Frequency Ectopia lentis is a rare condition. Incidence in the general population is unknown. The most common cause of ectopia lentis is trauma. Mortality/Morbidity Ectopia lentis may cause marked visual disturbance, depending the degree of lens displacement and the underlying etiologic abnormality. Sex Males appear more prone to ocular trauma than females; therefore, a male preponderance has been reported. Male and female frequency varies with the etiology of the lens displacement. Age Ectopia lentis can occur at any age. It may be present at birth, or it may manifest late in life.
Ectopia lentis and its low vision management
AND ITS LOW VISION MANAGMENT
Indra P Sharma
Master of Clinical Optometry (Year I)
Amity Medical School
To have a better understanding about ectopia
lentis with regard to its pathophysiology, etiology,
clinical manifestation and management.
To understand the management of ectopia lentis
with low vision aids
1. An overview
2. Signs and symptoms
3. Etiology of ectopia lentis
4. Workup and evaluation
6. Low Vision Management
Ectopia lentis is defined as displacement or malposition
of the crystalline lens of the eye.
1749 - Berryat first reported case of lens dislocation
1856- Stellwag coined the term “ectopia lentis”
Ectopia lentis can be :
2. Luxation (dislocated)
Condition when the
crystalline lens is
partially displaced but
contained within the
Few zonular attachment
Also known as luxated
When lens lies completely
outside the lens patellar
fossa, in the anterior
chamber, free-floating in
the vitreous, or directly on
Anatomy of zonules
The lens is suspended in its anatomic position by
ciliary zonules (zonules of Zinn or suspensory
ligament of Zinn)
Zonules fibers which run from ciliary body and
insert into the outer layer of the lens capsule
around the equator (1.5 mm anteriorly and 1mm
Stronger zonules in anterior capsule
Each zonule measures 5 to 30μm in diameter
and is composed of bundles of microfibrils
Disruption or dysfunction of
the zonular fibers of the
lens, regardless of cause
(trauma or heritable
The degree of zonular
impairment determines the
degree of lens
A rare condition.
Incidence in the general population is unknown.
Can cause marked visual disturbance, depending the
degree of lens displacement and the underlying etiology
More common in males.
At any age (At birth or it may manifest late in life)
Signs and Symptoms
Distance visual acuity (moderate to severe impairment)
depending on position of lens
1. Fluctuating vision dramatically as the vision may alternate
between phakia and aphakic
2.Progressive movement of the lens- Extreme hyperopic shift or
Poor near vision
Due to loss of accommodative power due to weakened,
stretched or broken zonules
Visual field defects (Due to lens induced secondary
A 1963 histological study found that crystalline lenses that
dislocated anteriorly were associated with glaucoma 77.2% of
the time and that crystalline lenses that were subluxed or
Source: Sihota R Sood NN, Argarwal He. 1995. Traumatic glaucoma.Acta Ophthalmol Scand
posteriorly were associated with glaucoma 87.5% of the time.
Painful red eye (secondary to trauma)
Glare and photophobia
Etiology of ectopia lentis
Traumatic dislocation is most common cause of ectopia
Hereditary ectopia lentis without
Single (isolated) ectopia lentis
Autosomal dominant inheritance
Genetic defect located on chromosome
15, causing a dysfunctional zonular
Microspherophakia is common.
Present at birth, can even onset late
Typically, supertemporal displacement
Ectopia lentis et pupillae
Usually is bilateral and typically autosomal
characterized by asymmetric eccentric pupils
that are displaced in the opposite direction of
the lens dislocation (toward the most
dysfunctional zonular fibers).
The irides often appear atrophic with
Pupil may be displaced in opposite
Single (isolated) ectopia lentis direction (ectopia lentis et pupillae)
Systemic conditions associated
with ectopia lentis
Marfan syndrome (most frequent cause of
hereditary ectopia lentis)
Homocystinuria (second most common cause of
hereditary ectopia lentis)
Transmitted as an autosomal dominant
Prevalence: approx. 5 per 100,000.
Mutations involving the fibrillin gene on
chromosomes 15 and 21 and may relate
to incompetent zonular fibers.
Systemic association of
• Pectus excavatum
• High-arched palate
• Aortic dilatation,
• Mitral valve prolapse
dislocation of a lens in 80%
Zonules usually intact
Axial myopia Lattice degeneration
Angle anomaly and
It is an inborn error of metabolism.
Most often caused by a deficiency of
cystathionine b-synthetase (the enzyme
that converts homocysteine to
Systemic and ocular
Malar flush and fine fair hair
Increased platelet stickiness
zonules Sharma IP
Rare syndrome characterized by skeletal
The inheritance pattern is not well
Pupillary block glaucoma is common;
therefore, prophylactic laser peripheral
iridotomies are recommended.
Systemic and ocular
Short stubby fingers
Anterior lens subluxation
Sulfite oxidase deficiency
Extremely rare disorder caused by a
defect in sulfur metabolism.
Salient features are:
Progressive CNS abnormalities that
develop within the first year of life
Extremely rare autosomal recessive
enzymatic defect of amino acid
Mental retardation and lens dislocation.
Diagnosis is made by demonstration of
increased plasma levels of lysine.
Workup and evaluation
History of ocular trauma.
