STELLATE MULTIFORM AMELANOTIC CHOROIDOPATHY: Clinical and Multimodal Imaging Features.

PURPOSE: To describe the clinical and multimodal imaging features of stellate multiform amelanotic choroidopathy (SMACH; also known as serous maculopathy due to aspecific choroidopathy). METHODS: Retrospective observational case series of eyes presenting with SMACH. Multimodal imaging including fundus photography, optical coherence tomography (OCT), OCT angiography (OCTA), and indocyanine green angiography (ICGA) was analyzed. RESULTS: Eighteen eyes from 18 patients (mean age: 28 ± 19 years) were included. The mean follow-up duration was 9 years. Ophthalmoscopy showed a yellowish orange, dendriform choroidal lesion. At presentation, subretinal fluid (SRF) was seen in 10 of 18 cases (56%). Eight patients (44%) showed no evidence of SRF during a mean follow-up of 6 years. Cross-sectional OCT showed hyperreflective fibrous-like changes within the inner choroid with choriocapillaris flow preservation on OCTA. En face OCT showed a hyperreflective choroidal lesion with finger-like projections oriented in a stellate configuration. On ICGA, SMACH showed early and late hypofluorescence. None of the cases showed lesion growth. CONCLUSION: SMACH seems to be a unilateral choroidopathy characterized by distinctive multimodal imaging features. As SRF was absent in some cases, while a dendriform pattern was a consistent finding in all eyes, the authors propose renaming this entity "stellate multiform amelanotic choroidopathy," a name that retains its previous abbreviation "SMACH."

Stellate nonhereditary idiopathic foveomacular retinoschisis.

PURPOSE: To describe a new classification of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR). DESIGN: Retrospective case series and literature review. PARTICIPANTS: A total of 17 patients from 5 institutions. METHODS: Detailed case history, multimodal imaging, and genetic testing were reviewed for patients with macular schisis without a known predisposing condition. Patients with a stellate appearance centered on the fovea with correlating confirmed expansion of the outer plexiform layer (OPL) by optical coherence tomography (OCT) were included. Exclusion criteria included a family history of macular retinoschisis, a known genetic abnormality associated with retinoschisis, myopic traction maculopathy, epiretinal membrane, vitreoretinal traction, optic or scleral pit, or advanced glaucomatous optic nerve changes. MAIN OUTCOME MEASURES: Clinical features, anatomic characteristics, and visual acuity. RESULTS: A total of 22 eyes from 16 female patients and 1 male patient with foveomacular schisis were reviewed from 5 institutions. Initial visual acuity was ≥ 20/50 in all eyes (mean, 20/27), but visual acuity in a single eye decreased from 20/20 to 20/200 after the development of subfoveal fluid. The refractive status was myopic in 16 eyes, plano in 3 eyes, and hyperopic in 2 eyes. Three eyes had a preexisting vitreous separation, and 19 eyes had an attached posterior hyaloid. Follow-up ranged from 6 months to >5 years. CONCLUSIONS: In this largest known series of patients with SNIFR, all patients demonstrated splitting of the OPL in the macula with relatively preserved visual acuity (≥ 20/40) except in a single patient in whom subretinal fluid developed under the fovea.

Time required for navigated macular laser photocoagulation treatment with the Navilas.

BACKGROUND: Navilas laser is a novel technology combining photocoagulation with imaging, including fluorescein angiographic (FA) images which are annotated and aligned to a live fundus view. We determine the time necessary for planning and treatment of macular edema utilizing the Navilas. METHODS: The screen recordings during treatments were retrospectively analyzed for treatment type, number of laser shots, the duration of planning (measured from the time the planning image was selected to time of marking the last planned treatment spot), and total time of laser application. RESULTS: A total of 93 treatments (30 grid, 30 focal and 33 combined treatments) by four physicians from three sites were included. An average of 125 spots were applied to each eye. The total time spent for each focal treatment - including the planning was 7 min 47 s (±3 min and 32 s). CONCLUSIONS: Navilas is a novel device providing a time efficient platform for evaluating FA images and performing threshold macular laser photocoagulation.

