Endothelial Corneal Dystrophy of Fuchs

Endothelial Corneal Dystrophy of Fuchs: An Overview

Endothelial Corneal Dystrophy of Fuchs (Fuchs' Dystrophy) is a genetic disorder that affects the endothelium, a layer of cells located at the back of the cornea, the clear outer layer of the eye that covers the iris and pupil. This disorder is caused by mutations in the COL8A2 gene, which is responsible for the production of type VIII collagen, a protein that helps to keep the cornea clear.

Symptoms and Diagn

Fuchs' Dystrophy typically presents in middle age and presents with symptoms such as:

  • Blurred vision, especially in the morning or after prolonged reading or computer use
  • Glare and halos around lights
  • Photophobia
  • Eye discomfort or pain
  • Sensitivity to bright lights

To diagnose Fuchs' Dystrophy, a comprehensive eye examination is typically performed. An ophthalmologist will examine the cornea using a slit lamp and may perform a corneal endothelial cell count to determine the health and density of the cells in the endothelial layer. Genetic testing may also be done to confirm the diagnosis and identify the specific gene mutation responsible for the disorder.

Types and Stages of Fuchs' Dystrophy

Fuchs' Dystrophy is classified into two types: Fuchs' Endothelial Corneal Dystrophy (FECD) and Posterior polymorphous corneal dystrophy (PPCD).

Fuchs' Endothelial Corneal Dystrophy (FECD): This is the most common form of Fuchs' Dystrophy, characterized by a gradual loss of endothelial cells which leads to a buildup of fluid in the cornea, resulting in corneal swelling and haze. This type is progressive and can cause visual impairment if left untreated.

Posterior polymorphous corneal dystrophy (PPCD) : This is a rarer form of Fuchs' Dystrophy, characterized by abnormal thickening and abnormal shape of the posterior corneal layers, it can be inherited in an autosomal dominant pattern and can cause corneal edema, visual impairment, and irregular astigmatism.

Fuchs’ dystrophy is classified in three stages:

  1. Asymptomatic stage: In this stage, there is no visible sign of corneal edema and no symptoms.
  2. Symptomatic stage: In this stage, the individual will start experiencing symptoms like blurred vision, glare, or halos around lights. The cornea is slightly thickened, and there may be visible guttae (small, yellowish spots) in the corneal endothelium.
  3. Advanced stage: In this stage, corneal edema is present, leading to severe visual impairment or blindness.

Treatment and Management

The treatment of Fuchs' Dystrophy varies depending on the type and stage of the disorder.

  1. Asymptomatic stage: In this stage, there is usually no treatment required, and the individual will be monitored for progression of the disorder.
  2. Symptomatic stage: In this stage, treatment options may include the use of hypertonic saline solutions and topical hyperosmotic agents to reduce corneal swelling, and medications to reduce the risk of infection. In more severe cases, a corneal transplant, specifically a Descemet's Stripping Automated Endothelial Keratoplasty (DSAEK) may be recommended.

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