Faculty Bibliography

The clinical presentation of an intraocular nematode unusually affixed to the posterior lens capsule is described. A 64-year-old female patient presented with a 7-year history of gradually declining vision and enlarging central scotoma, but no inflammation. On follow-up 2 years later, vision had further declined and a non-motile, 8-mm nematode was seen affixed to the posterior lens capsule that remained unchanged through final follow-up. The patient disclosed having resided in Africa as a child. Systemic review revealed no evidence of extraocular involvement. Nematode carcasses may remain preserved in the human eye for extended periods without ongoing inflammation.

Macular holes in the setting of macular telangiectasia type 2 can be difficult to manage. The rates of anatomical closure after macular hole surgery are less favorable in patients with macular telangiectasia than in those with idiopathic macular holes. These differences may be due to the influence of unique pathogenic mechanisms that modulate macular hole dynamics in patients with macular telangiectasia. In this report, the authors document the multimodal imaging findings of a patient with macular telangiectasia demonstrating spontaneous macular hole closure. These findings may improve the understanding of the natural course of this entity and may have relevance for clinical management. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:883-886.].

PURPOSE: To evaluate the vascular structure of eyes with macular telangiectasia type 2 (MacTel2) using volume-rendered optical coherence tomography angiography (OCTA). DESIGN: Retrospective cross-sectional study. PARTICIPANTS: A total of 14 consecutive patients (20 eyes) with MacTel2 who had a signal strength score >/=55 and could maintain fixation during the scan process. METHODS: The eyes were scanned using optical coherence tomography with split-spectrum amplitude decorrelation techniques to derive flow information. Data were extracted and used to create volume-rendered images of the retinal vasculature that could be rotated about 3 different axes for evaluation. MAIN OUTCOME MEASURES: Descriptive appraisal of the vascular abnormalities associated with MacTel2. RESULTS: Vessels posterior to the outer boundary of the deep retinal plexus were secondary to retinal thinning, vascular invasion, or a combination of both. These vessels had the same shape and distribution as the late staining seen during conventional fluorescein angiography. Lateral contraction in the temporal macula in 5 eyes created an appearance of vessels radiating from a central locus, which was the site of a right angle vein. Loss of macular tissue as part of the disease process led to a central amalgamation of the inner vascular plexus and the deep vascular plexus, which appeared to be in a state of decline. Subretinal neovascularization originated from the retinal circulation but involved not only the subretinal space but also could infiltrate the remaining, thinned, retina. CONCLUSIONS: Volume rendering of OCTA information preserves the 3-dimensional relationships among retinal vascular layers and provides opportunities to visualize retinal vascular abnormalities in unprecedented detail. The retinal vascular leakage and invasion in MacTel2 may arise as a consequence of loss of control with depletion of Muller cells and exposure of the remaining retinal vessels to the more hypoxic environment near the inner segments of the photoreceptors.

PURPOSE: To correlate postmortem histology with previously recorded multimodal imaging from a patient with type 1 neovascularization associated with an acquired vitelliform lesion in the setting of age-related macular degeneration (AMD). DESIGN: Case study. METHODS: Multimodal imaging that was obtained ante mortem was matched with ex vivo and high-resolution histological images of the preserved donor macula. Anatomic correlates for multimodal imaging findings were then defined. RESULTS: Spectral-domain optical coherence tomography (OCT) revealed a split in the retinal pigment epithelial-Bruch's membrane band. Type 1 NV in this case was comprised of 6 layered components: (1) Retinal pigment epithelium, (2) Basal laminar deposits, (3) Fibrovascular membrane, (4) Fibrocellular scar, (5) Hemorrhage, and (6) Bruch's membrane. The anatomic correlates for the hyporeflective band on spectral-domain OCT included a thick basal laminar deposit. Not all structures could be readily separated on the basis of their reflectivity patterns. CONCLUSIONS: This is an important clinicopathologic correlation of NV secondary to AMD in the spectral-domain OCT era. Our findings of 6 layers include and extend the anatomical framework encapsulated by the double-layer and triple-layer signs. The resolution of current devices does not always permit distinction of the different layers of NV tissue. Thick basal laminar deposits may appear hyporeflective on spectral-domain OCT and may be confused with fluid from a neovascular process. It will be important to perform a larger clinicopathologic series to aid our anatomical interpretation of spectral-domain OCT images.