The duration of effect of centrifuge concentrated intravitreal triamcinolone acetonide.

PURPOSE: To estimate the duration of activity for intravitreal triamcinolone injected with a new technique using centrifuge concentration (Centrifuge concentrated IntraVitreal Triamcinolone, C-IVT). METHODS: All injections were performed by a single surgeon (M.D.O.) using a 30-gauge needle. A vial of Triesence (triamcinolone; Alcon Laboratories, Fort Worth, TX) was drawn into a 1-mL syringe and the plunger cut off. The contents were spun in a centrifuge, and a second plunger was placed. Records of all patients receiving C-IVT with 0.05 mL or 0.1 mL from January 1, 2009, through December 31, 2009, were retrospectively reviewed. RESULTS: Eighty-four injections from 69 eyes of 57 patients were included. Sixty-nine injections from 54 eyes of 44 patients received 0.05 mL of C-IVT, whereas 15 injections from 15 eyes of 13 patients received 0.1 mL of C-IVT. Triamcinolone acetonide was still visualized in the vitreous on an average of 5.0 ± 2.4 months (median 5 months) after 0.05 mL of C-IVT and 8.3 ± 4.0 months (median 8 months) after 0.1 mL of C-IVT during follow-up visits. The longest duration recorded was 14 months for the 0.05-mL group and 18 months for the 0.l-mL group. CONCLUSION: The C-IVT results in a long duration of effect that seems to be greater than previously published techniques. It may be considered for patients requiring chronic steroid therapy, in which the benefits of long-term intravitreal steroids are believed to outweigh their risk.

Maculopathy in Patients with Monoclonal Gammopathy of Undetermined Significance.

PURPOSE: To describe clinical findings, laboratory values, and treatment response of patients with monoclonal gammopathy of undetermined significance (MGUS) demonstrating neurosensory macular detachment. DESIGN: Retrospective case series. PARTICIPANTS: Seven eyes of 4 patients (3 men and 1 woman; age range, 60-81 years) with neurosensory macular detachment, treatment-resistant submacular fluid, and vitelliform material. METHODS: We retrospectively reviewed the medical and ocular histories, ocular examination findings, retinal imaging, ocular disease course, and laboratory findings in 4 patients with submacular fluid associated with MGUS. MAIN OUTCOME MEASURES: Description of the macular findings and treatment courses of 4 patients diagnosed with MGUS maculopathy. RESULTS: Seven eyes of 4 patients demonstrated neurosensory macular detachment with treatment-resistant submacular fluid and vitelliform material. No eyes demonstrated signs of significant hyperviscosity retinopathy. Fluorescein angiography showed no definite leakage in any involved eye. Laboratory evaluation revealed immunoglobulin G MGUS in all 4 patients. All 4 patients were resistant to treatments aimed at resolving the subretinal fluid, including some combination of anti-vascular endothelial growth factor injections, photodynamic therapy, topical dorzolamide, oral dosing of eplerenone or acetazolamide, or some combination thereof. In 3 patients, MGUS underwent malignant transformation 24 to 144 months after diagnosis, in 1 patient to lymphoplasmacytic lymphoma and in 2 patients to multiple myeloma. The fourth patient showed no evidence of malignancy 8 years after diagnosis. CONCLUSIONS: Submacular fluid without fluorescein leakage and unresponsive to conventional treatment may suggest an underlying immunoproliferative disorder that we have termed monoclonal gammopathy of macular significance. Given the propensity for monoclonal gammopathy of macular significance to transform into malignant disease in our series, serum protein analysis should be considered in patients with neurosensory macular detachment not attributable to known causes.

Corticosteroids for blastomycosis-induced ARDS: a report of two patients and review of the literature.

