Peds- Congenital Abnormalities Flashcards Preview

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Flashcards in Peds- Congenital Abnormalities Deck (62)
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1

Omphalocele vs gastroschisis:
which one has a sac or covering?

Omphalocele
( cOvering = Omphalcele)

2

Omphalocele vs gastroschisis:
which one has no covering? just intestines poping out

gastroschisis
( G-sausage = Gastroschisis)

3

Omphalocele:
where is defect

base of umbilicus, within the umbilical cord
(the covering)

4

gastroschisis:
where is the defect

lateral to the umbilicus, periumbilical

5

gastroschisis:
has no amnion or covering what does this mean it is important for the anesthetist to do?

prevent hypothermia
Prevent infection
prevent dehydration

6

Omphalocele vs gastroschisis:
which one needs a cardiac eval prior to proceeding to sx

omphalocele

7

Omphalocele vs gastroschisis:
which one is NOT associated with other anomalies

gastroschesis

8

Omphalocele
is associated with what sysndrome

beckwith-woideman syndrome (giantism)

9

Omphalocele vs gastroschisis:
which one is associated with high gastric pressures post op and usually do not have primary closure

omphalocele

10

Omphalocele
with increases gastric pressures what 3 things can occur as a complication

dusky bowel
dead bowel
dead baby

11

Omphalocele vs gastroschisis:
which one has a better survival rate

gastroschisis

12

Omphalocele vs gastroschisis:
is anesthesia concerns the same or different for both

same

13

Omphalocele vs gastroschisis: Anesthesia concerns
what do you want to do before induction?

decompress stomach w/ NG tube

14

Omphalocele vs gastroschisis: Anesthesia concerns
what do you want to do with induction?

intubate awake/asleep
with or without paralysis
(basically any indiction works)

15

Omphalocele vs gastroschisis: Anesthesia concerns
should you use N2O?

Nope

16

Omphalocele vs gastroschisis: Anesthesia concerns
what is required to replace bowel into the and cavity?

muscle relaxation

17

Omphalocele vs gastroschisis: Anesthesia concerns
keep intubated how long post op

1-2 days

18

Omphalocele vs gastroschisis: Anesthesia concerns
should you be aggressive with fluid replacements?

yes

19

Omphalocele vs gastroschisis: Anesthesia concerns
what should you do with the ambient temperature of the OR

keep it warm ( > 24C or 75F)

20

Prune Belly Syndrome:
is associated with complications of what systems in the infant?

urinary tract
Bladder

21

Prune Belly Syndrome:
how to remember?

prUne Belly
Urinary tract
Bladder

22

Prune Belly Syndrome:
why is 95% of incidences associated w/ male babies?

b/c it is usually associated with bilateral undescended testicles

23

Prune Belly Syndrome:
you should always treat these pts as what

full stomachs

24

Intestinal malrotation and vulvulus:
what is it

spont abnormal rotation of the midgut around the mesentery

25

Pierre-Robin Syndrome:
what are complications associated with their palate?

cleft palate that is incomplete
possible high arched palate

26

Pierre-Robin Syndrome:
is the Lip involved with the cleft palate?

not in this case

27

Pierre-Robin Syndrome:
what is the abnormalities associated with the facial / oral structures? (what 5 things are Small)

small face
small glottis
small jaw
small tongue
small palate

28

Pierre-Robin Syndrome:
the chin is displaces where?

posteriorly

29

Pierre-Robin Syndrome:
what other problem is wrong with the tongue besides just being small?

glossoptsosis

30

Pierre-Robin Syndrome:
what can cause airway obstruction?

the tongue

31

Treacher Collins Syndrome:
they have a deformity of what structures?

facial mouth deformities

32

Treacher Collins Syndrome:
what abnormalities are associated with the face/oral structures? (what 3 things are small)

Small mouth
Facial hypoplasia
Pharyngeal hypoplasia

33

Treacher Collins Syndrome:
is associated with atresia of what?

choanal atresia
(tissue in back of nasal passage blocked)

34

Treacher Collins Syndrome:
what other abnormalities (structure related) do these pts have

ear malformation
Notching of lower eyelid
underdeveloped malar bones
coloboma (hole in an eye structure)

35

Treacher Collins Syndrome:
do these pts have cardiac defects

yes

36

Treacher Collins Syndrome Vs Pierre- Robbin syndrome:
which one os more sever?

Treacher Collins Syndrome

37

Treacher Collins Syndrome & Pierre- Robbin syndrome:
do they have difficult intubation?

umm yes

38

Treacher Collins Syndrome & Pierre- Robbin syndrome:
what intubation tech is best

Awake intubation
fully awake before extubation

39

Trisomy 21 Syndrome/Down's Syndrome:
they have and extra chromosome where

the 21st

40

Trisomy 21 Syndrome/Down's Syndrome:
describe their major abnormalities

Short neck
flat occiput
mental retardation
high arched palates
micrognathia (small jaw)
macroglossia
Alantoaxial instability

41

Trisomy 21 Syndrome/Down's Syndrome:
what is a subglottic anomaly that they will have the rest of their life and approx 2% of all pts will have it

subglottic stenosis

42

Trisomy 21 Syndrome/Down's Syndrome:
what are other associated anomalies?

congenital heart dz
TEF
floppy soft palate
ASD
VSD
PDA
OSA

43

Trisomy 21 Syndrome/Down's Syndrome: Anesthesia concerns
if there a difficult airway

yes

44

Trisomy 21 Syndrome/Down's Syndrome: Anesthesia concerns
should you use a smaller or larger OETT

smaller

45

Trisomy 21 Syndrome/Down's Syndrome: Anesthesia concerns
what should you pay close attention to post op

post op apnea and stridor (common)

46

Trisomy 21 Syndrome/Down's Syndrome: Anesthesia concerns
what is a huge concern for intubation

Alanto-occiptal disslocation

47

Trisomy 21 Syndrome/Down's Syndrome: Anesthesia concerns
what do you want to avoid on IV tubing and why?

air bubbles
poss R-L shunting

48

Cystic Fibrosis: Pulmonary
what happens to residual volume?

inscreased

49

Cystic Fibrosis: Pulmonary
what happens to airway resistance

increased

50

Cystic Fibrosis: Pulmonary
what happens to Vital capacity

decreased

51

Cystic Fibrosis: Pulmonary
what happens to expiratory flow rate

decreased

52

Cystic Fibrosis: Pulmonary
what happens to secretion

thick, viscous secretions

53

Cystic Fibrosis: Anesthetic concerns:
can you use anticholinergics?

controversal

54

Cystic Fibrosis: Anesthetic concerns:
what do you want to do on intubation so you don't stimulate mucus secretions

deep intubation

55

Cystic Fibrosis: Anesthetic concerns:
what is usually necessary d/t thick secreetions

agressive suctioning

56

Cystic Fibrosis: Anesthetic concerns:
do you want to avoid hyper or hypo ventilation

avoid hyperventilation

57

Scoliosis:
may alter the function of what 2 ogans

pullmonary and cardiac

58

Scoliosis:
what happened to chest wall compliance

reduced

59

Scoliosis:
there is a reduced PaO2 in these individuals.. why?

V/Q mismatching

60

Scoliosis:
an increase in what ABG parameter signals severe disease

PaCO2

61

Scoliosis:
elevated what form chronic hypoxia causes pulm hTN and right ventricular hypertrophy

PVR (peripheral vascular resistance)

62

Scoliosis: Anesthetic concerns:
what are 3 things you may want to get preop

ABG
PFTs
EKG

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