Matthew T. Witmer

Wide-field Imaging of the Retina

The retinal periphery is the site of pathology in several eye diseases. Imaging of the peripheral retina offers a way to diagnose, monitor, and evaluate responses to the treatment of these conditions. Traditional fundus cameras have offered a 30- to 50-degree field of view. Recent technology has advanced to provide up to a 200-degree field of view. The utility of this technology in clinical practice continues to be investigated; wide-field color photography, autofluorescence imaging, and fluorescein angiography have been used for imaging peripheral retinal disease. Due to the limitations of this imaging technology and the lack of normative data, however, the clinical role of wide-field imaging remains controversial.

Comparison of ultra-widefield fluorescein angiography with the Heidelberg Spectralis® noncontact ultra-widefield module versus the Optos® Optomap®

Purpose To compare ultra-widefield fluorescein angiography imaging using the Optos® Optomap® and the Heidelberg Spectralis® noncontact ultra-widefield module. Methods Five patients (ten eyes) underwent ultra-widefield fluorescein angiography using the Optos® panoramic P200Tx imaging system and the noncontact ultra-widefield module in the Heidelberg Spectralis® HRA+OCT system. The images were obtained as a single, nonsteered shot centered on the macula. The area of imaged retina was outlined and quantified using Adobe® Photoshop® C5 software. The total area and area within each of four visualized quadrants was calculated and compared between the two imaging modalities. Three masked reviewers also evaluated each quadrant per eye (40 total quadrants) to determine which modality imaged the retinal vasculature most peripherally. Results Optos® imaging captured a total retinal area averaging 151,362 pixels, ranging from 116,998 to 205,833 pixels, while the area captured using the Heidelberg Spectralis® was 101,786 pixels, ranging from 73,424 to 116,319 (P = 0.0002). The average area per individual quadrant imaged by Optos® versus the Heidelberg Spectralis® superiorly was 32,373 vs 32,789 pixels, respectively (P = 0.91), inferiorly was 24,665 vs 26,117 pixels, respectively (P = 0.71), temporally was 47,948 vs 20,645 pixels, respectively (P = 0.0001), and nasally was 46,374 vs 22,234 pixels, respectively (P = 0.0001). The Heidelberg Spectralis® was able to image the superior and inferior retinal vasculature to a more distal point than was the Optos®, in nine of ten eyes (18 of 20 quadrants). The Optos® was able to image the nasal and temporal retinal vasculature to a more distal point than was the Heidelberg Spectralis®, in ten of ten eyes (20 of 20 quadrants). Conclusion The ultra-widefield fluorescein angiography obtained with the Optos® and Heidelberg Spectralis® ultra-widefield imaging systems are both excellent modalities that provide views of the peripheral retina. On a single nonsteered image, the Optos® Optomap® covered a significantly larger total retinal surface area, with greater image variability, than did the Heidelberg Spectralis® ultra-widefield module. The Optos® captured an appreciably wider view of the retina temporally and nasally, albeit with peripheral distortion, while the ultra-widefield Heidelberg Spectralis® module was able to image the superior and inferior retinal vasculature more peripherally. The clinical significance of these findings as well as the area imaged on steered montaged images remains to be determined.

Optical coherence tomography predicts visual outcome in macula-involving rhegmatogenous retinal detachment

