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FUCHS ENDOTHELIAL DYSTROPHY

Writer's picture: Munib ur RehmanMunib ur Rehman

Updated: Feb 9, 2024


INHERITANCE PATTERN

AD (OR SPORADIC)

 

VISUAL ACUITY

Grossly affected in advanced cases.

It is worse in the morning and on a humid or rainy day.

 

CORNEA

Multiple corneal guttae on Descemet's membrane – often more marked in the central cornea – can be seen only in the early stage of dystrophy.

Stromal edema

Endothelial folds

Corneal haze and edema (thickening)

Microcysts or Bullae may be seen in the epithelium –

Fluorescein Staining - Microcysts are best seen after fluorescein is placed on the cornea, leaving areas of negative staining, whereas when a bulla has ruptured, its base is stained with fluorescein.

Sub-epithelial fibrosis and corneal vascularization – chronic corneal edema


Also Perform,

Pachymetry

Specular microscopy

 

LENS

Intraocular lens implant

(The dystrophy may be exacerbated by the implant, especially in the anterior chamber.

The patient alternatively may have pseudophakic bullous keratopathy.

 

FELLOW EYE

The condition is bilateral.

 

QUESTIONS:

 

How do you differentiate between Fuchs endothelial dystrophy and Pseudophakic bullous keratopathy?

Clinically, Fuchs endothelial dystrophy is indistinguishable from pseudophakic bullous keratopathy. Fuchs endothelial dystrophy tends to be bilateral.


What factors are responsible for the integrity of the cornea?

The water content within the cornea is delicately balanced by opposing forces. Here's how it works:

A. Inward Forces:

Swelling Pressure of the Stroma: The stromal layer of the cornea exerts pressure, pushing water inward.

Intraocular Pressure: The pressure inside the eye also contributes to water influx.

 

B. Factors Preventing Swelling: 

Endothelial Barrier and Metabolic Pump: The endothelium (innermost layer) acts as a barrier, preventing excessive water entry. It also maintains the cornea's health through a metabolic pump mechanism. 

Epithelial Barrier: The outermost epithelial layer provides additional protection against swelling.

Evaporation Control: Although less significant, evaporation from the corneal surface plays a role in maintaining equilibrium.

 

Consequences of Dysfunction:

If any of these factors fail or become damaged, corneal edema (swelling) occurs, increasing corneal thickness.


How would you treat Fuchs dystrophy?

The early stage requires no treatment, but caution must be exercised in considering such patients for cataract surgery, which may precipitate corneal decompensation. Early stromal edema may be helped by hyperosmotic agents and the use of warm, dry air to aid evaporation. Treat any ocular hypertension. The advanced disease requires a corneal graft.

 

What are the environmental risk factors for FED?

Environmental risk factors include smoking and body mass index.

Increasing visual deterioration may develop, sometimes weeks or months, after a successful Nd: YAG laser surgery for a PCO.

 

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