Vortex Keratopathy l 68 year old female l

Vortex Keratopathy l 68 year old female l Chief complaint: Blur at near in OD>OS Dryness OU l Medical Hx: Allergies Hypertension Irregular Heart Beat

Exam Findings l PERRL (–)APD l EOM: Full and Smooth l CF: FTFC l Rx: OD: +1. 50 -1. 00 x 085 OS: -0. 50 -1. 25 x 100 OU: +2. 25 Add l VA: 20/40 OD, 20/20 OS @ D/N

Slit Lamp Exam OD OS l Lids/Lashes: Clear l Conjunctiva: Clear l Cornea: INFERIOR WHORLS l Anterior Chamber: D&Q l Iris: Clear l IOP: 16 mm. Hg OU

Fundoscopy l Lens l C/D l A/V l Macula l Vitreous l Periphery OD OS NS 2, PSC 1+ PCIOL. 2/. 2 2/3 Clear PVD (-)Breaks/Tears

Corneal Finding

Corneal Verticillata l Whorl-like pattern of yellow or brown deposits confined to the inferior central cornea. l At the level of the deep epithelium(50 microns). l Limbal-sparing. l Delineate epithelial migration patterns.

Etiology l Fabry’s disease. l Reaction to systemic drug therapy including: Amiodarone Chloroquine Indomethacin Chlorpromazine Tamoxifen

Fabry’s Disease Fabry's disease is an X-linked recessive lipid storage disorder with an incidence of about 1 in 40, 000. l Deficiency of the enzyme alpha-galactosidase A results in the accumulation of ceramide trihexoside. l Symptoms include skin lesions and neurologic changes. Painful neuropathy is common; often associated with a low-grade fever. l Hypohydrosis is common and can lead to heatstroke. l

Fabry’s Disease Deposits of lipid in the myocardium can lead to arrhythmia, myocardial infarction and valvular dysfunction. Involvement of small cerebral vascular vessels can result in cerebral hemorrhage. l Deposits of lipid in the kidney can lead to progressive renal compromise and renal failure. l Confirmation with a blood test: Alphagalactosidase level (Normal range is 19 to 29). l

Fabrys’ Disease and the Eye l Corneal verticillata. l Conjunctival and retinal vessel tortuosity. l Oculomotor abnormalities. l Anterior subcapsular cataracts. l Periorbital edema.

Amiodarone hydrochloride is a Class III antiarrhythmic agent. l Usual dose is 200 -600 mg/ day. l Prolongs the action potential duration and refractory period of atrial, nodal and ventricular tissues. l Amiodarone increases coronary blood flow, decreases cardiac oxygen requirements and also suppresses ectopic pacemakers. l

Amiodarone and the Eye l Corneal verticillata. l Decrease in vision, rarely. l Photophobia. l Colored halos. l Dyschromatopsia. l Dry eye. l Anterior ischemic optic neuropathy*.

As it turns out… l Patient has ocular dryness, and anterior subcapsular cataract. l This patient has been on Amiodarone for several years for heart arrhythmia. l *Color vision testing may be helpful if medical history is unreliable.

Mechanism of Keratopathy l Amphiphilic drugs; amiodarone, chloroquines and phenothiazones deposit verticillata. l Verticillata form when drug complexes with phospholipids in the cells. l Complexes cannot be metabolized by lysosomal phospholipases and remain within the cornea.

Amiodarone Keratopathy l Grading System – 1 - Small, brown punctate epithelial opacities within inferior temporal cornea arranged in a single line. – 2 - Branching pattern to line of opacities. – 3 - Increase in branches to form a whorl. – 4 - Whorling with irregular clumps of brown pigment.

Amiodarone and AION l Patients on Amiodarone have a severe cerebrovascular deficit. An AION is most likely due to the illness rather than a reaction to the drug.

Studies… l Most studies suggest that all taking Amiodarone will develop verticillata. l Journal of AOA, 1985. Observations of 21 patients on a daily dosage of 200 -600 mg for periods ranging from six months to three years. Corneal deposits developed in all 21 patients and anterior lens opacities developed in 12 of 20 phakic patients.

Studies… l Cornea, 2001. Eleven patients on amiodarone therapy were observed. All patients showed the presence of high reflective, bright intracellular inclusions in the epithelial layers.

Studies… l Cardiology No. 5, 2003. Examination of 298 patients who received oral amiodarone for 1 – 122 months (mean 27, 3± 1, 5 months). l Signs of keratopathy were found in 280 patients (94%). Severity of keratopathy depended on cumulative drug dose and duration of administration.

Studies… Digital Journal of Ophthalmology, 2004. l Evaluated Amiodarone Keratopathy using the computer operated corneal topographers. l Corneal topography of 7 of 8 eyes revealed an unusual irregular astigmatism with generalized mild inferior temporal steepening consistent with the location of the corneal deposits. l Note: LASIK or PRK are not recommended. l

Topographic Scans

Treatment l There is no recommended treatment for verticillata. l Medication regimens are not altered based on the presence of corneal deposits. l If drug is stopped, most verticillata will eventually disappear within seven months. l Unknown history warrants investigation!