As noted above, congenital Horner syndrome is usually associated with heterochromia; that is, one iris differs in color from its fellow. If all patients with congenital Horner syndrome had one brown eye and one blue eye, detection of this condition would be simple. However, in some blue-eyed patients, the color difference between the irises is quite subtle and difficult to detect. Iris color should be checked in daylight (by the office window) rather than in fluorescent light. The difference thus revealed is often striking.

Both darker-eye and lighter-eye heterochromia are seen, although the latter is more common than the former. These symptoms can indicate several disorders other than Horner syndrome. Larger-sector pigmented areas are nevi and do not represent true heterochromia.

Darker-Eye Heterochromia

A diffuse iris melanoma is best evaluated with the slit lamp, since the darker brown area will appear as a mass rather than simply as pigmented iris stroma. In neurofibromatosis, associated signs help identify the disorder. A thorough physical examination is called for, with the physician looking for other neurofibromas and, particularly, for cafe au lait spots. If the physician finds five café au lait spots that are more than 2 cm in diameter each, the diagnosis of neurofibromatosis is almost a certainty, even if the patient lacks other signs and symptoms. A single café au lait spot may point to neurofibromatosis, but it is not conclusive evidence.

If hemosiderosis is present, a carefully taken history of trauma or ocular penetration, as well as slit-lamp, indirect ophthalmoscopic, and gonioscopic examinations, is mandatory.

Hemochromatosis of the iris is associated with a history of repeated bleeding in the anterior chamber.

Lighter-Eye Heterochromia

Lighter-eye heterochromia is seen more frequently than darker-eye heterochromia because the iris does not become pigmented until late in the first year of life. Iris pigmentation requires sympathetic stimulation. Congenital or neonatal sympathetic paralysis is a leading cause of heterochromia. The lack of pigmentation is not universally present.

Waardenburg syndrome is a variation of congenital Horner syndrome. In addition to lighter-eye heterochromia, the patient has a prominent white forelock, lateral displacement of the medial canthus, deafness, hypertrichosis, a broad nasal root, and lighter pigmentation of the ipsilateral fundus.

Fuchs' heterochromic cyclitis is worthy of comment because of its resistance to therapy. Most prolonged cases of iritis cause atrophy of the iris stroma and result in secondary heterochromia. Fuchs' iritis has specific corneal endothelial deposits that are stellate in shape. It has a low level of activity and is resistant to therapy of any kind. Secondary glaucoma and cataract also may develop, but they can be treated as the need arises.

Essential iris atrophy is a rare but interesting problem that occurs predominantly in young women. It is progressive. Since secondary glaucoma is the main complication, patients should be examined for glaucoma periodically.

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