Types of Cataracts & Diseases

Myotonic dystrophy is aka...

"floppy baby" syndrome

Pre-natal rubella infection can cause what other problems?

-hearing loss -nuclear cataracts -glaucoma -microcornea -fundus pigmentation -might have a MENTAL disability

what are intrauterine causes present at birth resulting in congenital cataracts? (5)

1. congenital rubella (measles) -> NC 2. intrauterine toxoplasmosis 3. CMV 4. opportunistic infection 5. maternal drug use (steroid, thalidomide)

what are the metabolic causes of cataracts?

1. diabetes 2. hypertension (medication) 3. galactosemia 4. galactokinase deficiency (from Graves disease) 5. Fabry's disease 6. Atopic dermatitis - really bad allergy; pts on long term steroid = PSC or stellate shaped cataract

what are lens size and shape abnormalities (3)?

1. lens coloboma - inferior nasal; associated iris coloboma; due to incomplete closure of embyonic fissure 2. microspherophakia - smaller diameter lens; spherical shape; opacification often present (ex: Weill Marchesani, Marfans - sometimes); bilateral, congenital 3. Lentiglobus - comparable to keratoconus; one specific area w/out-pouching of the lens; deformity of surface; conical, nipple-like

What is the #1 cause of cataracts causing a decrease in vision?

AGE

Lawrence-Moon-Biedl findings include: polydactyly, hypogonadism, retinitis pigmentosa, microcornea, iris coloboma & cataracts & MENTAL RETARD. Transmission?

AR

what is a pulverant cataract also known as?

Coppock's cataract or Doyne's discoid cataract

due to excessive heating of pre-lenticular iris; anterior capsule fibers "pull-off"/peel; may have weaker capsule or zonules

Exfoliation (true exfoliation is RARE - old furnace workers had this)

what is the surgical indication for Exfoliation?

Exfoliative glaucoma (lens fibers stuck in angle)

aka oculo-mandibulo-dyscephaly (bones of face/head not formed properly), nystagmus, microphthalmos, blue sclera, cognitive issues; not compatible w/life; BILATERAL CONGENITAL cataracts

Hallerman-Streif

hypermature cataract (cataract of any etiology that has reached advanced stages); imbibed water; whole cataract has progressed so fat that it begins to decompensate and cortical tissue LIQUEFIES so that the density of the lens sags; NUCLEUS DROPS out of center - poor VA

Intumescent

Arachnodactyly, skeletal abnormalities, slender bones, weak joints and connective tissue disorder; can be aortic/cardiac issues

Marfan's Syndrome

OLIGOPHRENIA (decreased development of the brain because the head is small - cranium space is limited) = MENTAL RETARDATION; cerebral ataxia, short stature, stabismus; BILATERAL CATARACTS

Marinesco-Sjogren's Syndrome

what is an intumescent cataract also known as?

Morgagnian type cataract

hypotonia w/wasting of musculature; facial diplegia, lacks expression, ptosis, chorioretinitis, retinal degeneration, cherry red macula, AD transmission & MENTAL RETARD

Myotonic Dystrophy

how does PS differ from PPM?

PPM is congenital

following a vitrectomy and other post seg procedures, what cataracts are commonly found due to oxidative stress and mechanical factors (gas tamponade or silicone oil)?

PSC initially then NS

Pseudo-exfoliation results in deposits in the anterior chamber - fine white substance. Associated w/?

Pigment dispersion Glaucoma - clog TM

mutation of the LOXL1 gene; synthesized from cells of CB, TM, iris, pre-equatorial lens; double ring appearance due to iris constricting and dilating between the two rings; anterior surface peels away (only superficial epithelial cells)

Pseudo-exfoliation

"peppercorn" presentation; small, dark, dense fetal opacity

Pulverant

associated w/small equatorial cortical opacities; look like isolated lines in the periphery; does not affect VA & will not progress

Riders

what type of cataracts present w/Wilson's disease?

SUNFLOWER

similar to an axial (Y-suture) opacity except that it is MUCH DENSER than an axial cataract; farther from fetal nucleus; does not change rapidly; lies along optic axis

Sutural cataract

mandibulofacial dysostosis (bad bone condition); AD; NO mental retardation; bird like faces; hearing loss; normal cognition

Treacher-Collins

how do cortical cataracts form?

