0% found this document useful (0 votes)
76 views38 pages

Ectopia Lentis and Cataract Overview

Uploaded by

rowan alaa
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
0% found this document useful (0 votes)
76 views38 pages

Ectopia Lentis and Cataract Overview

Uploaded by

rowan alaa
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd

S.

S-O [04]
The Lens

Dr. Wessam Salem, FRCS.


Assistant lecturer of Ophthalmology, NGU.
Intended learning Outcomes
1. Revisiting anatomy of the lens
2. Understand different types of cataract and their
morphology
3. Identify surgical management of cataract, the
preoperative assessment and operative
complications
4. Understand what is ectopia lentis and its systemic
associations.
Anatomy of the lens

[Link]/contene0https00459t/5/1/
Introduction
• Cataracts are clouding of the lens of
your eye, which is normally clear.
• Most cataracts develop slowly over
time, causing symptoms such as
blurry vision.
• Cataracts can be surgically removed
through an outpatient procedure
that restores vision in nearly
everyone.
Kanski's Clinical Ophthalmology - 9th Edition.
What is a cataract?
• A cataract develops when the lens in
your eye, which is normally clear,
becomes foggy.
• For your eye to see, light passes
through a clear lens. The lens is behind
your iris, The lens focuses the light on
the retina.
• When a cataract clouds over the lens,
your eye can’t focus light in the same
way. This leads to blurry vision.
• Your vision change depends on the
cataract’s location and size.

[Link]
Cataract can be congenital or
acquired.
Acquired cataract could be due to
• Age related cataracts
• Secondary to systemic or local
diseases A B
Cataract maturity
A. Immature cataract is one in which the
lens is partially opaque.
B. Mature in which the lens is totally
opaque
C. Hypermature cataract has a shrunken
and wrinkled anterior capsule due to
leakage of water out of the lens.

C
I. Congenital Cataract
Discussed in details before in CH Module
A. Cataract Associated with metabolic disorders:
1. Galactosemia
2. Lowe syndrome
3. Mannosidosis
B. Cataract Associated with intrauterine infections
1. Rubella
2. CMV
3. Varicella
4. Toxoplasmosis
C. Cataract Associated with Chromosomal Abnormalities
1. Down Syndrome (triosmy 21) Oil Droplet Cataract
[Link]
2. Edwards syndrome (trisomy 18)
II. Acquired Cataract
A. Age related cataract
Age related cataract are divided mainly into 3 types; Sub-capsular, Nuclear and Cortical.
Subcapsular cataract
• This type of cataract lies
directly under the lens and it
may be anterior subcapsular or
posterior.
• It is due to fibrous metaplasia
of the lens epithelium
• Due to its location at the nodal
point of the eye, a posterior
subcapsular opacity often has a
particularly profound effect on
vision.
[Link]
A. Age related cataracts
Nuclear Cataract
• It is considered an exaggeration of
the normal aging changes
• Nuclear sclerotic cataract is
characterized by a yellowish hue
due to deposition of urochrome
pigment.
• It is associated with myopia due
to an increase in the refractive
index of the nucleus, resulting in
some elderly patients being able Kanski's Clinical Ophthalmology - 9th Edition.

to read without spectacles again


(Second sight of the aged).
A. Age related cataracts
Cortical cataract
• Cortical cataract may involve
the anterior, posterior or
equatorial cortex.
• The opacities start as clefts and
vacuoles between lens fibers
due to cortical hydration
• Subsequent opacification
results in typical wedge-
shaped or radial spoke-like
opacities.
[Link]
B. Cataract in systemic diseases
• Diabetes mellitus
Glucose is metabolized into sorbitol, which accumulates
within the lens, resulting in secondary osmotic
overhydration.
In mild degree, this may affect the refractive index of
the lens with consequent fluctuation of refraction in
line with the plasma glucose level, hyperglycemia
resulting in myopia and vice versa.
Classic diabetic cataract, which is rare, consists of
snowflake cortical opacities.
• Myotonic dystrophy
About 90% of patients with myotonic dystrophy develop
fine wedge-shaped cortical opacities with star
configuration.

Kanski Clinical Ophthalmology- 9th edition


B. Cataract in systemic diseases
• Atopic dermatitis
About 10% of patients with severe atopic
dermatitis develop cataracts in the 2nd to 4th
decades , cataracts are Shield-like dense
anterior subcapsular plaque that wrinkles the
anterior capsule
• Neurofibromatosis 2
Neurofibromatosis type 2 is associated with
cataract in more than 60% of patients.
Opacities are posterior subcapsular, cortical
or mixed and tend to develop in early
adulthood.
Shield-like Cataract
Kanski Clinical Ophthalmology- 9th edition
C. Cataract in local diseases
• Chronic anterior uveitis • Acute congestive glaucoma
Chronic anterior uveitis is the most Acute congestive angle closure may
common cause of secondary cataract, the cause small anterior grey-white capsular
incidence being related to the duration opacity to form within the pupillary area
and intensity of inflammation. (glaukomflecken).

