Fuchs Endothelial Dystrophy

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Disease

  • Most frequently non-hereditary (C3), families with autosomal dominant inheritance
  • Onset usually in the 4th-5th decade of life, early forms starts in the 1st decade of life

Findings

  • Symptoms: Gradual deterioration of visual acuity, typically foggy vision in the morning due to stromal oedema
    • How many hours until vision improves? -> Indicator for corneal transplantation
  • Cornea guttata (endothelium like “hammered metal” in advanced cases) especially in central cornea
  • Endothelial cell count reduced (confocal microscopy)
  • Endothelial decompensation with central stromal oedema
  • May lead to epithelial oedema with microcysts and bullae, erosion

Management

  • Conservative:
    • NaCl gtt 5%
    • Bandage contact lens for ruptured bullae +/- antibiotic prophylaxis (e.g. Floxal gtt (Ofloxacin) 3x/d)
  • Operative:
    • Lamellar endothelial keratoplasty (DSAEK, DMEK) -> see also corneal transplantations
    • Penetrating keratoplasty (PKP)
    • Cataract surgery can lead to significant endothelial cell loss
      • Catataract surgery before keratoplasty; consider so-called “triple procedure” (phaco/IOL + keratoplasty)

Sources

  • EyeWiki Fuchs Endothelial Dystrophy
  • Weiss JS, Møller HU, Aldave AJ, Seitz B, Bredrup C, Kivelä T, Munier FL, Rapuano CJ, Nischal KK, Kim EK, Sutphin J, Busin M, Labbé A, Kenyon KR, Kinoshita S, Lisch W. IC3D classification of corneal dystrophies–edition 2. Cornea. 2015 Feb;34(2):117-59. doi: 10.1097/ICO.0000000000000307. Erratum in: Cornea. 2015 Oct;34(10):e32. PMID: 25564336.
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)