Crystalline Lens - Congenital Cataracts

Terms in this set (27)

What are congenital cataracts?
Cataracts present at birth or development within 3 mos after birth; can be bilateral/unilateral, stable/progressive, vary in severity
T/F: Most congenital cataracts are idiopathic?
T
What percentage of congenital cataracts are inherited and part of syndromes?
25%
Bilateral congenital cataracts are usually inherited or acquired?
Inherited
What is the most common inheritance pattern for congenital cataracts?
Autosomal dominant
What are the major cataractogenic agents for congenital cataracts?
1. ionizing radiation - greatest risk 1st trimester of preg; esp x-ray in pelvis radiation; 2. drugs - 1st trimester of preg, CORTs, AB sulfonamides; 3. mother's metabolic health - diabetes, galactosemia, hypocalcemia, parathyroid disorders connected with Ca metabolism; 4. maternal malnourishment - protein and AA deficiencies, niacin, thiamine, riboflavin; 5. intrauterine infection - transmitted through placenta to fetus; toxoplasmosis, rubella, CMV, syphillis
When is the risk for cataractogenic agents the highest?
1st trimester of pregnancy
Description of embryonic nuclear cataract
grayish, in visual axis, bilateral, dominantly inherited, does NOT affect vision
Description of cataracta centralis pulveralenta
granular central opacity, tiny little dots in center of lens "pulverized cataract"
Description of fetal cataract
1. opacification of either ant/post fetal nucleus; 2. gray opacity; 3. little or no affect on vision
Description of sutural cataract
1. usu bilateral; 2. 30% of all congenital cataracts; 3. Y-shaped, stellate, stalagmite, coralliform opacities; 4. little or no visual reduction
Desc of anterior polar cataract
1. cotton ball like opacity on ant sub-capsule underneath anterior capsule in visual axis; 2. composed of hyaline material, Ca, cholesterol, degen lens fibers; 3. sharp demarcated edge; 4. assoc with persistent pupillary membrane; 5. little to no visual decrease
Desc of posterior polar cataract
1. cotton ball like opacity on post polar capsule; 2. assoc with hyaloid remanant almost like Mittendorf dot; 3. assoc with capsule defect; 4. sharply demarcated edge; 5. DECREASE in vision due to posterior location closer to nodal pt; DDX: posterior subcapsular cataract related to age? or complicated cataract? or congenital cataract? Sometimes hard to tell
Desc of synechiae
1. Condition where iris adheres to either the cornea in ant synechia or lens in posterior synechia. 2. inflammation involving pupillary border of iris; 3. seen in persistent pupillary membrane.
Synechiae from inflammation would involve which portion of the iris?
pupillary border (iris collarette) - ex of persistent pupillary membrane - not usu broken with dilation no problem; vs posterior synechiae from inflammation - you want to break and may continue to form a 360 attachment of pupillary border to lens and interfere with aq outflow leading to secondary glaucoma;
When do you want to break the synechiae?
posterior synechiae want to break or it may continue to form a 360 attachment of pupillary border to lens and interfere with aq outflow leading to secondary glaucoma
In general, sharp demarcated borders of cataracts are acquired or congenital?
congenital
Desc pyramidal cataract
1. cone/pyramid shaped opacity; 2. apex protrudes into anterior chamber; 3. involves ant capsule; 4. minimal effect on vision
Zonular or lamellar cataract
1. most common congenital cataract 40%; 2. usu bilateral; 3. afffects one lamella or zone of lens fibers; 4. seen as circular zone with clear center surrounding nucleus; 5. may have spokes or "riders" of overlying affected fibers; 6. opacity appears disc-shaped when viewed frontally; 7. assoc with abnormal calcium metabolism - abnormalities in tooth enamel and formation common
Most common type of congenital cataract?
zonular or lamellar cataract
Congenital cataract that has spokes or riders of overlying lens fibers?
zonular or lamellar cataract
Congenital cataract that is associated with persistent pupillary membrane?
anterior polar cataract
Congenital cataract associated with abnormal calcium metabolism?
zonular or lamellar cataract
What are the 2nd most common type of congential cataracts?
sutural cataracts
Total diffuse cataracts
1. freq inherited, but can be related to trauma; 2. all lens fibers affected; 3. white calcified mass within the pupil; 4. white capsule shrinkage shriveled up
Juvenile cataracts
1. involves juvenile nucleus; 2. present 3 mo of age into adolescence and mid-teens; 3. VA minimally affected
Presenile cataracts
1. involves adult nucleus or cortex; 2. usu premature onset of "senile" or age-related cataracts
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