Asymmetric preservation of choroidal pigmentation simulating choroidal nevus in two siblings with Waardenburg syndrome type 2A.

INTRODUCTION: In addition to sensorineural hearing loss, Waardenburg Syndrome (WS) may present with variable pigmentation of skin and choroid, which may simulate other life-threating conditions (e.g. melanoma). CASE REPORT: Two siblings ostensibly presented with unilateral choroidal pigmentary abnormalities concerning for choroidal tumour. Serial ophthalmic examination documented no lesion growth (base or height) whilst the apparent syndromic features (i.e. iris hypochromia, profound sensorineural hearing loss, SNHL), family history (autosomal dominant inheritance) and positive genetic testing (pathogenic MITF variant) led to a revised diagnosis of Waardenburg Syndrome type 2A. CONCLUSION: Sectoral preservation of choroidal pigmentation in WS is rarely associated with choroidal malignancy. Awareness of syndromic features (e.g. SNHL) and access to genetic testing may facilitate early accurate diagnosis (i.e. allay concern for malignancy), enable treatment of modifiable features (e.g. SNHL) and identify other affected relatives.

Trends in Radiation Practices for Female Ocular Oncologists in North America: A Collaborative Study of the International Society of Ocular Oncology.

BACKGROUND: The aim of this study was to determine the known radiation exposure, attitudes, and consequent risk modifications among female ocular oncologists in North America who routinely administer radioactive plaque brachytherapy treatment and are members of the International Society of Ocular Oncology. METHODS: Nineteen female ocular oncologists completed an anonymous 17-question radiation exposure survey. RESULTS: Eleven of the participants chose to routinely wear lead protection during surgery; 8 did not. Fifteen of 19 participants reported using an unloaded "nonactive" template to prepare for plaque implantation. During pregnancy, 11 of 13 participants continued to perform plaque brachytherapy. Eight of these 11 undertook measures to decrease radiation exposure self-reported as lead wear and other. The average reported anxiety regarding fertility was 2.1 (SD, 2.2) on a scale from 1 to 10. CONCLUSION: This study corroborates prior literature that surgeons' exposure to radiation during plaque brachytherapy is minimal. Nonetheless, there remains some anxiety regarding exposure risk to women, due to potential effects on fertility and fetal health. We found variability in exposure monitoring, required training, and precautions during pregnancy amongst this group of surgeons. Improved education and clearer pregnancy guidelines may equip female ocular oncologists with optimal knowledge regarding risk of radiation exposure.

Intraocular lens power calculations after myopic laser refractive surgery: a comparison of methods in 173 eyes.

PURPOSE: To evaluate and compare published methods of intraocular lens (IOL) power calculation after myopic laser refractive surgery in a large, multi-surgeon study. DESIGN: Retrospective case series. PARTICIPANTS: A total of 173 eyes of 117 patients who had uneventful LASIK (89) or photorefractive keratectomy (84) for myopia and subsequent cataract surgery. METHODS: Data were collected from primary sources in patient charts. The Clinical History Method (vertex corrected to the corneal plane), the Aramberri Double-K, the Latkany Flat-K, the Feiz and Mannis, the R-Factor, the Corneal Bypass, the Masket (2006), the Haigis-L, and the Shammas.cd postrefractive adjustment methods were evaluated in conjunction with third- and fourth-generation optical vergence formulas, as appropriate. Intraocular lens power required for emmetropia was back-calculated using stable post-cataract surgery manifest refraction and implanted IOL power, and then formula accuracy was compared. MAIN OUTCOME MEASURES: Prediction error arithmetic mean ± standard deviation (SD), range (minimum and maximum), and percent within 0 to -1.0 diopters (D), ±0.5 D, ±1.0 D, and ±2.0 D relative to target refraction. RESULTS: The top 5 corneal power adjustment techniques and formula combinations in terms of mean prediction errors, standard deviations, and minimizing hyperopic "refractive surprises" were the Masket with the Hoffer Q formula, the Shammas.cd with the Shammas-PL formula, the Haigis-L, the Clinical History Method with the Hoffer Q, and the Latkany Flat-K with the SRK/T with mean arithmetic prediction errors and standard deviations of -0.18±0.87 D, -0.10±1.02 D, -0.26±1.13 D, -0.27±1.04 D, and -0.37±0.91 D, respectively. CONCLUSIONS: By using these methods, 70% to 85% of eyes could achieve visual outcomes within 1.0 D of target refraction. The Shammas and the Haigis-L methods have the advantage of not requiring potentially inaccurate historical information.

Intravitreal bevacizumab for iris neovascularization following proton beam irradiation for choroidal melanoma.

OBJECTIVE: To evaluate the safety and efficacy of intravitreal injections of bevacizumab as an eye-sparing treatment for iris neovascularization (NVI) following proton beam irradiation for choroidal melanoma. DESIGN: Retrospective interventional case series. PARTICIPANTS: Four patients who received intravitreal bevacizumab for NVI following proton beam irradiation for choroidal melanoma were identified in the Department of Ophthalmology archives at the University of British Columbia. METHODS: Clinical details were reviewed. Long-term follow-up of more than 2 years was detailed for each case. RESULTS: All 4 patients responded to a single injection of bevacizumab with regression of NVI. Neovascular glaucoma (NVG) was evident in 3 cases, 2 of which had stable intraocular pressure following treatment. NVI recurred following a single injection in all patients after an interval ranging from 1 month to 12 months. A longer period of regression was seen in patients with fewer systemic neovascular risk factors and earlier treatment. CONCLUSIONS: Regression of NVI following proton beam irradiation for choroidal melanoma was seen in all treated patients. Repeated treatments may be required to maintain regression of new vessels. This treatment modality may be a useful eye-sparing adjunct in the prevention and treatment of NVG following proton beam irradiation.

Histopathologic features of multiple myeloma involving the optic nerves.

We report a case of optic nerve involvement by multiple myeloma in which progressive visual loss heralded leukemic transformation and intracranial involvement. Imaging showed enhancing nodules in the intracranial segments of both optic nerves posterior to the optic canals and in the anterior optic tract, optic chiasm, and basal leptomeninges. Postmortem histopathologic examination disclosed malignant plasma cells in the subarachnoid spaces around the optic nerves and in the optic nerves. Infarctions were present in both optic nerves near their junction with the globes. Microscopic examination also showed malignant plasma cell infiltration of the leptomeninges of the cerebrum, brain stem, optic chiasm, pituitary gland, cranial bone marrow, and subarachnoid blood vessels. This is the first reported histopathologic examination in conjunction with MRI of multiple myeloma involving the anterior visual pathway. The mechanism of optic neuropathy in this case is probably related to infiltration of the optic nerve meninges by malignant plasma cells and impaired vascular supply caused by aggregated intraluminal plasma cells and monoclonal hypergammaglobulinemia.