ARDS secondary to blastomycosis is associated with a high mortality rate despite appropriate antifungal therapy. Corticosteroids are of proven benefit in the treatment of severe Pneumocystis jiroveci pneumonia and are recommended for the treatment of severe pulmonary infections with Histoplasma capsulatum. However, their role in the treatment of severe pulmonary infections with Blastomyces dermatitidis has not been established. We report the cases of two previously healthy men who presented with severe ARDS secondary to blastomycosis. Refractory hypoxemia developed in both patients despite adequate antifungal coverage with amphotericin B. Dramatic improvement was seen in each patient after initiation of corticosteroids in addition to amphotericin B. Both patients survived and did well on follow-up. We suggest that treatment with corticosteroids may be of benefit in patients with blastomycosis-induced ARDS. This may be due to a decrease in the severity of the inflammatory response.

Confirmation of Luteinizing Hormone (LH) in Living Human Vitreous and the Effect of LH Receptor Reduction on Murine Electroretinogram.

Luteinizing hormone (LH), produced in the anterior pituitary, has been detected in cadaver eyes and LH receptors (LHRs) have been identified in the retina, with the highest density in cone photoreceptors. Our aim was to confirm the presence of LH in the living, human eye as well as to examine the potential impact of a reduction in LHR signaling on visual processing. Vitreous samples were collected from 40 patients (23 diabetics, 17 non-diabetics) who were undergoing vitrectomies for various indications. LH concentration was quantified in each sample via an electro-chemiluminescence immunoassay and Meso Scale Discovery platform and normalized to total protein. In addition, full-field electroretinography (ERG) was performed on 11 adult LHR knockout heterozygous mice (B6;129X1-Lhcgrtm1Zmlei/J) and 11 wild types using the Celeris-Diagnosys system. The median LH values (pg/mg total protein) for non-diabetics, diabetics without proliferative diabetic retinopathy (PDR) and diabetics with PDR were 40.7, 41.9 and 167.8 respectively. LH levels were significantly higher in diabetics with PDR. In our ERG investigation, heterozygous LHRKOs were found to have significantly reduced amplitudes of a-wave and b-waves at high stimulus intensities with no significant change in a-wave or b-wave amplitudes at lower intensities; this is consistent with a selective impairment of cone-mediated responses. Our findings confirm LH is present in the adult human eye. Our findings also suggest that a reduction in LH receptor signaling negatively impacts visual processing of the cone photoreceptors. Overall, our study results support the theory that LH likely plays a physiologic role in the eye.

Safety and efficacy assessment of chimeric ribozyme to proliferating cell nuclear antigen to prevent recurrence of proliferative vitreoretinopathy.

OBJECTIVE: To determine the safety and efficacy of VIT100 (Immusol, Inc, San Diego, California), a ribozyme to proliferating cell nuclear antigen, in preventing recurrent proliferative vitreoretinopathy (PVR) in patients with established PVR who undergo vitrectomy for retinal reattachment repair. METHODS: A multicenter, double-masked, placebo-controlled, randomized clinical trial. One hundred seventy-five eyes from 175 patients with grade C or worse PVR were randomly assigned to receive high-dose VIT100, low-dose VIT100, or placebo by intravitreal injection at the conclusion of retinal reattachment surgery. MAIN OUTCOME MEASURES: The primary efficacy end point was recurrent retinal detachment secondary to PVR. The secondary end point was recurrent retinal detachment due to any cause. RESULTS: One hundred fifty-four patients completed the study. Forty-one patients (27%) developed recurrent retinal detachment due to PVR by 24 weeks, including 18 patients (33%) in the group receiving 0.75 mg, 13 patients (24%) in the group receiving 0.15 mg, and 10 patients (22%) in the placebo group. There was no statistically significant difference in patients reaching this end point by 24 weeks (P = .37). Ancillary statistical analyses are reported. CONCLUSIONS: VIT100 was not effective in preventing PVR recurrence in patients with established grade C or worse PVR. APPLICATION TO CLINICAL PRACTICE: To our knowledge, this is the most recent, meticulously designed clinical trial in PVR.

