Adaptive optics ophthalmoscopy, multifocal ERG and OCTA in unique case of suspected torpedo maculopathy presenting with vitelliform lesion.

PURPOSE: To report the case of a ten-year old girl with torpedo maculopathy with a complete vitelliform lesion and describe associated optical coherence tomography (OCT), OCT angiography (OCTA), multifocal electroretinogram (ERG) and adaptive optics ophthalmoscopy (AOO) imaging of the lesion. OBSERVATIONS: An asymptomatic ten-year old girl with visual acuity of 20/15 OU was referred for evaluation of possible Best's disease of her left eye. The unilaterality, location, and shape of the lesion was consistent with torpedo maculopathy. OCT and autofluorescence (AF) revealed that the entire lesion was composed of subretinal hyperreflective material that was hyperautofluorescent, consistent with vitelliform material. Within the boundary of the lesion, OCTA showed reduced choriocapillaris density while adjacent to the lesion, the choriocapillaris density was slightly increased. Microperimetry demonstrated normal sensitivity in both eyes, electrooculograms (EOG) were normal and multifocal ERG showed symmetrical mildly supernormal amplitudes. Additionally, AOO demonstrated that nasal to the lesion there were clusters of hyper-reflective areas, and immediately adjacent to the lesion cones were poorly resolved. However, there was a return to more normal photoreceptor architecture outside of the lesion. CONCLUSIONS AND IMPORTANCE: Torpedo maculopathy lesions typically present with outer retinal attenuation and retinal pigmented epithelium (RPE) atrophy. Vitelliform material was recently observed for the first time in association with Torpedo maculopathy in a case report that described small vitelliform material at the periphery of the lesion. We report the second case of torpedo maculopathy associated with a vitelliform lesion and the first description of a torpedo lesion composed fully of presumed vitelliform material. We also describe findings of OCTA, multifocal ERG and AOO imaging in torpedo maculopathy with vitelliform lesion.

Foveal involvement by acquired retinoschisis: long-term visual outcomes.

PURPOSE: To report the long-term findings of acquired retinoschisis whose posterior border was <10° from the fovea. METHODS: Retrospective case series. RESULTS: Over a 43-year period, 2 patients retained central vision when the foveola was involved by retinoschisis alone. In these two and in another four patients, the development of outer layer holes eventually was followed by either outer layer or full thickness retinal detachment into the foveola with loss of central vision. Laser treatment or vitrectomy prevented this in three patients. A seventh patient is being monitored by periodic spectral domain optical computerized tomography. CONCLUSION: Acquired retinoschisis that progress into the foveola without an outer layer detachment is compatible with preservation of some degree of central vision. If an outer layer detachment develops under the foveola, such patients lose central vision. Periodic monitoring by spectral domain optical computerized tomography should help to avoid or detect this complication.

Ocular coherence tomography in acute posterior multifocal placoid pigment epitheliopathy.

The authors report a case of typical acute posterior multifocal placoid pigment epitheliopathy in which an ocular coherence tomographic scan was performed through an acute lesion and then repeated through the same lesion 12 months later. The initial ocular coherence tomographic scan showed marked anterior displacement of both neuroretina and outer reflective band. A subsequent ocular coherence tomographic scan revealed resolution of the prior displacement, increased reflectance of the outer reflective band and mild disruption of the outer retinal layers. These findings are consistent with a primary choroiditis.

Long-term juxtafoveal retinal telangiectasia.

PURPOSE: To determine the long-term natural history of idiopathic juxtafoveal telangiectasia (IJRT). METHODS: Record review in 2 university-based and 2 private vitreoretinal practices sought patients with IJRT documented by color photographs and fluorescein angiograms (FAs) during the period January 1, 1980, to December 31, 1993. Patients then had repeated examinations and FAs. RESULTS: Twenty patients with IJRT in 32 eyes had follow-up examinations. Fifteen patients had color photographs and FAs, and one had color photographs alone. Follow-up varied from 10 years to 21 years (average, 15 years). Six eyes were treated by laser photocoagulation at onset. Twenty-four of the 26 untreated eyes lost vision as measured by Snellen testing. Visual loss and morphologic progression depended on IJRT type. Six of 8 untreated eyes with type IA IJRT lost vision by >/ or = 3 lines (Snellen), 4 to 20/70 or worse. Vision loss was caused by progressive telangiectatic changes and intraretinal edema. Fifteen of 20 initially untreated eyes with type IIA IJRT developed either central retinal pigment epithelium membranes or subretinal neovascularization with loss of vision to 20/80 or less. CONCLUSION: IJRT prognosis depends on type and clinical features. Long-term prognosis for central vision is poor.

Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies.

