Accepted Manuscript Paracentral Acute Middle Maculopathy in Nonischemic Central Retinal Vein Occlusion Ehsan Rahimy, David Sarraf, Michael L. Dollin, John D. Pitcher, Allen C. Ho PII:

S0002-9394(14)00227-X

DOI:

10.1016/j.ajo.2014.04.024

Reference:

AJOPHT 8901

To appear in:

American Journal of Ophthalmology

Received Date: 19 December 2013 Revised Date:

23 April 2014

Accepted Date: 23 April 2014

Please cite this article as: Rahimy E, Sarraf D, Dollin ML, Pitcher JD, Ho AC, Paracentral Acute Middle Maculopathy in Nonischemic Central Retinal Vein Occlusion, American Journal of Ophthalmology (2014), doi: 10.1016/j.ajo.2014.04.024. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

ACCEPTED MANUSCRIPT

Paracentral Acute Middle Maculopathy in Nonischemic Central Retinal Vein Occlusion Ehsan Rahimy1, David Sarraf2,3, Michael L. Dollin1, John D. Pitcher1, Allen C. Ho1 1

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Mid Atlantic Retina, The Retina Service of Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. 2 Retinal Disorders and Ophthalmic Genetics Division, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California 90095, USA 3 Greater Los Angeles Veterans Affairs Healthcare Center, Los Angeles, California 90073, USA

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Short Title: Paracentral Acute Middle Maculopathy in Vein Occlusion

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Key Words: central retinal vein occlusion, cilioretinal artery occlusion, optical coherence tomography, paracentral acute middle maculopathy, retinal ischemia Correspondence:

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David Sarraf, MD Retinal Disorders and Ophthalmic Genetics Division Jules Stein Eye Institute, University of California, Los Angeles 100 Stein Plaza Los Angeles CA 90095 Phone: (310) 794-9921 Fax: (310) 794-7904 Email: [email protected] Request for Reprints: same as correspondence

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Word Count (text): 3,047

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Abstract

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Purpose: To better characterize a novel spectral-domain optical coherence tomography (SD-OCT) presentation, termed paracentral acute middle maculopathy and to describe this finding in patients with nonischemic central retinal vein occlusion (CRVO), and to support a proposed pathogenesis of intermediate and deep retinal capillary ischemia. Design: Retrospective observational case series.

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Methods: Clinical histories, high-resolution digital color imaging, red-free photography, fluorescein angiography, near-infrared reflectance, and SD-OCT images of 484 patients with acute CRVO from two centers were evaluated for the presence of coexisting paracentral acute middle maculopathy.

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Results: Twenty-five (5.2%) of 484 patients diagnosed with CRVO demonstrated evidence of concurrent paracentral acute middle maculopathy. Patients averaged 51 years of age, and presented with complaints of paracentral scotomas. All patients demonstrated hyperreflective plaque-like lesions at the level of the inner nuclear layer with SD-OCT, and showed corresponding dark grey lesions on near-infrared reflectance and perivenular deep retinal whitening with color fundus photography. There was no fluorescein angiographic correlate to these lesions. On follow-up SD-OCT imaging, the lesions resolved into areas of inner nuclear layer atrophy with persistence of scotomas.

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Conclusions: Paracentral acute middle maculopathy refers to characteristic hyperreflective SD-OCT lesions involving the middle layers of the retina at the level of the inner nuclear layer that may develop in response to ischemia of the intermediate and deep capillary plexi. This series is the largest to describe this SD-OCT finding to date, and the first to associate it with nonischemic CRVO.

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Introduction Recently, we identified characteristic band-like hyperreflective spectral-domain optical coherence tomography (SD-OCT) lesions in patients with presumed acute macular neuroretinopathy involving the middle layers of the retina at the level of the inner nuclear layer, a novel finding we termed paracentral acute middle maculopathy.1, 2 Since these initial reports, a smaller case series additionally observed this finding in 4 patients, including one with underlying diabetic retinopathy.3 Retinal capillary ischemia has been proposed as the etiologic factor in the development of these lesions, which localize histologically at the level of the intermediate and deep retinal capillary plexuses flanking the inner and outer boundaries of the inner nuclear layer, respectively.1, 4

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This report will describe 25 new cases of paracentral acute middle maculopathy occurring in association with acute nonischemic central retinal vein occlusion (CRVO), and will help to further elucidate the underlying pathogenesis of these middle retinal lesions.

Methods Institutional review board approval for this retrospective two-center study was obtained through Wills Eye Hospital and the University of California, Los Angeles Office of the Human Research Protection Program. Research adhered to the tenets of the Declaration of Helsinki and was conducted in accordance with regulations set forth by the Health Insurance Portability and Accountability Act.

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Using ICD-9 billing codes for CRVO, two of our authors (ER, DS) reviewed the SD-OCT imaging database at each of their retinal care centers, and studied the findings from 484 cases of CRVO diagnosed between January 2010 and September 2013. We identified 25 cases of acute CRVO with simultaneous acute paracentral acute middle maculopathy lesions and assessed the clinical and imaging data for each case, primarily focusing on multimodal imaging correlations. Baseline and follow up demographic, clinical, and laboratory data were extracted from patient charts and analyzed. Statistical analysis was performed using a simple 2-tailed t-test using GraphPad Software (GraphPad, La Jolla, California).

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High-resolution digital color imaging, red-free photography, and fluorescein angiography at baseline presentation were analyzed, when available, for each patient. Initial nearinfrared reflectance and SD-OCT examinations utilizing the Heidelberg Spectralis (Heidelberg Engineering, Heidelberg, Germany) were evaluated at baseline in every patient and correlated with subsequent follow-up scans during the course of a patient’s follow-up when available. We used the registration feature specific to Heidelberg Spectralis, which allows each SD-OCT B-scan to be coupled to its exact location on the near-infrared reflectance image, facilitating point-to-point correlations of the retinal findings between the near-infrared reflectance and SD-OCT images.

Results

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Twenty-five (5.2%) of the 484 patients diagnosed with CRVO demonstrated evidence of concurrent paracentral acute middle maculopathy at the time of their initial office evaluation. Baseline patient demographics and ocular examination findings from these eyes are outlined in Table 1. The mean age at presentation was 51 years (range: 19 to 78, standard deviation (SD): 16.6) in the group with paracentral acute middle maculopathy, which was significantly younger than the 69.6 years (range: 20 to 95, SD: 13.9) in the remainder of the patients with CRVO (P

Paracentral acute middle maculopathy in nonischemic central retinal vein occlusion.

To better characterize a novel spectral-domain optical coherence tomography (OCT) presentation, termed paracentral acute middle maculopathy, to descri...
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