Optic Disc Edema with a Macular Star and Neuroretinitis


Optic Disc Edema with a Macular Star and Neuroretinitis

Optic disc edema with a macular star (ODEMS) is a descriptive term encompassing a heterogeneous group of disorders. In 1916, Leber described patients with idiopathic unilateral visual loss, optic disc edema, and macular exudate (Leber, 1916). He incorrectly theorized that the pathologic process was primarily retinal and called the condition “stellate retinopathy.” The condition subsequently has been called Leber’s stellate maculopathy, Leber’s idiopathic stellate neuroretinitis, or simply neuroretinitis. In 1977, Gass suggested that this syndrome was caused by a prelaminar disc vasculitis that results in leakage of disc capillaries and concluded that this entity was not a retinal vasculopathy but a primary optic neuropathy (Gass, 1977).

This syndrome is characterized by swelling of the optic disc, peripapillary and macular hard exudates that often occur in a star pattern, and (often) vitreous cells. Because the macular exudate likely results from primary optic nerve disease and not a true retinitis, we prefer the term idiopathic optic disc edema with a macular star (ODEMS) for idiopathic cases, and use the term neuroretinitis when optic disc swelling and a macular star are associated with retinitis, especially if an infectious cause is documented (Brazis, 1996).

What Are the Clinical Features of ODEMS and Neuroretinitis?

The clinical features of ODEMS have been described by a number of authors (Brazis, 1996; Hamard, 1994; King, 1991) and are summarized in Table 3–1. Patients are usually children or young adults, with the average age of onset being 20 to 40 years. Men and women are affected equally. Most cases are unilateral, but bilateral involvement has been noted to occur in up to a third of the cases. Most patients present with acute unilateral loss of vision. The condition is often painless, but retrobulbar pain, pain on eye movement, or associated headache may occur. A nonspecific viral illness precedes or accompanies the visual loss in approximately half of the cases.

Table 3–1. Clinical Characteristics of Optic Disc Edema with Macular Star (ODEMS)

Age at onset: childhood to young adult (6 to 50 years of age)

Gender: men and women affected equally

Bilateral involvement: 5–33%

Pain: occasional

Antecedent viral illness: approximately 50%

Initial visual acuity: variable (20/20–light perception)

Dyschromatopsia: often prominent

Visual field testing: central, cecocentral, arcuate, or altitudinal defects; possible generalized constriction

Relative afferent pupil defect present; but may be absent if bilateral involvement

Optic disc swelling present with subsequent optic atrophy

Macular star present but may take 1 or 2 weeks to develop

Vitreous cells common (90%)

Visual acuity with ODEMS may range from 20/20 to light perception, but most cases are in the 20/40 to 20/200 range. Dyschromatopsia is often present. Perimetry most often reveals a central or cecocentral scotoma, but other “optic nerve–type” field abnormalities may occur, including arcuate and altitudinal defects or generalized constriction. Most patients have a relative afferent papillary defect unless involvement is bilateral and relatively symmetric.

Optic disc edema is the earliest sign of ODEMS and may be severe. The disc edema tends to resolve over 2 weeks to 2 months, but in some patients optic atrophy ensues. Optic disc edema is associated with leakage of disc capillaries with the fluid spreading from the disc through the outer plexiform layer of the retina. The serous component of the fluid accumulation in Henle’s layer is reabsorbed, and the lipid precipitate forms a macular star. The macular star may be present at the onset of visual loss or may be noted only after 1 to 2 weeks following development of the disc edema. The macular star may even be observed only after the disc swelling is starting to resolve. Patients with acute disc swelling with a normal macula should thus be reexamined within 2 weeks to search for the presence of a macular star, especially because it is of prognostic importance for the patient’s subsequent risk of developing multiple sclerosis (see below). Fluorescein angiography typically shows leakage from the optic disc in the middle to late phases, with abnormal permeability of the deep capillaries in the optic nerve head but no perifoveal leakage (Ray, 2001).

ODEMS is often associated with cells in the vitreous. Other occasional findings include cells in the anterior chamber, chorioretinitis, inflammatory sheathing of the peripapillary veins, scleritis and uveitis, and (rarely) central or branch retinal artery occlusions (May, 1995). The association of ODEMS with these latter findings suggests a more diffuse vasculitis or an infectious cause.

