eyes ears nose throat

Question 1

What causes ROP

Answer 1

It is a two phase process:

1) due to relative hyperoxia in comparison to in utero –> oxygen depresses vegf and slows vascularization
2) then avascular zones experience hypoxia –> increasing vegf activity –> abnormal vessel development

Question 2

ROP staging

Answer 2

zone 1-3, starting from inner to outer retina

stage 0-5:

0- immature, no rop.

1 - line of demarcation between vascular and avascular.

2 - elevated line or ridge.

3 - extraretinal vascular proliferation.

4 - partial retinal detachment.

5 - complete retinal detacment.

Question 3

When and who do you screen for ROP

Answer 3

Screen VLBW <1500g or <30 w

Screen at 4w or 31w, whichever is later

Question 4

When do you treat ROP

Answer 4

• if Z1 - any plus disease or S3 w/o plus
• if z2 - S2 or S3 with plus

Question 5

What are causes of congenital ptosis?

Answer 5

1) simple (myopathic) ptosis - abnormal development of levator muscle, unilateral.
2) blepharophimosis - severe b/l ptosis, horizontally shortened palpebra.
3) markus-gunn jaw-winking: spastic elevation of levator when masseter is used

Question 6

what is leukocoria and what causes it

Answer 6

Leukocoria is a white pupil due to decreased red reflex from retina. Causes include retinoblastoma, retinal detachment/hemmorhage, and cataracts.

Question 7

What are syndromic and non-syndromic causes of cataracts and what are treatment options.

Answer 7

Syndromic (2/3) causes include trisomies and rubella

Nonsyndromic (1/3) includes Coppock cataract which is familial).

Tx : if significant, lensectomy at 2-3m age

Question 8

What is PETERs anomaly

Answer 8

Remembered by the acronym CHED: sclerocornea, dermoid, Congential Hereditary Endothelial Dystrophy

Question 9

What causes glaucoma, how does it present, and which conditions is it associated with?

Answer 9

Due to increased ocular pressure –> corneal edema, optic n. damage.

Presents as common triad: photophobia, blepharospasm, epiphora (excessive watering of eyes).

Assc with SW, NF1, Lowe (condition effecting eyes, brain and kidney in males)

Question 10

TORCH infections related to eye issues

Answer 10

cataract: rubella
keratitis: hsv, syphillis
retinopathy: syphilis, cmv, toxo, rubella

Question 11

What is the differential for conjunctivitis based on time of presentation?

Answer 11

0-2d: chemical

3d-3w: gonnorhea - purulent, tx w/ ceftriaxone

5-10d: chlamydia - clear, tx w/ erythromycin

Question 12

retinoblastoma: genetic locus, work up and treatment

Answer 12

genetic locus: RB1 mutations on ch 13, RB1 mutations

Consider MRI to rule out neuroectodermal tumor of pineal gland

tx: chemo and then local consolidation

Question 13

auditory brainstem response

Answer 13

for conduction hearing loss, text cochler n and brainstem electrical activity in response to tone stimuli

Question 14

new born hearing screen

Answer 14

should be done admission or by 1 m of life abr recommended for nicu if failed, repeat at 3m if at risk, repeat 16m for 3 y

Question 15

otoacoustic emissions

Answer 15

acoustic emissions generated by cochlear outer hair cells., most commonly used for nbhs- generated in response to tone

Question 16

laryngomalacia

Answer 16

most common cause of stridor. omega shaped epiglottis curling inwards

Question 17

vocal cord paralysis

Answer 17

second most common cause of stridor. if present, obtain mri to rule out chiari.

Question 18

neonatal airway differences

Answer 18

• larynx is higher in neck with more acute angle from oropharynx - cricoid cartilage is narrowest point

Question 19

cleft lip/palate

Answer 19

CL+P: failure of fusion of medial nasal processes (4-6w) CP: failure of suion of lateral palatine processes (8-12w) 95% have OM CL repair at 10 w, CP repair at 6-18m

Question 20

choanal atresia

Answer 20

failure of nasal buccal membrane to resorb at 5 weeks

Question 21

normal development with branchial/pharyngeal arches

Answer 21

Question 22

Pierre Robin sequence is associated with which branchial arch

Answer 22

1st

Question 23

DiGeorge is associated with which branchial structures

Answer 23

hypoplasia of first branchial arch and 3rd and 4th branchial pouches (associated with TEF, trachelmalacia, glottic web and hearing loss and Cleft palate)

Question 24

branchio-oto-renal syndrome

Answer 24

x

Question 25

branchial cleft anomalies

Answer 25

(1st branchial cleft) I: preauricular cyst

II: submandibular region

(2nd branchial cleft) most common type of branchial cleft cysts, painless fluctuant mass in anterior triangle of neck

(3rd bc): rare, mass in lower anterior neck

Question 26

thyroglossal duct cyst

Answer 26

most common congenital anomaly of neck, failure of obliteration of thyroglossal tract. painless midline neck mass close to hyoid bone that elevates with swallowing or tongue protrusion

Question 27

pseudotumor of infancy

Answer 27

fibrotic lesion of SCM, present with torticollis, associated with difficult delivery

Question 28

dermoid cyst

Answer 28

midline painless mass that *doesn’t* elevate with tongue protrusion

Question 29

teratoma

Answer 29

arise from misplace germ cells, all 3 layers. large midline mass, may cause respiratory compromise and require EXIT procedure. termed epignathus if arising from palate

Question 30

puillary light response

Answer 30

evident by 30-31 weeks, Absence is abnormal after 32 weeks

Question 31

infantile periocular hemangioma

Answer 31

mc childhood orbital tumor. spontaneous regression. gradually enlarging reddish mass in inner canths which becomes engorged when infant cries or strains.

Question 32

encephalocele

Answer 32

located midline on nasal bridge, higher than medial canthus. enlarges when crying and stranining, may be pulsatile. does not regress.

Question 33

dacrocystocele

Answer 33

congenital swelling soon after birth, bluish, due to trapped fluid in naso lacrimal duct and lacrimal sac. massage, observe, potentially drain. may cause nasal obstruction, adjacent but lower to medial canthus.