Anterio Seg Midterm 1.3

Front 1

What is anterior lenitconus caused by?

Back 1

-thinning of affected anterior capsule (fragile basement membrane)

Front 2

Is anterior lenticonous bi or unilateral

Back 2

bilateral

Front 3

Anterior lenticonus is commonly associated with

Back 3

Alport's syndrome

Front 4

What is the most common and earliest manifest of Alport Syndrome?

Back 4

Hematuria (gross of microscopic)

Front 5

T/F Microscopic hematuria is observed in all males and females.

Back 5

False
all males and 95% females

Front 6

What is the most common ocular fidning in Alport's Syndrome? (Hint: X-lined)

Back 6

dot and fleck retinopathy
85% males

Front 7

T/F Alport's/Anterior Lenticonus dec. VA.

Back 7

FALSE
spares the macula

Front 8

___ and ___ systemic conditions almost always accompany Alport's.

Back 8

renal failure and hearing loss

Front 9

Posterior polymorphous corneal dystrophy

Back 9

rare ocular manifestation in pts. with AS

Front 10

What is the difference between Anterior and Posterior Lenticonus

Back 10

Posterior Lenticonus is NOT associated w/ systemic problems and is unilateral

Front 11

If you see posterior lenticonus bilaterally, what should you suspect?

Back 11

inheritable condition

Front 12

Lentiglobus

Back 12

-unilateral
-can cause polar cataracts
-3-15yo
-globular rather than conical protrusion

Front 13

The most common cause of ectopia lentis is

Back 13

trauma

Front 14

What are the 1st and 2nd most common causes of hereditary ectopia lentis

Back 14

1-marfan
2-homcystinuria

Front 15

Weil-Marchesani

Back 15

rare skeletal malformation syndrome, limited joint mobility, and microspherophakia

Front 16

Marfan syndrome:
lens dislocation (75%) is where?

Back 16

bilateral, symmetrical, and supertemporal

Front 17

Homocystinuria:
lens dislocation is where?

Back 17

bilateral, symmetrical, and inferonasal

Front 18

How does Weil pt. subluxates?

Back 18

inferiorly

Front 19

Diagnostic Triad of Lowe's

Back 19

congenital cataracts, neonatal/infantile hypotonia, renal tubular dysfunction

Front 20

When do Lowe's pts. usually die?

Back 20

20s/30s

Front 21

Lens coloboma

Back 21

segmental agenesis rather than true coloboma

Front 22

Lens coloboma is associated with (3)

Back 22

3M's
Microphthalmia
Marfans
Morning Glory

Front 23

Mittendorf Dot

Back 23

lens vesicle is formed independent of closure of fetal fissure

Front 24

Mittendorf Dot may be associated with (3)

Back 24

-posterior lenticonus
-lentiglobus
-microspherophakia

Front 25

Posterior Embryotoxin

Back 25

-thickened, anteriorly displaces Schwalbe's line

Front 26

2 syndromes associated wtih Posterior Embryotoxin

Back 26

Axenfield-Rieger
Alagille's

Front 27

Axenfield's Anomaly

Back 27

describes findings of post embryotoxon coupled with peripheral iris processes extending to or beyond prominent Schwalbe's loine

Front 28

Axenfield Syndrome is when anaomlay is present along with _____.

Back 28

associated glaucoma

Front 29

glaucoma develops in __% of pts with Axenfield anomly

Back 29

50%

Front 30

Rieger's Anomaly Triad

Back 30

Axenfield's anomaly (post embryotoxin +iris strands) + iris,

Front 31

What is the most widely held theory on the development of Axenfield/Rieger's?

Back 31

developmental arrest of specific anterior segment tissues derived from neural crest cells
(retention of a primordial endothelial cell layer on portions of iris and angle structures)

Front 32

What is the difference between Type I and Type II Peter's Anomaly

Back 32

Type I- no lens involvement
Type II- lens involvement

Front 33

Glaucoma occurs in ___% of Peter's Anomaly pts.

Back 33

50-70%

Front 34

T/F Peter's Anomaly is usually unilateral

Back 34

False, Bilateral

Front 35

What is a key systemic feature of Alagilles?

Back 35

neonatal jaundiceT/F

Front 36

T/F Nearly ALL Alageille pts. have cardiac murmurs.

Back 36

true

Front 37

What is the most frequent ophthalmologic finding in Alagille?

Back 37

Posterior embryotoxon

Front 38

Miller's syndrome

Back 38

aniridia and Wilm's tumor

Front 39

Gillespie's Syndrome

Back 39

mentral retardation and cerebellar ataxia
(associated with automosomal recessive with inherited cases of aniridia)

Front 40

WAGR syndrome

Back 40

A rare genetic disorder that has
Wilms Tumor
Aniridia
Genitourinary malformations
Retardation

Front 41

What are the 2 types PPM?

Back 41

type I- has attachments only to the iris
type II- iridolenticular adhesions

Front 42

Wachendorf's membrane

Back 42

part of TVL

Front 43

Microphthalmos is defined as

Back 43

total axial length at least 2 standard deviations below age-similar controls

Front 44

There are 4 types of microphthalmos

Back 44

-simple: not associated with other major ocular malformations
-complex: associated with coloboma
-with cyst: associated with formation of orbital cyst
-posterior:decreased TAL, normal corneal diam., high hyeropia, retinal folds

Front 45

What are the two types of anophthalmos?

Back 45

-simple:
-with cysts: globe is replaced by cysts

Front 46

T/F Nanophthalmos is a small eye with NORMAL structures.

Back 46

N for Normal
TRUE