Diagnostic Errors in Initial Misdiagnosis of Optic Nerve Sheath Meningiomas | Neuro-ophthalmology | JAMA Neurology | JAMA Network
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Original Investigation
December 17, 2018

Diagnostic Errors in Initial Misdiagnosis of Optic Nerve Sheath Meningiomas

Author Affiliations
  • 1Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia
  • 2Department of Neurology, Emory University School of Medicine, Atlanta, Georgia
  • 3Department of Epidemiology, Emory School of Public Health, Atlanta, Georgia
  • 4Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia
  • 5Department of Neurological Surgery, Emory University School of Medicine, Atlanta, Georgia
JAMA Neurol. 2019;76(3):326-332. doi:10.1001/jamaneurol.2018.3989
Key Points

Question  What is the source of errors in the initial misdiagnosis of optic nerve sheath meningiomas?

Findings  In this medical record review that included 35 patients with optic nerve sheath meningiomas, 25 initially received a misdiagnosis that resulted in diagnostic delay, most frequently due to clinician assessment failures and errors in diagnostic testing.

Meaning  Education regarding diagnostic strategies and proper neuroimaging for optic neuropathies may prevent vision loss from the initial misdiagnosis of optic nerve sheath meningiomas.


Importance  Diagnostic errors can lead to the initial misdiagnosis of optic nerve sheath meningiomas (ONSM), which can lead to vision loss.

Objective  To identify factors contributing to the initial misdiagnosis of ONSM.

Design, Setting, and Participants  We retrospectively reviewed 35 of 39 patients with unilateral ONSM (89.7%) who were seen in the tertiary neuro-ophthalmology practice at Emory University School of Medicine between January 2002 and March 2017. The Diagnosis Error Evaluation and Research taxonomy tool was applied to cases with missed/delayed diagnoses.

Exposures  Evaluation in a neuro-ophthalmology clinic.

Main Outcomes and Measures  Identifying the cause of diagnostic errors for patients who initially received a misdiagnosis who were found to have ONSM.

Results  Of 35 patients with unilateral ONSM (30 women [85.7%]; mean [SD] age, 45.26 [15.73] years), 25 (71%) had a diagnosis delayed for a mean (SD) of 62.60 (89.26) months. The most common diagnostic error (19 of 25 [76%]) was clinician assessment failure (errors in hypothesis generation and weighing), followed by errors in diagnostic testing (15 of 25 [60%]). The most common initial misdiagnosis was optic neuritis (12 of 25 [48%]), followed by the failure to recognize optic neuropathy in patients with ocular disorders. Five patients who received a misdiagnosis (20%) underwent unnecessary lumbar puncture, 12 patients (48%) unnecessary laboratory tests, and 6 patients (24%) unnecessary steroid treatment. Among the 16 patients who initially received a misdiagnosis that was later correctly diagnosed at our institution, 11 (68.8%) had prior magnetic resonance imaging (MRI) results that were read as healthy; 5 (45.5%) showed ONSM but were misread by a non-neuroradiologist and 6 (54.5%) were performed incorrectly (no orbital sequence or contrast). Sixteen of the 25 patients (64%) had a poor visual outcome.

Conclusions and Relevance  Biased preestablished diagnoses, inaccurate funduscopic examinations, a failure to order the correct test (MRI brain/orbits with contrast), and a failure to correctly interpret MRI results were the most common sources of diagnostic errors and delayed diagnosis with worse visual outcomes and increased cost (more visits and tests). Easier access to neuro-ophthalmologists, improved diagnostic strategies, and education regarding neuroimaging should help prevent diagnostic errors.