BASAL CELL NEVUS SYNDROME PRESENTING AS EPIRETINAL MEMBRANE AND MYELINATED NERVE FIBER LAYER.

PURPOSE: To report a case of epiretinal membrane and myelinated nerve fiber layer, which preceded the diagnosis of basal cell nevus syndrome, in a young girl. METHODS: Observational case report. RESULTS: A 12-year-old girl was referred for an asymptomatic epiretinal membrane. Examination revealed epiretinal membrane in the right eye without posterior vitreous separation or vitreous abnormality and bilateral myelinated nerve fiber layer. Subsequent workup yielded pathologic diagnosis of multiple skin basal cell carcinoma and odontogenic keratocysts in the jaw. Genetic testing revealed a frameshift mutation in the PTCH1 gene. CONCLUSION: Basal cell nevus syndrome is a rare autosomal dominant disease that affects multiple organ systems, including the eyes. Recognition of common ocular findings in children with basal cell nevus syndrome can lead to systemic diagnosis. Early diagnosis is critical to initiate early screening for known neoplastic associations and lifelong minimization of sun exposure to reduce the incidence and severity of basal cell carcinoma.

SPONTANEOUS BILATERAL HEMORRHAGIC CHOROIDAL DETACHMENTS ASSOCIATED WITH MALIGNANT HYPERTENSION.

PURPOSE: To report the details of a patient with bilateral spontaneous suprachoroidal hemorrhages related to malignant hypertension. METHODS: Observational case report with review of relevant literature. RESULTS: A 62-year-old man with a history of hypertension was referred secondary to bilateral temporal scotomas and persistent headache for 3 days. Symptoms began during an inpatient admission for malignant hypertension. Examination revealed bilateral 360° hemorrhagic choroidal detachments without retinal hemorrhage or detachment. Choroidal hemorrhages underwent prompt resolution with blood pressure control. CONCLUSION: Spontaneous suprachoroidal hemorrhage is a rare event and should prompt a focused systemic workup including the evaluation of blood pressure.

Chorioretinal Perfusion in Persistent Placoid Maculopathy.

Persistent placoid maculopathy (PPM) is a rare clinical entity that has previously been reported in the literature with a characteristic appearance on multimodal imaging, including hypofluorescence observed on fluorescein angiogram (FA) and indocyanine green angiography (ICGA). The leading mechanisms proposed for this hypofluorescence include impaired choroidal vasculature with nonperfusion of the choriocapillaris and/or blockage of choroidal fluorescence by inflammatory deposits. This report demonstrates previously unreported characteristics of chorioretinal perfusion in a case of acute onset PPM in a 58-year-old woman. Optical coherence tomography angiography confirmed hypoperfusion of the choriocapillaris corresponding to the hypoperfusion seen on FA and ICGA. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:1026-1031.].

Fundus autofluorescence in patients with pseudoxanthoma elasticum.

PURPOSE: To evaluate the autofluorescence findings of patients with pseudoxanthoma elasticum, a disease resulting from a defect in a reputed transport protein encoded by the gene adenosine triphosphate-binding cassette subtype C number 6. DESIGN: Observational case series. METHODS: Color, red-free monochromatic, and autofluorescence photography and fluorescein angiography of patients with pseudoxanthoma elasticum seen in a referral practice were evaluated. MAIN OUTCOME MEASURES: Cataloging of the abnormalities as detected by autofluorescence photography. RESULTS: The 8 subjects ranged in age from 26 to 60 years (mean, 55+/-12), and their best-corrected visual acuity ranged from 20/20 to 5/400 (mean, 20/50). Of the 16 eyes of the 8 patients, all had abnormalities typical of pseudoxanthoma elasticum, including angioid streaks in 14, peau d'orange in 4, and choroidal neovascularization in 11. Angioid streaks appeared as hypoautofluorescent fissures, sometimes showing expansion of the hypoautofluorescence suggestive of retinal pigment epithelium (RPE) absence or atrophy. Peau d'orange had a stippled appearance of autofluorescence, and drusen of the optic nerve appeared as hyperautofluorescent bodies. In addition to the expansion of RPE atrophy around angioid streaks, 3 additional configurations of RPE atrophy were recognized as RPE rips in 6 eyes, multilobular areas of atrophy in 9 eyes, and broad areas of poorly demarcated atrophy in 5 eyes. Some eyes had more than one manifestation of RPE atrophy, but the latter 3 types of atrophy occurred in eyes with, but not necessary contiguous to, concurrent choroidal neovascularization. CONCLUSIONS: Autofluorescence photography demonstrated that patients with pseudoxanthoma elasticum have more widespread areas of RPE disturbance, particularly atrophy, than what is detectable by other means of ocular imaging, which suggests that the RPE disturbance may play a role in the pathogenesis of visual loss in patients with pseudoxanthoma elasticum.

