamber angle. The iris showed diffuse pigmentation in 10 cases and sectorial in 2. Mean ciliary body thickness of the affected eyes was 0.581 ± 0.058 mm (range 0.489-0.744) compared with 0.475 ± 0.048 mm (range 0.406-0.622) in the contralateral eye, which was found to be a statistically significant difference (P < .001). The affected ciliary body showed hyperreflectivity when compared with the unaffected eye. All affected eyes were graded as medium to high reflectivity compared with the unaffected eyes that showed a medium to medium/low reflectivity. CONCLUSION: Ciliary body involvement in ocular/oculodermal melanocytosis presents as increased thickness and higher ultrasound reflectivity on UBM when compared with the unaffected eye. UBM is helpful in imaging clinically undetectable areas of melanocytosis involving the ciliary body.

Ultrasound biomicroscopy diagnosis of an axenfeld loop.

A 67-year-old man was referred for evaluation of a small mass on the sclera of the right eye. Ultrasound biomicroscopy of the mass suggested the diagnosis of Axenfeld loop.

Clinical and ultrasound biomicroscopy features associated with growth in iris melanocytic lesions.

PURPOSE: To determine the clinical and ultrasound biomicroscopy (UBM) features associated with growth in iris melanocytic lesions. STUDY DESIGN: Retrospective case series analysis. METHODS: We included all iris melanocytic lesions that were monitored between January 2005 and November 2009. At the end of the analysis, 44 eyes of 44 patients were included in the final analysis. The clinical features analyzed were: iris color, largest base diameter, radial location of the lesion epicenter, circumferential location of the lesion epicenter, lesion configuration, lesion pigmentation, intrinsic vascularity within the lesion, presence of associated pigmentation, the impact on the pupil, presence of iris atrophy, and lesion-induced localized cataracts. The UBM features included lesion thickness, presence of corneal touch, presence of surface plaque, internal structure, and internal reflectivity. Regression analysis was performed to define the features associated with growth. RESULTS: Twenty-three percent of the lesions showed documented growth. Mean follow-up was 21.4 months (range: 10-48). Clinical features associated with growth were a large basal diameter at baseline (P = .004) and inferior location (P = .004). UBM features associated with growth were: a greater baseline thickness (P = .01), presence of corneal touch (P = .007), an irregular internal structure (P = .0001), and the presence of dots and linear streaks (P < .0001). Clinical features that were not associated with growth were the radial location of the lesion in the iris (P > .999), lesion configuration (P > .999), lesion pigmentation (P > .999), the presence of pigment dispersion (P = .70), iris freckles (P = .15), corectopia (P > .999), ectropion (P > .999), and intrinsic vascularity (P = .70). UBM features not associated with growth were the presence of a surface plaque (P = .07) and the internal reflectivity (P = .77). CONCLUSION: Substantial growth in iris melanocytic lesions is associated with original larger basal diameter and inferior lesion location. On UBM growth is associated with greater original thickness, presence of corneal touch, and an irregular internal structure. Presence of these features could modify the frequency of observation of those lesions.

Intraocular extension of conjunctival invasive squamous cell carcinoma after pterygium surgery and cataract extraction.

OBJECTIVES: Conjunctival squamous dysplasia can often be confused with pterygium and pinguecula. Incomplete excision of dysplastic tissue can lead to recurrence and rarely intraocular invasion. This study describes two cases in which invasive squamous cell carcinoma (SCC) of the conjunctiva was originally partially resected as pterygium and eventually required enucleation for intraocular invasion. METHODS: In this clinicopathologic small case series, two cases of intraocular SCC managed at a single tertiary ocular oncology institution are described. Clinical features, pathologic characteristics, and relevant imaging are described. RESULTS: In both cases, incomplete excision of conjunctival SCC was followed by rapid regrowth of the conjunctival lesion and signs of intraocular inflammation. An intraocular mass within the substance of the ciliary body was identified using ultrasound biomicroscopy in both the cases. Enucleation was performed. Pathologic features were typical to SCC. CONCLUSIONS: Intraocular spread on conjunctival SCC occurs only rarely but tends to follow recurrence of the conjunctival lesion after attempted excision. Modes of invasion may include direct invasion through sclera, along the tract of the anterior ciliary vessels, or inoculation through intraocular surgery incision.

Factors predictive of radiation retinopathy post (125)Iodine brachytherapy for uveal melanoma.

