🌫️ Fuchs Endothelial Dystrophy (FED): From Guttae to Grafts

By Dr. Hemali Kanabar | July 9, 2025

πŸ‘οΈ What is Fuchs Endothelial Dystrophy?

Fuchs Endothelial Dystrophy (FED) is a progressive, bilateral, and typically asymmetric corneal dystrophy that affects the endothelial layer of the cornea. It leads to corneal decompensation and visual loss due to fluid accumulation and corneal thickening.

🧬 Pathophysiology

In FED, the corneal endothelial cells (which pump out excess fluid) progressively degenerate. This leads to:

  • Formation of guttae (excrescences on Descemet’s membrane)
  • Loss of endothelial cell density
  • Stromal and epithelial oedema
  • Painful bullae in advanced stages

πŸ” Clinical Features

StageFindings
EarlyGuttae seen on specular reflection, minimal symptoms
ModerateDecreased vision (especially in morning), corneal thickening
AdvancedStromal oedema, epithelial bullae, painful vision loss

πŸ› οΈ Tip: Use a slit-lamp with specular reflection or confocal microscopy to detect early guttae.

πŸ”¬ Diagnosis

  • Slit-lamp exam: Guttae, stromal haze, epithelial bullae
  • Specular microscopy: Reduced endothelial cell count
  • Pachymetry: Corneal thickening (>640 ΞΌm may indicate decompensation)
  • OCT of anterior segment: Helps assess corneal layers

πŸ“ˆ Disease Progression and Cataract Coexistence

FED often presents in older adults, and many patients also have cataracts. This creates a management dilemma: Which to treat first β€” the cornea or the lens?

Here’s a simplified management approach

🧠 Management Strategy in Patients with FED and Cataract

Clinical ScenarioSuggested Management
No cataract + mild corneal oedema🟒 Conservative medical management (hypertonic saline, lubricants)
Dense cataract + severe corneal oedemaπŸ”„ Triple procedure (phacoemulsification + IOL + endothelial keratoplasty)
No cataract + severe corneal oedemaπŸ” Keratoplasty (DMEK/DSAEK) first β†’ cataract surgery later if needed
Dense cataract + clear corneaπŸ‘οΈ Cataract extraction first β†’ keratoplasty only if decompensation occurs

πŸ’‰ Surgical Options

  • DMEK (Descemet Membrane Endothelial Keratoplasty): Replaces only Descemet’s membrane and endothelium. Quicker recovery, excellent outcomes.
  • DSAEK (Descemet Stripping Automated Endothelial Keratoplasty): Easier technique, but more stromal interface haze.
  • Triple Procedure: Phacoemulsification + IOL + DMEK/DSAEK in the same sitting.

⚠️ Postoperative Considerations

  • Monitor intraocular pressure
  • Watch for graft dislocation
  • Long-term steroid therapy may be needed
  • Visual recovery is typically excellent if macula is healthy

🧾 FRCOphth Exam Tip

In OSCEs or viva, always assess the extent of corneal oedema and coexisting cataract. Be ready to justify your decision-making in sequential vs. combined approaches.

πŸ“š Summary

  • FED is a progressive endothelial dystrophy leading to corneal oedema.
  • The presence and severity of cataract and corneal changes determine management.
  • Triple procedure is ideal for advanced cases with coexisting cataract and oedema.
  • DMEK is the gold standard for endothelial replacement.

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