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An Atypical Case of Optic Neuropathy

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

A 17-year-old previously healthy girl presented with 3 months of decreased vision in the right eye associated with throbbing pain with eye movement. The patient had no history of weakness, incontinence, or severe headaches. Initial presentation was notable for hand motion visual acuity with a 2+ relative afferent pupillary defect on the right; fundus examination revealed grade IV disc edema with obscuration of all major vessels on the disc (Figure, A). A magnetic resonance image (MRI) of the brain with and without gadolinium contrast was degraded by motion and orthodontic artifact but was suggestive of optic nerve enhancement consistent with optic neuritis. Results of serology tests and cerebral spinal fluid analysis were negative for infectious and inflammatory etiologies, including syphilis, sarcoidosis, Lyme disease, viral etiologies, multiple sclerosis, and lupus.

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Pilocytic astrocytoma with anaplastic transformation

A. Transorbital biopsy of the right optic nerve for grading and immunohistochemistry

The patient’s progression was more rapid than would be expected with a typical optic pathway glioma, as optic nerve gliomas do not tend to grow as rapidly. Given the rate of aggressive progression and risk of vision loss to the left eye, the best decision is to proceed with biopsy of the right optic nerve. Complete surgical excision was not possible because of the involvement of the tumor at the chiasm; the biopsy was to evaluate the grade and immunohistochemistry of the tumor prior to starting radiation. Performing the biopsy via a transorbital (choice A) rather than transcranial route would allow radiation to start more expeditiously. Transcranial biopsy (choice B) is not preferred because of longer recovery and potential delay of radiotherapy. Chemotherapy and/or radiation prior to knowing the grade of the tumor (choice C) would not be preferred. Observation of a tumor with chiasmal involvement places the patient at significant risk for visual loss on the left side (choice D). Tumor at the chiasm, described as “masslike expansion,” would potentially threaten the entering fibers from the left optic nerve.

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Article Information

Corresponding Author: Katherine J. Williams, MD, Department of Ophthalmology, Baylor College of Medicine/Cullen Eye Institute, 1977 Butler Blvd, 2nd Floor, Houston, TX 70030 (katherine.williams@bcm.edu).

Published Online: November 18, 2021. doi:10.1001/jamaophthalmol.2021.1466

Conflict of Interest Disclosures: Dr Allen has received personal fees from Horizon Pharma. No other disclosures were reported.

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

References
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Azad  A , Deb  S , Cher  L .  Primary anaplastic pilocytic astrocytoma.   J Clin Neurosci. 2009;16(12):1704-1706. doi:10.1016/j.jocn.2009.04.012PubMedGoogle ScholarCrossref
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Rodriguez  FJ , Scheithauer  BW , Burger  PC , Jenkins  S , Giannini  C .  Anaplasia in pilocytic astrocytoma predicts aggressive behavior.   Am J Surg Pathol. 2010;34(2):147-160. doi:10.1097/PAS.0b013e3181c75238PubMedGoogle ScholarCrossref
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Fried  I , Tabori  U , Tihan  T , Reginald  A , Bouffet  E .  Optic pathway gliomas: a review.   CNS Oncol. 2013;2(2):143-159. doi:10.2217/cns.12.47PubMedGoogle ScholarCrossref
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Yong  EX , McKelvie  P , Murphy  M , Wang  YY .  Anaplastic pilocytic astrocytoma.   J Clin Neurosci. 2014;21(11):1993-1996. doi:10.1016/j.jocn.2014.02.014PubMedGoogle ScholarCrossref
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Peters  KB , Cummings  TJ , Gururangan  S .  Transformation of juvenile pilocytic astrocytoma to anaplastic pilocytic astrocytoma in patients with neurofibromatosis type I.   J Pediatr Hematol Oncol. 2011;33(5):e198-e201. doi:10.1097/MPH.0b013e318205e230PubMedGoogle ScholarCrossref
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Ishii  N , Sawamura  Y , Tada  M ,  et al.  Absence of p53 gene mutations in a tumor panel representative of pilocytic astrocytoma diversity using a p53 functional assay.   Int J Cancer. 1998;76(6):797-800. doi:10.1002/(SICI)1097-0215(19980610)76:6<797::AID-IJC5>3.0.CO;2-TPubMedGoogle ScholarCrossref
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Apanisile  I , Karosi  T .  Surgical management of pilocytic astrocytoma of the optic nerve: a case report and review of the literature.   Case Rep Oncol Med. 2017;2017:4283570. doi:10.1155/2017/4283570 PubMedGoogle Scholar
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 CME points in the American Board of Surgery’s (ABS) Continuing 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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