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Lens Abnormalities
Ocular Disease II-Exam II
65
Medical
Graduate
11/04/2012

Additional Medical Flashcards

 


 

Cards

Term
Correlate crystallines (alpha, beta, gamma) with age
Definition
insoluble crystallines increase with age
Term
Lens glucose metabolism is mainly....
Definition
anaerobic
Term
lens develops from which tissue
Definition
surface ectoderm
Term
What is another name for basement membrane of lens epithelium
Definition
Anterior Lens Capsule
Term
Compare and adult lens to a child's lens
Definition

Thicker

Brighter

More spherical

Term
Lack of lens at birth
Definition
Congenital Aphakia
Term
Differentiate between primary and secondary congenital aphakia
Definition

Primary-no lens develops from surface ectoderm at all

Secondary-Lens resorbed/expelled in utero

Term
Describe the vision of someone with congenital aphakia
Definition

Severely reduced/blindness

nystagmus

amblyopia

Term
Describe how the eyes look in congenital aphakia
Definition

Microphthalmos

Microcornea

lack of fully formed anterior segment

small palpebral fissure

Term
Smaller than normal diameter lens
Definition
Microphakia
Term
Which syndrome is associated with Microphakia? What is the shape of the lens?
Definition

Lowe's syndrome

Disc shaped lens

Term
Name 4 causes of microspherophakia
Definition

Familial

Weill-Marchesani syndrome

Marfan's syndrome

Hyperlysinemia

Term
Refractive error associated with microspherophakia
Definition

Severe myopia

 

Term
Describe developmental basis for lens coloboma
Definition
Incomplete closure of ocular fetal fissure
Term
Subjective findings with lens coloboma
Definition
reduced VAs, irregular astigmatism, accommodative dysfunction
Term
Objective findings with lens coloboma
Definition
Iris, choroid, retina and optic nerve may also show coloboma
Term
How is lens coloboma treated?
Definition

Spectacles

prosthetic iris contact lens

Term
A rare condition with a circumscribed round bulge at posterior axial zone of lens that increases in size with age
Definition
Posterior Lenticonus
Term
Axial projection of central 3-4mm of lens associated with Alport's syndrome
Definition
Anterior Lenticonus
Term
Lens completely dislocated from pupillary space
Definition
Luxated Ectopia Lentis
Term
Lens partially dislocated from pupillary space
Definition
Subluxated ectopia lentis
Term
5 ways in which ectopia lentis may be acquired
Definition

trauma (zonular rupture)

Very large eyes

Anterior uveal tumours

Syphilis

Hypermature cataract

Term
Systemic triad of Marfan's syndrome
Definition

Cardiac Anomalies

Skeletal Anomalies

Muscular Underdevelopment

Term
Specify the cardiac anomalies associated with Marfan's syndrome
Definition

Aneurysm of ascending aorta

aortic regurgitation

Term
Specify the skeletal anomalies in Marfan syndrome
Definition

Long limbs disproportionate to trunk

long fingers

high arched palate

mild joint laxity

Term
Why do people with Marfan's syndrome often develop hernias?
Definition
due to their muscular underdevelopment
Term
What direction does the lens tend to displace in Marfan's syndrome?
Definition
Up
Term
List the ocular features of Marfan's syndrome
Definition

Bilateral, nonprogressive, upward lens subluxation

Angle anomaly (dense iris processes and thick trabecular sheets)

Retinal detachment

Hypoplasia of dilator pupillae muscle

Flat corneas

Axial myopia (big globes)

Term
  • Dystrophia mesodermalis hyPOplasia
Definition
Marfans
Term
Dystrophia mesodermalis hyPERplasia
Definition
Weill-Marchesani Syndrome
Term
Genetic Inheritance of both Marfan's and Weill-Marchesani
Definition
Autosomal dominant
Term
Systemic features of Weill-Marchesani
Definition

Small, short stubby fingers (brachydactylyl)

Mental handicap

Term
Direction of lens dislocation in Weill-Marchesani syndrome
Definition
Downward
Term
Ocular features of Weill Marchesani syndrome
Definition

