Lens Abnormalities

presbyopia

physiological decrease in amplitude of accommodation associated with aging

cataract

any opacity of the lens, small local opacity or diffuse general loss of transparency

pseudophakia

S/P cataract extraction w/ IOL

aphakia

absence of lens: congenital or acquired

subluxation

partial displacement of lens

luxation

total displacement of lens

Vossius’ ring

pigment left on the anterior lens capsule from the iris after trauma

rosette cataract

anterior or posterior subcapsular cataract due to trauma of the lens

smoking, UV exposure, DM, prolonged steroid use, ocular trauma, high myopia

what are some risk factors for cataract development?

intumescent

swelling of lens

immature cataract

scattered opacities separated by tranparent fibers

mature cataract

opaque cortex w/ intact capsule

hypermature cataract

liquefication of lens fibers w/ leakage of H2O & protein

sutural opacities/cataracts

• congenital cataract

• may be small isolated opacity along the Y suture or dense opacities along partial or total branches of Y sutures

• do not progress

• found in fetal nucleus

cerulean ‘blue dot’ opacities

• congenital cataract

• small, blush punctate opacities in cortex

• non-progressive

• no visual loss

• symmetrical

lamellar/zonular cataract

• congenital cataract

• most common infantile cataract

• bilateral, non-progressive

• secondary to insult during fetal development

• opacities occupy spherical lamellae

• U shaped “riders”

• normally patient still sees fairly well, but if interfering w/ vision needs to be removed quickly to prevent amblyopia

coronary ‘crown’ cataracts

• congenital cataract

• single/multiple club-shaped peripheral cortical opacities

• no vision loss

• often associated w/ cerulean opacities

anterior polar cataract

• congenital cataract

• dense, circular, pyramidal, well-defined opacity

• symmetric & bilateral

• typically no visual effect

posterior polar cataract

• congenital cataract

• dense, circular, pyramidal, well-defined opacity

• symmetric & bilateral

• can affect VA

nuclear sclerosis

• change in nucleus

• slow development

• signs/sx:

  • poor hue discrimination

  • reduced contrast sensitivity

  • myopic shift

  • monocular diplopia

1. slight yellowing, expected for age

2. definite yellowing

3. yellow ++

4. yellow-orange

5. orange-brown

describe the color changes for grades 1-4 of an NS cataract

cortical (cuneiform) cataract

• wedge-shaped opacities in anterior & posterior cortex

• slowly progressive

• begins inferior-nasal quadrant

• signs/sx:

  • increased glare

  • decreased contrast sensitivity

  • variable VA reduction (depends on if spokes go through visual axis)

  • lenticular, lamellar clefts-spoke opacities

  • sheet-like zones of peripheral opacification

retrodots

• secondary to oxidative stress

• risk factors:

  • alcohol consumption

  • high serum levels of HDL cholesterol

  • smoking

• associated w/ nuclear cataracts

vacuoles

• secondary to oxidative stress

• clear, spherical, fluid-filled spaces w/in the lens cortex or subcapsular space

• vary in size

• minimal effect on vision

1. <10%

2. 10-50%

3. 50-90%

4. >90%

describe the % obscuring visual axis for cortical cataracts grade 1-4

posterior subcapsular cataract

• cataracts that have a significant effect on vision, especially with a small pupil

  • surgery may be necessary earlier on

• no effect on RE

• etiology: migration & thickening of lens epithelial cells in the posterior subcapsular space

  • trauma, chronic uveitis, chronic systemic steroid use

1. 3%

2. 30%

3. 50%

4. >50%

describe the % of affected posterior capsule in PSC grade 1-4

epicapsular stars

• star-shaped deposits on anterior lens capsule

• remnants of tunica vasculosa lentis

persistent pupillary membrane

• remnant of tunica vasculosa lentis

• attached to iris collarette

• flow freely or are attached to anterior lens capsule

• may have subcapsular lens opacities

Mittendorf’s dot

• remnant of tunica vasculosa lentis

• remnant of hyaloid artery on posterior capsule

• typically inferior-nasal

• may have a tail

persistent hyperplastic primary vitreous (aka persistent fetal vasculature)

• hyperplasia of tunica vasculosa lentis

• unilateral

• decreased VA

• secondary PSC

• strabismus

• microphthalmos

• leukocoria

coloboma

• notch at inferior lens equator

• loss of zonules in region (subluxation upward)

congenital primary aphakia

• abnormality occurs during 4-5th week of fetal development

• failed induction of surface ectoderm during embryogenesis

• may be AR (mutations in FOXE3 gene)

• may be associated with severe ocular & systemic developmental anomalies

  • microphthalmia

  • aniridia

  • sclerocornea

secondary aphakia

• lens has developed but been resorbed or extruded before or during birth

• often associated w/ congenital infections like rubella

lenticonus

• lens has a cone shape

• more common posteriorly

• unilateral

• etiology: traction of hyaloid remnants or capsule weakness

• anterior is associated w/ Alport syndrome

• oil droplet appearance on retroillumination

• distorted, myopic reflex

lentiglobus

• lens has a spherical shape

• more common posteriorly

• unilateral

• etiology: traction of hyaloid remnants or capsule weakness

• oil droplet appearance on retroillumination

• distorted, myopic reflex

Alport syndrome

what systemic condition is anterior lenticonus associated w/?

