congenital defects

Question 1

diaphragmatic hernia

Answer 1

hole in diaphragm

bowel in chest

lung cant expand = hypoplastic

Question 2

scaphoid abdomen

Answer 2

diaphragmatic hernia

Question 3

where does diaphragmatic hernia usually occur?

Answer 3

left MC than right

back MC than lateral

Question 4

diaphragmatic hernia dx

Answer 4

xray

Question 5

diagphargmatic hernia tx

Answer 5

surgical

corticosteriods (dev. lung)

Question 6

gastroschis/ omphalocele pathogenesis

Answer 6

gut comes out of baby twist goes back in

Question 7

extrophy cc bladder

Answer 7

omphalacele with bladder involved

• wet with urine
• shiny and red
• no bowel

Question 8

gastroschisis def

Answer 8

defect of bowel off to side right of midline

• angry nasty looking

Question 9

omphalacele

Answer 9

• midline bowel protuding

- contained in a hernia sac

Question 10

gastroschis/omphalacele

Answer 10

clinical dx

silo surgical procedure

Question 11

extrophy of bladder dx

Answer 11

clinical

Question 12

extrophy requires ___

Answer 12

surgical repair

Question 13

biliary atresia sx

Answer 13

worsening jaundice at 7-14 days

direct hyperbilirubinemia

Question 14

biliary atresia def

Answer 14

biliary ducts never made

bile cant get from liver to duodenum

Question 15

biliary atresia diagnostic

Answer 15

1. u/s no ducts

2. hida scan after 5-7 days of phenobarbital

Question 16

treat biliary atresia

Answer 16

resect and fix

Question 17

cleft lip / cleft palate

patho

Answer 17

• failure to grow
• failure to fuse
• soft, hard, palate, lip
• bilateral, unilateral, midline, superficial, deep etc

Question 18

cleft lip / palate sx

Answer 18

FTT if feeding problem

recurrent infxn

cosmetic

Question 19

cleft lip surgery timing

Answer 19

11-12 wks

Question 20

palate surgery

Answer 20

11-12 months

Question 21

Neural tube defects

Answer 21

patho: genetic disorders

folate deficiency

Question 22

***mom should be on folate supplements prior to pregnancy!!

Answer 22

neural tube defects

Question 23

dx NTD

Answer 23

quad screen elevated afp

u/s shows defect

Question 24

NTD sx

Answer 24

tuft of hair

meningomyelocele

Question 25

meningomyelocele

Answer 25

CSF and nerves protruding out posterior spine in skin sac

Question 26

meningocele

Answer 26

just CSF protruding posterior spine in skin sac

Question 27

spina abifida occulta

Answer 27

some posterior spine breakdown with skin surrounding segments, may only see tuft of hair

Question 28

NTD also associated with...?

Answer 28

arnold malformation II hydrocephalus FND below lesion