Corneal Epithelial Basement Membrane Dystrophy by gdf57j

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									Corneal Epithelial Basement Membrane Dystrophy

Darshana B Rathod, Anjali D Nicholson, Bhavisha Bid


Abstract
A 38 year old female presented with recurrent redness, pain, watering in both eyes since last 6-
7 yrs having taken antibiotics, steroids, lubricating drops, anti-viral (acivir), hypersol, (ban-
dage contact lenses) BCL for treatment with no positive family history. Her best corrected
visual acuity were 6/9 in both eyes for distance and N/6 for near with right eye showing lid
oedema and conjunctival congestion. Corneal sensations and Schirmer’s test were normal.



Introduction                                              acuity were 6/9 in both eyes for distance and N/6 for
                                                          near with right eye showing lid oedema and

C     orneal map-dot-fingerprint dystrophy is
     by far the most common corneal
dystrophy and is named from the appearance
                                                          conjunctival congestion. Corneal sensations and
                                                          Schirmer’s test were normal.
                                                             On examination, slit lamp findings suggested of
of its characteristic slit lamp findings. Map-            an oval corneal epithelial defect measuring 3 mm x 1
dot-fingerprint dystrophy also is known as                mm, with positive fluoroscein staining and inferior
epithelial basement membrane dystrophy,                   limbal vascularisation in the right eye and left eye
                                                          showing sub-epithelial map and dot pattern opacities.
and Cogan microcystic epithelial dystrophy.
It usually is classified as a dystrophy but fits              Patient was treated locally with mild steroid-
                                                          fluromethanole and lubricating eye drops. Systemic
more accurately into the corneal
                                                          Doxycycline 100 mg BD was prescribed for 6 weeks.
degeneration category.
                                                          Pathophysiology
  Corneal       dystrophies       usually    are
hereditary, bilateral, progressive, and not                  Corneal abnormalities associated with
associated with systemic or local disease.1               map-dot-fingerprint dystrophy are the result
Map-dot-fingerprint dystrophy has been found              of a faulty basement membrane, which is
in several families with a presumed                       thickened, multilaminar, and misdirected into
autosomal dominant pattern, but in most                   the epithelium. Maps histologically represent
cases, it is not familial. It is not progressive          areas of multilaminar basement membrane,
but rather variable and fluctuating in its                which extend into the epithelium. Dots are
course. Usually, it is bilateral but can be               intraepithelial microcysts that contain
unilateral    or    very       asymmetric      in         nuclear, cytoplasmic, and lipid debris.
presentation. 2                                           Fingerprints are curvilinear clusters of
                                                          reduplicated and thickened basement
Case Report
                                                          membrane and fibrillogranular material.
   A 38 year old female presented with recurrent
                                                          Blebs, a less common manifestation are
redness, pain, watering in both eyes since last 6-7 yrs
having taken antibiotics, steroids, lubricating drops,    localized areas of fibrillogranular material or
anti-viral (acivir), hypersol, BCL for treatment with     thickened basement membrane.3
no positive family history. Her best corrected visual        Frequency: In the US prevalence of map-
                                                          dot-fingerprint dystrophy range from 2-43%
Ophthalmology Department, B Y L Nair Charitable
                                                          of the general population and is found to be
Municipal Hospital, Mumbai 400 008.

126                                                          Bombay Hospital Journal, Vol. 51, No. 1, 2009
  Fig. 1 : Oblique illumination showing corneal subepithelial map and dot dystrophy.

more common in females and is in all age
group except children.
   Mortality/Morbidity: Patients may be
asymptomatic, experience painful recurrent
erosions, decreased vision, or both.

