Ectopia Lentis

Anatomi lensa
a biconvex, avascular, colorless, and almost completely transparent structure,
about 4 mm thick and 9 mm in diameter
suspended behind the iris by the zonule (suspensory ligament )
connects it with the ciliary body
Anterior to the lens is the aqueous; posterior to it, the vitreous
A subcapsular epithelium is present anteriorly
lens nucleus is harder than the cortex
With age, subepithelial lamellar fibers continuously produced, so that the lens
gradually becomes larger and less elastic throughout life.
The lens consists of about 65% water, about 35% protein
Potassium is more concentrated in the lens than in most tissues. Ascorbic acid and
glutathione are present in both oxidized and reduced forms.

Background
Ectopia lentis is defined as displacement or malposition
of the crystalline lens of the eye
considered dislocated or luxated when

it lies completely outside the lens patellar fossa

in the anterior chamber
free-floating in the vitreous
directly on the retina.

In the absence of trauma, ectopia lentis should evoke

suspicion for concomitant hereditary systemic disease
or associated ocular disorders.[4, 5]

Pathophysiology
Disruption or dysfunction of the zonular fibers
degree of zonular impairment determines the degree of
lens displacement.

Epidemiology
The most common cause of ectopia lentis istrauma,

Mortality/Morbidity
visual disturbance
underlying etiologic abnormality.

Sex
more prone to ocular trauma than females
Male and female frequency varies with the etiology of the lens
displacement.

Age
Ectopia lentis can occur at any age.[6]It may be present at
birth, or it may manifest late in life.

History
Common presenting symptoms (visual disturbance) include the following:

Red painful eye (secondary to trauma)

Decreased distance visual acuity (secondary to astigmatism or myopia)
Poor near vision (loss of accommodative power)
Monocular diplopia

Determine if there is a history of ocular trauma.

Possible systemic disease associations
Cardiovascular disease (eg, Marfan syndrome)
Skeletal problems -Marfan syndrome, Weil-Marchesani syndrome, or
homocystinuria
Pertinent family history (autosomal recessive conditions) homocystinuria,
hyperlysinemia, ectopia lentis et pupillae, [7]or sulfite oxidase deficiency

Ocular examination
Vision

evaluasi lens position

identify phacodonesis or cataract.
Measure intraocular pressure

visually debilitating aries with the degree of

malpositioning of the lens.
Amblyopia common cause of decreased vision in
congenital ectopia lentis and preventable and
treatable.

External ocular examination

orbital anatomy eg. hereditary
malformations(marfan syndrome enophthalmos
with facial myopathic appearance)
Measure corneal diameter (Marfan syndrome
megalocornea)
Strabismus is not uncommon (secondary to
amblyopia).

Retinoscopy and refraction

myopia with astigmatism
Keratometry may degree of corneal astigmatism.

Slit lamp examination

pupillary block
phacoanaphylaxis or phacolytic
posttraumatic angle recession
poorly developed angle structures
lens in the anterior chamber

Dilated fundus examination

Retinal detachment is one of the

most serious consequences of a
dislocated lens.

Causes
Traumatic dislocation (the most
common cause)
Hereditary ectopia lentis
without systemic
manifestations
autosomal dominant inheritance
with the genetic defect located on
chromosome 15 dysfunctional
zonular apparatus
characterized by asymmetric
eccentric pupils
Usually is bilateral and typically
autosomal recessive

Systemic conditions commonly associated with ectopia lentis

Marfan syndrome is the most frequent

cause
75% of patients with Marfan syndrome
and usually is bilateral, symmetrical, and
supertemporal
Homocystinuria is the second most
common cause of hereditary ectopia
lentis
inborn error of metabolism near absence of
cystathionine b-synthetase (converts
homocysteine to cystathionine)
usually is bilateral, symmetrical, and
inferonasal

Weil-Marchesani is a rare syndrome

characterized by skeletal malformations
(eg, short stature

 A high level of homocysteine in the blood (

hyperhomocysteinemia) makes a person more prone to
endothelial cellinjury, which leads toinflammationin
the blood vessels, which in turn may lead to
atherogenesis, which can result inischemic injury.[3]

Laboratory Studies
a hereditary ondition is suspected
cardiac evaluation for Marfan syndrome,
check serum and urine levels of homocysteine or methionine
for homocystinuria)

Komplikasi
most common ocular complications of ectopia lentis
include

Amblyopia
Uveitis
Glaucoma
retinal detachment

surgery
Indications for lensectomy include the following:

Lens in the anterior chamber

Lens-induced uveitis
Lens-induced glaucoma
Lenticular opacity with poor visual function
Anisometropia or refractive error not amenable to optical
correction (eg, in a child to prevent amblyopia)
Impending dislocation of the lens

Marfan Syndrome

Marfan Syndrome
connective tissue disorder
manifestations involving primarily the ocular, skeletal,
and cardiovascular systems,
caused by the deficiency of a structural extracellular
matrix protein, fibrillin-1,