Untitled spreadsheet - peds

Question 1

Pediatrics

ddx of poor vision with normal exam

Answer 1

LCA, achromatopsia, csnb, albinism, optic nerve hypoplasia, CVI

Question 2

Pediatrics

AL in kids

Answer 2

17mm

Question 3

Pediatrics

cornea in kids

Answer 3

9.5-10.5

Question 4

Pediatrics

associations with nanophthalmos

Answer 4

hyperopopia, choroidal effusion

Question 5

Pediatrics

location of dermoid

Answer 5

supertemporal quadrant near brow nea rsuture, filled with keratin

Question 6

Pediatrics

lethal association with capillary hemangioma

Answer 6

kassabach-Merrit syndrome: consumptaite coagulaopathy, high output CHF

Question 7

Pediatrics

poptosis increasing with crying

Answer 7

varix

Question 8

Pediatrics

albright’s syndrome

Answer 8

fibrous dysplasia, short stature, diplopia, decreased vision

Question 9

Pediatrics

tumors of fibrous connective tissue, cartilage, and bone

Answer 9

fibrous dysplasia, short stature, diplopia, decreased vision

Question 10

Pediatrics

most common form of rhabdo

Answer 10

embryonal

Question 11

Pediatrics

most lethal rhabdo

Answer 11

alveolar

Question 12

Pediatrics

best prognosis

Answer 12

pleomorphic

Question 13

Pediatrics

lytic lesion of orbital roof causing progressive proptosis

Answer 13

histiocytosis x

Question 14

Pediatrics

benign local solitary bone lesion superiortemporal bony orbit

Answer 14

eosinophilic grnuloma

Question 15

Pediatrics

proptosis, lytic skull defects, DI

Answer 15

Hand schuller christian disease

Question 16

Pediatrics

multisystem histiocytosis x

Answer 16

Lettere-siwe disease

Question 17

Pediatrics

burkitt’s lymphoma

Answer 17

ffects maxilla in black children, related to eBV

Question 18

Pediatrics

increased interpupillary distance due to increased sitance between medial orbital walls

Answer 18

hypertelorism

Question 19

Pediatrics

lateral displacement of inner canthi,c onfluent eybrows, sensineural deafness, white forelock.

Answer 19

Waardenburg’s syndrome

Question 20

Pediatrics

RD, congenital galucoma, cataracts, high myopia

Answer 20

Pierre Robin sequence

Question 21

Pediatrics

partial or complete fusion of lip margins

Answer 21

ankloblepharon

Question 22

Pediatrics

absent lid crease, ptosis telecanthus, epicanthus inversis, ectropion

Answer 22

blepharophimosis

Question 23

Pediatrics

different types of epicanthus (medial canthal verticsl skin folds)

Answer 23

TIPS (tarsalis- upper, inversus lower, palpebral U=L, supracilus above the cilia).

Question 24

Pediatrics

euryblepharon

Answer 24

horizontal idening of palpebral fissure

Question 25

# Pediatrics definition of telcanthus

Answer 25

widened intercanthal distance due to long medial canthal tendons

Question 26

# Pediatrics associations of telcanthus

Answer 26

fetal alcohol, Waardenburg's blepahrophimosis.

Question 27

# Pediatrics where does dacryocystocele present

Answer 27

inferior and nasal to medial canthus.

Question 28

# Pediatrics bilateral pseudomembraneou conjuncitivitis in kids

Answer 28

ligneus conjuncitivitis

Question 29

# Pediatrics two forms of CHED

Answer 29

AR: nystagmus, at birth, AD: later, pain tearing no nystagmus

Question 30

# Pediatrics autonomic dysfunction to block of NE production

Answer 30

decreased corneal sensation, emotional lability

Question 31

# Pediatrics external findings of syphilis

Answer 31

hutchinson's teeth, mulberry molars, saber shins, frontal bossing, saddle nose

Question 32

# Pediatrics aniridia, mr and ataxia

Answer 32

guillespie's syndrome

Question 33

# Pediatrics where are iris colobomas

Answer 33

inferonasal

Question 34

# Pediatrics ddx of microspherophakia

Answer 34

weill marchesani, alport, rubella, marfan, lowe's peters.rx. Cycloplegia

Question 35

# Pediatrics iris mass tumor arising from nonpigmented epithelium

Answer 35

medulloepitheliuoma, can have teratoid eleemnts

Question 36

# Pediatrics syndrome with anterior lenticonus

Answer 36

Alport's (hereditaroy nephritis, deafness)