microphakia

• abnormally small lens diameter

• high myopia

• subluxation & iridodonesis

• potential for pupil block & secondary glaucoma

• systemic associations:

  • Lowe’s syndrome

microspherophakia

• abnormally small lens diameter with spherical shape

• high myopia

• subluxation & iridodonesis

• potential for pupil block & secondary glaucoma

• systemic associations:

  • hyperlysinemia

  • Weill-Marchesani syndrome

  • Marfan’s syndrome

ectopia lentis

• signs/sx:

  • increased astigmatism

  • monocular diplopia

  • cataracts

  • glaucoma

• systemic associations:

  • aniridia

  • Marfan’s syndrome

  • homocystinuria

  • Weill-Marchesani syndrome

Marfan’s syndrome

• etiology: AD w/ variable expression, abnormality of connective tissue

• ocular signs/sx:

  • bilateral, superior subluxation

  • microspherophakia

  • angle anomaly → secondary glaucoma

  • segmental hypoplasia of iris dilator muscle

  • severe myopia

  • retinal detachment

• systemic associations:

  • cardiac anomalies

  • skeletal anomalies

  • normal intelligence

Weill-Marchesani syndrome

• etiology: rare, AR

• ocular signs/sx:

  • microspherophakia

  • high myopia

  • inferior subluxation

  • may precipitate acute glaucoma

• systemic associations:

  • short stature w/ broad hands & fingers

  • joint sitiffness

  • decreased mobility

  • carpal tunnel syndrome

myotonic dystrophy

• AD, mutation in mitochondrial DNA

• systemic signs:

  • impaired contraction & relaxation of skeletal muscles

  • frontal baldness

  • cardiac anomalies

• ocular signs:

  • polychromatic cortical opacities

  • christmas tree cataracts

  • bilateral ptosis

  • chronic progressive external ophthalmoplegia

  • retinal pigmentary changes

atopic dermatitis

• bilateral, posterior/anterior stellate opacities

• mature rapidly

• chronic keratoconjunctivitis

• keratoconus

Down’s syndrome

• ocular signs:

  • punctate lenticular opacities

  • narrowed & slanted palpebral fissures

  • esotropia

  • high RE

  • keratoconus

• systemic signs:

  • intellectual disability

  • defective, awkward gait

  • small stature

  • congenital heart defects

diabetes

• +FHx

• ocular signs:

  • fluctuations in RE

  • senile cataracts

  • bilateral, white punctate snowflake opacities that can mature w/in days, due to marked increase in blood sugar

rubella

• positive IgM antibodies

• 15% of women of childbearing age at risk

• systemic signs:

  • deafness

  • congenital heart defects

  • intellectual disability

• ocular signs:

  • nuclear/lamellar cataracts

  • RPE pigmentary retinopathy

  • high RE

  • strabismus

  • microphthalmos

  • glaucoma

  • optic atrophy

  • nystagmus

• preventable w/ vaccination

chlorpromazine toxic cataracts

• etiology: antipsychotic medication

• ocular signs:

  • fine, stellate pigmentation under anterior capsule w/in the pupillary zone

  • photosensitive

  • corneal endothelial & stromal pigmentation

  • retinopathy with high doses (rare)

• dose dependent

corticosteroids (PSC), chlorpromazine (anterior stellate), infrared radiation (glass blower’s cataract), x-ray radiation (PSC), UV radiation (cortical/nuclear cataract)

what things can cause toxic cataracts & what type of cataract is formed?

pseudoexfoliation syndrome

• etiology: amyloid amorphous material secreted by CB epithelium, deposited on the anterior lens capsule, posterior iris, ciliary processes, & TM

• usually bilateral

• can lead to secondary glaucoma

glaucomflecken

• diagnosis of previous acute angle closure glaucoma

• small gray-white anterior subcapsular or capsular opacities w/in pupillary zone

• no visual loss

phacolytic uveitis & glaucoma

• etiology:

  • hypermature cataract or ruptured capsule

  • inflammation secondary to the release of lens proteins

  • macrophages respond to the proteins & obstruct the TM

• uveitis ocular signs

• tx:

  • initial suppression of immune response w/ corticosteroids, then cataract extraction

Vossius ring

rosette cataract

lens subluxation

mature cataract

hypermature cataract

sutural cataract

sutural cataract

sutural cataract

cerulean cataract

cerulean cataract

lamellar/zonular cataract

lamellar/zonular cataract

lamellar/zonular cataract

coronary cataract

anterior polar cataract

posterior polar cataract

nuclear sclerotic cataract

NS cataract

NS cataract

cortical cataract

retrodots

vacuoles

cortical cataract

PSC

PSC

PSC

NS & cortical cataract

epicapsular stars

PPM

Mittendorf dot

Mittendorf dot & tail

PHPV/PFV

PHPV

PHPV

coloboma

posterior lenticonus & polar cataract

posterior lenticonus

lenticonus

microphakia

microphakia

subluxation

subluxation (Marfan’s)

subluxation (Weill-Marchesani)

christmas tree cataract (myotonic dystrophy)

christmas tree cataract (myotonic dystrophy)

christmas tree cataract (myotonic dystrophy)

stellate anterior subcapsular cataract (chlorpromazine)

pseudoexfoliation syndrome