OBJECTIVE: To report the host, tumour, and radiation-related predictive factors for developing radiation retinopathy post (125)Iodine brachytherapy for uveal melanoma. DESIGN: A retrospective clinical case series. PARTICIPANTS: Three hundred consecutive patients with uveal melanoma treated with (125)Iodine brachytherapy. METHODS: Electronic chart review of demographic, clinical, treatment, and follow-up data. Proliferative and (or) nonproliferative radiation retinopathy patients were included. Cumulative incidence rates were calculated using Kaplan-Meier estimates. Univariate and multivariate statistical regression analyses were performed to identify factors predictive of radiation retinopathy. RESULTS: The mean follow-up period was 48 months. Radiation retinopathy occurred in 107 patients (36%). Actuarial incidence of radiation retinopathy was 30% (CI 24%-36%) at 24 months post-treatment. In the multivariate model, the predictive factors were a younger age (hazard ratio [HR] 0.98, p < 0.03), diabetes (HR 2.17, p < 0.007), and hypertension (HR 2.17, p < 0.004). Tumour-related factors were proximity to optic disc (HR 0.95, p < 0.02) and proximity to foveola (HR 0.96, p < 0.02). Pretreatment tumour dimensions, other tumour characteristics, and total radiation dose did not demonstrate statistically significant risks for developing radiation retinopathy. CONCLUSIONS: Radiation retinopathy affects 30% of patients 2 years following (125)Iodine brachytherapy for uveal melanoma. The predictive factors for radiation retinopathy are a younger age, comorbidity with diabetes or hypertension, and proximity of the tumour margin to the optic disc or foveola. Identifying the predictive factors for developing radiation retinopathy can modify follow-up for patients at risk, which may permit earlier management of the developing radiation-induced ischemic retinal changes.

Ultrasound biomicroscopic imaging of iris melanoma: a clinicopathologic study.

PURPOSE: To demonstrate the correlation of ultrasound biomicroscopy (UBM) features of iris melanoma with histopathology. DESIGN: Retrospective analysis of medical records. METHODS: The medical records of patients that underwent surgery for iris melanoma at the Princess Margaret Hospital, University of Toronto, from June 1990 to October 1998 were reviewed. The clinical features, as well as the UBM findings prior to surgical intervention, were evaluated. The anatomic features noted on UBM were correlated with histopathologic features seen in the surgical specimens. RESULTS: Fourteen cases met the inclusion criteria and were included in the final analysis. The ultrasound acoustic characteristics showed a broad spectrum of findings among iris melanomas. Tumor acoustic parameters correlated well with histologic features, including tumor vascularity, surface plaque, extrascleral extension, ciliary body involvement, and integrity of iris pigment epithelium. CONCLUSIONS: UBM is a useful imaging technique for the in vivo assessment of primary iris melanoma and can provide detailed imaging of the tumor's interface with the angle structures. The preoperative assessment of these tumors by UBM may aid the surgeon in choosing the most appropriate technique to ensure total removal.

Long-term follow-up of 42 patients with small ciliary body tumors with ultrasound biomicroscopy.

PURPOSE: To establish the growth behavior of small ciliary body tumors in a relatively large cohort of patients over an extended period. DESIGN: Retrospective, noncomparative case series. METHODS: Ciliary body tumors less than 4 mm in size within the penetration power of ultrasound biomicroscopy (UBM) were included. Tumor height was assessed by ultrasound biomicroscopy. Tumor growth was defined as an increase in height of at least 20% from baseline, as measured on 2 consecutive UBM readings. The data were collected longitudinally, and a statistical analysis was performed. RESULTS: Forty-two patients were included in the study with a median follow-up of 9.0 years (range, 1.0 to 17.2 years). The median age was 59 years (range, 17 to 82 years). Median initial tumor height was 2.05 mm (range, 1.11 to 3.80 mm). The overall average rate of growth was 0.0014 mm per year (P = .68). The 5- and 10-year accumulative tumor growth rates were 12% and 29%, respectively. In the first 3 years after diagnosis, the growth rate of ciliary body lesions with an initial tumor thickness less than or equal to 2 mm was 0.054 mm per year (P = 0.0001); thereafter, tumor size appeared to stabilize. Tumors with an initial thickness greater than 2 mm showed a small but significant rate of regression of 0.0125 mm per year (P = 0.04). CONCLUSIONS: Most small tumors of the ciliary body show little growth over an extended period and can be managed conservatively without invasive diagnostic interventions. However, long-term follow-up is required. Indications for treatment include growth in height or lateral extension, extrascleral extension or the need for cataract surgery.

Ultrasound biomicroscopic analysis of iris-sutured foldable posterior chamber intraocular lenses.

