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Optic Disc Edema with a Macular Star
Andrew G. Lee, MD
Optic disc edema with a macular star (ODEMS) is a descriptive term for the ophthalmoscopic findings seen in a heterogeneous group of disorders. The clinical findings include swelling of the optic disc head, hard macular exudates in a partial or complete star pattern, and, in infectious or inflammatory etiologies, vitreous cells.
Because macular exudates typically arise from the optic nerve and not from inflammation (i.e., retinitis) of the macula, I prefer the descriptive term ODEMS at initial evaluation and reserve the term "neuroretinitis" for ODEMS proven to be due to an idiopathic or infectious etiology.
Patients with infectious or idiopathic ODEMS are often children or young adults. The idiopathic form has been referred to in the literature as Leber's idiopathic stellate neuroretinitis. No gender predilection exists. The majority of cases are unilateral; however, bilateral involvement has been reported in up to one-third of patients.
Nonspecific prodromal viral or flu-like symptoms may precede the visual loss in up to one-half of patients. Patients typically present with painless, central loss of vision. Retrobulbar pain, which is pain on eye movement, or associated headache may occur. Usually, no other neurologic signs or symptoms are present.
Visual acuity is variable and may range from 20/20 to light perception. Loss of color vision is often an accompanying feature. Typically, visual field testing reveals a central or cecocentral scotoma, but other nerve fiber layer field defects may occur (e.g., arcuate, altitudinal defects or generalized constriction). A relative afferent pupillary defect is present in unilateral or bilateral but asymmetric cases.
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Typically, the optic disc is markedly swollen with secondary leakage of subretinal fluid into the outer plexiform layer of the retina. After the serous fluid in Henle's layer is reabsorbed, a lipid precipitate produces the partial or complete star figure of hard macular exudates (Slide 1).
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Although the macular star figure may be present at the onset of symptoms, it may appear later in the course of the disease (e.g., 1 to 2 weeks after the acute optic disc edema). In some patients, the macular star figure may appear after the optic disc swelling is resolving or has resolved (Slide 2).
Anterior segment inflammation and vitreous cells may be present in the infectious etiologies of ODEMS. Associated chorioretinitis, inflammatory sheathing of the peripapillary vessels, scleritis or central or branch retinal artery occlusions should suggest an associated infectious or inflammatory (e.g., vasculitis) etiology for the ODEMS.
Although many cases of ODEMS are idiopathic (i.e., Leber's stellate neuroretinitis), benign, and self-limited, a number of infectious agents and inflammatory diseases have been reported to cause ODEMS. Table 1 lists the potential infectious etiologies for ODEMS. In my experience, cat scratch disease, Lyme disease, syphilis, and toxoplasmosis are the most common causes of infectious ODEMS. Also, tuberculosis is an uncommon but treatable etiology.
The patient history should focus on risk factors for these infectious agents including recent travel history, tick bite (Lyme disease), consumption of unpasteurized or uncooked foods (toxoplasmosis), sexually transmitted disease (syphilis), tuberculosis exposure, and pet contacts (cat scratch fever). Physical examination evidence for these infectious etiologies may reveal an associated rash, fever, or lymphadenopathy.
A complete ocular examination should be performed in all patients with ODEMS. The clinician should perform a careful slit lamp biomicroscopy examination of the anterior and posterior segment and specific comment should be made for vitreous cells. Formal perimetry is helpful in documenting the visual field loss.
Patients with acute optic disc swelling with a normal macula at onset should be re-examined within several weeks to rule out the development of the macular star figure. In the Optic Neuritis Treatment Trial, the appearance of the macular star figure was less likely to be associated with multiple sclerosis. The typical optic disc appearance in demyelinating optic neuritis is either a normal optic nerve (i.e., retrobulbar optic neuropathy) or mild hyperemia or disc edema (i.e., papillitis).
Fluorescein angiography shows fluorescein leakage from the optic disc in the mid to late phases and abnormal permeability of the deep capillaries in the optic nerve head. No perifoveal leakage occurs in the macula. I do not typically perform a fluorescein angiogram unless suspicion for vasculitis or an arterial occlusion is present.
