Cornea

Fuchs' Corneal Dystrophy

Overview

Fuchs' corneal dystrophy is an inherited condition that affects the delicate inner layer (endothelium) of the cornea, the transparent dome of layered cells overlying the colored iris of the eye. The endothelium functions as a pump mechanism, constantly removing fluids from the cornea to maintain its clarity. Patients gradually lose these endothelial cells as the Fuchs' corneal dystrophy progresses. Once lost, the endothelial cells do not grow back, but instead spread out to the fill empty spaces. The pump system becomes less efficient and the fluid starts to build up, causing the cornea to become swollen and cloudy, losing its crystal-clear transparency.

 

Fuchs' dystrophy usually develops over two stages:

  • Stage 1: In this early stage, the dystrophy may produce no symptoms or only mild symptom such as glare and light sensitivity. The vision may seem blurred in the morning and sharper later in the day because closing your eyes during sleep keeps moisture from evaporating out of the cornea. Swelling of the corneal cells occurs in the morning then tends to clear as the day progresses.

  • Stage 2: Once the disease has progressed to Stage 2, vision no longer gets better later in the day. People with Stage 2 Fuchs' dystrophy may have pain and sensitivity to light. Extreme climate conditions such as high humidity can worsen the condition.

Over time, some people with Stage 2 Fuchs' dystrophy develop scarring at the center of their cornea. The patient may become more comfortable once scarring is present, but the scar tissue over the cornea reduces vision.

It can take 10 to 20 years or longer for Fuchs' dystrophy to progress from its early to late stage. If the end stage of Fuchs' dystrophy results in significant vision loss, Dr. Kim may recommend DMEK corneal transplant surgery to restore vision.

Symptoms

Symptoms of Fuchs' dystrophy may include:

  • Hazy or cloudy vision that is often most pronounced in the morning

  • Glare when looking at lights

  • Light sensitivity

  • Sandy, gritty sensation

  • Fluctuating vision

  • Halos around certain objects

  • Reduced visual acuity

  • Reduced ability to discern contrasts

  • Difficulty driving at night

  • Pain in the eye, if the condition is more advanced and blisters have formed

Detection and Diagnosis

Fuchs' dystrophy is detected by examining the cornea with a slit lamp microscope that magnifies the endothelial cells. The health of the endothelium is evaluated and monitored by measuring the thickness of a cornea, known as pachymetry.

Treatment

Treatment of Fuchs' dystrophy will vary depending on the stage at which it was diagnosed. The frequency of follow-up visits to your doctor will also vary. Early stage Fuchs' dystrophy may involve annual visits, but more advanced cases or certain treatments may call for visits every few months or even more frequently.

Fuchs' dystrophy cannot be cured, but certain medications control corneal swelling and resulting blurred vision. Salt solutions such as sodium chloride drops or ointment are often prescribed to draw fluid from the cornea and to reduce swelling. In advanced cases, partial thickness corneal transplant, DMEK surgery, may be advised.

 

Only a thin layer of Descemet’s membrane and endothelium is replaced in DMEK surgery, allowing for an exact anatomical replacement of the damaged cells. No sutures are used, as a specialized air bubble technique will secure the new corneal tissue.

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