Sequenced combined intravitreal triamcinolone and indocyanine green angiography-guided photodynamic therapy for retinal angiomatous proliferation.

OBJECTIVE: To study sequenced combined therapy using intravitreal triamcinolone acetonide followed by photodynamic therapy for the treatment of retinal angiomatous proliferation. METHODS: Patients newly diagnosed as having retinal angiomatous proliferation underwent intravitreal triamcinolone injection to reduce intraretinal and subretinal exudation, followed 7 to 14 days later by indocyanine green angiography-guided photodynamic therapy with verteporfin. Complete ocular examination, fluorescein angiography, indocyanine green angiography, and optical coherence tomography were performed at baseline and at standard intervals thereafter. RESULTS: Twenty-seven eyes of 26 patients underwent this sequenced combined treatment and were followed up for 12 months. The triamcinolone injection reduced the cystoid edema before photodynamic therapy. Complete resolution of the angiographic leakage was achieved in 89% of eyes. Visual acuity improved in 37% and was stable in 52% of eyes. Eight eyes developed recurrent leakage after 3 to 11 months. Complete resolution of leakage was observed after subsequent treatment. CONCLUSIONS: This sequenced combined treatment in patients with retinal angiomatous proliferation was effective in reducing or eliminating the edema, achieving rapid regression of neovascularization, and stabilizing or improving visual acuity. To our knowledge, no study to date has achieved such promising results in the management of retinal angiomatous proliferation. A randomized clinical trial is under way to compare sequential and simultaneous combined therapy.

Measurement of the actual dose of triamcinolone acetonide delivered by common techniques of intravitreal injection.

PURPOSE: To measure the actual dose of triamcinolone acetonide (TA) delivered during intravitreal injection performed by several common techniques. DESIGN: Experimental study. METHODS: A 0.1-ml, 40-mg vial of TA (Kenalog-40; Bristol-Myers-Squibb, Peapack, New Jersey, USA) was prepared according to one of four protocols and the mass determined after drying overnight on waxed paper. In group 1, a 0.1-ml aliquot of TA was dispensed with a 30-gauge needle after shaking the vial 10 or 30 times. Group 2 used a 27-gauge needle. In group 3, the supernatant was removed from the crystals. Group 4 passed the suspension through a 0.2-microm micropore filter and rinsed the crystals with saline. RESULTS: There was no statistically significant difference between 30- or 27-gauge needles (P = 0.83, t test) or between shaking the vial 10 or 30 times before withdrawing the drug (P = 0.99). A statistically significant difference (t test, P = 0.001) was found between TA delivered from the initial 60% of each syringe (mean +/- SD, 2.7 +/- 1.0 mg) to that drawn from the last 40% of each syringe (7.8 +/- 3.6 mg). Group 3 had a mean weight of 32.1 +/- 7.0 mg and group 4, 10.6 +/- 2.1 mg. CONCLUSIONS: Efforts to achieve a 4.0-mg dose of TA, regardless of method used, are variable and inconsistent. Injecting through a small-gauge needle appears to concentrate the remaining suspension. Techniques to concentrate TA or remove aqueous preservatives by filtering effectively increase the concentration, but these results are variable.