History investigating possible systemic disease
Cardiovascular disease in Marfan syndrome
Skeletal problems in Marfan syndrome,Weil-Marchesani
syndrome or homocystinuria
Pertinent family history
Unexplained deaths at young age
Amblyopia is a common cause of decreased
vision in congenital ectopia lentis.
External ocular examination
Orbital anatomy for hereditary malformations
(eg, enophthalmos with facial myopathic
appearance seen in Marfan syndrome).
Measure corneal diameter (megalocornea in
Strabismus is common (secondary to amblyopia).
Retinoscopy and and keratometry
Careful retinoscopy and refraction is essential, often
revealing myopia with astigmatism.
Keratometry may help ascertain degree of corneal
Slit lamp examination
Evaluate lens position, and identify phacodonesis or
Measure intraocular pressure.
Dilated fundus examination
To rule out retinal detachment
Perform appropriate diagnostic and laboratory
evaluation, if a hereditary condition is suspected
Cardiac evaluation for Marfan syndrome
Check serum and urine levels of homocysteine
or methionine for homocystinuria.
Axial length measurement may be of benefit
(patients with Marfan syndrome have large
For any other associated vitrous and retinal
The key to managing lens subluxation optically is
perform two refractions:
1.For that portion of the pupil covered by the
crystalline lens (myopic astigmatism zone) and
2. For that portion of the pupil not covered by
the crystalline lens (highly hyperopic zone).
For moderate to severe subluxation
Option: An aphakic prescription with bifocals
and a pharmacologic dilation
For lens is subluxed out of the visual axis
(bilateral) Best option: An aphakic prescription
Both spectacle and contact lens may be
If no, history of trauma, patients may
possess a systemic disease with potentially
deleterious effects; therefore,
comanagement with pediatrician or internist
Dietary restriction: partially effective in
Treatment of glaucoma is dependent on the
Pupillary block requires laser peripheral iridotomy
or iridectomy and raised IOP treated medically.
Prophylactic laser iridotomy in microspherophakia.
Dislodged lens into the AC is initially
pharmacological (mydriasis/cycloplegia) in
conjunction with ocular massage through a closed
Treatment of a dislocated lens in the vitreous is
Sharma IP surgical.
1.Nd:YAG laser zonulysis
to displace lens out of visual axis
Lensectomy is technically challenging and are indication in the
Lens in the anterior chamber
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
Prevention and prognsis
Early diagnosis of ectopia lentis with appropriate optical
correction can prevent amblyopia.
The most common ocular complications include
amblyopia, uveitis, glaucoma, and retinal detachment
Depending on the degree of lens dislocation, the age of
onset, and its associated secondary complications, most
patients do well.
Trauma-associated ectopia lentis – poor visual
Low vision management
Rule of thumb
Provide LVA to help releive the main
Distance viewing devices
Why do we need it?
To compensate for high hypermetropia or
High power lenses
High plus spectacle
(Silsoft/silsoft super plus)
Roving Ring Scotoma
Circular restriction in the peripheral VF due to
prismatic effect of high plus lenses.
reduces this scotoma
Near optical devices
Why is it required?
To compensate for high hyperopia and loss of
Preferable near viewing devices
1. Spectacle-mounted Reading Lenses
4. Electronic Devices- CCTV
made to focus at
Illuminated and non-illuminated
Hand held and stand magnifiers
Visual field enhancing
To compensate for VF
defects due to secondary
Relative size and larger assistive devices
Glare, contrast, and lighting control
Handwriting and written communication
Medical management devices
Orientation and mobility management
techniques and devices
Relative size and larger assistive
Books, magazines, newspaper, dictionaries, thesauri,
atlas, cookbooks, encyclopedias, bibles
Photocopy machines ( e.g. A4 size to A3 size)
Computer with large prints font
Computer software program
Large print typewriters
Telephone dial, bank checks, watches, clocks, calculators etc
Games like cross word puzzles, playing cards, chess
checkers, bingo etc.
Magic 8.0 Large print
Glare, contrast, and lighting
Aphakia allows for increased transmission of UV
Tennis shades, caps, hats, side-shields, visors
NoIR and CPF lenses
Sunglasses/ photochromatic lenses to reduce
Typoscopes – a reading guide cut
Glare cutter ( 390-410nm)-
cuts 100% UVB, 99% UVA
Diplopia eliminating devices
Aperture control contact lens
Color and contrast
Environmental modification Painted edges of Staircase
If a hereditary condition is discovered,
appropriate genetic counseling recommended.
All relatives with potential risk should be
Advised against playing contact sports or doing
physically strenous activities.
Take home message
Managing patients with ectopia lentis including
refraction is usually difficult and needs
expertise to manage them well.
As most patients with ectopia lentis are
syndromic, co-management with other
specialist is essential.
While managing patient with low vision we
need to looks at various cause and other
ocular association like glaucoma, retinal
detachment and amblyopia.
An optometrist needs to understand the need
of the patient and prescribe according.
Jonathan A Jackson, Low Vision Mannual,2007, Butterworth
William J Benjamin,2006, Borish’s Clinical Refraction,
Butterworth Heineman Elsevier. 20: 816-829
Lighthouse International, The Lighthouse Clinician’s Guide
to Low Vision Practice
Brilliant Richard L, 1999, Essentials of Low vision Practice,
Butterworth Heineman Elsevier. 220.127.116.11