Peds- Congenital Abnormalities Flashcards Preview

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Flashcards in Peds- Congenital Abnormalities Deck (62)
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1
Q

Omphalocele vs gastroschisis:

which one has a sac or covering?

A

Omphalocele

cOvering = Omphalcele

2
Q

Omphalocele vs gastroschisis:

which one has no covering? just intestines poping out

A

gastroschisis

G-sausage = Gastroschisis

3
Q

Omphalocele:

where is defect

A

base of umbilicus, within the umbilical cord

the covering

4
Q

gastroschisis:

where is the defect

A

lateral to the umbilicus, periumbilical

5
Q

gastroschisis:

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

A

prevent hypothermia
Prevent infection
prevent dehydration

6
Q

Omphalocele vs gastroschisis:

which one needs a cardiac eval prior to proceeding to sx

A

omphalocele

7
Q

Omphalocele vs gastroschisis:

which one is NOT associated with other anomalies

A

gastroschesis

8
Q

Omphalocele

is associated with what sysndrome

A

beckwith-woideman syndrome (giantism)

9
Q

Omphalocele vs gastroschisis:

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

A

omphalocele

10
Q

Omphalocele

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

A

dusky bowel
dead bowel
dead baby

11
Q

Omphalocele vs gastroschisis:

which one has a better survival rate

A

gastroschisis

12
Q

Omphalocele vs gastroschisis:

is anesthesia concerns the same or different for both

A

same

13
Q

Omphalocele vs gastroschisis: Anesthesia concerns

what do you want to do before induction?

A

decompress stomach w/ NG tube

14
Q

Omphalocele vs gastroschisis: Anesthesia concerns

what do you want to do with induction?

A

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

15
Q

Omphalocele vs gastroschisis: Anesthesia concerns

should you use N2O?

A

Nope

16
Q

Omphalocele vs gastroschisis: Anesthesia concerns

what is required to replace bowel into the and cavity?

A

muscle relaxation

17
Q

Omphalocele vs gastroschisis: Anesthesia concerns

keep intubated how long post op

A

1-2 days

18
Q

Omphalocele vs gastroschisis: Anesthesia concerns

should you be aggressive with fluid replacements?

A

yes

19
Q

Omphalocele vs gastroschisis: Anesthesia concerns

what should you do with the ambient temperature of the OR

A

keep it warm ( > 24C or 75F)

20
Q

Prune Belly Syndrome:

is associated with complications of what systems in the infant?

A

urinary tract

Bladder

21
Q

Prune Belly Syndrome:

how to remember?

A

prUne Belly
Urinary tract
Bladder

22
Q

Prune Belly Syndrome:

why is 95% of incidences associated w/ male babies?

A

b/c it is usually associated with bilateral undescended testicles

23
Q

Prune Belly Syndrome:

you should always treat these pts as what

A

full stomachs

24
Q

Intestinal malrotation and vulvulus:

what is it

A

spont abnormal rotation of the midgut around the mesentery

25
Q

Pierre-Robin Syndrome:

what are complications associated with their palate?

A

cleft palate that is incomplete

possible high arched palate

26
Q

Pierre-Robin Syndrome:

is the Lip involved with the cleft palate?

A

not in this case

27
Q

Pierre-Robin Syndrome:

what is the abnormalities associated with the facial / oral structures? (what 5 things are Small)

A
small face
small glottis
small jaw
small tongue
small palate
28
Q

Pierre-Robin Syndrome:

the chin is displaces where?

A

posteriorly

29
Q

Pierre-Robin Syndrome:

what other problem is wrong with the tongue besides just being small?

A

glossoptsosis

30
Q

Pierre-Robin Syndrome:

what can cause airway obstruction?

A

the tongue

31
Q

Treacher Collins Syndrome:

they have a deformity of what structures?

A

facial mouth deformities

32
Q

Treacher Collins Syndrome:

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

A

Small mouth
Facial hypoplasia
Pharyngeal hypoplasia

33
Q

Treacher Collins Syndrome:

is associated with atresia of what?

A
choanal atresia
(tissue in back of nasal passage blocked)
34
Q

Treacher Collins Syndrome:

what other abnormalities (structure related) do these pts have

A

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

35
Q

Treacher Collins Syndrome:

do these pts have cardiac defects

A

yes

36
Q

Treacher Collins Syndrome Vs Pierre- Robbin syndrome:

which one os more sever?

A

Treacher Collins Syndrome

37
Q

Treacher Collins Syndrome & Pierre- Robbin syndrome:

do they have difficult intubation?

A

umm yes

38
Q

Treacher Collins Syndrome & Pierre- Robbin syndrome:

what intubation tech is best

A

Awake intubation

fully awake before extubation

39
Q

Trisomy 21 Syndrome/Down’s Syndrome:

they have and extra chromosome where

A

the 21st

40
Q

Trisomy 21 Syndrome/Down’s Syndrome:

describe their major abnormalities

A
Short neck
flat occiput
mental retardation
high arched palates
micrognathia (small jaw)
macroglossia
Alantoaxial instability
41
Q

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

A

subglottic stenosis

42
Q

Trisomy 21 Syndrome/Down’s Syndrome:

what are other associated anomalies?

A
congenital heart dz
TEF
floppy soft palate
ASD
VSD
PDA
OSA
43
Q

Trisomy 21 Syndrome/Down’s Syndrome: Anesthesia concerns

if there a difficult airway

A

yes

44
Q

Trisomy 21 Syndrome/Down’s Syndrome: Anesthesia concerns

should you use a smaller or larger OETT

A

smaller

45
Q

Trisomy 21 Syndrome/Down’s Syndrome: Anesthesia concerns

what should you pay close attention to post op

A

post op apnea and stridor (common)

46
Q

Trisomy 21 Syndrome/Down’s Syndrome: Anesthesia concerns

what is a huge concern for intubation

A

Alanto-occiptal disslocation

47
Q

Trisomy 21 Syndrome/Down’s Syndrome: Anesthesia concerns

what do you want to avoid on IV tubing and why?

A

air bubbles

poss R-L shunting

48
Q

Cystic Fibrosis: Pulmonary

what happens to residual volume?

A

inscreased

49
Q

Cystic Fibrosis: Pulmonary

what happens to airway resistance

A

increased

50
Q

Cystic Fibrosis: Pulmonary

what happens to Vital capacity

A

decreased

51
Q

Cystic Fibrosis: Pulmonary

what happens to expiratory flow rate

A

decreased

52
Q

Cystic Fibrosis: Pulmonary

what happens to secretion

A

thick, viscous secretions

53
Q

Cystic Fibrosis: Anesthetic concerns:

can you use anticholinergics?

A

controversal

54
Q

Cystic Fibrosis: Anesthetic concerns:

what do you want to do on intubation so you don’t stimulate mucus secretions

A

deep intubation

55
Q

Cystic Fibrosis: Anesthetic concerns:

what is usually necessary d/t thick secreetions

A

agressive suctioning

56
Q

Cystic Fibrosis: Anesthetic concerns:

do you want to avoid hyper or hypo ventilation

A

avoid hyperventilation

57
Q

Scoliosis:

may alter the function of what 2 ogans

A

pullmonary and cardiac

58
Q

Scoliosis:

what happened to chest wall compliance

A

reduced

59
Q

Scoliosis:

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

A

V/Q mismatching

60
Q

Scoliosis:

an increase in what ABG parameter signals severe disease

A

PaCO2

61
Q

Scoliosis:

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

A

PVR (peripheral vascular resistance)

62
Q

Scoliosis: Anesthetic concerns:

what are 3 things you may want to get preop

A

ABG
PFTs
EKG

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