Photodynamic therapy for focal retinal pigment epithelial leaks secondary to central serous chorioretinopathy.

PURPOSE: To report the use of photodynamic therapy with verteporfin as a treatment for patients with focal retinal pigment epithelial leaks secondary to central serous chorioretinopathy (CSC). DESIGN: Noncomparative, nonrandomized, retrospective interventional case series. PARTICIPANTS: Nine eyes of 9 symptomatic patients with acute focal retinal pigment epithelial leaks secondary to CSC, confirmed with fluorescein angiography, evaluated at 1 of 3 referral retina practices. METHODS: Patients were treated with photodynamic therapy using verteporfin. Best-corrected visual acuity (VA) was recorded at presentation and follow-up visits. MAIN OUTCOME MEASURES: Resolution of neurosensory detachment, status of fluorescein leakage, and VA. RESULTS: Neurosensory detachment and fluorescein leakage resolved in all patients within 1 month. Visual acuity improved from 1 to 6 lines in 7 eyes and remained unchanged in 2. At 6 months, there was a statistically significant improvement in mean VA (P = 0.012, Wilcoxon signed ranks test), and mean VA improved from 20/80 to 20/40. No patient lost vision or suffered any treatment-related complications. CONCLUSION: The treatment of acute CSC with photodynamic therapy may result in prompt resolution of neurosensory detachment and fluorescein leakage, which can be associated with rapidly improved vision. Although this case series is limited in follow-up and number of patients, the encouraging results and lack of visually significant complications suggest that further investigation is warranted.

Congenital uveal malignant melanoma: a case report.

CASE REPORT: We present a case of congenital choroidal mass in a male infant with multiple cutaneous pigmented lesions. Enucleation performed in the first weeks of life confirmed the diagnosis of diffuse uveal malignant melanoma with extraocular extension. The patient was also treated with 5 cycles of chemotherapy. He subsequently developed cutaneous and ocular pigmented lesions, including 2 choroidal nevi located within the posterior pole and a benign conjunctival lesion in the opposite eye, in addition to malignant melanoma of the skin. COMMENTS: At 10 years follow-up, the child is clinically well with no evidence of further malignancy. We believe this represents the fourth case ever reported in the literature and the longest follow-up of a congenital malignant melanoma originating within the eye.

The effects of cataract surgery on patients with wet macular degeneration.

PURPOSE: To explore whether cataract surgery contributes to the progression of wet age-related macular degeneration (wet AMD). DESIGN: Retrospective cohort study. METHODS: Retrospective review was performed of consecutive patients with wet AMD who underwent cataract surgery at the midpoint of a 1-year study window. A control arm included wet AMD eyes treated with anti-vascular endothelial growth factor (VEGF) injections that did not undergo cataract surgery for a 1-year period. Best-corrected visual acuity (BCVA), number of anti-VEGF injections, and optical coherence tomography (OCT) features were compared between the 2 arms. RESULTS: Forty eyes in the surgical group and 42 in the nonsurgical group were included. BCVA was equivalent in the first half of the study, and became significantly better in the surgical group vs the nonsurgical group (0.23 ± 0.65 vs 0.11 ± 0.59 logMAR improvement, P = .049). There was no change in the number of injections given 6 months before vs after the midpoint in the surgical group (P = .921). The mean OCT central retinal thickness became greater in postsurgical eyes compared to nonsurgical eyes (265.4 ± 98.4 µm vs 216.4 ± 58.3 µm, P = .011). Surgical eyes were more likely to develop new or worse cystoid changes after the study midpoint (13 surgical eyes [54.2%] vs 9 nonsurgical eyes [28.1%], P = .048). CONCLUSIONS: Cataract surgery leads to vision improvement and does not appear to contribute to worsening of wet AMD. However, anatomic changes based on OCT analysis suggest a subclinical susceptibility to postoperative cystoid macular edema or exacerbation of choroidal neovascularization.