PURPOSE: To report ultrasound biomicroscopic (UBM) findings of iris-sutured foldable posterior chamber intraocular lenses (PCIOLs). DESIGN: Prospective, noninterventional consecutive case series. METHODS: Fifteen eyes with foldable acrylic IOL implantation using peripheral iris suture fixation in the absence of capsular support were included. UBM was used to determinate the haptic position in relation to the ciliary sulcus and ciliary body in these eyes. Additionally, anterior chamber depth, lens tilt, site of suture fixation, focal iris or angle abnormalities, and relationship of iris to lens were determined. Main outcome measures were haptic position, anterior chamber depth, and iris anatomic changes. RESULTS: Of the 30 haptics imaged, 16 (53.3%) were positioned in the ciliary sulcus. Nine (30%) haptics were found over the ciliary processes, and 5 (16.7%) were over pars plana. No patients were found to have peripheral anterior synechiae present at the haptic position. The mean (+/- standard deviation) depth of the anterior chamber was 3.84 +/- 0.36 mm. The iris profile was altered in all patients at the iris-haptic suture fixation site. No angle abnormalities or tilted lenses were found. CONCLUSIONS: Iris-sutured PCIOL haptics were found to be in the ciliary sulcus or over the ciliary body with no significant tilt on UBM analysis. The procedure respects the angle anatomy, and no evidence of angle closure was found. The anterior chamber was deeper than has been reported previously for scleral sutured PCIOLs and was similar to that of pseudophakic eyes. This may have implications for surgical technique, IOL power calculations, and postoperative complications.

Management of iris melanomas with 125Iodine plaque radiotherapy.

PURPOSE: To report the experience of the Princess Margaret Hospital/University Health Network with the treatment of iris melanoma (IM) with (125)Iodine plaque brachytherapy. DESIGN: Retrospective noncomparative case series. METHODS: All cases of IM submitted to (125)Iodine plaque radiotherapy were included. Patients' demographic, clinical, management, and follow-up data were reviewed. Outcome measures included rates of tumor control, eye preservation, systemic metastases, and brachytherapy-related complications. RESULTS: Fourteen IMs were included in the study. All patients had blue/green irises. Mean largest basal dimension and thickness were 7.1 +/- 2.1 mm (range, 4.0 to 11.5 mm) and 2.2 +/- 0.8 mm (range, 1.0 to 3.5 mm), respectively. Ten patients (71%) had seeding and 2 (14%) had glaucoma at presentation. Median follow-up was 26.6 +/- 19.5 months (range, 6 to 72 months). Tumor control was achieved in 100% of the cases and no eye was enucleated because of radiation-induced complications. At last visit, all patients were alive and free of metastasis. Final visual acuity was the same as or better than before treatment in 9 patients (75%). Cataract was the most common complication (8; 75%), followed by persistent glaucoma (2; 17%) and anterior uveitis (1; 8%). No other significant complication was seen during the follow-up period. CONCLUSIONS: Plaque radiotherapy is a safe and effective conservative treatment option for IM, although cataract is a common, yet treatable, complication. This treatment scheme circumvents an intraocular procedure and may avoid the dissemination of malignant cells, and provides a margin of safety in the treatment of clinically undetectable disease.

Management of ciliary body melanoma with iodine-125 plaque brachytherapy.

OBJECTIVE: Because ciliary body melanoma often defies early diagnosis, and its treatment is controversial, we report our experience with the clinical presentation and management of ciliary body melanoma, treated with iodine-125 plaque brachytherapy. STUDY DESIGN: A retrospective noncomparative case series. PARTICIPANTS: Forty-two patients with ciliary body melanoma treated with iodine-125 brachytherapy. METHODS: Electronic chart review of ciliary body melanoma patients treated at Princess Margaret Hospital, University of Toronto, Toronto, Ont. Patients' demographics and clinical, management, and follow-up data, including brachytherapy-related complications, were reviewed. Outcome measures included rates of tumour control, eye preservation, systemic metastases, and brachytherapy-related complications. RESULTS: Median age at diagnosis was 58 years and median follow-up was 43 months. Ciliary body melanoma was asymptomatic in 55% of patients, whereas floaters or flashes were the main symptom in 33%. Median tumour thickness before brachytherapy was 5.2 mm and after brachytherapy was 3.3 mm. Radiation-induced cataract was the main complication in 55% of patients, radiation retinopathy in 24%, and neovascular glaucoma in 15%, which was controlled by topical treatment in half of the cases. Two patients developed metastases and 3 required secondary enucleation. CONCLUSIONS: Medium-sized ciliary body melanoma produced no or minimal symptoms on presentation in 88% of the patients. Iodine-125 plaque brachytherapy offered 98% tumour control at 43 months' follow-up. Radiation-related complications are generally manageable in most patients, which permits retention of the treated eye.