PURPOSE: To present a case of an HIV-positive patient with acute exudative polymorphous vitelliform maculopathy (AEPVM) and evaluate the presence of specific spectral-domain optical coherence tomography (SD-OCT) findings. METHODS: Case report. RESULTS: We reviewed the AEPVM cases reported in the literature and compared those to our patient to determine if there is a correspondence between the etiology that leads to the onset of AEPVM and clinical and SD-OCT findings. CONCLUSIONS: Acute exudative polymorphous vitelliform maculopathy is a disease that involves the outer retinal layers with lipofuscin deposits and serous detachment of the neuroepithelium with or without intraretinal cysts. Not much is known about the etiology and pathogenesis, and not many cases have been described. A review of the few clinical cases reported in the literature does not show a specific correspondence between etiology and SD-OCT findings.

BACKGROUND:: To report the presence of transient peripapillary serous detachments in multiple evanescent white dot syndrome. METHODS:: Retrospective case series. RESULTS:: Four eyes of four patients diagnosed with multiple evanescent white dot syndrome presented with peripapillary serous detachments. Diagnosis was based on clinical presentation, fundus findings, and angiographic findings. All 4 were female with age ranges between 24 and 40 years and presented with photopsias, an enlarged scotoma contiguous with the blind spot, and chorioretinal white dots in the posterior pole with characteristic angiographic features. All of the serous detachments resolved or were greatly reduced concomitantly with the resolution of the patient's other clinical symptoms and fundus findings. CONCLUSION:: The authors report peripapillary serous detachments as a previously unreported manifestation of multiple evanescent white dot syndrome. These seem to be self limited with concurrent resolution with the rest of the patient's symptoms.

PURPOSE:: To report the ocular phenotype in patients with autosomal recessive bestrophinopathy and carriers, and to describe novel BEST1 mutations. METHODS:: Patients with clinically suspected and subsequently genetically proven autosomal recessive bestrophinopathy underwent full ophthalmic examination and investigation with fundus autofluorescence imaging, spectral domain optical coherence tomography, electroretinography, and electrooculography. Mutation analysis of the BEST1 gene was performed through direct Sanger sequencing. RESULTS:: Five affected patients from four families were identified. Mean age was 16 years (range, 6-42 years). All affected patients presented with reduced visual acuity and bilateral, hyperautofluorescent subretinal yellowish deposits within the posterior pole. Spectral domain optical coherence tomography demonstrated submacular fluid and subretinal vitelliform material in all patients. A cystoid maculopathy was seen in all but one patient. In 1 patient, the location of the vitelliform material was seen to change over a follow-up period of 3 years despite relatively stable vision. Visual acuity and fundus changes were unresponsive to topical and systemic carbonic anhydrase inhibitors and systemic steroids. Carriers had normal ocular examinations including normal fundus autofluorescence. Three novel mutations were detected. CONCLUSION:: Three novel BEST1 mutations are described, suggesting that many deleterious variants in BEST1 resulting in haploinsufficiency are still unknown. Mutations causing autosomal recessive bestrophinopathy are mostly located outside of the exons that usually harbor vitelliform macular dystrophy-associated dominant mutations.

PURPOSE:: To describe multimodal imaging findings in a patient with foveal red spot syndrome. METHODS:: We report a case of a 57-year-old man with foveal red spot syndrome. Multimodal imaging techniques, including fundus color and red-free photographs, fluorescence angiography (Topcon 50DX; Topcon, Tokyo, Japan), MultiColor scanning laser imaging, spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany), swept source optical coherence tomography (DRI OCT-1 Atlantis; Topcon, Tokyo, Japan), adaptive optics (RTX-1; Imagine Eyes, Orsay, France), and microperimetry (MP1 Microperimeter; Nidek Technologies, Padua, Italy), were performed to confirm the diagnosis and determine the anatomical abnormalities related to the disease. RESULTS:: Conventional clinical examination and imaging studies failed to explain the patient's visual dysfunction and this specific macular abnormality. Using advanced ophthalmic technologies, including MultiColor imaging, spectral domain optical coherence tomography with high-density acquisition, swept source optical coherence tomography, adaptive optics, and microperimetry, we identified the nature of the macular manifestation accounting for pathology of the foveal red spot syndrome and his visual dysfunction. CONCLUSION:: When conventional clinical examination and imaging techniques fail to identify the presence of and visual symptoms in foveal red spot syndrome, advanced technologies may be used to confirm the diagnosis and explain the etiology of the abnormality.