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Granular Corneal Dystrophy

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Grancular Corneal Dystrophy

Grancular Corneal Dystrophy

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  • 1. Grand Rounds Eric K. Chiu, M.D. Department of Ophthalmology and Visual Science University of Chicago 2/2/05
  • 2. Case Presentation
    • 43 y.o. male presenting with blurred vision bilaterally
    • Pt states he is tired of glasses and contacts and is interested in corrective surgery
    • No pain/redness/flashes/floaters
  • 3. Clinical Presentation
    • PMH: none
    • All: NKDA
    • Meds: none
    • Past ocular history:
      • myopia
  • 4. Clinical Presentation
    • SLE
      • See photo
    • DFE
      • wnl
  • 5. Case Presentation
  • 6. Clinical Course
    • What would you advise the patient?
  • 7. Differential Dx.
    • Granular dystrophy
      • Stromal dystrophy
      • Autosomal dominant
    • Reis-Bücklers dystrophy
      • Anterior membrane dystrophy
      • Affects Bowman’s layer and anterior stroma
      • Autosomal dominant
    • Macular dystrophy
      • Stromal dystrophy
      • Dense diffuse stromal opacities
      • Autosomal recessive
  • 8. Clinical Course
    • Pt underwent LASIK surgery bilaterally
    • 9 years later pt presents to U. Chicago with blurred vision RE>LE
  • 9. Clinical Course
    • VA SC
      • OD 20/400 PH 20/80
      • OS 20/80 PH 20/50
    • IOP GA
      • OD 17mmHg
      • OS 17mmHg
  • 10. Clinical Course
    • PKP recommended
    • Risks/benefits/alternatives discussed
  • 11. Clinical Course
    • One year later pt returns to clinic p/w decreased vision RE and LE
    • Pt notes increased light sensitivity
    • VA SC
      • OD 20/300 PH 20/200
      • OS 20/200 PH 20/60
  • 12. Clinical Course
    • Pt decided to undergo PKP in RE
    • Last seen 3 weeks post-op
    • VA SC
      • OD 20/300 PH 20/70
      • OS 20/80 PH 20/50
  • 13. Clinical Pathology
  • 14. Clinical Pathology
  • 15. Clinical Pathology
  • 16. Clinical Pathology
  • 17. Clinical Pathology
  • 18. Clinical Pathology
  • 19. Clinical Course
    • Pt underwent PKP left eye for decreased vision secondary to stromal opacities
  • 20. Granular Dystrophy
    • What is the definition of a corneal dystrophy?
  • 21. Granular Dystrophy
    • Dystrophy
      • Bilateral
      • Progressive
      • Genetic component
      • Little no relationship to environmental or systemic factors
  • 22. Granular Dystrophy
    • Dystrophy
      • Bilateral
      • Progressive
      • Genetic component
      • Little no relationship to environmental or systemic factors
    • General
      • Stromal dystrophy
    • Genetics
      • Autosomal dominant
      • Chromosome 5q31
        • Lattice
        • Avellino
        • Reis-B ücklers
      • BIGH3 gene
        • Formation of keratoepithelin
  • 23. Clinical
    • Type I
      • Most frequent
      • Early onset in life with crumblike opacities
        • Broaden into disciform appearance in teens
      • Do not extend to limbus
      • Can extent anteriorly through breaks in Bowman’s layer
      • Slowly progressive
        • Vision rarely drops to 20/200 after age 40
  • 24. Clinical
    • Type II
      • Presents in 2 nd decade
      • Fewer, larger ring/disc-shaped deposits in anterior stroma
      • Clear areas
      • Deposits progressively deeper with age
      • Erosions infrequent
      • Vision usually better than 20/70
  • 25. Clinical
    • Type III
      • Presents in infancy with epithelial erosions
      • More superficial
      • Granular deposits confined to Bowman’s layer or anterior stroma
      • Resembles Reis-B ücklers but distinct mutation of BIGH3 gene
  • 26. Management
    • Early in disease process no treatment needed
    • Recurrent erosions
      • Contact lenses
      • Superficial keratectomy
      • PTK
    • Decreased VA
      • Good prognosis with PKP
  • 27. Management
    • PKP
      • Recurrence in graft may occur after many years as fine subepithelial opacities varying from original presentation
  • 28. Pathology
    • Pathogenesis
      • Granular material consists of hyaline
      • Stains bright red with Masson trichrome stain
      • Electron dense material made up of rod-shaped bodies immersed in an amorphous matrix
      • Noncollagenous protein from corneal epithelium and/or keratocytes
  • 29. ACGME considerations…check please
    • New patient Comprehensive Eye Exam-No referral 48056
      • Facility charge 152.00
      • Professional charge 201.00
    • External slit lamp photos 48034
      • Facility charge 75.00
      • Professional charge 32.00
    • -------------------------------------------
    • Collected  460.00 (paid by check)
  • 30. ACGME considerations…check please
    • PKP CPT 65730
      • Facility charge $19,326.00
      • Professional charge $
      • ------------------------------------
      • Collected  pending
  • 31. References
    • Ophthalmology. Yanoff. 2nd edition, p 439-440
    • Akhtar S et al. Deposits and proteoglycan changes in primary and recurrent granular dystrophy of the cornea. Archives of Ophthalmology . 1999;117:310-321
    • Rapuano et al. Recurrence of corneal dystrophy after excimer phototherapeutic keratectomy. Ophthalmology . 1999 Aug; 106(8):1490-7
    • Marcon et al. Recurrence of Corneal Stromal Dystrophies after Penetrating Keratoplasty. Cornea . Jan. 2003 22(1):19-21
    • Seitz et al. Morphometric analysis of deposits in granular and lattice corneal dystrophy: histopathologic considerations for phototherapeutic keratectomy. Cornea . 2004 May;23(4): 380-385

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