Researchers Identify Factors Associated With Misdiagnosis of Optic Nerve Sheath Meningiomas

Unnecessary laboratory tests, steroid treatment, and lumbar puncture were noted in 48%, 24%, and 20% of patients, respectively.

A study has identified factors commonly associated with the initial misdiagnosis of optic nerve sheath meningiomas (ONSM), including biased preestablished diagnoses, erroneous funduscopic assessments, failure to order correct tests, and failure to interpret magnetic resonance imaging (MRI) results correctly. The findings were published in JAMA Neurology.

Researchers from the Emory School of Medicine retrospectively reviewed patient cases of unilateral ONSM recorded at 1 neuro-ophthalmology clinic of a tertiary healthcare institution. All cases with missed/delayed diagnoses had the Diagnosis Error Evaluation and Research taxonomy tool applied, and all patient cases were characterized according to the type and location of diagnostic error. The primary outcome of the study was the identification of factors influencing the misdiagnosis of ONSM in patients subsequently found to have meningiomas.

A total of 35 patients with unilateral ONSM (mean [SD] age, 45.26 [15.73] years) were included in the final analysis. Approximately 71% (n=25) of patients had an ONSM diagnostic delay of a mean (SD) of 62.60 (89.26) months. Clinician assessment failure was found to be the most common diagnostic error (76%), followed by diagnostic testing errors (60%). Optic neuritis was the most common initial misdiagnosis in roughly half (48%) of cases.

Unnecessary laboratory tests, unnecessary steroid treatment, and unnecessary lumbar puncture were noted in 48%, 24%, and 20% of patients, respectively. In addition, the majority of patients who were initially misdiagnosed and were correctly diagnosed later had prior MRI results recorded as healthy. In 45.5% of patients, MRI results were misread by a nonneuroradiologist, and 54.5% of misdiagnosed patients had MRIs that were performed incorrectly.

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Limitations of the study include its retrospective nature, as well as the small number of patients enrolled from a single center.

“Education regarding diagnostic strategies and proper neuroimaging for optic neuropathies may prevent vision loss from the initial misdiagnosis of optic nerve sheath meningiomas,” the researchers concluded.


Kahraman-Koytak P, Bruce BB, Peragallo JH, Newman NJ, Biousse V. Diagnostic errors in initial misdiagnosis of optic nerve sheath meningiomas [published online December 17, 2018]. JAMA Neurol. doi: 10.1001/jamaneurol.2018.3989