Sohel Somani

Stereotactic Radiotherapy for Treatment of Juxtapapillary Choroidal Melanoma: 3-Year Follow-up.

Aims: To report the treatment outcomes and complication rates of stereotactic radiotherapy in the management of patients with juxtapapillary choroidal melanoma. Methods: A retrospective review of 64 consecutive patients with juxtapapillary choroidal melanoma, located within 2 mm of the optic disc, treated with stereotactic radiotherapy at Princess Margaret Hospital between October 1998 and January 2006. Results: The median age was 63 years. The median tumour height was 4.2 mm, and median largest basal diameter was 9.8 mm. The prescribed radiation dose was 70 Gy in five fractions over 10 days, and the median follow-up was 37 months. Post-treatment, the actuarial rates of local tumour control, metastases and survival at 37 months were 94%, 15% and 90%, respectively. Actuarial rates of radiation-induced complications at 37 months were neovascular glaucoma 42%, cataract 53%, retinopathy 81% and optic neuropathy 64%. Secondary enucleation was necessary for 10 patients (16%), in four patients for tumour recurrence and in six for painful neovascular glaucoma. Conclusions: Stereotactic radiotherapy offers a non-invasive alternative to enucleation and brachytherapy in the management of juxtapapillary choroidal melanoma with a high tumour control rate, however, at the expense of a significant rate of long-term ocular complications.

Intracameral bevacizumab in the treatment of neovascular glaucoma and exudative retinal detachment after brachytherapy in choroidal melanoma.

Choroidal melanoma is the most common primary intraocular tumor in adults. Plaque brachytherapy is a common treatment option for medium-sized tumors. Radiation retinopathy and its sequellae, including neovascularization of the iris (NVI), neovascular glaucoma (NVG), and exudative retinal detachment (ERD), are among the known side effects of radiation treatment. NVI can develop as soon as 9 months after radiation (mean time of 27 months) and secondary NVG develops in up to 23% of eyes at 5 years. ERD occurs following plaque radiotherapy in up to 16% of patients. Treatment options for managing NVG secondary to brachytherapy include medical therapy, panretinal photocoagulation, cyclodestructive procedures, anti–vascular endothelial growth factor (VEGF) therapy, and, in some cases, enucleation. Typically, there are no specific treatments directed to ERDs in treated choroidal melanoma patients. Multiple reports have shown that intravitreal bevacizumab (Avastin, Genentech Inc, San Francisco, Calif.) an antiVEGF agent, reduces neovascular activity and vascular permeability in ocular tissues. Bevacizumab can be administered intravitreally or intracamerally to reduce NVI secondary to diabetes and other retinal vascular diseases. We report the use of intracameral bevacizumab in a patient with NVG and ERD following brachytherapy for a medium-sized choroidal melanoma. A 45-year-old woman was treated with iodine-125 (I-125) brachytherapy for a medium-sized choroidal melanoma (thickness of 6.8 mm, 10 mm at its largest diameter). There was no retinal detachment (RD) at the time of diagnosis. Fourteen months post treatment, a total ERD was noted despite local tumor control (4.26 mm thickness). Three months later, she developed a painful left eye and decreased visual acuity to no light perception (NLP).The intraocular pressure (IOP) was 50 mm Hg. Corneal edema was present, with 360° of NVI; the angle was open and neovascularization of the angle was noted. The tumor height was 4.35 mm by ultrasound. Maximum medical therapy was initiated to treat the elevated IOP. Panretinal photocoagulation could not be performed because of the extent of the RD. Bevacizumab (1.25 mg) was injected intracamerally since the intravitreal approach was deemed unsafe in the presence of an RD. One week post injection, the IOP was 36 mm Hg. On examination, there was regression of the iris and angle neovascularization, which was evident on iris angiography (Fig. 1). One month post injection, visual acuity remained NLP, IOP was 20 mm Hg, and some iris vessels were visible. Intracameral bevacizumab injections were repeated

Visual field expansion in patients with retinitis pigmentosa.

BACKGROUND To determine the effectiveness of using spectacle-mounted prisms for field expansion in patients with retinitis pigmentosa (RP). METHODS Vision-related activities of daily living (V-ADL) questionnaire scores and functional visual field score (FFS) measurements were conducted before and after a one-month trial of spectacle-mounted prisms in those patients with RP who had residual central visual fields of less than 10 degrees. RESULTS 16 patients were recruited who met study inclusion criteria. Mean V-ADL and FFS at baseline were 67.6 (73%) and 22.9 (46%), respectively. After a 1-month trial using spectacle-mounted prisms, V-ADL and FFS demonstrated significant improvement to 73.4 (80%, p < 0.05) and 27.0 (54%, p < 0.001), respectively. INTERPRETATION Spectacle-mounted prisms effectively create visual field expansion and noticeable spatial orientation benefits in patients with RP. This may provide an adjunctive tool in low vision rehabilitation and should be considered in all cases with RP with less than 10 degrees of visual field.