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The evaluation of ODEMS should be directed by the history and clinical examination. Vasculopathic and older aged patients with unilateral ODEMS (especially without constitutional features suggesting infection and lacking vitreous cells) are more likely to have non-arteritic anterior ischemic optic neuropathy than infectious neuroretinitis. Toxoplasmosis typically presents with an adjacent peripapillary chorioretinitis (Slide 3) or old chorioretinal scar and more florid vitreous inflammation rather than the optic disc edema with a macular star.
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Typically, I do not perform a neuroimaging study (e.g., magnetic resonance imaging [MRI]) for unilateral ODEMS. Patients with atypical or bilateral ODEMS should undergo a cranial and orbit MRI with contrast and fat suppression to exclude papilledema or enhancement of the optic nerve. Measurement of systemic blood pressure is recommended to rule out malignant hypertension with optic disc edema (Slide 4).
Verm and Lee reported a case of papilledema due to an intracranial arteriovenous malformation that was misdiagnosed as "neuroretinitis" because of the presence of the macular star figure. It cannot be over-emphasized that ODEMS is an ophthalmoscopic description of a finding and not by itself a diagnosis. As stated above, atypical features should prompt neuroimaging.
I order a cat scratch titer (Bartonella henselae) in patients with ODEMS regardless of the presence or absence of cat exposure, fever, or lymphadenopathy. A syphilis serology, a chest radiograph, and, in patients with a suggestive history (e.g., tick bite, rash, travel to endemic area), a Lyme titer are reasonable additional tests. A complete blood count and erythrocyte sedimentation rate may be useful for acute infectious etiologies.
Toxoplasmosis titer is performed for patients with evidence for chorioretinitis. I do not perform a lumbar puncture, but I will proceed with cerebrospinal fluid analysis for bilateral cases especially if other signs or symptoms of increased intracranial pressure are present.
ODEMS due to infectious etiologies should be differentiated from other mimics of infectious neuroretinitis (e.g., anterior ischemic optic neuropathy, posterior hyaloid detachment, branch or central retinal artery occlusion, hypertension, diabetes, polyarteritis nodosa, inflammatory bowel disease, and Eales' disease).
Bilateral cases, especially those presenting with signs of increased intracranial pressure or with an enlarged blind spot rather than a visual field suggestive of an optic neuropathy should be considered to be papilledema until proven otherwise. Vasculopathic patients with unilateral ODEMS are more likely to have anterior ischemic optic neuropathy than infectious neuroretinitis especially in absence of vitreous cells.
The treatment of ODEMS should be directed at the underlying etiology. Infectious etiologies of ODEMS (i.e., neuroretinitis) should be treated with appropriate antibiotic therapy and consultation with an infectious disease specialist may be helpful as antibiotic regimens change from year to year.
Patients with non-infectious etiologies of ODEMS (e.g., anterior ischemic optic neuropathy, papilledema, hypertension) should have therapy directed at the underlying causes. No proven treatment for idiopathic (i.e., Leber's stellate neuroretinitis) ODEMS is available.
Corticosteroids have been used anecdotally, but no proof of efficacy exists and the natural history of ODEMS is typically recovery of vision. Aggressive immunosuppressive agents may be considered in the rare recurrent cases of ODEMS especially with persistent or progressive visual loss.
Post-infectious (i.e., neuroretinitis) or idiopathic (i.e., Leber's stellate neuroretinitis) ODEMS is usually a benign condition that resolves spontaneously with or without treatment. The optic disc edema and the macular star figure tend to resolve slowly over several weeks to months. Residual exudates and the macular star, however, may persist for up to 1 year. Optic atrophy and macular retinal pigment epithelium changes may occur over time.
Most patients have complete visual recovery, but some have significant residual visual disability depending on the degree of macular retinal pigment epithelial change and optic atrophy. Recurrences in the same or fellow eye are uncommon but have been described in idiopathic as well as infectious cases, especially reactivation of toxoplasmosis.
Purvin and Chioran reported recurrent ODEMS in patients who experienced from two to seven attacks of ODEMS at intervals ranging from 1 to 10 years. Patients with this recurrent form may not experience improvement and may require more aggressive immunosuppression to prevent further attacks.
