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."

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.

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.

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.].

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.

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.

Nd:YAG membranotomy for preretinal hemorrhage secondary to valsalva retinopathy.

PURPOSE: To report four cases of premacular hemorrhage secondary to valsalva retinopathy treated with Nd:YAG membranotomy and discuss techniques as well as the literature. DESIGN: Retrospective case series. METHODS: A retrospective review was conducted for four patients with vision obstructing hemorrhage secondary to valsalva retinopathy. These patients were all treated with Nd:YAG membranotomy. RESULTS: Four patients with premacular hemorrhage secondary to valsalva retinopathy were treated with Nd:YAG laser creating a membranotomy to drain the hemorrhage. Power settings ranged from 1.7 to 3.8 mJ. Visual acuity at presentation ranged from 20/400 (1 patient) to count fingers (3 patients). Visual acuity improved in three out of four patients after laser treatment. Final visual acuity ranged from 20/20 to 20/30 in these three patients. One patient was lost to follow up after performing laser membranotomy and therefore visual acuity after treatment was not obtained. No complications were noted. CONCLUSION: Nd:YAG membranotomy is a non-invasive, office-based treatment option that may be successfully used to treat premacular hemorrhage secondary to valsalva retinopathy.

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.

Immunogammopathies and acquired vitelliform detachments: a report of four cases.

PURPOSE: To describe the nature and evolution of acquired macular detachments in patients with immunogammopathies and to propose a mechanism for their development. DESIGN: Retrospective observational case series. METHODS: Three patients with multiple myeloma and 1 with light chain deposition disease were diagnosed with vitelliform macular detachments based on clinical examination, fundus autofluorescence, fluorescein angiography, and optical coherence tomography. These patients were followed over time and their clinical examinations and imaging studies were compared and contrasted. RESULTS: Three patients (5 eyes) with multiple myeloma and 1 patient (2 eyes) with light chain deposition disease presented with acquired macular yellowish subretinal deposits on funduscopic examination that corresponded to hyperautofluorescent lesions on fundus autofluorescence imaging and subretinal hyperreflective material on spectral-domain optical coherence tomography. One patient (2 eyes) had diffuse serous retinal detachments involving not only the macular region but also the midperiphery of the retina. These acquired macular vitelliform detachments were not associated with signs of hyperviscosity retinopathy in 5 eyes and resolved after successful treatment of the multiple myeloma in 6 eyes. CONCLUSION: Patients with an immunogammopathy such as multiple myeloma or light chain deposition disease may develop serous elevations of the macula that we classify as acquired vitelliform detachments using multimodal imaging. Appropriate evaluation including serum protein electrophoresis and hematology consultation should be considered in the management of patients with acquired vitelliform detachments of uncertain etiology.

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.

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 expanded spectrum of focal choroidal excavation.

OBJECTIVE: To describe the clinical and imaging findings in patients with focal choroidal excavation. METHODS: Retrospective observational case series. The medical records of 12 patients (13 eyes) with focal choroidal excavation were reviewed. Clinical histories and imaging findings (including color photography, fundus autofluorescence imaging, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and enhanced depth imaging spectral-domain optical coherence tomography) were analyzed. RESULTS: The mean age of the patients was 45 years (range, 22-62 years). Four patients were Asian. Mean visual acuity was 20/31 (range, 20/20 to 20/100). Mean refractive error was -3.54 diopters (D) (range, 6.00 to -8.00 D). One patient had bilateral involvement. All patients manifested varying degrees of foveal pigmentary changes that were usually hypoautofluorescent on fundus autofluorescence images. Fluorescein angiographic findings varied with degree of retinal pigment epithelial alterations. Indocyanine green angiography revealed relative hypofluorescence. In 7 eyes, spectral-domain optical coherence tomography revealed outer retinal layers conforming to retinal pigment epithelial alterations within the excavation. In the other 6 eyes, spectral-domain optical coherence tomography revealed a separation between the outer retina and the retinal pigment epithelium within the excavation. In 7 eyes studied with enhanced depth imaging spectral-domain optical coherence tomography, there was no evidence of scleral ectasia. Mean choroidal thickness of the uninvolved choroid was thicker than normal at 319 µm (range, 244-439 µm). All lesions remained stable except for in 1 eye, which had findings of central serous chorioretinopathy and secondary type 2 (subretinal) neovascularization. CONCLUSION: Focal choroidal excavation is a newly described idiopathic entity in eyes having 1 or more focal areas of choroidal excavation. In some patients, there may be an association with central serous chorioretinopathy. Although most lesions remain stable, secondary choroidal neovascularization may occur.