Management of Uveal Melanoma: Updated Cancer Care Alberta Clinical Practice Guideline.

OBJECTIVE: The purpose of this guideline update is to reassess and update recommendations in the prior guideline from 2016 on the appropriate management of patients with uveal melanoma. METHODS: In 2021, a multidisciplinary working group from the Provincial Cutaneous Tumour Team, Cancer Care Alberta, Alberta Health Services was convened to update the guideline. A comprehensive review of new research evidence in PubMed as well as new clinical practice guidelines from prominent oncology groups informed the update. An enhancement in methodology included adding levels of evidence and strength of recommendations. The updated guideline was circulated to all members of the Provincial Cutaneous Tumour Team for review and endorsement. RESULTS: New and modified recommendations address provider training requirements, diagnostic imaging for the detection of metastases, neo-adjuvant pre-enucleation radiotherapy, intravitreal anti-vascular endothelial growth factor agents for radiation retinopathy, genetic prognostic testing, surveillance following definitive local therapy, and systemic therapy for patients with metastatic uveal melanoma. DISCUSSION: The recommendations represent evidence-based standards of care agreed to by a large multidisciplinary group of healthcare professionals.

Two cases of spontaneous filtering blebs, one idiopathic and one associated with Terrien marginal degeneration.

PURPOSE: To report a case of an idiopathic unilateral filtering bleb in an otherwise well patient and a case of bilateral spontaneous filtering blebs in association with Terrien marginal degeneration. METHODS: In this retrospective observational study of 2 cases, clinical notes, ultrasound biomicroscopy and gonioscopy images were reviewed. RESULTS: Case 1: A 32-year-old woman presented with a 1-year history of a gradually growing cyst in her right eye. No changes in vision were reported. The patient had no previous ocular or systemic history, trauma, or surgical interventions. Slit-lamp examination revealed a multilobulated cyst at the supranasal limbus OD. Diagnosis was an idiopathic spontaneous limbal filtering bleb of unclear etiology because there was no inciting event or obvious cause. Case 2: A 31-year-old man, with 2 past episodes of spontaneous filtering bleb development, affecting the left, then the right, eye, presented with his third event in May 2012. The visual acuity was decreased to 20/400 OD. The right eye was hypotonous and ultrasound biomicroscopy confirmed a shallow anterior chamber, a superior peripheral corneal defect, and an echolucent space suggestive of subconjunctival fluid. Limbal thinning was noted bilaterally, consistent with Terrien marginal degeneration. CONCLUSIONS: This is a rare case of an idiopathic filtering bleb occurring in a healthy patient with no precipitating cause, other ocular abnormalities, or physical malformations, and the only reported case of bilateral sequential filtering blebs associated with an atypical presentation of Terrien marginal degeneration.

Multiple bilateral iridociliary cysts: ultrasound biomicroscopy and clinical characteristics.

BACKGROUND: To describe the utility of ultrasound biomicroscopy in diagnosing multiple bilateral iridociliary cysts and to determine the clinical significance of this condition. METHODS: A retrospective review of 73 patients referred for ultrasound biomicroscopy who were found to have bilateral iridociliary cysts. RESULTS: Seventy-three patients were identified who demonstrated 2 or more cysts in 1 eye and at least 1 in the other with ultrasound biomicroscopy. A solitary iris elevation was the most common reason for referral (84.9%). There were 44 patients for whom sufficient follow-up data were available, with a mean follow-up of 40.4 months. Clinical sequelae included 1 case of sectoral cataract (2.3%) and 4 cases of cyst-related glaucoma (9.1%). INTERPRETATION: Multiple bilateral iridociliary cysts is a common condition with occasional clinical significance. Ultrasound biomicroscopy is a valuable technique in diagnosing this condition. Patients who have significant angle compromise should be followed and treated as required for glaucoma.

Postmortem microscopic evaluation and clinical correlation of a pseudophakic eye with pseudoexfoliation and loss of zonular support.