UVB changes the H20 balance in the lens, causing a separation of fibers; opacities start as vacuoles and water clefts between lens fibers; begin inferiorly then progress

"inverted marfans" (short bones); brachydactyly (stubby fingers, short statue), ectopia lentis, spherophakia, microphakia (small lens), microcornea

Weill-Marchesani

hepatolenticular degeneration w/Kayser Fleischer ring in cornea; LIVER problems w/processing copper

Wilson's Disease

what should you look for w/concussive injuries?

angle recession; vossius ring (imprint of iris on lens - pigment deposition)

congenital absence of iris (iris root may or may not be present); associated w/lens deformity; can be associated w/glaucoma or albanism

aniridia

what cataracts are found with chronic anterior uveitis?

anterior and posterior subcapsular --> progress more quickly when found w/PS

In acute angle closure glaucoma (glaukomflecken), the acute spike in IOP causes a cataract where?

anterior lens capsule

anterior or posterior Y-suture opacity (not as dense as sutural cataracts); central lens - lies along the optic axis

axial cataract

how are cortical cataracts graded?

based on quadrants 25% = grade 1 50% = grade 2 75% = grade 3 100% = grade 4

opacity or cloudiness of the lens that may prevent a clear image from forming on the retina

cataract

cortical opacity; present in the far equator; speckled areas in lens peripher, bluish tint

cerulean

AR; associated w/skeletal abnormalities - malformation of cartilage, usually short stature & short life span; bones calcify near joints; BILATERAL CONGENITAL CATARACTS

chrondrodystrophia calcificans - congenital

What is the most common cause of secondary cataracts (result of other eye diseases)?

chronic anterior uveitis

1/3 of _______ cataracts are hereditary

congenital (pt was born w/it)

birth defect due to German Measles

congenital rubella

"crown-like" - stem w/a dot; present at equator; involves cortex; aka "blue dot" cataract

coronary

what is the most common lens opacity seen in clinical practice & the least clinically significant?

cortical cataracts

Toxic cataracts are caused by medications and enhanced by environmental factors (smoking, diet, alcohol, UV*). Ex of medications?

corticosteroids anti-psychotics phototherapy anti-neoplalstics anti-arrhythmias miotics RA meds

occur at any layer; similar to "christmas tree type"; usually unilateral; reflective and colorful; little effect on VA

crystalline cataract

FB makes contact w/lens

direct penetration (monitor - may have no effect on VA)

Lens Luxation due to:

dislocation of lens displacement of lens weakened/broken zonule fibers Marfans (superior luxation) Weill marchesani Trauma

high myopia can be associated w/posterior lens opacities & __________.

early nuclear sclerosis

What are the ocular problems associated w/Marfans?

ectopia lentis, lens subluxation

pupillary lens; pisciform (fish-tail shaped); little to no effect on acuity

epicapsular stars

what are two other capsular opacities (other than cataracts)?

epicapsular stars mittendorf dots (posterior capsule; deposition of PTVL)

other conditions causing cataracts?

hypocalcemia hypoparathyroidism albright's hereditary osteodystrophy pseudohypoparathyoidism

any opacity affecting any given lamella (layer) of the lens; DEVELOPMENTAL cataract --generic term

lamellar cataract

why pseudo-ex problematic for cataract surgery?

may have made zonules weaker = cataract surgery more challenging

What can result from lens luxation?

monocular diplopia RE shift VA shift

advanced lens changes can cause what kind of RE shift?

myopic - narrowing the anterior chamber

why is this cataract visually threatening?

near vision diminished more than distance (centrally located cataract); nodal point is critical

what type of cataract results from congenital rubella (15%)?

nuclear

changes occurring at the level of the nucleus; brunescent changes; relucent light;

nuclear cataract

Treacher-Collins presents w/what type of cataracts?

nuclear sclerosis

benign iris strands; can have any connection of the iris; visually significant if crosses pupil; prenatal reduction of the pupillary membrane

persistent pupillary membrane (PPM)

what must we worry about w/intumescent cataracts?

phacolytic glaucoma -> lens leaks, which causes inflammatory response and increased IOP = glaucoma; anterior chamber becomes shallow **IMMEDIATE ATTENTION needed

variable shapes; pigment deposition on the anterior lens surface or posterior cornea (Kruckenberg spindles on the endothelium); pigment in the angle; pseudoexfoliation syndrome

pigment dispersion

dense capsule opacity; anterior or posterior; can be AD; lies along the optic axis; due to toxic, electric, or traumatic

polar cataract

findings of LOWE's Syndrome (Lowe's oculo-cerebral-renal syndrome)? transmission (AR, AD, X)?

posterior lenticonus megalocornea bupthalmos dense bilateral cataracts; mother = punctate MENTAL RETARDATION X-LINKED

adhesion of posterior iris to the lens; can disrupt anterior capsule and cause cataracts; due to trauma or uveitis

posterior synechia

hereditary fundus dystrophies may result in early onset cataracts. Ex?

retinitis pigmentosa

a cataract associated w/a concussion -> anterior cortical (in cortex); petalloid appearance, not usually symptomatic

rosette cataract

what is a sutural cataract also known as?

stellate cataract

fibrous metaplasia of the anterior lens epithelium; posterior migration of epithelial cells

subcapsular cataracts

what kind of cataracts can Myotonic dystrophy cause?

subcapsular, cortical opacities polychromatic opacity (christmas tree cataract) spoke-like opacities in central lens (stellate - like)

most common cause of unilateral cataract especially in young

traumatic

specific type of lamellar cataract where a single layer is involved; metabolic etiology; not associated w/fibers

zonular cataract