Stereotactic radiotherapy in the treatment of juxtapapillary choroidal melanoma: 2-year follow-up

OBJECTIVE To evaluate the efficacy and complications of stereotactic radiotherapy in the management of patients with juxtapapillary choroidal melanoma. DESIGN Retrospective review. PARTICIPANTS 64 patients with juxtapapillary choroidal melanoma. METHODS Consecutive patients with juxtapapillary choroidal melanomas located within 2 mm of the optic nerve, treated with stereotactic radiotherapy at Princess Margaret Hospital from October 1998 to January 2006, were reviewed for treatment effect and complication rates. RESULTS Median age was 63 years. Median tumor height was 4.2 mm, and median maximum tumor diameter was 9.8 mm. The prescribed radiation dose was 70 Gy in 5 fractions over 10 days, and the median follow-up was 26 months. After treatment, there was local tumor recurrence in 3 patients, and in 8 patients there was systemic progression. Actuarial rates of local tumor control, metastases, and survival at 26 months were 94%, 12%, and 94%, respectively. Rates of radiation-induced neovascular glaucoma, cataract, retinopathy, and optic neuropathy at 26 months were 28%, 45%, 80%, and 52%, respectively. Enucleation was necessary for 7 patients. CONCLUSIONS Stereotactic radiotherapy offers a noninvasive alternative with acceptable ocular toxicity rates to enucleation and brachytherapy in the management of juxtapapillary choroidal melanoma.

The Ocular Manifestations of Jacobsen Syndrome: A Report of Four Cases and a Review of the Literature

Purpose: We report ophthalmic manifestations in four Jacobsen syndrome cases, review the literature, and suggest phenotype-genotype correlations. Methods: Chart review of Ocular Genetics Program patients at The Hospital for Sick Children, Toronto, Canada. Results: Four del11qter cases are presented. Hypertelorism/telecanthus, abnormally slanted palpebral fissures, abnormal retinal findings, nasolacrimal duct obstruction, anomalous extraocular muscles, amblyopia, and microcornea were found. Conclusions: We report typical findings and novel ocular presentations. Visual prognosis is generally good. Retinal dysplasia and coloboma seem associated with del11q23. ABCG4, NCAM, and Mfrp are candidate genes in this region that theoretically may be disrupted.

Long-term outcomes in half-dose verteporfin photodynamic therapy for chronic central serous retinopathy

Objective To evaluate the short- and long-term outcomes of half-dose verteporfin with photodynamic therapy (PDT) in the treatment of chronic central serous retinopathy (CSR). Design Retrospective case series. Participants 45 eyes in 39 patients with chronic CSR were included. Diagnosis of chronic CSR was confirmed by fluorescein angiography and persistence of subretinal fluid by optical coherence tomography for a minimum of 3 months duration. Methods Each patient underwent treatment with half-dose verteporfin with full-fluence PDT; initial follow-up was defined as a 6–8 week visit following the treatment, and final follow-up ranged from 5 to 70 months. Results The average follow-up period for treatment was 19.3 months. Best-corrected visual acuity increased from logMAR means of 0.52 to 0.42 (p<0.05). Central retinal thickness and choroidal thickness also significantly decreased at last follow-up (p<0.05). Eight of 45 eyes (18%) demonstrated a recurrence of CSR following treatment within the follow-up period. At the final follow-up, 41 out of the 45 eyes (91%) had complete resolution of subretinal fluid accumulation. Conclusion Half-dose PDT is an effective treatment option for chronic CSR in a Canadian population, and it is both safe and durable. The positive treatment effect is realized rapidly, with the initial 6-week result highly correlated with the final follow-up result.

Outcomes of hypofractionated palliative radiotherapy for choroidal metastases.

194 Background: External beam radiotherapy (RT) is an effective palliative treatment for choroidal metastases aimed at preserving vision and obtaining local tumor control. Delivery of 30-40 Gy in 2 Gy daily fractions is a standard approach in many centers. At our center, a shorter, more convenient schedule of 20 Gy in 5 fractions has been used in this palliative setting. This study reports the efficacy and toxicity of this hypofractionated RT approach. METHODS We conducted a retrospective review of patients treated in the Ocular Oncology clinic at Princess Margaret Cancer Center who received RT (20 Gy in 5 fractions) for choroidal metastases between January 1, 1999 and November 30, 2012. Primary outcome measures were change in visual acuity and tumor response. Secondary outcomes included toxicities of RT, tumor control, and overall survival from the date of choroidal metastases diagnosis. RESULTS A total of 55 patients with 71 involved eyes were included. Decreased vision was the presenting symptom in 43 eyes (61%). Visual acuity improved from a median of 20/70 to 20/40 between baseline assessment and last follow-up, and remained stable or improved in 56 eyes (80%). On ultrasound, tumor regression was observed in 64 eyes (91%) with complete response in 47 eyes (67%). Metastases progressed in 4 eyes (6%) despite RT with 1 eye requiring enucleation. Median survival after diagnosis of choroidal metastases was 13 months with estimated overall survival at 1, 2, and 3 years to be 50% (36-62), 23% (12-35), and 8% (3-18), respectively. Forty-nine patients (89%) did not experience any acute complications. Mild acute toxicities included transitory dryness in 5 patients and episcleritis in 1 patient. Cataracts developed in 4 eyes (6%), retinopathy in 1 eye, optic neuropathy in 7 eyes (10%), pigmentary maculopathy in 5 eyes (7%), and neovascular glaucoma in 1 eye. CONCLUSIONS A short fractionation schedule of 20 Gy in 5 fractions is a well-tolerated treatment that effectively preserves vision and gains local tumor control for many patients with choroidal metastases. This hypofractionated approach would help reduce the burden of a longer treatment course in this palliative patient population.