Keratoendotheliitis fugax hereditaria

Keratoendotheliitis Fugax Hereditaria is a rare, hereditary eye condition characterized by transient episodes of corneal inflammation and opacities. This condition primarily affects the corneal endothelium, leading to temporary vision disturbances.

Clinical Presentation

Patients with Keratoendotheliitis Fugax Hereditaria typically experience recurrent episodes of corneal edema and opacities. These episodes can cause symptoms such as blurred vision, photophobia, and mild eye discomfort. The opacities usually resolve spontaneously within a few days, but they may recur periodically.

Pathophysiology

The exact pathophysiological mechanism of Keratoendotheliitis Fugax Hereditaria is not fully understood. It is believed to involve an inflammatory process affecting the corneal endothelium, possibly triggered by environmental factors or stress. The hereditary nature of the condition suggests a genetic component, although specific genetic mutations have not been conclusively identified.

Diagnosis

Diagnosis of Keratoendotheliitis Fugax Hereditaria is primarily clinical, based on the characteristic episodic nature of the corneal opacities and the patient's family history. Slit-lamp examination during an episode can reveal the presence of corneal edema and opacities. Other diagnostic tests, such as corneal topography and specular microscopy, may be used to assess the corneal endothelium.

Management

There is no specific treatment for Keratoendotheliitis Fugax Hereditaria. Management focuses on alleviating symptoms during episodes. Patients may be advised to use topical corticosteroids or nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation and discomfort. Regular follow-up with an ophthalmologist is recommended to monitor the condition.

Prognosis

The prognosis for individuals with Keratoendotheliitis Fugax Hereditaria is generally good, as the condition does not typically lead to permanent vision loss. However, the recurrent nature of the episodes can impact the quality of life, and patients may require ongoing management to control symptoms.

Related Pages

• Corneal diseases
• Hereditary eye diseases
• Corneal endothelium

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  Keratoendotheliitis fugax hereditaria