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CLINICAL MANAGEMENT GUIDELINES
Fuchs Endothelial Corneal Dystrophy
Condition
Aetiology
Predisposing
factors
Symptoms
Signs
Fuchs Endothelial Corneal Dystrophy
 thickening of posterior part of Descemet’s membrane with localised
excrescences (guttata)
 thinning, degeneration and loss of endothelial cells
 reduced endothelial function produces corneal oedema
 resultant stromal and epithelial oedema leads to epithelial bullae
 supposed association with OHT / open angle glaucoma is controversial
 cases with no known inheritance are most common, although an
autosomal dominant inheritance pattern has been described
 usually a slowly progressive disease of older patients (symptoms rare
under age 50)
 smoking
 one of the more common corneal dystrophies, especially in women
 a rare early-onset variant begins in the first decade of life
 none in the early stages
 glare and blurred vision especially on waking (symptoms accentuated by
overnight corneal oedema)
 diurnal changes in refraction (relatively myopic on waking)
 sharp pain (when epithelial bullae rupture)
 bilateral (may be asymmetrical)
 central cornea affected (may extend to periphery in time)
o cystic epithelial oedema, ground glass appearance (bullous
keratopathy)
o stromal oedema and thickening (endothelial decompensation)
o thickened Descemet’s membrane
o fine pigment dusting on endothelium
o excrescences on posterior surface (guttata) produce ‘beaten
metal’ appearance and interrupt specular image of endothelium
 loss of contrast sensitivity
 reduced corneal sensitivity
The stages of Fuchs Dystrophy have been graded by a number of authorities,
for example by Krachmer, whose system has six levels
Differential
 pseudophakic or aphakic bullous keratopathy
diagnosis
 Posterior Polymorphous Dystrophy
 Cornea guttata (often listed as a dystrophy, but not necessarily
progressive)
 Corneal hydrops (in keratoconus)
 contact lens overwear
Management by Optometrist
Practitioners should recognise their limitations and where necessary seek further advice or refer the
patient elsewhere
Non
 photography and imaging, including specular microscopy (if available)
pharmacological
 measurement of central corneal thickness (CTT) for monitoring
progression of disease (see evidence base)
 where there is pain, a therapeutic contact lens may protect exposed
corneal nerves and reduce friction between lid margins and bullae on
blinking (see evidence base)
 monitor for glaucoma and/or ocular hypertension (controversial: see
evidence base)
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
Fuchs Endothelial Corneal Dystrophy
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Date of search 08.11.14; Date of revision 16.12.14; Date of publication 16.02.15; Date for review 07.11.16
© College of Optometrists
CLINICAL MANAGEMENT GUIDELINES
Fuchs Endothelial Corneal Dystrophy
Pharmacological Gutt. sodium chloride 5% (in severe cases with persistent oedema) applied in
the mornings and as necessary. The dosage will need to be varied as
usefulness or otherwise in controlling the patient’s symptoms becomes apparent
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
B1: initial management, possible routine referral (modified statement)
Management
Category
Possible management by Ophthalmologist
 confirmation of diagnosis
 photography and imaging, including specular microscopy
 measurement of CTT for monitoring progression of disease
 repeat assessment to establish progression
 when condition no longer responsive to management of symptoms, may
offer surgery
o posterior lamellar transplantation (e.g. Descemet’s Stripping
Automated Endothelial Keratoplasty, DSAEK; Descemet’s
Stripping Endothelial Keratoplasty, DSEK; Descemet’s Membrane
Endothelial Keratoplasty, DMEK)
o penetrating keratoplasty
o either of the above procedures may be combined with cataract
surgery if this is clinically indicated
(NB: evidence from randomised controlled clinical trials [RCTs] does not support
superiority of newer endothelial keratoplasty procedures over traditional
penetrating keratoplasty)
Evidence base
*GRADE: Grading of Recommendations Assessment, Development and
Evaluation (see http://www.gradeworkinggroup.org/index.htm)
Sources of evidence
Coster DJ, Lowe MT, Keane MC, Williams KA; Australian Corneal Graft Registry
Contributors. A comparison of lamellar and penetrating keratoplasty outcomes: a
registry study. Ophthalmology 2014;121(5):979-87
Knezović I, Dekaris I, Gabrić N, Cerovski J, Barisić A, Bosnar D, Rastegorac P,
Parać A. Therapeutic efficacy of 5% NaCl hypertonic solution in patients with
bullous keratopathy. Coll Antropol. 2006;30:405-8
Nanavaty MA, Wang X, Shortt AJ. Endothelial keratoplasty versus penetrating
keratoplasty for Fuchs endothelial dystrophy. Cochrane Database Syst Rev.