Question 37

# Pediatrics pathology of posterior migration of lens epithelium

Answer 37

posterior polar cataracts

Question 38

# Pediatrics oil droplet cataract

Answer 38

glactosemia (AR)

Question 39

# Pediatrics defect in galactosemia

Answer 39

galactokinase UDP galactose

Question 40

# Pediatrics rx for galactosemia:

Answer 40

elminate lactose, also have MR, hepatomegaly, jaundaice

Question 41

# Pediatrics cornea verticillata, spoke like cataract

Answer 41

Fabry's disease

Question 42

# Pediatrics defect in Fabry's

Answer 42

alpha galactosidase A

Question 43

# Pediatrics findings in Fabry's

Answer 43

angiokeratomas, CV events

Question 44

# Pediatrics inheritance of Lowe's

Answer 44

X linked

Question 45

# Pediatrics findings in Lowe's

Answer 45

bilateral cataracts, renal tubular acidosis, MR, glaucoma

Question 46

# Pediatrics anterior lenticonus, deafness and hemorrhagic neophropathy and renal failure

Answer 46

Alport's

Question 47

# Pediatrics syndrome with glaucoma and cataract

Answer 47

Hallermann-Strieff syndrome : hypoplasia of mandible with birdlike faces, microphakia, microconia

Question 48

# Pediatrics Workup for bilateral congenital cataracts

Answer 48

UA ( amino acids: Lowe's, galactosemia), blood tests: calcium TORCH, hypoglycemia, Karotyping, audiograms: congenital rubella, Alport's.

Question 49

# Pediatrics ddx of congenietl cataractws and glaucoma

Answer 49

Lowe's, Hallermann-Strieff, rubella

Question 50

# Pediatrics findings of congeneital rubella

Answer 50

cardiac efets, deafnesss, MR.

Question 51

# Pediatrics complication of pedi cataract surgery

Answer 51

aphakic glaucoma (15%).

Question 52

# Pediatrics define monofixation syndrome

Answer 52

central facultative scotoma, peripheral fusion.

Question 53

# Pediatrics treacher collins is hypoplasia of what structure?

Answer 53

first and 2nd brachial arches

Question 54

# Pediatrics difference between apert's and crouzon's

Answer 54

syndactyly of hands, cleft palate, and facial deformaties in Apert.

Question 55

# Pediatrics origin of inferior oblique

Answer 55

floor or orbit.

Question 56

# Pediatrics what immune problems with ATM

Answer 56

low levels of Iga

Question 57

# Pediatrics eye findings in ATM

Answer 57

supranuclear palsies and telangiectasias.

Question 58

# Pediatrics ocular associations with aniridia

Answer 58

glaucoma, congenital nystagmus, lenticular, pannus, foveal hypoplasia

Question 59

# Pediatrics what is miller's syndrome

Answer 59

aniridia, wilm's tumor, deletion of short arm of 11

Question 60

# Pediatrics features of lowe's syndrome

Answer 60

cataract glaucoma, aminoaciduria, microphakia

Question 61

# Pediatrics features of alport's

Answer 61

anterior lenticonus, cataract, albipunctatus like retinal lesions

Question 62

# Pediatrics rx for neonatal chlamydial infection

Answer 62

oral erythomycin, and oral tetra or erytho for parents.

Question 63

# Pediatrics what refractive erros associated with CSNB and LCA

Answer 63

LCA: hyperopia, X linked: myopia