We describe a pseudophakic patient with pseudoexfoliation who developed late intraocular lens (IOL) instability manifested by pseudophakodonesis, a change in refraction, and loss of glaucoma control. The patient subsequently required glaucoma surgery that was complicated by vitreous loss. Preoperative ultrasound biomicroscopy (UBM) failed to provide useful information regarding the zonular status. The patient died as a result of a malignancy, and the eye was donated for research. Anatomical evaluation confirmed the clinical impression of IOL placement in the bag and zonular laxity. Postmortem UBM helped explain some of the technical difficulties in examining zonules in the pseudophakic state.

Small ciliary body tumors: ultrasound biomicroscopic assessment and follow-up of 42 patients.

PURPOSE: Detecting and following small tumors of the ciliary body is a particular challenge because of their location. Recently high-frequency ultrasound biomicroscopy (UBM) has been used to assist in this task. The aim of this study was to evaluate this subset of small ciliary body tumors through the use of UBM. DESIGN: Retrospective, noncomparative case series. METHODS: Forty-two patients with small ciliary body tumors (less than 4 mm) from our institution were included in the study with a median follow-up of 4.3 years. UBM was used to assess tumor characteristics including height, location, and internal and external features. The data were collected longitudinally, and statistical analysis was performed. RESULTS: Median initial tumor height was 2.05 mm (range 1.1 to 3.8 mm) as measured by UBM. By 5 years after diagnosis, five tumors (12%) exhibited growth. The overall mean growth rate was 0.026 mm per year (P = .00007). The most rapid period of growth was in the first year after diagnosis (growth rate 0.128 mm per year), after which the mean tumor size appeared to stabilize. CONCLUSIONS: UBM is a valuable tool for detecting and following small ciliary body tumors (less than 4 mm), as these lesions may go undetected by other methods. Despite the potential for local extension into the iris or choroid, few of the tumors in this study exhibited growth, suggesting that many of these tumors can be managed conservatively. UBM can be used to assess various internal tumor features; however, arriving at a specific diagnosis without histologic correlation is difficult.

Ultrasound biomicroscopy for the assessment of zonules after ocular trauma.

PURPOSE: To evaluate the role of ultrasound biomicroscopy (UBM) as a method for assessing the zonules after trauma to the anterior segment. These patients might develop traumatic cataract or glaucoma requiring surgery. If occult zonular defects can be identified preoperatively, this might lead to a modified surgical technique and improved outcomes. DESIGN: Retrospective observational case series. PARTICIPANTS: Fifty-nine patients who were referred for UBM after anterior segment trauma. METHODS: UBM examinations were performed using the Humphrey Instruments Ultrasound Biomicroscope equipped with a 50-mHz transducer. A plastic eyecup filled with 2% methylcellulose solution was used in each case. The chart and UBM images of all anterior segment trauma patients examined between 1995 and 2000 were reviewed. The age, gender, and mechanism of injury were recorded for each patient. Follow-up data were obtained from the referring physician in patients who subsequently underwent ocular surgery. MAIN OUTCOME MEASURES: The number of studies judged to be of adequate quality to evaluate zonules was noted, and the number of cases in which zonular defects were found was recorded. The number of cases in which surgery was subsequently performed was noted, and postoperative results were obtained. RESULTS: Fifty-nine charts and 60 UBM studies were reviewed. Forty-nine studies were judged to be adequate for imaging zonules. Most poor-quality studies were in the early years of the study. Further analysis was carried out on the reliable studies. Occult zonular defects were identified in 21 of 49 patients (42.9%). Thirty-one patients went on to have cataract, filtering, and/or vitreoretinal surgery. One surgeon (ACS) performed 25 of the surgical cases, for which a summary is provided. Referring surgeons found the information helpful in surgical planning and anticipating complications in reliable study cases. CONCLUSIONS: UBM is an effective method for identifying occult zonular damage in patients with anterior segment trauma. There is a significant learning curve in the examination technique. The ability to diagnose zonular rupture preoperatively is of significant benefit to the surgeon and might reduce the chance of intraoperative complications.