A 30-year-old man presents with acute onset of unilateral optic disc edema with a macular star figure of exudate OD. The vision was 20/200 OD, there is right RAPD, and a cecocentral scotoma on visual field testing OD. There is mild vitreous cell OD. The ophthalmologist writes'“papilledema” on the chart and refers the patient to a neurologist for further testing. The neurologist orders a head MRI which is normal and performs a lumbar puncture that shows a normal opening pressure and cerebrospinal fluid analysis. The patient sees another ophthalmologist for a second opinion and the correct diagnosis of cat scratch neuroretinitis is made based upon a markedly elevated Bartonella henselae titer (IgG 1:1024).\nPapilledema for neuro-ophthalmologist represents a term that is best reserved for optic disc swelling due to elevated intracranial pressure. It is my recommendation that all other causes of optic disc swelling should be referred to by etiology (e.g., optic neuritis, or anterior ischemic optic neuropathy) or simply by describing the ophthalmoscopic finding of “optic disc edema” Clinicians should use the term “papilledema” carefully because it is potentially confusing to your colleagues in neurology, neurosurgery, and medicine if you misuse the term. This inappropriate charting could lead to inappropriate testing? (e.g., the MRI and lumbar puncture in this case). It also delays testing for the real culprit for the patient's optic disc edema and a macular star figure (i.e., infectious cat scratch neuroretinitis in this case). The misleading documentation could also lead to medicolegal implications of the missed diagnosis.\nIn general the clinician can use the clinical presentation to differentiate the three major forms of optic disc edema: papilledema, anterior ischemic optic neuropathy, and optic neuritis. Papilledema can occur at any age, is typically associated with relatively preserved central visual acuity (unless there is macular exudate, fluid, hemorrhage, or chronic or long standing papilledema), is bilateral (although markedly asymmetric or frankly unilateral cases do occur), and usually produces a big blind spot initially on visual field testing followed by nerve fiber layer loss over time. There might also be associated signs and symptoms of increased intracranial pressure (e.g., headache, nausea, vomiting, pulse synchronous tinnitus, or diplopia from a nonlocalizing sixth nerve palsy). In contrast to papilledema, optic neuritis typically affects younger patients, is often associated with decreased central acuity and dyschromatopsia, is typically unilateral (although bilateral cases can occur especially in children), and the visual field defect is usually a central scotoma (although any type of optic nerve related visual field loss may occur). Finally, in an older adult, anterior ischemic optic neuropathy is the most common unilateral cause for optic disc edema. The patient may have vasculopathic risk factors, an altitudinal visual field defect (but any optic nerve type of defect can occur), and again is usually unilateral. Thus the clinician should be wary of making the diagnosis of bilateral simultaneous optic disc edema related to either optic neuritis or non-artertic anterior ischemic optic neuropathy and probably should exclude papilledema in these clinical settings.\nThe evaluation of papilledema should include a check of the systemic blood pressure as malignant hypertension can mimic papilledema (i.e., hypertensive optic disc edema). Neuroimaging (preferably an MRI with contrast and an MR venogram to exclude venous sinus thrombosis) should be considered as the initial study in papilledema. If the neuroimaging study is normal then a lumbar puncture with an opening pressure and cerebrospinal fluid analysis should be performed. If the intracranial pressure is elevated, the neuorimaging is normal, and the CSF analysis is normal then the diagnosis of idiopathic intracranial hypertension can be confirmed but should be considered a diagnosis of exclusion. Although a CT scan might be the initial emergent neuroimaging study performed, there are many etiologies for increased ICP that may be missed on CT scan especially a noncontrast study in the emergency room. These intracranial etiologies include cerebral venous sinus occlusion, arteriovenous or dural malformation, infiltrative neoplasm or disorders that produce meningeal enhancement (e.g., inflammatory, infectious, infiltrative, or neoplastic etiologies).\nAlthough routine, typical, and medication responsive papilledema from idiopathic intracranial hypertension might be managed by a general ophthalmologist, a referral to neuro-ophthalmology might still be necessary if the patient fails maximum medical therapy or presents with acute, severe visual loss. These patients might require surgical intervention (e.g., shunting procedure or optic nerve sheath fenestration) to prevent irreversible papilledema related visual loss. In addition, the diagnosis should be made with caution in the following atypical circumstances: the patient is thin rather than obese, elderly or a child rather than a young adult, and male rather than female. Consultation is also recommended for patients with progressive visual loss despite treatment, signs or symptoms not due to increased intracranial pressure, or any inflammatory intraocular signs that might suggest an inflammatory rather than idiopathic etiology.
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.