Purpose Visual recovery after rhegmatogenous retinal detachment (RRD) repair depends upon various anatomical factors. We investigated spectral-domain optical coherence tomography (SD-OCT) abnormalities, pre- and postoperatively, in patients with nontraumatic RRD and correlated these findings with visual outcome. Methods The medical records of all patients presenting to Weill Cornell Medical College with nontraumatic macula-involving RRD from August 2010 to September 2011 were retrospectively reviewed in this single-center, consecutive case series. All patients underwent pre- and postoperative visual acuity (VA) testing, slit-lamp biomicroscopy, and dilated fundus examination. Spectral domain optical coherence tomography was obtained preoperatively in twelve patients and postoperatively in ten patients. Results Twelve patients (12 eyes) were included in the final analysis. Preoperative optical coherence tomography revealed that the inner segment/outer segment (IS/OS) junction was disrupted in 10/12 eyes (83%), the external limiting membrane (ELM) was disrupted in 9/12 (75%) eyes, cystoid macular edema (CME) was present in 10/12 (83%) eyes, an epiretinal membrane (ERM) was present in 2/12 eyes (17%) and outer retinal corrugation was present in 7/12 (58%) eyes. In postoperative imaging of 10 eyes, the IS/OS junction was disrupted in 4/10 (40%), the ELM was disrupted in 3/10 (30%) eyes, CME was present in 2/10 (20%), and an ERM in 1/10 (10%). All retinas were attached postoperatively. Outer retinal corrugation was the most predictive of worse preoperative (P = 0.0016) and 1-month postoperative visual acuity (P = 0.05). Conclusion Preoperative SD-OCT demonstrating outer retinal corrugation in macula involving RRD predicts poor visual acuity outcome in nontraumatic RRD. Such findings may have implications for the urgency for these eyes to undergo surgical repair.

Peripheral autofluorescence findings in age‐related macular degeneration

Purpose:  To describe the peripheral autofluorescent findings in patients with age‐related macular degeneration (AMD) using ultrawide‐field imaging.

Central serous chorioretinopathy in patients receiving exogenous testosterone therapy.

Purpose: To report an association between central serous chorioretinopathy (CSCR) and exogenous testosterone therapy. Methods: This is a retrospective case series from two institutions. Patients who presented with fluorescein angiography and optical coherence tomography findings consistent with CSCR were included. All patients were concurrently being treated with exogenous testosterone therapy and lacked other known risk factors for CSCR. Results: Nine patients presented with CSCR after beginning exogenous testosterone therapy. Two patients stopped therapy with resolution of symptoms and subretinal fluid. Conclusion: Exogenous testosterone may be an independent risk factor for the development of CSCR.

Spectrum of Ocular Manifestations in CLN2-Associated Batten (Jansky-Bielschowsky) Disease Correlate with Advancing Age and Deteriorating Neurological Function

Background Late infantile neuronal ceroid lipofuscinosis (LINCL), one form of Batten’s disease is a progressive neurodegenerative disorder resulting from a CLN2 gene mutation. The spectrum of ophthalmic manifestations of LINCL and the relationship with neurological function has not been previously described. Methods Patients underwent ophthalmic evaluations, including anterior segment and dilated exams, optical coherence tomography, fluorescein and indocyanine green angiography. Patients were also assessed with the LINCL Neurological Severity Scale. Ophthalmic findings were categorized into one of five severity scores, and the association of the extent of ocular disease with neurological function was assessed. Results Fifty eyes of 25 patients were included. The mean age at the time of exam was 4.9 years (range 2.5 to 8.1). The mean ophthalmic severity score was 2.6 (range 1 to 5). The mean neurological severity score was 6.1 (range 2 to 11). Significantly more severe ophthalmic manifestations were observed among older patients (p<0.005) and patients with more severe neurological findings (p<0.03). A direct correlation was found between the Ophthalmic Severity Scale and the Weill Cornell Neurological Scale (p<0.002). A direct association was also found between age and the ophthalmic manifestations (p<0.0002), with older children having more severe ophthalmic manifestations. Conclusions Ophthalmic manifestations of LINCL correlate closely with the degree of neurological function and the age of the patient. The newly established LINCL Ophthalmic Scale may serve as an objective marker of LINCL severity and disease progression, and may be valuable in the evaluation of novel therapeutic strategies for LINCL, including gene therapy.

Oral anticoagulation and the risk of vitreous hemorrhage and retinal tears in eyes with acute posterior vitreous detachment.

