Keratoendotheliitis Fugax Hereditaria: Causes, Symptoms, and Treatment

Keratoendotheliitis Fugax Hereditaria: Causes, Symptoms, and Treatment

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Focused Health Topics
Contributed byAlexander Enabnit+2 moreJun 22, 2023

Introduction

Keratoendotheliitis Fugax Hereditaria (KEFH) is a rare genetic disorder that affects the corneal endothelium, which is the innermost layer of the cornea. This article will explore the causes, symptoms, and treatments of KEFH.

KEFH is a rare genetic disorder that affects the corneal endothelium. The corneal endothelium is a single layer of cells that line the inner surface of the cornea, which is the transparent front part of the eye. The corneal endothelium helps to maintain the clarity of the cornea by regulating the amount of fluid that enters and leaves it.

Causes:

KEFH is caused by a genetic mutation that affects the function of the corneal endothelial cells, leading to recurrent episodes of corneal edema. It is an autosomal dominant genetic disorder, which means that it is caused by a mutation in a single copy of the gene inherited from one parent. The exact gene involved in KEFH is not yet known.

Symptoms:

The symptoms of KEFH are characterized by recurrent episodes of corneal edema, which is the swelling of the cornea due to the accumulation of fluid. These episodes can last from a few hours to a few days and usually resolve spontaneously. The symptoms of KEFH may include:

  • Blurred or hazy vision
  • Sensitivity to light
  • Halos or glare around lights
  • Mild discomfort or pain

Treatment

There is currently no cure for KEFH, but the symptoms can be managed with various treatment options. The treatment options for KEFH include:

  • Hypertonic saline drops: These are eye drops that help to draw fluid out of the cornea and reduce swelling.
  • Miotics: These are medications that help to constrict the pupil and improve the drainage of fluid from the cornea.
  • Corneal transplant: In severe cases of KEFH, a corneal transplant may be necessary to replace the damaged cornea with a healthy donor cornea.

Prevention

Since KEFH is a genetic disorder, there is no surefire way to prevent it. However, genetic counseling and testing can be useful for individuals with a family history of the disorder who are planning to have children.

Conclusion

KEFH is a rare genetic disorder that affects the corneal endothelium and leads to recurrent episodes of corneal edema. The symptoms of KEFH can be managed with various treatment options, including hypertonic saline drops, miotics, and corneal transplant. Genetic counseling and testing may be useful for individuals with a family history of KEFH who are planning to have children.

Hashtags: #KEFH #CornealEdema #GeneticDisorder #EyeHealth

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Alexander Enabnit picture
Author

Alexander Enabnit

Senior Editorial Staff
Alexandra Warren picture
Author

Alexandra Warren

Senior Editorial Staff

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