Uveitic Cataract
Glaukomflecken
[Link] [Link]
C. Cataract in local diseases
• High myopia
High (pathological) myopia can be associated with
posterior subcapsular cataract and early-onset
nuclear sclerosis, which ironically may increase the
myopic refractive error.
• Hereditary fundus dystrophy
such as retinitis pigmentosa, Leber congenital
amaurosis and Stickler syndrome
• Secondary to medications
Systemic and topical steroids can lead to cataract
formation, its usually posterior subcapsular.
Posterior Subcapsular cataract
[Link]
D. Traumatic cataract
Trauma is the most common cause of
unilateral cataract in young individuals.
• Penetrating trauma
• Blunt trauma
• Electric shock
• Infrared/ionizing radiations

Traumatic cataract may be accompanied with other


ocular injuries e.g. Iridodialysis (Separation of the iris
from its attachment to the ciliary body).
Traumatic Cataract with Iridodialysis
[Link]
[Link]
What are the symptoms of a cataract?
1. Vision that’s cloudy, blurry, foggy or filmy.
2. Sensitivity to bright sunlight, lamps or headlights.
3. Glare (seeing a halo around lights) especially when you drive at night
with oncoming headlights.
4. Changes of glasses prescription
including sudden near sightedness.
5. Need for brighter light to read.
6. Difficulty seeing at night.

[Link]
Management of age-related cataract
Indication of surgery
1. Visual improvement, which is by far the most important indication whether if
the vison is deteriorating affecting the daily activity or in clear lens exchange for
management of refractive errors.
2. Indications in which a cataract is adversely affecting the health of the eye.
Examples include phacolytic glaucoma or clearing the view for fundus
pathology e,g Retinal detachment.
Systemic preoperative assessment For elective surgery, a general medical history is
taken and any problems managed accordingly.
Cataract surgery
• The word cataract comes from the Greek for waterfall. Until the mid 1700s, it was thought that a
cataract was formed by opaque material flowing, like a waterfall, into the eye
• The oldest written record of eye diseases dates to the year 1500 B.C., numerous diseases of the eye
are referred to in the Ebers Papyrus discovered between the legs of a mummy in the Theban
Necropolis in Egypt by the German professor Georg Ebers in 1872.
• The earliest written reference to cataract surgery is found in Sanskrit manuscripts dating from the
5th century BC, They are thought to have been written by the Hindu surgeon Susruta.
1. Reclination / Couching
2. Intracapsular cataract extraction (ICCE)
3. Extracapsular cataract extraction (ECCE)
4. Phacoemulsification (conventional Surgery nowadays)
5. Catarex / Femtocataract; using femtosecond laser in some steps of phacoemulsification eg:
corneal incisions.
Concept of cataract surgeries
Reclination / Couching
• This is the first known cataract surgery..
• The technique called COUCHING
consisting of pushing the lens in the
back of the eye, which gave a better,
but not a normal vision by any means.
• A cataractous lens was displaced away
from the pupil to lie in the vitreous
cavity in the back of the eye.
• Reclination was practiced in Egypt,
India and in ancient Greece.

UCL materials
• ICCE
Cryo-surgery was introduced by Krawicz of Poland
in 1961 to remove the lens with a freezing Cryo
probe that attached to the surface of the cataract.
The whole lens is removed including its capsule.

• ECCE
Meaning the lens is removed and the lens capsule
is left intact to allow for implantation of
Intraocular lens (IOL).
It requires large corneal incision 9-13mm and uses
of stitches.
Its indicated in patients with very hard cataracts.
[Link]
87482034/
Phacoemulsification
• In the late 1960s Charles Kelman of New York
developed a technique for emulsifying the
lens contents using ultrasonic vibrations and
aspirating the emulsified cataract.
• A new IOL is implanted through a 2-3 mm
incision, the procedure takes usually takes 15
minutes and done under local anesthesia.

