Kayser-Fleischer rings are caused by copper deposition in the Descemet membrane of the cornea. This copper accumulation occurs due to an excess of copper in the body, which stems from conditions like cholestasis, liver disease, or errors in copper metabolism.
Understanding the Mechanism
Kayser-Fleischer rings are visually distinctive. They appear as brown or grayish-green rings that encircle the peripheral cornea, overlying the iris.
- Copper Deposition: The root cause is the buildup of copper within the Descemet membrane, a layer in the cornea.
- Underlying Conditions: Several medical conditions can lead to this abnormal copper accumulation:
- Cholestasis: This refers to a reduction or blockage of bile flow from the liver. The impaired bile flow disrupts the body's ability to remove copper, leading to a buildup.
- Liver Disease: Various liver diseases can impede the processing and excretion of copper. When the liver's function is impaired, copper builds up in other tissues, including the cornea.
- Errors in Copper Metabolism: Inborn metabolic errors disrupt the normal handling of copper, causing an excess of copper in the system, which may manifest as Kayser-Fleischer rings.
Visual Manifestation
These rings are not always easily visible to the naked eye, sometimes requiring specific examination techniques such as a slit-lamp examination by an ophthalmologist to be fully appreciated.
Treatment and Management
Addressing the underlying cause is the primary approach to managing Kayser-Fleischer rings. This usually involves treating the condition that is causing copper to accumulate, such as liver disease.
| Cause | Description |
|---|---|
| Cholestasis | Reduced or blocked bile flow leading to copper accumulation. |
| Liver Disease | Liver dysfunction impairs copper processing and excretion. |
| Copper Metabolism Error | Inborn metabolic errors disrupt copper handling, resulting in a buildup. |
