Vortex Keratopathy

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Vortex keratopathy ( cornea verticillata ) in a Fabry disease patient viewed with a slit lamp. The cream-colored swirl-like pattern appearing on both sides does not impair visual acuity.

As cornea verticillata ( lat. Corneal deposits , corneal =, cornea ') is called vortex-like deposits in the corneal epithelium of the eye . In most cases it is a side effect of certain drugs .

description

The cornea verticillata is a corneal degeneration that occurs in both eyes and is characterized by eddy-shaped deposits in the corneal epithelium.

etiology

In most cases, the cause of vortex keratopathy is the long-term use of certain medications. For example, 90% of patients who take the antiarrhythmic amiodarone for at least six months develop cornea verticillata . Visual acuity is only very rarely impaired. 1 to 10% of affected patients notice a slight blue tinge to their vision.

The drug chloroquine , which is used for the treatment and prophylaxis of malaria , can also cause vortex keratopathies. The tyrosine kinase inhibitor vandetanib (ZD6474) can obviously also lead to vortex keratopathy.

Almost all hemizygous patients suffering from the hereditary disease Fabry's disease have vortex keratopathy. It does not affect visual acuity either, but serves as an important symptom in diagnosing the disease. The cloudiness is caused by the inclusion of the sphingolipid globotriaosylceramide (Gb3).

diagnosis

Vortex keratopathy can normally be reliably diagnosed by an ophthalmologist using a slit lamp through the characteristic vortices. The distinction between whether the vortex keratopathy is medicated or induced by Fabry disease can be made in vivo using confocal microscopy .

therapy

In the case of vortex keratopathy caused by drugs, the drug causing the problem can be discontinued in the case of pronounced visual impairments, for example optical neuropathies with visual field deficits. In the case of amiodarone, some cardiologists consider vortex keratopathy to be an indication of good compliance .

In Fabry disease, enzyme replacement therapy can reduce Gb3 deposits in the corneal epithelium.

further reading

  • M. Erdurmus, Y. Selcoki et al. a .: Amiodarone-induced keratopathy: full-thickness corneal involvement. In: Eye & contact lens. Volume 34, Number 2, March 2008, pp. 131-132, doi : 10.1097 / ICL.0b013e31814934c0 . PMID 18327053 .
  • A. Dosso, E. Rungger-Brändle: In vivo confocal microscopy in hydroxychloroquine-induced keratopathy. In: Graefe's archive for clinical and experimental ophthalmology. Volume 245, Number 2, February 2007, pp. 318-320, doi : 10.1007 / s00417-006-0365-8 . PMID 16738856 .
  • DA Hollander, AJ Aldave: Drug-induced corneal complications. In: Current opinion in ophthalmology. Volume 15, Number 6, December 2004, pp. 541-548, PMID 15523201 . (Review).
  • M. Ciancaglini, P. Carpineto, et al. a .: In vivo confocal microscopy of patients with amiodarone-induced keratopathy. In: Cornea. Volume 20, Number 4, May 2001, pp. 368-373, PMID 11333323 .
  • GK Shah, HL Cantrill, EJ Holland: Vortex keratopathy associated with atovaquone. In: American journal of ophthalmology. Volume 120, Number 5, November 1995, pp. 669-671, PMID 7485371 .
  • SD Jaanus: Ocular side effects of selected systemic drugs. In: Optometry clinics. Volume 2, Number 4, 1992, pp. 73-96, PMID 1363080 . (Review).
  • F. Taylor: Drugs affecting the eye. In: Australian family physician. Volume 14, Number 8, August 1985, pp. 744-745, PMID 2864912 .
  • LJ Kaplan, WE Cappaert: Amiodarone-induced corneal deposits. In: Annals of ophthalmology. Volume 16, Number 8, August 1984, pp. 762-766, PMID 6497223 .
  • DJ D'Amico, KR Kenyon, JN Ruskin: Amiodarone keratopathy: drug-induced lipid storage disease. In: Archives of ophthalmology. Volume 99, Number 2, February 1981, pp. 257-261, PMID 6258544 .

