Corneal verticillata, also known as vortex keratopathy or whorl keratopathy, is a distinct ocular condition characterized by the appearance of whorl-like patterns on the cornea. These fine, golden-brown or grayish lines often spiral outward from the center of the cornea, forming a striking, yet subtle pattern that can be detected through an eye examination. Corneal verticillata is a non-inflammatory condition and is typically asymptomatic, meaning that it often goes unnoticed by those who have it. However, its presence can be indicative of underlying systemic conditions or the use of certain medications, making it an essential marker for eye care professionals.

The causes of corneal verticillata can be broadly classified into two categories: drug-induced and disease-related.

• Drug-Induced Corneal Verticillata: Many cases of corneal verticillata are associated with the long-term use of certain medications. The most commonly implicated drugs include:
  • Amiodarone: A medication used to treat arrhythmias or irregular heartbeats. Amiodarone deposits in the cornea lead to the formation of whorl-like patterns, typically within months of use.
  • Chloroquine and Hydroxychloroquine: These antimalarial drugs are also used to treat autoimmune conditions like lupus and rheumatoid arthritis. Prolonged use can cause corneal deposits that result in verticillata.
  • Tamoxifen: A drug used to treat breast cancer. It can also lead to corneal deposits, though these are less common.
  • Indomethacin: A non-steroidal anti-inflammatory drug (NSAID) used for arthritis, which can also cause this condition.
  • In most cases, drug-induced corneal verticillata is reversible and does not cause long-term damage. The deposits tend to disappear once the offending medication is discontinued.
• Disease-Related Corneal Verticillata: Apart from medications, corneal verticillata can also be a sign of an underlying metabolic disorder, most notably Fabry disease. Fabry disease is a rare, inherited disorder caused by the deficiency of an enzyme called alpha-galactosidase A. This enzyme deficiency leads to the buildup of glycosphingolipids in various tissues, including the cornea.
  Patients with Fabry disease often exhibit corneal verticillata as one of the early signs of the condition, making eye examinations crucial for diagnosis. Unlike drug-induced verticillata, the corneal changes seen in Fabry disease tend to persist, even with enzyme replacement therapy.

In most cases, corneal verticillata is asymptomatic, meaning that individuals may not experience any noticeable changes in their vision. This is because the deposits usually do not interfere with the optical path of light entering the eye. However, in rare instances where the deposits become denser or more widespread, patients may report mild symptoms such as:

• Blurred vision
• Halos or rings around lights
• Glare, especially at night

These symptoms are typically mild and do not progress to severe vision impairment. The condition is often discovered during routine eye exams, especially in patients taking the medications known to cause verticillata or those with Fabry disease.

Diagnosing corneal verticillata involves a comprehensive eye examination, including slit-lamp biomicroscopy, which allows the eye care professional to visualize the characteristic whorl-like patterns on the cornea. The spiraling lines are usually located in the inferior interpalpebral region of the cornea and are more visible under specific lighting conditions.

If corneal verticillata is detected, the eye doctor may ask about the patient’s medical history, focusing on the use of medications such as amiodarone, hydroxychloroquine, or tamoxifen. If no drug-related cause is identified, further investigation may be required to rule out metabolic disorders like Fabry disease.

In suspected cases of Fabry disease, additional diagnostic tests such as enzyme activity assays, genetic testing, and a systemic evaluation may be necessary to confirm the diagnosis. Early diagnosis of Fabry disease is essential to prevent complications and to initiate enzyme replacement therapy if appropriate.

In cases of drug-induced corneal verticillata, treatment typically involves discontinuing or switching the medication responsible for the deposits. Once the offending drug is stopped, the whorl-like patterns on the cornea often fade over time. The timeline for resolution can vary depending on the duration of drug use and the individual’s response to discontinuation. In most cases, the verticillata do not cause permanent damage to the cornea, and vision returns to normal without intervention.

For patients with Fabry disease, corneal verticillata is considered a marker for the systemic involvement of the condition. While enzyme replacement therapy is available to address the underlying enzyme deficiency in Fabry disease, the corneal deposits themselves are not typically treated unless they cause significant visual disturbances. Regular follow-up with an eye care professional is recommended to monitor the condition and ensure that no other ocular complications arise.

The long-term outlook for individuals with corneal verticillata is generally favorable. In drug-induced cases, the condition is reversible, and once the medication is discontinued, the cornea usually returns to its normal state without lasting effects on vision. Even in cases related to Fabry disease, corneal verticillata tends to remain stable over time and does not lead to progressive vision loss.

However, for individuals with Fabry disease, it is essential to recognize that corneal verticillata may be just one of the many systemic manifestations of the condition. Early detection and management of Fabry disease can help prevent more serious complications affecting the heart, kidneys, and nervous system.

Corneal verticillata, while often asymptomatic, is a valuable clinical sign that can provide insights into a patient’s medication use or underlying systemic conditions. For patients taking medications like amiodarone or hydroxychloroquine, regular eye exams are essential to monitor for the development of verticillata. In cases where Fabry disease is suspected, corneal verticillata can serve as an early diagnostic clue, prompting further investigation and treatment. Ultimately, corneal verticillata is a manageable condition with a good prognosis, especially when addressed early by an eye care professional.

World Eye Care Foundation’s eyecare.live brings you the latest information from various industry sources and experts in eye health and vision care. Please consult with your eye care provider for more general information and specific eye conditions. We do not provide any medical advice, suggestions or recommendations in any health conditions.