Intraocular lens
(IOL)

Phacoemulsification
[Link]
[Link]
Video
Ophthalmic Preoperative assessment
1. Visual Acuity testing using Snellen chart
2. Cover test a hetertropia may indicate amblyopia which carries
guarded prognosis
3. Pupillary reflex as cataracts doesn’t produce RAPD and its presence
implies a posterior segment pathology.
4. Cornea examination by specular microscopy of the endothelial cell
count (risk of corneal decompensation) or a corneal opacity which is
associated with decreased clarity during surgical operation.
5. Anterior chamber (AC) depth as shallow AC can render surgery
difficult.
Ophthalmic Preoperative assessment
6. Lens as nuclear cataract are harder than cortical cataract in
removal, also to detect any subluxation.
7. Fundus examination to exclude associated pathology as retinal
detachment (RD) or Age-related macular degeneration (AMD)
Other preoperative measure
• Informed Consent
• Biometry : a device that aids in calculation of the desired IOL power
for postoperative refraction.
Operative complications
1. Rupture of the posterior capsule of the lens
2. Loss of some lens fragments in the vitreous cavity
3. Dislocation of the IOL
4. Suprachoroidal hemorrhage
5. Acute postoperative endophthalmitis
Operative complications; TEST YOURSELF

Rupture posterior Capsule


[Link]
-aug/rupture-during-polishing-of-
the-posterior-capsule/

IOL dislocation
[Link]
g=IndianJOphthalmol_2011_59_4_32
Endophthalmitis 7_82011_f2.jpg
[Link]
ssues/2016/october-
2016/endophthalmitis-in-the-
modern-era
Ectopia Lentis
Ectopia Lentis
Refers to a hereditary or acquired displacement of the lens from its normal position.
The lens may be completely dislocated, rendering the eye functionally aphakic (luxated), or partially
displaced, remaining partly within the pupillary area (subluxated).

Clinical presentation: The early stages of


subluxation may manifest with a tremulous
lens (phacodonesis).
Causes:
A. Acquired
• Trauma.
• Inflammation, e.g. chronic uveitis.
• Hypermature cataract.
• Large eye, e.g. high myopia, buphthalmos.
• Anterior uveal tumors.
Inferior subluxation
[Link]
left-eye-showing-the-subluxated-crystalline-lens
B. Hereditary
A. Without systemic associations
2. Familial ectopia lentis is an AD
1. Ectopia lentis et pupillae is a rare congenital condition characterized by bilateral
bilateral disorder with AR inheritance
characterized by displacement of the pupil and symmetrical supertemporal
the lens in opposite directions. The pupils are displacement of the lens. It may
small and dilate poorly. manifest congenitally or later in life.

Kanski's Clinical Ophthalmology - 9th Edition. [Link]


bisecting-the-pupil_fig2_283362680
B. With systemic associations
1. Marfan syndrome Bilateral ectopia lentis (80%); subluxation is most frequently
superotemporal. The zonules are frequently intact so that accommodation is retained.
Other ocular features: glaucoma and retinal detachment, strabismus.
Musculoskeletal features include a tall, thin stature with disproportionately long limbs (arm
span > height), long fingers and toes (arachnodactyly), a narrow high-arched palate.
Cardiovascular lesions include mitral valve prolapse and aortic aneurysm formation.

Superotemporal subluxation High-arched palate Arachnodactyly


[Link] Kanski's Clinical Ophthalmology - 9th Kanski's Clinical Ophthalmology - 9th
image-in-a-patient-with-Marfan-syndrome-with-superior- Edition. Edition.
lens-subluxation
B. With systemic associations

2. Weill–Marchesani syndrome
Ectopia lentis (50%). Subluxation is
Brachydactyly
in an inferior direction.
• Microspherophakia (lens is small
and spherical) is common, so that
pupillary block with angle closure
may ensue.
• Systemic features include short
stature, short fingers and toes
(brachydactyly) and learning Dislocation of
microspheric lens into
difficulties. the anterior chamber

Kanski's Clinical Ophthalmology - 9th Edition.


B. With systemic associations

3. Homocystinuria Ectopia lentis, typically


inferonasal, is almost universal by the age of 25
years in untreated cases.
Coarse blonde hair
The zonules, which normally contains high levels
of cysteine (deficient in homocystinuria),
disintegrate so that accommodation is often lost.
Other ocular features include cataract, myopia
and retinal detachment.
Systemic features include coarse blond hair, blue
irises, malar flush.
Treatment involves oral pyridoxine, folic acid and Inferonasal
vitamin B12 to reduce plasma homocysteine and subluxation
methionine levels.

Kanski's Clinical Ophthalmology - 9th Edition.


Management:

Mainly depends on treatment and preventing Complications resulting from the lens
tilt which includes refractive error (mainly astigmatism), glaucoma and Uveitis.
1. Spectacle correction of Astigmatism
2. Surgical removal of the lens if glaucoma or uveitis developed or large error of
refraction.
Key Messages
• Worldwide, Cataract is
responsible for 47-50% of all
global blindness and/or visual
impairment.
• Cataract surgery is effective in
improvement visual functions
specially distance visual acuity.
• Phacoemulsification is the
conventional surgery nowadays
with reduced operation time,
smaller surgical incision and
faster recovery and visual
rehabilitation.
• Ectopia lentis might be an
indicator of an associated
systemic disease or vice versa.
Thank you.

You might also like