Purpose: To determine if oral anticoagulation alters the association between vitreous hemorrhage (VH) and retinal tears in eyes with acute, posterior vitreous detachment (PVD). Methods: In this retrospective chart review, the complete records of consecutive patients with spontaneous, symptomatic acute PVD from a single referral-based practice were reviewed. The use of oral anticoagulants, the presence of a VH, and the presence of a retinal tear or detachment were recorded. Results: A total of 336 consecutive eligible patients (336 eyes) were included in the final analysis. Vitreous hemorrhage occurred in 118 (35%) eyes; in 43% of patients taking aspirin, clopidogrel, or warfarin versus 31% not taking these medications (P = 0.03). Retinal tears occurred in 46% of patients with VH versus 27% of patients without VH (P = 0.0007). Retinal tears occurred in 39% of patients with VH taking aspirin, clopidogrel, or warfarin compared with 52% of patients not taking these medications. (P = 0.20) A decreased proportion of patients with acute PVD taking one or more of the oral anticoagulant medications studied, regardless of the presence of VH, were diagnosed with a retinal tear (P = 0.0017) or retinal detachment (P = 0.0001). Conclusion: Retinal tears are commonly found (46%) in the eyes of patients who present with symptoms and signs of acute PVD and VH. Patients taking aspirin, clopidogrel, or warfarin who develop an acute PVD are more likely to present with VH. No statistically significant association was demonstrated between the use of oral anticoagulants in patients with acute PVD and VH and the presence of retinal tears or retinal detachment.

Ophthalmic Artery Ischemic Syndrome Associated With Neurofibromatosis and Moyamoya Syndrome

Author Affiliations: Department of Ophthalmology, Hôpitaux Universitaires, Geneva (Dr Vaclavik), and JulesGonin Eye Hospital, University of Lausanne, Lausanne (Drs Vaclavik, Borruat, Ambresin, and Munier), Switzerland. Correspondence: Dr Munier, Jules-Gonin Eye Hospital, Avenue de France 15, 1004 Lausanne, Switzerland (francis.munier@fa2.ch). Author Contributions: Drs Vaclavik and Borruat contributed equally to the work and share first authorship. Conflict of Interest Disclosures: None reported. Funding/Support: This work was supported by grant 320030-127558 from the Swiss National Science Foundation (Dr Munier). Previous Presentation: This paper was presented at the Atlantic Coast Fan Club Meeting; January 20, 2012; New York, New York; and the Société Française d’Oculogénétique Francophone Annual Meeting; December 2-3, 2011; Lausanne, Switzerland.

Isolated foveal hypoplasia without nystagmus

We report the case of a 23-year-old healthy Caucasian male with isolated foveal hypoplasia without nystagmus. Clinical examination and spectral-domain optical coherence tomography demonstrated the bilateral absence of a foveal depression and the patient was diagnosed with isolated foveal hypoplasia. This is a rare condition which is probably under-diagnosed since it can exist without nystagmus and low vision.

Characteristics of intraretinal deposits in acute central serous chorioretinopathy

Purpose To describe the temporal and spatial characteristics of intraretinal deposits in patients with acute central serous chorioretinopathy (CSC) using spectral domain optical coherence tomography (OCT). Materials and methods We retrospectively reviewed the medical records of all patients that presented with acute CSC to Weill Cornell Medical College from January 2012 to May 2013. Acute CSC was defined as a diagnosis of CSC within 4 months of the onset of symptoms. Only one eye per patient was included in the study. Each patient was imaged with spectral domain OCT at the initial office visit. The decision to reimage these patients was made by the treating physician. Results A total of 25 patients (25 eyes; 17 men and eight nonpregnant women) were included in this review. Seven of 25 patients (28%) demonstrated intraretinal deposits within the outer plexiform layer during the initial OCT, with deposits appearing as early as the same day as the onset of symptoms. A total of 25 of 25 patients (100%) demonstrated intraretinal deposits in the outer nuclear layer upon initial (76%) or follow-up OCT, as early as 2 days after the onset of symptoms. A total of 24 of 25 patients (96%) demonstrated deposits in the external limiting membrane upon a follow-up OCT, as early as 7 days from symptoms appearing. A total of 24 of 25 patients (96%) developed intraretinal deposits in the inner segment/outer segment layer upon follow-up OCT, appearing as early as 14 days after symptom onset. At the time of resolution of subretinal fluid, 20 of 25 patients (80%) demonstrated intraretinal deposits. Conclusion Intraretinal deposits are present in the outer retinal layers in patients with acute CSC, with the deposits appearing progressively deeper within the retina as the condition evolves. Upon resolution of subretinal fluid, the deposits slowly resolve.