Although optic neuritis is a risk factor in the development of multiple sclerosis, ODEMS is believed to be less likely to be associated with demyelinating disease. In the Optic Neuritis Treatment Trial (ONTT), multiple sclerosis was less likely to occur in patients who had swollen discs and retinal exudates than in patients who had swollen discs but did not have retinal exudates. Williams and Johnson reported three of 35 patients with neuroretinitis who were diagnosed with multiple sclerosis.
In summary, ODEMS is an ophthalmoscopic description of findings that may be due to a number of underlying etiologies. In typical patients with "neuroretinitis" the evaluation should focus on infectious etiologies. Atypical patients should undergo neuroimaging and further evaluation as needed. Older aged patients with vasculopathy may have non-arteritic anterior ischemic optic neuropathy, especially if other signs of intraocular inflammation (e.g. anterior or posterior cells) are absent. The presence or development of the star figure of exudates makes it less likely to that the optic neuropathy is associated with demyelinating disease.
Akura J, Ikeda T, Sato K, Ikeda N. Macular star associated with posterior hyaloid detachment. Acta Ophthalmol Scand. 2001;79:317-318.
Bafna S, Lee AG. Bilateral optic disc edema and multifocal retinal lesions without loss of vision in cat scratch disease. Arch Ophthalmol. 1996;114:1016-1017.
Bar S, Segal M, Shapira R, Savir H. Neuroretinitis associated with cat scratch disease. Am J Ophthalmol. 1990;110:703-705.
Bhatti MT, Asif R, Bhatti LB. Macular star in neuroretinitis. Arch Neurology. 2001;58:1008-1009.
Brazis PW, Lee AG. Optic disk edema with a macular star. Mayo Clin Proc. 1996;71:1162-1166.
Brazis PW, Stokes HR, Ervin FR. Optic neuritis in cat scratch disease. J Clin Neuro-ophthalmol. 1986;6:172-174.
Carithers HA, Margileth AM. Cat-scratch disease. Acute encephalopathy other neurologic manifestations. Am J Dis Child. 1991;145:98-101.
Chrousos GA, Drack AV, Young M, et al. Neuroretinitis in cat scratch disease. J Clin Neuroophthalmol. 1990;10:92-94.
Cohen SM, Davis JL, Gass DM. Branch retinal arterial occlusions in multifocal retinitis with optic nerve edema. Arch Ophthalmol. 1995;113:1271-1276.
Conrad DA. Treatment of cat-scratch disease. Curr Opin Ped. 2001;13:56-59.
Cunningham ET, Koehler JE. Perspective: ocular bartonellosis. Am J Ophthalmol. 2000;130:340-349.
Cunningham ET Jr, Schatz H, McDonald HR, Johnson RN. Acute multifocal retinitis. Am J Ophthalmol. 1997;123:347-357.
Cunningham ET Jr, McDonald HR, Schatz H, et al. Inflammatory mass of the optic nerve head associated with systemic Bartonella henselae infection. Arch Ophthalmol. 1997;115:1596-1597.
Dhar MY, Goel JL, Sota LD. Optic neuroretinitis, a rare manifestation of herpes zoster ophthalmicus: a case report. J Communicable Diseases. 1997;29:57-61.
Earhart KC, Power MH. Bartonella neuroretinitis. New Eng J Med. 2000;343:1459.
Fish RH, Hogan RN, Nightingale SD, Anand R. Peripapillary angiomatosis associated with cat-scratch neuroretinitis. Arch Ophthalmol. 1992;110:323.
Fish RH, Hoskins JC, Kline LB. Toxoplasmosis neuroretinitis. Ophthalmology. 1993;100:1177-1182.
Foster RE, Lowder CY, Meisler DM, et al. Mumps neuroretinitis in an adolescent. Am J Ophthalmol. 1990;110:91-93.
Friedrich Y, Feiner M, Gawi H, Friedman Z. Diabetic papillopathy with macular star mimicking clinically significant diabetic macular edema. Retina. 2001;21:80-82.
Garcia-Arumi J, Salvador F, Corcostegui B, Mateo C. Neuroretinitis associated with melanocytoma of the optic disk. Retina. 1994;14:173-176.
Gass JDM. Diseases of the optic nerve that may simulate macular disease. Trans Am Acad Ophthalmol Otolaryngol. 1977;83:763-770.