PURPOSE: Paraneoplastic and autoimmune retinopathies are immunologically mediated retinal degenerations that are associated with antibodies directed against any of several retinal proteins, including alpha-enolase. We report the clinical and electrophysiological features of antienolase retinopathy in contrast to the features of antirecoverin retinopathy. DESIGN: Retrospective, observational case series. METHODS: Patients were referred for evaluation of unexplained acquired visual symptoms, including photopsias, and loss of visual acuity or field considered of possible retinal origin. Full-field and multifocal electroretinograms (ERGs) were performed. Sera from patients were examined for antiretinal antibodies by Western blot analysis using proteins extracted from human retinas and by immunohistochemistry; antienolase was confirmed by incubating patient sera with purified alpha-enolase. RESULTS: Of 87 patients with unexplained retinal visual symptoms associated with abnormal ERGs, 37 (43%) demonstrated autoantibodies to retinal antigens, including 12 against alpha-enolase, of whom 4 had cancer. Initial visual loss was typically central and often asymmetric. The ERGs demonstrated mostly normal rod responses but central cone abnormalities (evident on multifocal ERG) and, for many, global cone abnormalities. Seven patients developed optic disk pallor. Corticosteroid and immunosuppressive therapy, when attempted, was clinically ineffective. CONCLUSIONS: Antienolase retinopathy is a protean autoimmune retinopathy that characteristically presents with cone dysfunction. The visual impairment and course vary from relative stability for years to slow progression with loss of central vision. With time, optic disk pallor can evolve, presumably from attrition of ganglion cells.

A simple device for teaching direct ophthalmoscopy to primary care practitioners.

PURPOSE: Ophthalmoscopy, a valuable skill for primary care practitioners, can be challenging to learn. A simple and inexpensive device for teaching direct ophthalmoscopy to primary care practitioners is described. DESIGN: Device description. METHODS: Cylindrical plastic canisters were altered to have an artificial pupil at one end and a replaceable fundus photograph at the other end to simulate the mechanics of performing direct ophthalmoscopy on a real eye. These were tested for ease of use by primary care students. RESULTS: The devices to aid in teaching ophthalmoscopy proved to be simple and inexpensive to construct. They allowed students to practice direct ophthalmoscopy technique and identification of funduscopic abnormalities. CONCLUSION: This simple device for teaching direct ophthalmoscopy to primary care practitioners is inexpensive to create and is a valuable aid for teaching direct ophthalmoscopy to primary care practitioners.

Clinical features and natural history of the acute idiopathic enlarged blind spot syndrome.

OBJECTIVE: To study the clinical pattern and natural history of patients with the symptom of an enlarged blind spot. DESIGN: A retrospective case series. Twenty-one patients were collected from a neuro-ophthalmologic practice during the period January 14, 1983, to July 1, 1996, and four consecutive patients were added from three vitreoretinal practices during the period April 14, 1986, to June 7, 1999. PARTICIPANTS: Twenty-six eyes of 25 patients were studied at onset and at repeat visits from 1 year and 7 months to 15 years and 6 months later. METHODS: The first visit was composed of a complete neuro-ophthalmologic examination with fundus photos and fluorescein angiography in 12 of the 26 eyes. Follow-up examination consisted of an interval history, ophthalmologic examination, visual fields, fundus photographs, fluorescein angiograms in eight eyes, indocyanine green angiograms in seven eyes, and multifocal electroretinograms (mfERG) in both eyes of seven patients. MAIN OUTCOME MEASURES: The major outcome measures were onset and long-term visual field characteristics, disc and peripapillary features, association with other chorioretinal diseases, and mfERG features. RESULTS: Twenty-one eyes had clinical features of chorioretinal syndromes, which are usually associated with an enlarged blind spot. Five eyes were examined too late after onset to expect such features. The visual field defect regressed in all but 12 eyes but never to an unequivocally normal-sized blind spot. Four of the 12 had chorioretinal scarring that corresponded to the permanent field defect. Twenty-one of 26 eyes had peripapillary scarring. The peripapillary scarring appeared the same no matter what the associated chorioretinal disease or type or size of field defect was. mfERG testing of seven patients at follow-up revealed first-order and second-order abnormalities long after clinical recovery, abnormalities that were bilateral even when the clinical signs had been unilateral. CONCLUSIONS: If an eye with an enlarged blind spot is examined within 2 weeks of onset, signs of a chorioretinal disease will usually be present. Beyond that period, signs such as disc congestion, disc staining, peripapillary retinitis, foveal changes, and peripheral retinal spots may not be present. Although the patient usually becomes asymptomatic, the blind spot is slightly and permanently enlarged, and there is usually peripapillary disc scarring. mfERG testing indicates that retinal damage is more widespread, bilateral, and permanent than the visual field and clinical features would indicate. Chorioretinal syndromes that are associated with a temporal field defect have some features in common and others that are distinctive.