Microspherophakia

Lens dislocation

Angle anomaly 

Term
Describe homocystinuria
Definition
Cystathione synthetase deficiency at birth that results in high levels of homocystine in plasma and urine
Term
What causes the vascular complications in homocystinuria
Definition
Increase in platelet stickiness causes thrombosis
Term
Describe systemic features of Homocystinuria
Definition
Like Marfan's but with less arachnodactylyl, vascular complications, fine/light hair and malar flush
Term
Ocular features of Homocystinuria
Definition

Downward lens subluxation

disintegration of zonules

Secondary glaucoma-due to pupil block

Term
lysine dehydrogenase deficiency that results in mental handicap, retardation and microspherophakia
Definition
Hyperlysinemia
Term
Fleck/wedge cataracts and ectopia lentis in which disorder?
Definition
Stickler's syndrome
Term
Collagen disorder from hydroxylysine deficit that results in hyperelastic skin, joints, aneurysms, angioid streaks, keratoconus etc. 
Definition
Ehler's-Danlos syndrome 
Term
Secretion of amyloid-like fibrillogranular material off the iris, ciliary body, zonules and lens epithelium
Definition
Pseudoexfoliation
Term
Objective findings with pseudoexfoliation
Definition

bulls-eye pattern on lens

flake material/pigment in angle

pupillary ruff atrophy

iris transillumination defects

krukenberg spindle

Term
Treatment process for pseudoexfoliation
Definition

Treat secondary glaucoma

Trabeculoplasty to prevent clogging

Cataract surgery

Lens extraction

Term
Secondary cataract due to acute congestive acute angle closure glaucoma
Definition
Glaukomflecken
Term
Causes development of oil droplet cataract within first few days/weeks of life due to diet
Definition
Galactosemia
Term
Differentiate between Galactosemia and Galactokinase deficiency
Definition

Galactosemia-cannot use galatose metabolically, severe disease and even death with the wrong diet.

Galactokinase def.-cannot metabolize galactose, those affected are still healthy

Term
Cataract type associated with galactokinase deficiency
Definition
Lamellar cataracts
Term
Cataract associated with Mannosidosis
Definition
Spoke-like posterior subcapsular Cataract
Term
Mannose-rich oligosaccharide accumulation due to alpha-mannosidase deficency
Definition
Mannosidosis
Term
Ocular Manifestations of Lowe's syndrome
Definition

Microphakia with lens opacities (capsular, nuclear or total)

50% have congenital glaucoma

Term
Describe cataracts in hypocalcemic syndromes
Definition
multicolored crystals/ flecks in lens that seldom progress to maturity
Term
Cataracts with  myotonic dystrophy
Definition

subcapsular and cortical polychromatic glistening granules (blue-dot) cataract

-->PSC

-->Total

Term
What is the most common cause of congenital cataracts?
Definition
Rubella
Term
5 Classic congenital rubella signs
Definition

Sensorineuronal defects

Cataracts in 15%

Heart defect (PDA)

Microcephaly

Severe mental retardation

Term

Which type of cataract can form as a result of uveitis (due to either the inflammation or treatment)

 

Definition
Posterior or Anterior Subcapsular
Term
Type of cataract most common in Hereditary Fundus Dystrophies
Definition
Posterior subcapsular
Term
Cataract formation associated with aniridia
Definition
Posterior or anterior subcapsular
Term
What can cause true lens exfoliation?
Definition
Infrared Radiation
Term
Name some essential vitamins and minerals in which deficency can lead to cataract formation
Definition

Vit B, A, C,E

zinc

Selenium

lutein

xeathanthin

Term
Cataract associated with steroids
Definition

Posterior Subcapsular

Anterior may also form eventually

Term

Which drug is associated with 5 stages of cataract development

 

Definition
Chlorpromazine
Term
Amiodarone-cataract formation
Definition

fine anterior subcapsular lens opacities

do not affect VAs

Term
What are Psoralen compounds?
Definition
Photosensitizing (UV absorbing) drugs
Term
Correlation of Rheumatoid arthritis and cataracts
Definition

Some use Chrysiasis to treat, which is colloidal gold.

Can cause fine yellow dust-like deposits in the lens and corneal stroma

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