[OCT Article] Multimodal Analysis of Cornea Verticillata by OCT

Anamnesis

We report the case of an 80-year-old female patient, without specific complaints, presenting with bilateral inferior keratopathy affecting the basal membrane of the epithelium, with a fern-like distribution. The patient has a history of treatment with amiodarone.

Clinical Results

Biomicroscopy reveals fine golden-brown or gray opacities in the basal epithelium that branch in the inferior cornea. The deposits do not stain with fluorescein examination and are bilateral.

High-Resolution Corneal OCT B-scan with Solix® shows, at the apex, a pseudo-duplication aspect of Bowman's membrane, appearing hyporeflective and associated with hyperreflectivity of the basal membrane of the corneal epithelium in both eyes.

The Enhanced Cornea mode, with a 200% zoom on the Y-axis, highlights more clearly the pseudo-duplication of Bowman's membrane and the abnormal reflectivity of the basal membrane of the corneal epithelium, as well as subtle superficial involvement of the anterior stroma.

The corneal en face OCT (3D Cornea, 8 × 8 mm), with a slab focused on the corneal epithelium, reveals diffuse opalescence of the epithelium with a fern-like appearance, involving the basal membrane. The opacity is more pronounced in the inferior region, with a distribution that appears more transverse and symmetrical in both eyes.

The B-scan provides information on the depth of the involvement, while the en face OCT offers insights into the distribution of the condition, constituting a multimodal OCT approach alongside clinical and biomicroscopic examination.

This aspect is compatible with a vortex keratopathy (cornea verticillata) of exogenous origin related to the use of amiodarone.

This aspect can also be found with an endogenous origin in the context of Fabry disease, a rare X-linked disorder caused by an enzymatic deficiency in α-galactosidase A. It involves the accumulation of glycosphingolipids (Gb3) in lysosomes, leading to tissue and organ damage. Moreover, this chronic disease can present with multisystemic manifestations, including ophthalmological signs such as lens opacities, conjunctival vessel telangiectasias and tortuosities, which are associated with vortex keratopathy.

Conclusion

High-resolution B-scan OCT and "en face" OCT imaging allow us to enhance the semiology of this condition, which affects not only the epithelium but also Bowman's membrane. OCT quantification will enable better monitoring of these corneal deposits during amiodarone therapy.

References

1.Kaya E, Yılmaz SG, Onay MP, Eğrilmez S, Yağcı A. Correlation of Biomicroscopic Findings with Confocal Microscopy in Eyes with Amiodarone-Induced Cornea Verticillata. Turk J Ophthalmol. 2014 Jan;44(1):63-67. doi:10.4274/tjo.98704.

2.Falke K, Büttner A, Schittkowski M, Stachs O, Kraak R, Zhivov A, Rolfs A, Guthoff R. The microstructure of cornea verticillata in Fabry disease and amiodarone-induced keratopathy: a confocal laser-scanning microscopy study. Graefes Arch Clin Exp Ophthalmol. 2009 Apr;247(4):523-34. doi: 10.1007/s00417-008-0962-9. Epub 2008 Oct 18. PMID: 18931853. 

3.Ciancaglini M, Carpineto P, Zuppardi E, Nubile M, Doronzo E, Mastropasqua L. In vivo confocal microscopy of patients with amiodarone-induced keratopathy. Cornea. 2001 May;20(4):368-73. doi: 10.1097/00003226-200105000-00007. PMID: 11333323. 

Adil El Maftouhi is an orthoptist specializing in ocular imaging and exploration at the Centre Ophtalmologique de Rive Geneva in Switzerland and the Institut Parisien d'Ophtalmologie in Paris, France. He is the author of international publications on OCT and OCT-A. He has also published several peer-reviewed articles, such as "OCT and Dry Eye Disease" and "OCT: The Intelligence of the Epithelium."

Adil El Maftouhi works on the development and utilization of various imaging systems available in ophthalmology to maximize the potential of each system for clinical benefit. At the same time, he contributes to the development of new imaging applications and software, particularly in OCT technology. Mr. El Maftouhi is also involved in clinical and pharmacological research projects in the fields of medical retina, glaucoma, and low vision.

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