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Sudden Fixed Swirl Circles in the Vision of a Young Healthy Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 20s was referred by the optician to our acute and emergency department for suspected optic neuritis in the right eye. She reported a sudden onset of photopsia and visual disturbances described as “fixed swirl circles” in the central vision of both eyes noticed on awakening 5 days prior and persisting since that time. Her medical history included migraine and polycystic ovaries. She had a flulike episode 1 week prior to the onset of symptoms. The patient was taking oral contraceptives and denied the use of recreational drugs. Ocular movements were full but the patient reported pain and discomfort during movements of the right eye. Pupils were equal and reactive to light and there was no afferent pupillary defect. Best-corrected visual acuity was 20/20 OU; color vision was 17/17 OU on Ishishara plates. The anterior segment was unremarkable and intraocular pressure was 14 mm Hg in both eyes. Dilated fundus examination revealed a healthy optic disc and no obvious abnormalities at the macula of both eyes. Near-infrared reflectance imaging and spectral-domain optical coherence tomography (OCT) scans of the right eye are shown in the Figure.

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Acute macular neuroretinopathy (AMN)

B. Observe the patient

Sudden-onset paracentral scotoma, good visual acuity, and the presence of wedge-shaped paracentral hyporeflective lesions on infrared reflectance imaging associated with hyperreflectivity of the junction of the outer plexiform layer and the outer nuclear layer on OCT were consistent with AMN. Observation (choice B) was the appropriate next step for this case. Fluorescein angiography (choice A) would not be the preferred answer as the next step because AMN is typically not detected on fluorescein angiography. Magnetic resonance imaging of the brain (choice C) was not recommended as the next step because diagnosis of AMN per se does not require brain imaging unless there are symptoms and/or signs suggestive of central nervous system involvement. There is no proven treatment for AMN; thus, treatment with oral corticosteroids (choice D) was not indicated.

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Article Information

Corresponding Author: Giuseppe Casalino, MD, FEBO, Moorfields Eye Hospital NHS Foundation Trust, 162 City Road, London EC1V 2PD, United Kingdom (peppecasalino@gmail.com).

Published Online: February 7, 2019. doi:10.1001/jamaophthalmol.2018.5919

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Vaphiades  MS, Grondines  B.  Occult acute macular neuroretinopathy.  Neuroophthalmology. 2017;41(4):219-223. doi:10.1080/01658107.2017.1297999PubMedGoogle ScholarCrossref
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Agarwal  A. Chapter 11: acute macular neuroretinopathy.  Gass' Atlas of Macular Diseases. Vol. 2. 5th ed. Elsevier; 2012:994-997.
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Fawzi  AA, Pappuru  RR, Sarraf  D,  et al.  Acute macular neuroretinopathy: long-term insights revealed by multimodal imaging.  Retina. 2012;32(8):1500-1513. doi:10.1097/IAE.0b013e318263d0c3PubMedGoogle ScholarCrossref
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Munk  MR, Jampol  LM, Cunha Souza  E,  et al.  New associations of classic acute macular neuroretinopathy.  Br J Ophthalmol. 2016;100(3):389-394. doi:10.1136/bjophthalmol-2015-306845PubMedGoogle ScholarCrossref
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Thanos  A, Faia  LJ, Yonekawa  Y, Randhawa  S.  Optical coherence tomographic angiography in acute macular neuroretinopathy.  JAMA Ophthalmol. 2016;134(11):1310-1314. doi:10.1001/jamaophthalmol.2016.3513PubMedGoogle ScholarCrossref
8.
Lee  SY, Cheng  JL, Gehrs  KM,  et al.  Choroidal features of acute macular neuroretinopathy via optical coherence tomography angiography and correlation with serial multimodal imaging.  JAMA Ophthalmol. 2017;135(11):1177-1183. doi:10.1001/jamaophthalmol.2017.3790PubMedGoogle ScholarCrossref
9.
Casalino  G, Arrigo  A, Romano  F, Munk  MR, Bandello  F, Parodi  MB.  Acute macular neuroretinopathy: pathogenetic insights from optical coherence tomography angiography.  [published online May 29, 2018].  Br J Ophthalmol. 2018;bjophthalmol-2018-312197. doi:10.1136/bjophthalmol-2018-312197PubMedGoogle Scholar
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