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Ophthalmology > CORNEA
Dystrophy, Map-dot-fingerprint
Article Last Updated: Dec 12, 2005
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: David D Verdier, MD, Clinical Professor, Department of Surgery, Michigan State University College of Human Medicine
David Verdier is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and Eye Bank Association of America
Editors: Fernando H Murillo-Lopez, MD, Senior Surgeon, Unidad Privada de Oftalmologia CEMES; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Hospital; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Author and Editor Disclosure
Synonyms and related keywords:
map-dot-fingerprint dystrophy, anterior basement membrane dystrophy, epithelial basement membrane dystrophy, Cogan's microcystic dystrophy, slit lamp, corneal dystrophy, corneal degeneration
Background
Corneal map-dot-fingerprint dystrophy is by far the most common corneal dystrophy and is named from the appearance of its characteristic slit lamp findings. Map-dot-fingerprint dystrophy also is known as epithelial basement membrane dystrophy, and Cogan microcystic epithelial dystrophy. It usually is classified as a dystrophy but fits more accurately into the corneal degeneration category.
Corneal dystrophies usually are hereditary, bilateral, progressive, and not associated with systemic or local disease. Map-dot-fingerprint dystrophy has been found in several families with a presumed autosomal dominant pattern, but in most cases, it is not familial. It is not progressive but rather variable and fluctuating in its course. Usually, it is bilateral but can be unilateral or very asymmetric in presentation.
Pathophysiology
The corneal epithelium produces and adheres to its underlying basement membrane. Corneal abnormalities associated with map-dot-fingerprint dystrophy are the result of a faulty basement membrane, which is thickened, multilaminar, and misdirected into the epithelium. Deeper epithelial cells that normally migrate to the surface can become trapped. Epithelial cells anterior to aberrant basement membrane may have difficulty forming viable hemidesmosomes and basement membrane complexes, which attach to the underlying stroma, resulting in recurrent erosions. Irregular epithelium centrally can cause decreased vision.
Maps histologically represent areas of multilaminar basement membrane, which extend into the epithelium. Dots are intraepithelial microcysts that contain nuclear, cytoplasmic, and lipid debris. Fingerprints are curvilinear clusters of reduplicated and thickened basement membrane and fibrillogranular material. Blebs, a less common manifestation of map-dot-fingerprint dystrophy, are localized areas of fibrillogranular material or thickened basement membrane.
Frequency
United States
Estimates of the prevalence of map-dot-fingerprint dystrophy range from 2-43% of the general population. Of patients with map-dot-fingerprint dystrophy, 10-33% have recurrent corneal erosions. As many as 50% of patients with recurrent corneal erosions have map-dot-fingerprint dystrophy.
Mortality/Morbidity
Patients with map-dot-fingerprint dystrophy may be asymptomatic. Others experience painful recurrent erosions, decreased vision, or both.
Sex
This condition is slightly more common in females than in males.
Age
This condition is uncommon in children.
History
- Most patients are asymptomatic.
- Past eye history - May be positive for recurrent corneal erosions
- Visual symptoms are usually mild and occasionally debilitating. Vision is variable and fluctuating due to migratory and intermittent corneal involvement. Refractions often are unstable and are not the fault of doctor or patient. Visual complaints include the following:
- Blurred vision
- Ghosting or monocular diplopia
- Glare
- Pain symptoms
- Foreign body sensation
- Photophobia
Physical
- Visual acuity - Ranges from 20/15 to 20/200
- Refraction - May be uncertain endpoint due to irregular astigmatism
- Slit lamp examination - Pathology is at the epithelial and basement membrane level. Areas of pathology often are identified best by broad-beam illumination, fluorescein with cobalt blue light to identify areas of negative staining, or retroillumination following dilation. Slit lamp findings include the following:
- Corneal maps are irregular geographic shape, faint gray-white patches that may contain clear oval areas. They vary greatly in size (usually 1 mm to several mm) and are seen best with broad oblique illumination.
- Corneal dots are gray-white, puttylike opacities, which can be round, comma-shaped, or irregular. They usually are 0.05-1.0 mm in size.
- Corneal fingerprints are clusters of contoured concentric lines 0.25-4.0 mm long. They are seen best with retroillumination.
- Corneal blebs are clear, round, bubblelike defects 0.05-0.2 mm in diameter. They are seen best with retroillumination.
- Keratometry - Look for irregular astigmatism.
- Computerized topography - Look for irregular astigmatism. (A Placido disk or keratometer often demonstrates irregularity better than computerized topography.)
Causes
- Other diagnostic considerations: Corneal pseudofingerprints or shift lines (a manifestation of corneal epithelial edema) can be seen with elevated intraocular pressure and/or corneal decompensation, in conditions such as the following:
- Glaucoma
- Pseudophakic or aphakic bullous keratopathy
- Fuchs corneal endothelial dystrophy
- Congenital hereditary endothelial dystrophy
- Posterior polymorphous dystrophy
- Keratouveitis
- Corneal trauma with endothelial cell damage
Corneal Abrasion
Dystrophy, Fuchs Endothelial
Herpes Simplex
Herpes Zoster
Other Problems to be Considered
Corneal trauma
Diabetes mellitus (can be thickened and redundant basement membrane)
Corneal suture lines (junction of a healing corneal epithelial defect)
Radial keratotomy (can have corneal epithelial opacities clinically indistinguishable from map-dot-fingerprint dystrophy)
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 by this author, 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.
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Hyperosmolar agents
Create osmotic gradient that draws water out of the cornea.
| Drug Name | Sodium chloride (Muro 128, AK-NaCl) |
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| Description | Used for temporary relief of corneal inflammation. |
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| Adult Dose | Solution: 1-2 gtt into affected eye tid
Ointment: 0.25-inch ribbon to inside of affected eye(s) qhs |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
|
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| Precautions | May cause temporary burning and irritation upon use; if pain, change in vision, continued redness, or irritation of the eye(s) occur, or if initial condition/problem worsens or persists, reevaluate therapy; do not use product if it changes color or becomes cloudy |
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Deterrence/Prevention
- Lubricating hypertonic saline or bland ointment at bedtime is often helpful to prevent recurrent erosions.
Complications
- Recurrent erosions predispose the cornea to infection.
Prognosis
- Map-dot-fingerprint dystrophy findings may fluctuate but tend not to progress over time. The great majority of patients are able to maintain sufficient vision and comfort for reading, driving, and other visual tasks, except during episodes of corneal erosions.
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.
- Map-dot-fingerprint dystrophy is a relative contraindication for refractive procedures, such as LASIK (laser in situ keratomileusis) or LASEK (laser epithelial keratomileusis). Trauma from the microkeratome sliding over the epithelial surface or from flap manipulation is more likely to occur in patients with map-dot-fingerprint dystrophy because of the poorly adherent epithelium. Epithelial sloughing can lead to epithelial ingrowth and stromal melts. Surface ablation (photorefractive keratectomy [PRK]) may be a better refractive procedure option for these patients.
| Media file 1:
Corneal maps. Best seen with broad illumination beam. |
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| Media file 3:
Corneal fingerprints. Best seen in retroillumination. |
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| Media file 4:
Pseudofingerprints (shift lines) in a patient with Fuchs corneal dystrophy. |
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Media type: Photo
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Dystrophy, Map-dot-fingerprint excerpt Article Last Updated: Dec 12, 2005
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