Gass JDM, Harbin TS Jr, Del Piero EJ. Exudative stellate neuroretinopathy and Coat's syndrome in patients with progressive hemifacial atrophy. Eur J Ophthalmol. 1991;1:2-10.
Ghauri RR, Lee AG. Optic disk edema with a macular star. Surv Ophthalmology. 1998;43:270-274.
Golnik KC, Marotto ME, Fanous MM, et al. Ophthalmic manifestations of Rochalimaea species. Am J Ophthalmol. 1994;118:145-151.
Gray AV, Reed JB, Wendel RT, Morse LS. Bartonella henselae infection associated with peripapillary angioma, branch retinal artery occlusion, and severe visual loss. Am J Ophthalmol. 1999;127:223-224.
Halperin LS. Neuroretinitis due to seronegative syphilis associated with human immunodeficiency virus. J Clin Neuro-ophthalmol. 1992;12:171-172.
Karma A, Seppala I, Mikkila H, et al. Diagnosis and clinical characteristics of ocular Lyme borreliosis. Am J Ophthalmol. 1995;119:127-135.
Kerkoff FT, Bergmans AM, van Der Zee A, Rothova A. Demonstration of Bartonella grahamii DNA in ocular fluids of a patient with neuroretinitis. J Clin Microbiol. 1999;37:4034-4038.
King MH, Cartwright MJ, Carney MD. Leber's idiopathic stellate neuroretinitis. Ann Ophthalmol. 1991;23:58-60.
Kodama T, Masuda H, Ohira A. Neuroretinitis associated with cat-scratch disease in Japanese patients. Acta Ophthalmol Scand. 2003;81:653-657.
Kosmorsky GS, Prayson R. Primary optic pathway sarcoidosis in a 38-year-old white man. J Neuro-ophthalmol. 1995;16:188-190
Leavitt JA, Pruthi S, Morgenstern BZ. Hypertensive retinopathy mimicking neuroretinitis in a twelve-year-old girl. Surv Ophthalmol. 1997;41:477-480.
Lee AG, Beaver HA. Acute bilateral optic disk edema with a macular star figure in a 12-year-old girl. Surv Ophthalmol. 2002;47:42-49.
Lee AG, Brazis PW. Poor visual outcome following optic disc edema with a macular star (neuroretinitis). J Neuro-Ophthalmology. 1998;19:57-61.
Lee AG, Orenga-Nania SD, Brazis PW, Lech EM. Poor visual outcome following optic disc edema with a macular star ("neuroretinitis"). Neuro-Ophthalmology. 1998;19:57-61.
Lee WR, Chawla JC, Reid R. Bacillary angiomatosis of the conjunctiva. Am J Ophthalmol. 1994;118:152-157.
Lesser RL, Kornmehl EW, Pachner AR, et al. Neuro-ophthalmologic manifestations of Lyme disease. Ophthalmology. 1990;97:699-706.
Matsuda A, Chin S, Ohashi T. A case of neuroretinitis associated with long-standing polyarteritis nodosa. Ophthalmologica. 1994;208:168-171.
May EF, Levi L, Ng JD, Truxal AR. Rochalimaea neuroretinitis and retinal vasculitis. Presented at: the Meeting of the North American Neuro-Ophthalmology Society. 1995; Tucson, AZ.
McCleish WM, Pulido JS, Holland S, et al. The ocular manifestations of syphilis in the human immunodeficiency virus type 1-infected host. Ophthalmology. 1990;97:196-203.
McCrary B, Cockerhan W, Pierce P. Neuroretinitis in cat-scratch disease associated with the macular star. Ped Infect Dis J. 1994;13:938-939.
McCrary B, Cockerham W, Pierce P. Neuroretinitis in cat scratch disease associated with macular star. J Mississippi State Med Assoc. 1997;38:158-159.
Moreno RJ, Weisman J, Waller S. Neuroretinitis: an unusual presentation of ocular toxoplasmosis. Ann Ophthalmol. 1992;24:68-70.
Ninomiya H, Hamada T, Akiya S, Kazama H. Three cases of acute syphilitic neuroretinitis. Folia Ophthalmol Jpn. 1990;41:2088-2094.
