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Macular Star, Optic Disc Swelling, and Lethargy in a Child

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

A 13-year-old girl with a 7-day history of painless vision loss and central scotoma in her left eye was referred to the department of ophthalmology. Three weeks prior, she had presented with mild fever and flulike symptoms, associated with a severe frontal headache that was mildly relieved by analgesics. Her medical history was positive for relapsing urinary tract infections since early infancy without adequate follow-up. Findings of an ophthalmological examination 1 year prior were reported as normal.

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A 13-year-old girl with a 7-day history of painless vision loss and central scotoma in her left eye was referred to the department of ophthalmology. Three weeks prior, she had presented with mild fever and flulike symptoms, associated with a severe frontal headache that was mildly relieved by analgesics. Her medical history was positive for relapsing urinary tract infections since early infancy without adequate follow-up. Findings of an ophthalmological examination 1 year prior were reported as normal.

At initial examination, she was conscious but somewhat lethargic. Her best-corrected visual acuity was 20/20 OD and 20/80 OS. Pupillary light responses, extrinsic ocular motility, anterior segment biomicroscopy, and intraocular pressure were normal in both eyes. Dilated fundus examination revealed a bilateral sectorial macular star that was more extended in the left eye, with retinal veins slightly dilated and tortuous, attenuated arterioles, some juxta and peripapillary nerve fiber layer infarcts, and optic disc edema with marked papillary telangiectasia; in addition, some faint, small, tan-yellow dots were observed at the level of the retinal pigment epithelium in the posterior pole, some of them already grayish and with a hypopigmented halo (Figure 1). Optical coherence tomography displayed elevated optic discs and hyperreflective material in the outer plexiform layer temporal to the optic disc in both eyes and a macular neurosensory detachment in the left eye. Results of visual field testing were normal in the right eye and showed a central scotoma in the left eye.

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Article Information

Corresponding Author: Julio A. Urrets-Zavalia, MD, PhD, Department of Ophthalmology, University Clinic Reina Fabiola, Universidad Catolica de Cordoba, Jacinto Rios 554, Piso 7, 5000 Cordoba, Argentina (julioaurrets@gmail.com).

Published Online: September 21, 2023. doi:10.1001/jamaophthalmol.2023.4216

Conflict of Interest Disclosures: None reported.

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

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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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