A 65-year-old woman with hypertension and diabetes presents with new awareness of blurred vision OD for two days. The visual acuity was 20/40 OD and 20/20 OS. No visual field is performed. The pupil examination showed a relative afferent pupillary defect (RAPD) OD. Ophthalmoscopy showed a normal optic nerve OD. The remainder of the eye exam was normal OS. A diagnosis of “posterior ischemic optic neuropathy” (PION) is made and the patient is instructed to return for a “next available” visual field test. The patient returns two months later and reports that “his vision is worse”. The vision is now 20/400 OD and 20/25 OS. There is a right RAPD. The Goldmann visual field showed a bitemporal hemianopic visual field loss with a superimposed central loss OD. There is now optic atrophy OD. A cranial MRI showed a pituitary adenoma compressing the optic nerve OD. A new eye doctor sees the patient and tells her that “If only you'd come to me sooner you would not be blind.”\nThe diagnosis of posterior ischemic optic neuropathy (PION) is one of exclusion. PION does occur and can be classified as arteritic (i.e., related to giant cell arteritis) or non-arteritic. The non-arteritic form is generally seen in the setting of perioperative or post-operative ischemic optic neuropathy after spine or cardiovascular surgery. Post-operative PION however has been seen after multiple surgical procedures.\nSadda et al. reported a retrospective chart review of 72 patients (98 eyes) with a clinical diagnosis of PION: perioperative (28 patients), arteritic (6 patients), and nonarteritic systemic vascular disease (38 patients). Among eyes with nonarteritic PION, 34% experienced improvement in vision, 28% remained stable, and 38% worsened.\nIt is my opinion that before the diagnosis of PION can be made two conditions must be excluded. First giant cell arteritis and second a compressive lesion producing a retrobulbar optic neuropathy. The key feature differentiating AION from PION is the presence of optic disc edema in the acute setting. Retrobulbar optic neuritis is typically a disorder of young patients and most commonly associated with multiple sclerosis. Retrobulbar optic neuritis however is rare in the elderly and the diagnosis should not be made without an imaging study. Compressive lesions can mimic an acute onset as seen in ischemic disease. In addition, corticosteroids a common treatment of retrobulbar optic neuritis can be disastrous in a patient harboring a fungal orbital apex lesion producing the retrobulbar optic neuropathy. Thus the diagnosis of retrobulbar “optic neuritis” should be made with extreme caution in the elderly. I generally recommend that a fluorescein angiogram be considered in elderly patients with suspected PION. The finding of choroidal perfusion deficit in the setting of PION is strongly suggestive of giant cell arteritis. Corticosteroids might be useful empirically for both arteritic and nonarteritic PION but an imaging study as mentioned above might be advisable prior to starting treatment.\nI would accept the diagnosis of PION of the clinical findings are consistent with an optic neuropathy (i.e., visual loss, RAPD, optic neuropathy related visual field), there is a clear etiology (i.e., post-surgical PION or giant cell arteritis), and optic atrophy develops over time. If there is no clear etiology for the PION, then a neuroimaging study is recommended (preferably cranial MRI with contrast and orbital fat suppressed views).
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.