Download CASE TWO - Better ONE or two

Vortex Keratopathy

68 year old female
 Chief complaint: Blur at near in OD>OS
Dryness OU
 Medical Hx: Allergies
Hypertension
Irregular Heart Beat
Exam Findings

PERRL (–)APD
 EOM: Full and Smooth
 CF: FTFC
 Rx: OD: +1.50-1.00x085
OS: -0.50-1.25x100
OU: +2.25 Add
 VA: 20/40 OD, 20/20 OS @ D/N
Slit Lamp Exam
OD
OS
 Lids/Lashes:
Clear
Clear
 Conjunctiva:
Clear
Clear
 Cornea:
INFERIOR WHORLS
 Anterior Chamber: D&Q
D&Q
 Iris:
Clear
Clear
 IOP:
16mmHg OU
Fundoscopy

Lens
 C/D
 A/V
 Macula
 Vitreous
 Periphery
OD
OS
NS2, PSC 1+
PCIOL
.2/.2
.2/.2
2/3
2/3
Clear
Clear
PVD
PVD
(-)Breaks/Tears
Corneal Finding
Corneal Verticillata

Whorl-like pattern of yellow or brown
deposits confined to the inferior central
cornea.
 At the level of the deep
epithelium(50microns).
 Limbal-sparing.
 Delineate epithelial migration patterns.
Etiology
Fabry’s disease.
 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.
 Deficiency of the enzyme alpha-galactosidase A
results in the accumulation of ceramide
trihexoside.
 Symptoms include skin lesions and neurologic
changes. Painful neuropathy is common; often
associated with a low-grade fever.
 Hypohydrosis is common and can lead to
heatstroke.
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.
 Deposits of lipid in the kidney can lead to
progressive renal compromise and renal failure.
 Confirmation with a blood test: Alphagalactosidase level (Normal range is 19 to 29).
Fabrys’ Disease and the Eye

Corneal verticillata.
 Conjunctival and retinal vessel tortuosity.
 Oculomotor abnormalities.
 Anterior subcapsular cataracts.
 Periorbital edema.
Amiodarone

Amiodarone hydrochloride is a Class III
antiarrhythmic agent.
 Usual dose is 200-600mg/ day.
 Prolongs the action potential duration and
refractory period of atrial, nodal and ventricular
tissues.
 Amiodarone increases coronary blood flow,
decreases cardiac oxygen requirements and also
suppresses ectopic pacemakers.
Amiodarone and the Eye

Corneal verticillata.
 Decrease in vision, rarely.
 Photophobia.
 Colored halos.
 Dyschromatopsia.
 Dry eye.
 Anterior ischemic optic neuropathy*.
As it turns out…

Patient has ocular dryness, and anterior
subcapsular cataract.
 This patient has been on Amiodarone for
several years for her heart arrhythmia.
 *Color vision testing may be helpful if
medical history is unreliable.
Mechanism of Keratopathy

Amphiphilic drugs; amiodarone,
chloroquines and phenothiazones deposit
verticillata.
 Verticillata form when drug complexes with
phospholipids in the cells.
 Complexes cannot be metabolized by
lysosomal phospholipases and remain
within the cornea.
Amiodarone Keratopathy

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

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…

Most studies suggest that all taking
Amiodarone will develop verticillata.
 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…

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…

Cardiology No.5, 2003. Examination of 298
patients who received oral amiodarone for
1–122 months (mean 27,3±1,5 months).
 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.
 Evaluated Amiodarone Keratopathy using the
computer operated corneal topographers.
 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.
 Note: LASIK or PRK are not recommended.
Topographic Scans
Treatment

There is no recommended treatment for
verticillata.
 Medication regimens are not altered based
on the presence of corneal deposits.
 If drug is stopped, most verticillata will
eventually disappear within seven months.
 Unknown history warrants investigation!