Individual evidence

  1. ↑ AP Burlina, KB Sims u. a .: Early diagnosis of peripheral nervous system involvement in Fabry disease and treatment of neuropathic pain: the report of an expert panel. In: BMC neurology. Volume 11, 2011, p. 61, doi : 10.1186 / 1471-2377-11-61 . PMID 21619592 . PMC 3126707 (free full text).
  2. ↑ a b D. P. Germain: Fabry disease. In: Orphanet Journal of Rare Diseases. Volume 5, 2010, p. 30, doi : 10.1186 / 1750-1172-5-30 . PMID 21092187 . PMC 3009617 (free full text). (Review in Open Access ).
  3. ↑ K. Falke, A. Büttner u. a .: The microstructure of cornea verticillata in Fabry disease and amiodarone-induced keratopathy: a confocal laser-scanning microscopy study. In: Graefe's archive for clinical and experimental ophthalmology. Volume 247, Number 4, April 2009, pp. 523-534, doi : 10.1007 / s00417-008-0962-9 . PMID 18931853 .
  4. ↑ E. Chew, M. Ghosh, C. McCulloch: Amiodarone-induced cornea verticillata. In: Canadian journal of ophthalmology. Journal canadien d'ophtalmologie. Volume 17, Number 3, June 1982, pp. 96-99, PMID 7116220 .
  5. ↑ KU Seiler, HJ Thiel, O. Wassermann: The chloroquinkeratopathy as an example of a drug-induced phospholipidosis (at the same time a contribution to the pathogenesis of the cornea verticillata). In: Clinical monthly sheets for ophthalmology. Volume 170, Number 1, January 1977, pp. 64-73, PMID 15159 .
  6. ^ S. Yeh, HA Fine, JA Smith: Corneal verticillata after dual anti-epidermal growth factor receptor and anti-vascular endothelial growth factor receptor 2 therapy (vandetanib) for anaplastic astrocytoma. In: Cornea. Volume 28, Number 6, July 2009, pp. 699-702, doi : 10.1097 / ICO.0b013e3181922146 . PMID 19512898 .
  7. ↑ Caprelsa ® (vandetanib) (PDF; 141 kB) Information from the Drugs Commission of the German Medical Association (AkdÄ), as of June 12, 2012.
  8. ↑ C. Orssaud, J. Dufier, D. Germain: Ocular manifestations in Fabry disease: a survey of 32 hemizygous male patients. In: Ophthalmic genetics. Volume 24, Number 3, September 2003, pp. 129-139, PMID 12868031 .
  9. ↑ TT Nguyen, T. Gin et al. a .: Ophthalmological manifestations of Fabry disease: a survey of patients at the Royal Melbourne Fabry Disease Treatment Center. In: Clinical & experimental ophthalmology. Volume 33, Number 2, April 2005, pp. 164-168, doi : 10.1111 / j.1442-9071.2005.00990.x . PMID 15807825 .
  10. ^ A. Sodi, AS Ioannidis et al. a .: Ocular manifestations of Fabry's disease: data from the Fabry Outcome Survey. In: The British journal of ophthalmology. Volume 91, Number 2, February 2007, pp. 210-214, doi : 10.1136 / bjo.2006.100602 . PMID 16973664 . PMC 1857640 (free full text).
  11. ↑ J. Wasielica-Poslednik, N. Pfeiffer u. a .: Confocal laser-scanning microscopy allows differentiation between Fabry disease and amiodarone-induced keratopathy. In: Graefe's archive for clinical and experimental ophthalmology [electronic publication before printing] July 2011, doi : 10.1007 / s00417-011-1726-5 . PMID 21720814 .
  12. ↑ J. Wasielica-Poslednik, S. Pitz, N. Pfeiffer: Cornea Verticillata in Fabry disease and amiodarone intake - a differentiation using in vivo confocal microscopy. In: 107th DOG Congress from 24.-27. September 2009.