Corneal endothelium dystrophy

Corneal Endothelium Dystrophy is a group of genetic disorders affecting the cornea, specifically the innermost layer known as the corneal endothelium. These disorders are characterized by the progressive loss of endothelial cells, which are essential for maintaining the cornea's clarity and thickness. The dysfunction or degeneration of these cells leads to corneal edema (swelling), resulting in vision impairment or loss. Corneal Endothelium Dystrophy is primarily classified into three main types: Fuchs' Endothelial Corneal Dystrophy (FECD), Posterior Polymorphous Corneal Dystrophy (PPCD), and Congenital Hereditary Endothelial Dystrophy (CHED).

Types

Fuchs' Endothelial Corneal Dystrophy (FECD)

FECD is the most common form of Corneal Endothelium Dystrophy, typically manifesting in the later stages of life. It is characterized by the gradual thinning and loss of endothelial cells, leading to the accumulation of fluid within the cornea. This condition is more prevalent in women than in men.

Posterior Polymorphous Corneal Dystrophy (PPCD)

PPCD is a rare condition that can present at any age, characterized by abnormal growth of endothelial cells. These cells take on an epithelial-like appearance and function improperly, leading to corneal swelling and blisters on the cornea's surface.

Congenital Hereditary Endothelial Dystrophy (CHED)

CHED is a rare, inherited disorder present at birth. It results in severe corneal clouding due to the dysfunction of endothelial cells, significantly affecting vision from a young age.

Symptoms

Common symptoms of Corneal Endothelium Dystrophy include:

• Blurred vision
• Sensitivity to light
• Pain and discomfort in the eye
• A feeling of grittiness or a foreign body sensation in the eye
• Visual distortion

Diagnosis

Diagnosis of Corneal Endothelium Dystrophy involves a comprehensive eye examination, including:

• Visual acuity test
• Slit lamp examination
• Pachymetry to measure corneal thickness
• Specular microscopy to assess the condition of the endothelial cells

Treatment

Treatment options vary depending on the severity of the condition and may include:

• Saline solutions and eye drops to manage symptoms
• Contact lenses to improve vision
• Corneal transplantation in severe cases, such as Penetrating Keratoplasty (PK) or Endothelial Keratoplasty (DSEK, DMEK)

Prevention

There is no known prevention for Corneal Endothelium Dystrophy as it is largely a genetic condition. However, regular eye examinations can help in early detection and management of the disease.

Corneal endothelium dystrophy Resources
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