Endothelial corneal dystrophy
What is it?
Fuchs’ endothelial dystrophy is a non-inflammatory, sporadic or autosomal dominant, dystrophy involving the endothelial layer of the cornea. With Fuchs’ dystrophy the cornea begins to swell causing glare, halo, and reduced visual acuity. The damage to the cornea in Fuchs’ endothelial dystrophy can be so severe as to cause corneal blindness.
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Endothelial corneal dystrophy.
Additional names
This group contains additional names:
- Fuchs’ Endothelial Dystrophy
Signs & symptoms
Some of the earliest symptoms of Fuchs’ endothelial dystrophy include reduced contrast sensitivity and mild reduction of visual acuity. Patients will often notice glare around a point source of light or have difficulty with nighttime driving. As the dystrophy is often slow in onset, patients may not even notice that their vision is reduced. Once fluid begins to collect in the stroma, patients will start to notice fluctuation in vision, typically worse in the early AM and improving toward the end of the day. Vision may be worse on humid or rainy days, and better on dry days. Eventually the vision stops fluctuating and becomes constantly blurry. Slowly, the vision continues to worsen and eventually intermittent pain can be felt if bullae form and rupture leaving corneal epithelial defects.
Diagnosis
The diagnosis of Fuchs’ endothelial dystrophy is clinical. The diagnosis is relatively easy in early disease as you can see the endothelial changes as will as mild corneal stromal edema. In severe cases, where you cannot see the endothelium, the diagnosis can be more challenging and the diagnosis may need to be based on the contralateral eye or by history.
Diagnostic procedures
The diagnosis of Fuchs’ endothelial dystrophy is clinical; however, there are some diagnostic tests that can be helpful. Pachymetry, or measurement of the central corneal thickness, is helpful in following a patient with Fuchs’ dystrophy. Over time you will see increasing corneal thickness as the disease worsens. The rate at which you see increasing corneal thickness can help with counseling patients. The corneal thickness also may help with risk/benefit analysis of any other surgery that may be necessary (such as cataract surgery). Endothelial cell counts can also be helpful when counseling patients as to how quickly their dystrophy may progress as well as how safe any other intra-ocular surgery may be. In even moderate Fuchs’ dystrophy, the cell count can be very difficult to obtain. Evaluation of the endothelium by specular microscopy can demonstrate classic changes of Fuchs' endothelial dystrophy, including guttatta, variation in cell size and shape, and low cell count per unit area.
Laboratory test
Fuchs’ endothelial dystrophy is diagnosed clinically. Specular microscopy to visualize the endothelium can corroborate the typical endothelial changes associated with this dystrophy.
Treatment
Medical treatment of Fuchs’ dystrophy begins once patients notice fluctuations in vision. The early treatment is usually in the form of hypertonic saline (such as Muro 128 or sodium chloride) eye drops and/or ointments. Use of the hypertonic saline may stabilize or improve vision by drawing extra water out of the cornea. Any activity that helps to evaporate fluid off the cornea will help shorten the time to visual recovery. Such activities may include pointing car vents toward the face or blowing air by the eyes using a hair dryer at arm's length. Bandage contact lenses can also be quite helpful in management of painful ruptured bullae in more severe disease.
As Fuchs’ dystrophy progresses, medical treatment may fail and surgical management becomes necessary.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
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Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:
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