Map-Dot-Fingerprint

The condition is referred to as Cogan's dystrophy.

The symptoms among patients may vary widely in severity and include:

Light sensitivity

Glare

Fluctuating vision

Blurred vision

Irregular astigmatism (uneven corneal surface)

Mild to extreme irritation and discomfort that is worse in the morning

Corneal tear or rip with sharp rip stinging pain often lasting several days.

Among the various forms of care for this dystrophy, the following are the more common:

Medical Care: Numerous treatment options are available, and like the disease itself, results are variable and differ from patient to patient.

• Hypertonic drops or ointment often are the first line of treatment. They may help both irregular astigmatism and recurrent corneal erosion problems. Sodium chloride (5%) drops at breakfast, lunch, and dinner; and ointment at bedtime is recommended.

• Nonhypertonic lubricating drops or ointment: The only prospective study to date detected no difference in results of bland versus hypertonic lubricating treatment.

• Consider patching for acute episodes of associated corneal erosions.

• Bandage extended wear soft contact lens occasionally may be useful but risk of infectious keratitis makes this a secondary choice.

• Hard or gas-permeable contact lens may improve vision by masking corneal irregular astigmatism but often is poorly tolerated because of increased corneal fragility/erosion problems.

Surgical Care:

• Debridement/superficial keratectomy is preferred, for both significant visual loss from associated irregular astigmatism and recurrent corneal erosions, if treatment with hypertonic drops and ointment fails. Combined debridement and superficial keratectomy can be completed easily in the office setting, at the slit lamp, using topical proparacaine or a similar anesthetic drop. Place a lid speculum, then debride (with a rather blunt Kimura spatula) the entire extent of any loosely adherent epithelium or basement membrane level opacities. With sweeping and pushing motions using the trailing or leading edges of the instrument, keeping nearly parallel to the corneal plane, redundant basement membrane level material can be massaged away, while maintaining the integrity of the Bowman layer.

• Diamond burr superficial keratectomy is very useful for recurrent erosions associated with map-dot-fingerprint dystrophy that does not respond to keratectomy with a Kimura spatula. Following epithelial debridement, a 4- or 5-mm diameter diamond-dusted burr very gently is used to polish the basement membrane throughout the area of epithelial debridement.

• Excimer laser phototherapeutic keratectomy is an excellent treatment for recurrent corneal erosions associated with map-dot-fingerprint dystrophy, with results similar to above superficial keratectomy procedures (but much more expensive in most settings).

• Corneal anterior stromal needle puncture is useful for recurrent corneal erosions from trauma that recur in the same location. This procedure is not as successful for recurrent erosions associated with map-dot-fingerprint dystrophy, which is usually more diffuse and often migratory.

Perhaps the most frustrating aspect of Cogan's dystrophy, (also known as map-dot-fingerprint dystrophy) is the Following SPECIAL CONCERN.

Special Concerns:

• While patients with map-dot-fingerprint dystrophy may be bothered by painful recurrent erosion episodes and/or decreased vision, they typically are most frustrated by the unpredictability of the condition. They may have an episode of pain and poor vision the day of their wedding or of an important presentation after being asymptomatic for weeks or months. Physicians need to understand this important concern, as do others. Patients under such circumstances should not be pressured to perform or do activities while they are suffering from the pain of their erosion episode.