CASE REPORT
Case report of Cornea Verticillate a 16-year-old boy was referred to the department of ophthalmology at a hospital. During the investigation of Marfan Syndrome. He was under evaluation complaining of pain and paresthesia in both feet for six months.
He had been previously evaluated by a pediatrician and performed a rheumatologic screening with negative results.
He had no ophthalmological complaints and his past medical and ocular history were unremarkable. He also had no familial history of genetic disorders or ocular diseases, but the ophthalmological exam revealed uncorrected visual acuity of 20/20 in both eyes and slit-lamp evaluation disclosed bilateral cornea verticillata.
DISEASE of Cornea Verticillate
This disease also called vortex keratopathy, whorl keratopathy, or Fleischer vortex describes a whorl-like pattern of golden brown or gray opacities in the cornea. It is termed cornea verticillata from the Latin noun “verticillus,” meaning “whorl”.
Cornea Verticillata is most commonly associated with amiodarone and Fabry disease.
Usually asymptomatic, it is caused by the deposition of medication, material, and disease byproducts in the basal epithelial layer of the cornea.
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From Choroida — the team behind this siteMANAGEMENT of Cornea Verticillate
There is no recommended treatment for this disease. The deposits are typically not visually significant and typically resolve with the cessation of the responsible agent.
No alteration in medication regimen or further work-up is required for an isolated finding of cornea verticillata. Several case reports have described the resolution of cornea verticillata with the use of topical heparin.
If Cornea Verticillata is associated with a drug that is known to produce retinal toxicities—most notably hydroxychloroquine, chloroquine, chlorpromazine, and tamoxifen—patients should be routinely monitored with automated visual fields plus spectral-domain optical coherence tomography (SD-OCT).
The presence of Cornea Verticillata does not correlate with retinal toxicity. The possibility of optic neuropathy should also be considered if patients taking amiodarone or tamoxifen present with reduced vision.
For these patients, a reduction in dose or a switch to a different medication may be necessary.
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From Choroida — the team behind this siteREFERENCES
- Koh S, Hamano T, Ichii M, Yatsui K, Maeda N, Nishida K. Transient Cornea Verticillata of Unknown Etiology. Cornea. 2019; 38 (5): e16–e17. doi: 10.1097/ICO.0000000000001913.
- Raizman M. B., Hamrah P., Holland E. J., Kim T., Mah F. S., Rapuano C. J., & Ulrich R. G. (2017). Drug-induced corneal epithelial changes. Survey of ophthalmology, 62(3), 286–301. 10.1016/j.survophthal.2016.11.008.
- Shah GK, Cantrill HL, Holland EJ. Vortex keratopathy is associated with atovaquone. Am J Ophthalmol, 120 (5) (1995), pp. 669-671.
- D’Amico DJ, Kenyon KR. Drug-induced lipidoses of the cornea and conjunctiva. Int Ophthalmol, 4 (1–2) (1981), pp. 67-76.
- King JA, Affeldt JC, Agarwal MR. Etiologic profile of vortex keratopathy. Investigative Ophthalmology and Visual Science. 2003;44(13):1381.
- Chong EM, Campbell JR, Bourne WM. Vortex keratopathy in a patient with multiple myeloma. Cornea, 16 (5) (1997), pp. 592-594.
- Eng CM, Germain DP, Banikazemi M, et al. Fabry disease: guidelines for the evaluation and management of multi-organ system involvement. Genet Med, 8 (9) (2006), pp. 539-548.
Test yourself
A few questions straight from this article.
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Which corneal layer holds the deposits that produce cornea verticillata?
The whorl pattern arises from medication, material and disease byproducts depositing in the basal epithelial layer of the cornea. -
Cornea verticillata is most commonly associated with which drug and which metabolic disease?
The article names amiodarone and Fabry disease as the two commonest associations of cornea verticillata. -
Which set of alternative names refers to cornea verticillata?
Cornea verticillata is also called vortex keratopathy, whorl keratopathy or Fleischer vortex, all describing the same whorl-like corneal opacity pattern. -
What colour are the corneal opacities described in cornea verticillata?
The article describes cornea verticillata as a whorl-like pattern of golden brown or gray opacities in the cornea. -
What is the usual symptomatic impact of cornea verticillata on the patient?
Cornea verticillata is usually asymptomatic, and the deposits are typically not visually significant. -
What is the recommended treatment for isolated cornea verticillata?
There is no recommended treatment; the deposits are not visually significant and typically resolve once the responsible agent is stopped. -
Topical use of which agent has been reported to resolve cornea verticillata in case reports?
Several case reports have described resolution of cornea verticillata with topical heparin. -
Which drugs causing cornea verticillata also warrant routine retinal toxicity monitoring?
Hydroxychloroquine, chloroquine, chlorpromazine and tamoxifen are known to produce retinal toxicity, so these patients need routine surveillance. -
Which two tests monitor patients taking drugs with known retinal toxicity?
Patients on retinotoxic drugs should be routinely monitored with automated visual fields plus spectral-domain optical coherence tomography. -
What is the relationship between cornea verticillata and drug-induced retinal toxicity?
The presence of cornea verticillata does not correlate with retinal toxicity, so it cannot be used as a marker of retinal damage. -
Reduced vision in a patient taking amiodarone or tamoxifen should raise suspicion of which complication?
The possibility of optic neuropathy should be considered when patients on amiodarone or tamoxifen present with reduced vision, and dose reduction or a drug switch may be needed.