O'Halloran HS, Draud K, Minix M, et al. Leber's neuroretinitis in a patient with serologic evidence of Bartonella elizabethae. Retina. 1998;18:276-278.
Optic Neuritis Study Group. The 5-year risk of MS after optic neuritis. Experience of the optic neuritis treatment trial. Neurology. 1997;49:1404-1413.
Optic Neuritis Study Group. High- and low-risk profiles for the development of multiple sclerosis within 10 years after optic neuritis: experience of the optic neuritis treatment trial. Arch Ophthalmol. 2003;121:944-949.
Ormerod LD, Dailey JP. Ocular manifestations of cat-scratch disease. Curr Opin Ophthalmol. 1999;10:209-216.
Ormerod LD, Skolnick KA, Menosky MM, et al. Retinal and choroidal manifestations of cat-scratch disease. Ophthalmology. 1998;105:1024-1031.
Perrotta S, Nobili B, Grassia C, et al. Bilateral neuroretinitis in a 6-year-old boy with acquired toxoplasmosis. Arch Ophthalmol. 2003;121:1493-1496.
Purvin VA, Chioran G. Recurrent neuroretinitis. Arch Ophthalmol. 1994;112:365-371.
Ray S, Gragoudas E. Neuroretinitis. Int Ophthalmol Clin. 2001;41:83-102.
Reed JB, Scales DK, Wong MT, et al. Bartonella henselae neuroretinitis in cat scratch disease. Diagnosis, management, and sequelae. Ophthalmology. 1998;105:459-466.
Rosen BS, Barry CJ, Nicoll AM, Constable IJ. Conservative management of documented neuroretinitis in cat scratch disease associated with Bartonella henselae. Australian New Zealand J Ophthalmol. 1999;27:153-156.
Schwartzman WA, Patnaik M, Barka NE, et al. Rochalimaea antibodies in HIV associated neurologic disease. Neurology. 1994;44:1312-1316.
Schwartzman WA, Patnaik M, Angula FJ, et al. Bartonella (Rochalimaea) antibodies, dementia, and cat ownership among men infected with immunodeficiency virus. Clin Infect Dis. 1995;21:954-959.
Soheilian M, Markomichelakis N, Foster CS. Intermediate uveitis and retinal vasculitis as manifestations of cat scratch disease. Am J Ophthalmol. 1996;122:582-584.
Solley WA, Martin DF, Newman NJ, et al. Cat scratch disease. Posterior segment manifestations. Ophthalmology. 1999;106:1546-1553.
Stechschulte SU, Kim RY, Cunningham ET. Tuberculous neuroretinitis. J Neuro-Ophthalmology. 1999;19:201-204.
Suhler EB, Lauer AK, Rosenbaum JT. Prevalence of serologic evidence of cat scratch disease in patients with neuroretinitis. Ophthalmology. 2000;107:871-876.
Thompson PK, Vaphiades MS, Sacccente M. Cat-scratch disease presenting as neuroretinitis and peripheral facial palsy. J Neuro-Ophthalmology. 1999;19:240-241.
Ulrich GG, Waecker NJ Jr, Meister SJ, et al. Cat scratch disease associated with neuroretinitis in a 6-year-old girl. Ophthalmology. 1992;99:246-249.
Vaphiades MS. Rocky Mountain Spotted Fever as a cause of macular star figure. J Neuroophthalmol. 2003;23:276-278.
Verm A, Lee AG. Bilateral optic disk edema with macular exudates as the manifesting sign of a cerebral arteriovenous malformation. Am J Ophthalmol. 1997;123:422-424.
Wade NK, Po S, Wong IG, Cunningham ET Jr. Bilateral Bartonella-associated neuroretinitis. Retina. 1999;19:355-356.
Wade NK, Levi L, Jones MR, et al. Optic disk edema associated with peripapillary serous retinal detachment: an early sign of systemic Bartonella henselae infection. Am J Ophthalmol. 2000;130:327-334.
Williams KE, Johnson LN. Neuroretinitis in patients with multiple sclerosis. Ophthalmology. 2004;111:335-340.
Zacchei AC, Newman NJ, Sternberg P. Serous retinal detachment of the macula associated with cat scratch disease. Am J Ophthalmol. 1995;120:796-797.
