This document discusses Fuchs endothelial dystrophy and pseudophakic bullous keratopathy. It describes the characteristics, classification, pathology, signs, and treatment of Fuchs endothelial dystrophy. It also covers the etiology, clinical features, evaluation techniques including specular microscopy and pachymetry, and management including corneal transplant and conjunctival flap procedures for pseudophakic bullous keratopathy resulting from traumatic endothelial damage following cataract surgery.

1. FUCHS DYSTROPHIES AND
PSEUDOPHAKIC BULLOUS
KERATOPATHY
Resident of ophthalmology
3rd
year : UN Leng

2. CHARACTERISTICS OF CORNEAL
DYSTROPHIES
 Bilateral, symmetric, inherited, no environmental or
systemic involvement
 Early onset, and become clinically apparent until later
 Central corneal location
 Classified: genetic pattern, severity, histopathologic
features, biochemical characteristic or anatomical
location

3. CLASSIFICATION
A. Anterior dystrophies
1. Epithelial basement membrane dystrophy (EBMD)
2. Meesman’s dystrophy
3. Reis Buckler dystrophy
4. Lisch dystrophy
A. Stromal dystrophie
1. Granular dystrophies
2. Lattice dystrophy
3. Macular dystrophy
4. Crystalline dystrophy
C. Posterior dystrophies
1.1. Fuchs` endothelial dystrophyFuchs` endothelial dystrophy
2. Posterior polymorphous dystrophy
3. Congenital hereditary endothelial
dystrophy

4. FUCHS ENDOTHELIAL DYSTROPHY
 Bilateral accelerated endothelial cell loss
 More common in women
 Inheritance: sporadic and AD
 Onset: slowly progressive disease in old age ( > 50 ys )

5. PATHOLOGY
 Microscopy: endothelial cells larger (polymegathism) &
more polymorphic (pleomorphism) + disrupted by
excrescences of excess collagen in Descemet’s
membrane=>
 Dysfunction of endothelial cell => increase corneal swelling
=> reduce Na+
/k+
-ATPase pump

6. FUCHS ENDOTHELIAL DYSTROPHY
 Signs:
 Cornea guttata
 Endothelial
decompensate =>
central stromal edema
and blurred vision, worse
in the morning and
clearing later in the day

7. FUCHS ENDOTHELIAL DYSTROPHY
 Signs:
 Beaten metal appearance
 Develop epithelium edema => persistent edema in the form of
microcysts and bullae
 Rupture => pain and discomfort

8. SPECULAR MICROSCOPY

9. TREATMENT
1. conservative options:
 Topical sodium chloride 5% drop or ointment
 Reducing IOP
 Using hair dryer to speed corneal dehydration in the morning
1. Bandage contact lens:
 comfort by protecting exposed nerve endings and flattening bullae

10. TREATMENT
3. Penetrating keratoplasty or Descemet stripping
endothelial keratoplasty ( DSEAK )
 Success rate high and not be delayed
4. Other options
 Poor vision eye: conjunctiva flaps and amniotic membrance
transplants

11. PSEUDOPHAKIC BULLOUS KERATOPATHY

12. CATARACT SURGERY
 accelerate endothelial cell loss => decompensation
 Triple procedure induce corneal epithelial edema:
 Cataract surgery
 Lens implatation
 Keratoplasty
 Should measure corneal thickness with pachymetry
 < 640 µm => good visual outcome
 > 620 - 640 µm => risk of cornea decompensate

13. ETIOLOGY
 Corneal endothelial damage
 Intraocular inflammation
 Vitreous or subluxation intraocular lens
 Preexisting endothelial dysfunction

14. In cases of TRAUMATIC conditions:
pseudophakic bullous keratopathy:
 The resulting endothelium is characterised by decreased cell number
and enlarged and irregularly shaped cells showing polymegathism and
pleomorphism .
 When the cell density falls below 200-400 cells/mm2 ,their pump
function begins to fail and stroma begins to swell.
PATHOPHYSIOLOGY

15. CLINICAL FEATURES
 Corneal edema
 Cornea bullae & Descemet folds
 Pain ( rupture of bullae )
 Erosive symptoms:
 Discomfort, FB sensation, photophobia and watering
 Cornea scare and neovascularization
 Cystoid macular edema

16. EVALUATION TECHNIQUE
1. Slit- lamp examination
 Corneal bullae
 Position of IOL
 Vitreous touches endothelium
 IOP
 Fundus examination: Look for CME ( FFA or OCT )
2. Corneal pachymetry (ultrasonic or optic):
 measures corneal thickess .[normal:500-550 microns]
 If 650 microns suggest a higher risk for edema after intra-ocular surgery
 If 700 microns suggest corneal decompensation

17. 3. Specular microscopy
demonstrates reduced endothelial cell density and abnormal
morphology
Its helpful in detecting
 `warts or guttae` in fuchs dystrophy
 polymegathism and pleomorphism .
EVALUATION TECHNIQUE

18. MANAGEMENT
1. Topical sodium chloride 5% drop and ointment
2. Reduce IOP
3. Rupture epithelial bullae:
 Antibiotic ointment
 Cycloplegic
 BSCL
 Recurrent ruptured bullae: anterior stromal micropuncture or PTK

19. MANAGEMENT
4. Full-thickness corneal transplant or endothelial
keratoplasty ( DSEK )
5. Conjunctival flap or amniotic membrane graft