What Is the Etiology and Differential of ODEMS and Neuroretinitis?

Most cases of ODEMS are idiopathic and thought to be the result of nonspecific viral infection or some immune-mediated process. In general, ODEMS is usually a benign, self-limited inflammatory process. A number of infectious agents and inflammatory diseases, however, have been reported to cause ODEMS and neuroretinitis. Infectious etiologies are listed in Table 3–2. Some of these infectious agents have been implicated in single case reports, but it appears that syphilis, cat-scratch disease, Lyme disease, and perhaps toxoplasmosis are the most common causes of ODEMS and neuroretinitis in cases where an etiologic agent can be identified. Infectious agents should be aggressively sought in cases of ODEMS and neuroretinitis because appropriate antibiotic treatment might be indicated. Ray and Gragoudas recommended special emphasis on recent patient travel history (Lyme endemic areas), consumption of unpasteurized or uncooked foods (toxoplasmosis), sexually transmitted disease exposure (syphilis), and animal contacts (cat scratch) (Ray, 2001).

ODEMS or neuroretinitis may occur as part of syphilitic meningitis (usually bilateral), or may occur as an isolated entity in patients with secondary syphilis, in which case it may be associated with unilateral or bilateral uveitis (Halperin, 1992; McCleish, 1990; Ninomiya, 1990). ODEMS or neuroretinitis is common manifestation of cat-scratch disease (Bar, 1990; Bhatti, 2001; Carithers, 1991; Chrousos, 1990; Cunningham, 2000; Earhart, 2000; Fish, 1992; Ghauri, 1998; Golnik, 1994; Gray, 1999; King, 1991; Labalette, 2001; May, 1995; McCrary, 1994, 1997; Ormerod, 1998; Reed, 1998; Rosen, 1999; Schwartzman, 1994, 1995; Solley, 1999; Suhler, 2000; Ulrich, 1992; Wade, 1999; Zhao, 1991). In fact, optic neuritis (papillitis) without a macular star has only rarely been reported with this disease (Brazis, 1986; Golnik, 1994). Cat-scratch disease may cause ODEMS or may cause a neuroretinitis with chorioretinitis at times associated with uveitis, cells in the anterior chamber, and even branch or central retinal artery occlusions (Fish, 1992; Golnik, 1994; May, 1995; Ulrich, 1992; Zhao, 1991). This disease may also cause a multifocal retinitis with optic disc edema (without macular star), branch retinal artery occlusion, and vitreitis (Cohen, 1995). Optic disc edema associated with peripapillary serous retinal detachment, even without macular star formation, may be an early sign of cat-scratch disease (Wade, 2000). Solley et al studied 24 patients (35 eyes) with choroidal, retinal, or optic disc manifestations of cat-scratch disease and found that discrete white retinal or choroidal lesions were the most common posterior segment finding (46% of eyes, 63% of patients) followed by macular star (43% of eyes, 63% of patients) (Solley, 1999). Vascular-occlusive events were also seen (14% of eyes, 21% of patients) and the site of occlusion was found to be intimately associated with the aforementioned retinal lesions. Final visual acuity was 20/25 or better in 26 (74%) of 35 eyes and was similar in both treated and untreated patients. Cat-scratch disease, therefore, should be considered in any patient who presents with ODEMS or neuroretinitis, especially if there is associated lymphadenopathy or retinal artery occlusion (class III, level B). The treatment (Conrad, 2001) of cat-scratch disease is quite variable in the literature and has included various antibiotic regimens including penicillins, cephalosporins, aminoglycosides, tetracyclines, macrolides, quinolones, trimethoprim-sulfamethoxazole, and rifampin (class III, level C). Reed et al reported seven cases, and concluded that, compared to historic controls, doxycycline and rifampin shortened the course of the disease and improved visual recovery (class III, level C) (Reed, 1998). The ophthalmologic manifestations of cat-scratch disease are outlined in Table 3–3.

Table 3–2. Infectious Etiologies of Optic Disc Edema with Macular Star or Neuroretinitis


Hepatitis B

Herpes simplex

Herpes zoster (Dhar, 1997)

Epstein-Barr virus

Influenza A

Mumps (Foster, 1990)

Coxsackie B


Cat-scratch disease (Bartonella henselae)

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Jun 4, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Optic Disc Edema with a Macular Star and Neuroretinitis
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