Drug-Induced Cornea Verticillata : About 2 Cases.

Cornea verticillata describes a whorl-like pattern of golden-brown or gray opacities in the corneal epithelium. It is called cornea verticillata from the Latin name “verticillus”, which means “whorl”.

Usually asymptomatic, it is caused by the deposition of drugs, metabolic substrates, or disease byproducts in the basal epithelial layer of the cornea.

It is most associated with amiodarone and Fabry disease. 

It can also be caused by a variety of other drugs, the most common of which are: Chloroquine and Hydroxychloroquine ; Indomethacin ; Phenothiazines.

we report two cases of drug - induced cornea verticillata (Amiodarone ; Hydroxychloroquine), to understand its pathophysiology, describe its clinical classification and discuss its treatment.

The first case is a 57 year old male with a known history of atrial fibrillation and hypertension, under amiodarone 200mg/d for 7 years.

the seconde one is a 65-year-old woman with a known history of atrial fibrillation and rheumatoid arthritis, under amiodarone 200mg/d for 5 years and Hydroxychloroquine 200 mg/d for 10 years.

Both patients presented a bilateral and progressive decrease in visual acuity. 

Corneal whorls are recognizable by fine golden-brown or gray opacities in the basal epithelium that branch out from a central whorl, usually through the inferior cornea. The deposits do not stain and are almost always bilateral.

Three grades have been described:

Grade 1: inferior linear deposits,
Grade 2: appearance like cat's whiskers;
Grade 3: appearance occupying most of the cornea.
The slit lamp examination found: grade 1 in the first case and grade 2 in the second one.

Cornea verticillata results from the centripetal migration of deposition-laden limbal stem cells as the corneal epithelium undergoes natural growth and repair. The drugs that produce this pathology share cationic and amphiphilic properties that allow them to enter the lysosomes of the basal epithelial layer of the cornea, where they bind to cellular lipids.

These drug-lipid complexes resist enzymatic degradation and accumulate as deposits in the cornea. Specifically, amiodarone and hydroxychloroquine inhibit lysosomal phospholipase A2. 

At an effective tissue concentration, 100% of patients will present (after six months of treatment with amiodarone) with bilateral corneal involvement. Regarding hydroxychloroquine, a cumulative dose greater than 1000 grams, a treatment duration greater than 5 years or a daily dose greater than 400 mg/day or 6.5 mg/kg/day increases the risk of corneal and retinal toxicity.

There is no recommended treatment for Cornea verticillata. The deposits are generally not visually significant and generally disappear with the cessation of the causative agent. No change in medication regimen is necessary for an isolated finding of whorled cornea. Several case reports have described resolution of Cornea verticillata with the use of topical heparin.