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?

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?