Fuchs endothelial corneal dystrophy
The cornea's inner pump cells slowly fail, so the cornea swells and vision is worst on waking. A thin endothelial transplant restores clarity.
What it is
An inherited, slowly progressive loss of corneal endothelial cells, with wart-like deposits (guttae) on Descemet membrane. As the endothelial pump fails, the cornea swells, first in the morning, and vision becomes cloudy with glare. It is the most common indication for corneal transplantation in many countries.
Who gets it
Usually appears after 50, more often in women; often familial.
Causes
- Genetic predisposition (often linked to the TCF4 gene)
- Age-related endothelial decline
Risk factors
- Female sex
- Age over 50
- Family history
- Cataract surgery can unmask it
Pathophysiology
Abnormal endothelial cells produce guttae and die faster than normal. Remaining cells enlarge but eventually cannot pump fluid out of the stroma; overnight, with eyes closed, less water evaporates, so swelling and blur are worst on waking. Later, blisters form under the epithelium and rupture painfully.
Classification
- Early: guttae without swelling
- Moderate: stromal oedema, morning blur
- Advanced: epithelial bullae, scarring
Symptoms and signs
- Blurred vision worst on waking, improving during the day
- Glare and halos
- Pain from ruptured epithelial blisters in advanced disease
Diagnosis
- Clinical
- Slit lamp shows guttae (a 'beaten-metal' endothelium) and stromal or epithelial oedema.
- Tests
- Corneal thickness measurement (pachymetry) and endothelial cell count (specular microscopy).
- Imaging
- Specular microscopy and corneal tomography document severity.
Differential diagnosis
- Corneal oedema after cataract surgery
- Other endothelial dystrophies
- Herpetic endotheliitis
- Acute angle closure
Goal of treatment
Keep the cornea clear and, when vision is limited, replace the endothelium.
Treatment
- Conservative
- Hypertonic saline drops and ointment and, in the morning, gently drying the cornea (for example with warm air) to reduce swelling.
- Surgical
- Endothelial keratoplasty (transplant of the endothelium and Descemet membrane only) for visually significant disease.
- Surgical
- Planning of combined or staged cataract and corneal surgery.
Complications
- Corneal decompensation
- Painful bullous keratopathy
- Corneal scarring
Prognosis
Slowly progressive; endothelial keratoplasty gives rapid, good visual recovery in most patients.
Test yourself
Think of the answer, then tap the question to see it.
Why is vision worst on waking in Fuchs dystrophy?
Because with the eyes closed overnight less water evaporates and the failing endothelium cannot clear the corneal swelling.
What are guttae?
Wart-like deposits on Descemet membrane produced by abnormal endothelial cells.
What is the surgical treatment?
Endothelial keratoplasty, replacing only the diseased endothelium.
In ophthalmology
- Corneal transplant (keratoplasty) and the artificial cornea
- Corneal topography, tomography and specular microscopy
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Fuchs Endothelial Dystrophy (StatPearls Publishing (NCBI Bookshelf), 2025).
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Representative model, for educational purposes only.