Peripapillary Neuroendocrine Carcinoma Metastasis: A Novel Approach to Treatment.

Peripapillary and circumpapillary retinal intraocular metastases are rare and present a treatment challenge for ophthalmologists because of the high risk of iatrogenic injury to the optic nerve. There are no clear guidelines on the management of these lesions, and many clinicians will initially observe for improvement of the metastases with systemic chemotherapy before considering local therapy with external beam radiation. Radiation to the optic disc carries a significant risk of injuring the optic nerve, leading to worsening of vision. Alternative treatment approaches are needed. We present a patient with large-cell neuroendocrine carcinoma with metastasis to the peripapillary retina who was treated with intravitreal topotecan and with intravitreal aflibercept. Serial fundus photos, ultrasound, and optical coherence tomography demonstrated a reduction in size of the lesion and a decrease in subretinal fluid with intravitreal topotecan and aflibercept. In addition, visual acuity was stabilized during treatment. Intravitreal chemotherapy for intraocular metastases in vision-sensitive areas such as the peripapillary retina may be a viable alternative for patients who seek to preserve their vision and maintain their quality of life.

Elevated Choroidal Thickness and Central Serous Chorioretinopathy in the Fellow Eyes of Patients with Circumscribed Choroidal Hemangioma.

OBJECTIVE: To determine whether increased choroidal thickness (CT) is associated with circumscribed choroidal hemangioma (CCH) and whether patients with CCH are predisposed to central serous chorioretinopathy (CSCR). METHODS: We performed a retrospective consecutive observational study of 15 fellow eyes of 15 patients who had CCH. A- and B-scan ultrasonography, fluorescein angiography (FA), and optical coherence tomography (OCT) findings were reviewed to confirm the diagnosis of CCH. Fellow eye CT was measured using enhanced depth imaging OCT. FA, indocyanine green angiography, and OCT images of fellow eyes were reviewed for signs of CSCR. RESULTS: Mean subfoveal CT was significantly greater in the fellow eyes of patients with CCH than in age-matched normal eyes (p = 0.004). Three of 15 (20.0%) fellow eyes of CCH patients had retinal pigment epithelium disruptions and/or pigment epithelial detachments indicative of previous asymptomatic CSCR. CONCLUSION: In this pilot study, elevated CT was associated with a risk of developing CCH as well as CSCR in patients of varying ages. Patients diagnosed with CCH should be screened for CSCR in the fellow eye. Further exploration of this association may reveal useful clues about the biology of abnormally elevated choroidal hyperpermeability and its various clinical manifestations.

Association between traditional clinical high-risk features and gene expression profile classification in uveal melanoma.

PURPOSE: To evaluate the association between traditional clinical high-risk features of uveal melanoma patients and gene expression profile (GEP). METHODS: This was a retrospective, single-center, case series of patients with uveal melanoma. Eighty-three patients met inclusion criteria for the study. Patients were examined for the following clinical risk factors: drusen/retinal pigment epithelium (RPE) changes, vascularity on B-scan, internal reflectivity on A-scan, subretinal fluid (SRF), orange pigment, apical tumor height/thickness, and largest basal dimensions (LBD). A novel point system was created to grade the high-risk clinical features of each tumor. Further analyses were performed to assess the degree of association between GEP and each individual risk factor, total clinical risk score, vascularity, internal reflectivity, American Joint Committee on Cancer (AJCC) tumor stage classification, apical tumor height/thickness, and LBD. RESULTS: Of the 83 total patients, 41 were classified as GEP class 1A, 17 as class 1B, and 25 as class 2. The presence of orange pigment, SRF, low internal reflectivity and vascularity on ultrasound, and apical tumor height/thickness ≥ 2 mm were not statistically significantly associated with GEP class. Lack of drusen/RPE changes demonstrated a trend toward statistical association with GEP class 2 compared to class 1A/1B. LBD and advancing AJCC stage was statistically associated with higher GEP class. CONCLUSIONS: In this cohort, AJCC stage classification and LBD were the only clinical features statistically associated with GEP class. Clinicians should use caution when inferring the growth potential of melanocytic lesions solely from traditional funduscopic and ultrasonographic risk factors without GEP data.

Purtscher-like retinopathy associated with pemphigus vulgaris.

PURPOSE: To report a case of Purtscher-like retinopathy associated with pemphigus vulgaris. METHOD: Case report of a 25-year-old Hispanic male who was referred for sudden and severe reduction of vision from his right eye shortly after an exacerbation of pemphigus vulgaris. RESULTS: Fundus examination of the right eye revealed severe macular edema, nerve fiber layer infarcts, flame-shaped hemorrhages, and venous dilation. Fluorescein angiography and optical coherence tomography findings of flecken, ischemia with subsequent nerve fiber layer atrophy, and capillary leakage were consistent with Purtscher-like retinopathy. The patient was treated with 5 consecutive monthly intravitreal injections of 0.5 mg of ranibizumab. Visual acuity improved with resolution of macular edema, and the patient was started on systemic mycophenolate mofetil. Continued ocular stability was achieved with 11 subsequent intravitreal injections over the following 2 years. CONCLUSION: Purtscher-like retinopathy may have been associated with pemphigus vulgaris through a complement activation process that is previously undescribed. Retinal findings were successfully mitigated with the use of intravitreal anti-vascular endothelial growth factor therapy.

Lucentis using Visudyne study: determining the threshold-dose fluence of verteporfin photodynamic therapy combined with intravitreal ranibizumab for exudative macular degeneration.

PURPOSE: Combination verteporfin photodynamic therapy (vPDT) and antivascular endothelial growth factor (anti-VEGF) therapy may decrease the need for injections while maintaining visual acuity in exudative age-related macular degeneration. This pilot study was designed to determine the threshold fluence dose of vPDT (the dose required to demonstrate an effect on choroidal perfusion) combined with ranibizumab. METHODS: Seven patients were randomized to sham vPDT (two patients), 20% fluence vPDT (two patients), or 40% fluence vPDT (three patients) in combination with three-monthly intravitreal 0.5 mg ranibizumab injections. Intravitreal ranibizumab was reinjected if disease activity was seen on fluorescein angiography, optical coherence tomography, or clinical examination. Indocyanine green-determined choroidal hypoperfusion was graded in a masked fashion. RESULTS: Patients with 20% vPDT had mild hypoperfusion defects at seven days that resolved by week 4 (threshold dose); patients with 40% fluence vPDT had marked hypoperfusion at seven days that persisted as long as 12 months. Recruitment was stopped after limited efficacy was observed. One patient with 20% fluence vPDT lost 19 letters at one year; no other patient lost or gained >10 letters. Central retinal thickness decreased in six of seven patients, but ranibizumab injections did not decrease. CONCLUSION: This pilot study shows that the threshold fluence dose of vPDT (when combined with ranibizumab) is approximately 20% standard fluence, and that mild and transient choroidal hypoperfusion can occur. Forty percent fluence vPDT causes a more prolonged and striking hypoperfusion. Despite hypoperfusion, no decrease in visual acuity or injections required was noted, suggesting that even higher fluence levels of vPDT may be necessary to decrease the number of anti-VEGF injections.

Selected clinical comparisons of spectral domain and time domain optical coherence tomography.

The authors report four cases where spectral domain optical coherence tomography (SD-OCT) imaged pathology not captured by time domain optical coherence tomography (TD-OCT). These cases include one of angioid streaks, two of juxtafoveal telangiectasia, and one of age-related macular degeneration. In each case, the improved images provided by SD-OCT changed either the management of the patient or the counseling of their disease process.