2014;2:CD008420
Nagarsheth M, Singh A, Schmotzer B, Babineau DC, Sugar J, Lee WB, Ivengar
SK, Lass JH; Fuchs Genetics Multi-Center Study Group. Relationship Between
Fuchs Endothelial Corneal Dystrophy Severity and Glaucoma and/or Ocular
Hypertension. Arch Ophthalmol. 2012;130:1384-8
Kopplin LJ, Przepyszny K, Schmotzer B et al (for the Fuchs Endothelial Corneal
Dystrophy Genetics Multi-Center Study Group). Relationship of Fuchs
Endothelial Corneal Dystrophy Severity to Central Corneal Thickness. Arch
Ophthalmol. 2012;130:433-9
Rice GD, Wright K, Silverstein SM. A retrospective study of the association
Fuchs Endothelial Corneal Dystrophy
Version 11, Page 2 of 3
Date of search 08.11.14; Date of revision 16.12.14; Date of publication 16.02.15; Date for review 07.11.16
© College of Optometrists
CLINICAL MANAGEMENT GUIDELINES
Fuchs Endothelial Corneal Dystrophy
between Fuchs’ endothelial dystrophy and glaucoma. Clin Ophthalmol.
2014;8:2155-9
Note on Corneal Dystrophies
Corneal dystrophies are progressive, usually bilateral, mostly inherited, alterations in corneal
transparency that develop without inflammation. In the past, the nomenclature of the many
dystrophies has often caused confusion. A new classification (the IC3D Classification) has been
proposed which integrates up-to-date information on phenotypic description, histopathological
examination and genetic analysis:
http://www.corneasociety.org/sites/default/files/publications/ic3d_class_cornealdystrophies.pdf
In everyday clinical practice, the most commonly encountered corneal dystrophies are Epithelial
Basement Membrane Dystrophy (Map-Dot-Fingerprint Dystrophy, Cogan’s Dystrophy), which affects
the epithelium and predisposes to Recurrent Corneal Epithelial Erosion (see the Clinical Management
Guideline of that name) and Fuchs Endothelial Corneal Dystrophy, the subject of this Clinical
Management Guideline.
LAY SUMMARY
A corneal dystrophy is a condition in which the cornea loses its normal transparency. It is usually
inherited, affects both eyes and is progressive. There is no inflammation or infection. This particular
corneal dystrophy was first described by an Austrian ophthalmologist, Ernst Fuchs, in 1910, and it has
borne his name ever since. In this condition the cells lining the back of the cornea (the endothelial
cells), which normally pump water out of the cornea, keeping it clear, no longer work properly. As a
result the cornea becomes water-logged and cloudy and the vision becomes hazy. The condition is
painless at first, but if, at a later stage, blisters form on the surface of the cornea and then burst, sharp
pain may result. No treatment is available to reverse this condition but if the vision is greatly affected
or the eye is painful, the ophthalmologist may recommend a corneal transplant procedure. This has a
good chance of improving the vision and making the eye comfortable.
Fuchs Endothelial Corneal Dystrophy
Version 11, Page 3 of 3
Date of search 08.11.14; Date of revision 16.12.14; Date of publication 16.02.15; Date for review 07.11.16
© College of Optometrists