 Clinical Relevance
  Refraction is uncertain due to irregular
astigmatism.
  Slit lamp findings includes the following:
   Corneal maps seen as 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.           Fig. 2 : Retro illumination view of basement
                                                            membrane dystrophy.
   Corneal dots seen as gray-white, puttylike
opacities, which can be round, comma-shaped,         computed tomography.
or irregular. They usually are 0.05-1.0 mm
                                                        Conditions of elevated intraocular pressure
in size.                                             and corneal decomposition which gives rise
  Corneal fingerprints seen as clusters of           to corneal epithelial oedema mimicking
contoured concentric lines 0.25-4.0 mm long.         corneal pseudo fingerprints or shift lines can
  They are seen best with retroillumination.         cause diagnostic dilemma.
  Corneal blebs are clear, round, bubble like        Treatment
defects 0.05-0.2 mm in diameter. They are
                                                     Medical Care
seen best with retroillumination.
                                                        Hypertonic drops or ointment help both
  Keratometry shows irregular astigmatism.
                                                     irregular astigmatism and recurrent corneal
  A placido disk or keratometer often
                                                     erosion problems. Sodium chloride (5%) drop
demonstrates irregularity better than                four times a day and ointment at bedtime is

Bombay Hospital Journal, Vol. 51, No. 1, 2009                                                  127
recommended.                                       helpful to prevent recurrent erosions.
   Nonhypertonic lubricating drops or              Complications
ointment is also preferred as it is found that
                                                      Recurrent erosions predispose the cornea
there is no difference between hypertonic and
                                                   to infection.
nonhypertonic ointment.
   Capsule Doxycycline (100 mg) BD for six         Prognosis
weeks helps in the adhesion between cells of          Map-dot-fingerprint dystrophy findings
epithelial basement membrane, mechanism            fluctuate but tend not to progress over time.
of which is not known.                             Majority of patients are able to maintain
   Patching is done in case of acute episodes      sufficient vision and comfort for reading,
of corneal erosions.                               driving, and other visual tasks, except during
                                                   episodes of corneal erosions.
   Bandage extended wear soft contact lens
is useful but risk of infectious keratitis makes   Special Concerns
this a secondary choice.                              Patient with this dystrophy may be
   Hard or gas-permeable contact lens is used      bothered by painful recurrent erosion
to improve vision by masking corneal               episodes and or decreased vision but are most
irregular astigmatism but is poorly tolerated      frustrated by the unpredictability of the
because of increased corneal fragility/erosion     condition.
problems.                                             Map-dot -fingerprint dystrophy is a relative
Surgical Care                                      contraindication for refractive procedures,
                                                   such as LASIK or LASEK. Trauma from the
   Debridement/superficial keratectomy is
                                                   microkeratome sliding over the epithelial
done in case of significant visual loss from
                                                   surface or from flap manipulation is more
associated irregular astigmatism and
                                                   likely to occur in patients with map-dot-
recurrent corneal erosions.
                                                   fingerprint dystrophy because of the poorly
  Diamond burr superficial keratectomy:            adherent epithelium. Epithelial sloughing can
After epithelial debridement a diamond-            lead to epithelial ingrowth and stromal melts.
dusted burr is used to polish the basement         Surface ablation [PRK]) may be a better
membrane.                                          refractive procedure option for these patients.
   Excimer     laser      phototherapeutic         References
keratectomy is an excellent treatment for
                                                   1. Dinh R, Rapuano CJ, Cohen EJ, et al. Recurrence
recurrent corneal erosions associated with            of corneal dystrophy after excimer laser
map-dot-fingerprint dystrophy. 4                      phototherapeutic. Keratectomy. Ophthalmology
   Corneal anterior stromal needle puncture:          1999; 106 : 1490-97.

This procedure is not as successful for            2. Bron A J. Genetics of the corneal dystrophies:
                                                      What have we learned in the past twenty-five
recurrent erosions associated with map-dot-
                                                      years. Cornea.2000; 19 : 699-711.
fingerprint dystrophy, which is usually more
                                                   3. Klintworth GK. Advances in the molecular
diffuse and often migratory.
                                                      genetics of corneal dystrophies. Am J
Prevention                                            Ophthalmology 1999; 128 : 747-54.
                                                   4. External Disease and cornea-section 8.American
   Lubricating hypertonic saline or bland
                                                      Academy of Ophthalmology.2006-2007; 311-13
ointment at bedtime is most of the time


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