A 55-year-old man with hypertension and diabetes presents with new awareness of blurred vision OD for two months. The visual acuity was 20/25 OD and 20/20 OS. Goldmann perimetry demonstrated an inferior altitudinal visual field loss OD. The pupil examination showed a relative afferent pupillary defect (RAPD) OD. Ophthalmoscopy showed a pale optic nerve OD. The eye exam was normal OS. A diagnosis of “optic atrophy” OD was made and the patient was instructed to return in 3 months for a repeat visual field. The patient returns in 3 months and the vision is now counting fingers OD. Magnetic resonance imaging of the head and orbit showed optic nerve sheath enhancement OD consistent with an optic nerve sheath meningioma.\nOptic atrophy is not a diagnosis. The clinician should document a cause for the optic atrophy and if no etiology is established a neuroimaging study and other evaluation should be performed. In a 55-year-old vasculopathic male the most likely etiology for a unilateral optic neuropathy with optic atrophy is non-arteritic anterior ischemic optic neuropathy (NA-AION). The “anterior” in NA-AION refers to optic disc edema and the absence of documented disc edema phase should prompt consideration for evaluation for alternative etiologies. In this case the patient experienced acute awareness of his visual loss OD rather than acute onset and the disc edema phase was not seen. Although serial visual field and follow up for a presumptive diagnosis of optic atrophy due to NA-AION is reasonable the clinical plan should be well documented in the chart, the patient should be informed of the possibility for a compressive or other etiology, and imaging and other evaluation should be pursued if the patient has progressive signs or symptoms (as in this case).\nLee et al. previously reported on the diagnostic yield for the evaluation of isolated and unexplained optic atrophy. This study was a retrospective review of all charts with the diagnosis of optic atrophy. Included patients were adults with isolated, but unexplained, optic atrophy and patients were excluded if they were children, had incomplete or inadequate documentation of the findings, had non-neurologically isolated optic atrophy (e.g., other localizing findings), or had a history (e.g., prior neuroimaging study showed a compressive lesion, prior ischemic optic neuropathy) or examination (e.g., central retinal artery occlusion) evidence for an etiology for the optic atrophy. A total of 1110 charts with the diagnosis of optic atrophy were reviewed from the two participating institutions (368 from the University of Cincinnati and 742 from the University of Iowa). Of these 1110 charts, 91 (8%) with isolated unexplained optic atrophy were included, and 1019 charts (92%) were excluded. Of the 91 cases included 18 (20%) had an underlying compressive lesion causing optic atrophy and 73 (80%) cases had no etiology for the optic atrophy on neuroimaging. Of the 18 patients with abnormal neuroimaging studies (e.g., meningioma, pituitary adenoma, or craniopharyngioma) 11 had bilateral and 7 had unilateral optic atrophy. Five of the 18 patients had progressive visual loss, 3 had hemianopic visual field loss, and 11 were younger than 50 years old. This study demonstrates that the risk of missing a compressive lesion in unexplained optic atrophy is high (20%) and that clinicians should not make the diagnosis of optic atrophy alone without an underlying proven or postulated mechanism.
A 14-year-old girl presents with headache, visual loss to counting fingers vision, and severe bilateral disc edema with macular star figure OU. The outside ophthalmologist makes the diagnosis of “bilateral neuroretinitis” due to cat scratch disease and sends the Bartonella titer. There is an incomitant esotropia and the secondary diagnosis of “sensory esotropia” is made. The cat scratch titer returns 2 weeks later but is completely negative. The patient is referred to the neuro-ophthalmology service where an MRI reveals a large intracranial tumor producing papilledema OU with significant leakage of exudate into the macula bilaterally.\nBilateral optic disc edema with a macular star figure (ODEMS) is NOT by itself diagnostic for infectious neuroretinitis. The ophthalmoscopic findings are not pathognemonic and papilledema can mimic the fundus appearance of ODEMS. It is my recommendation to consider neuroimaging for all bilateral optic disc edema cases.