Cornea verticillata

Morbus Fabry Cornea verticillata 01

Cornea verticillata, also known as vortex keratopathy, is a condition characterized by the accumulation of lipid or other substances in the corneal epithelium, leading to a distinctive whorl-like pattern on the cornea. This condition can be associated with various systemic diseases and medications, and while it primarily affects the cornea's appearance, it rarely impacts vision significantly.

Causes and Associations

Cornea verticillata is most commonly associated with the use of certain medications, particularly Amiodarone, an antiarrhythmic agent used to treat various types of cardiac arrhythmias. Other drugs that have been linked to this condition include chloroquine, hydroxychloroquine, indomethacin, tamoxifen, and fabry disease, a genetic disorder. In the case of Fabry disease, cornea verticillata is considered a hallmark sign and can be an important clue in the diagnosis of this condition.

Clinical Presentation

Patients with cornea verticillata typically present with a bilateral, symmetric whorl-like pattern of corneal deposits that originate from the central or paracentral cornea and radiate outward. These deposits are best visualized with slit-lamp examination, a common ophthalmologic diagnostic tool. Despite the apparent changes in the cornea, most patients remain asymptomatic, with no significant impact on visual acuity. However, in rare cases, patients may report slight blurring of vision or halos around lights.

Diagnosis

The diagnosis of cornea verticillata is primarily clinical, based on the characteristic appearance of the corneal deposits during slit-lamp examination. Additional tests are generally not necessary but may be conducted to investigate potential underlying causes, such as Fabry disease or adverse effects from medication use.

Management and Treatment

Management of cornea verticillata focuses on monitoring the condition and addressing the underlying cause. If the corneal deposits are linked to medication use, discontinuation or substitution of the medication, under the guidance of a healthcare professional, may lead to the resolution of the deposits. However, this decision must be carefully weighed against the benefits of the medication for the underlying condition. In cases associated with Fabry disease, enzyme replacement therapy may help in reducing corneal deposits.

Prognosis

The prognosis for patients with cornea verticillata is generally good, as the condition rarely affects vision. However, monitoring is essential to ensure that any potential underlying diseases are managed appropriately.