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. Meesmans 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 Descemets 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 m => good visual outcome > 620 - 640 m => 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