Peds- Congenital Abnormalities Flashcards by Josh Newby

Q

Omphalocele vs gastroschisis:

which one has a sac or covering?

A

Omphalocele

cOvering = Omphalcele

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis:

which one has no covering? just intestines poping out

A

gastroschisis

G-sausage = Gastroschisis

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele:

where is defect

A

base of umbilicus, within the umbilical cord

the covering

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

gastroschisis:

where is the defect

A

lateral to the umbilicus, periumbilical

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis:

which one needs a cardiac eval prior to proceeding to sx

A

omphalocele

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis:

which one is NOT associated with other anomalies

A

gastroschesis

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele

is associated with what sysndrome

A

beckwith-woideman syndrome (giantism)

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis:

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

A

omphalocele

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele

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

A

dusky bowel
dead bowel
dead baby

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis:

which one has a better survival rate

A

gastroschisis

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis:

is anesthesia concerns the same or different for both

A

same

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis: Anesthesia concerns

what do you want to do before induction?

A

decompress stomach w/ NG tube

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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)

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis: Anesthesia concerns

should you use N2O?

A

Nope

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis: Anesthesia concerns

what is required to replace bowel into the and cavity?

A

muscle relaxation

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis: Anesthesia concerns

keep intubated how long post op

A

1-2 days

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis: Anesthesia concerns

should you be aggressive with fluid replacements?

A

yes

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Omphalocele vs gastroschisis: Anesthesia concerns

what should you do with the ambient temperature of the OR

A

keep it warm ( > 24C or 75F)

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Prune Belly Syndrome:

is associated with complications of what systems in the infant?

A

urinary tract

Bladder

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Prune Belly Syndrome:

how to remember?

A

prUne Belly
Urinary tract
Bladder

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Prune Belly Syndrome:

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

A

b/c it is usually associated with bilateral undescended testicles

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Prune Belly Syndrome:

you should always treat these pts as what

A

full stomachs

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Intestinal malrotation and vulvulus:

what is it

A

spont abnormal rotation of the midgut around the mesentery

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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

cleft palate that is incomplete | possible high arched palate

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

not in this case

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 ```

Pierre-Robin Syndrome: | the chin is displaces where?

posteriorly

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

glossoptsosis

Pierre-Robin Syndrome: | what can cause airway obstruction?

the tongue

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

facial mouth deformities

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

Small mouth Facial hypoplasia Pharyngeal hypoplasia

Treacher Collins Syndrome: | is associated with atresia of what?

``` choanal atresia (tissue in back of nasal passage blocked) ```

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)

Treacher Collins Syndrome: | do these pts have cardiac defects

yes

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

Treacher Collins Syndrome

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

umm yes

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

Awake intubation | fully awake before extubation

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

the 21st

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 ```

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

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

``` congenital heart dz TEF floppy soft palate ASD VSD PDA OSA ```

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

yes

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

smaller

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

post op apnea and stridor (common)

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

Alanto-occiptal disslocation

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

Cystic Fibrosis: Pulmonary | what happens to residual volume?

inscreased

Cystic Fibrosis: Pulmonary | what happens to airway resistance

increased

Cystic Fibrosis: Pulmonary | what happens to Vital capacity

decreased

Cystic Fibrosis: Pulmonary | what happens to expiratory flow rate

decreased

Cystic Fibrosis: Pulmonary | what happens to secretion

thick, viscous secretions

Cystic Fibrosis: Anesthetic concerns: | can you use anticholinergics?

controversal

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

deep intubation

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

agressive suctioning

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

avoid hyperventilation

Scoliosis: | may alter the function of what 2 ogans

pullmonary and cardiac

Scoliosis: | what happened to chest wall compliance

reduced

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

V/Q mismatching

Scoliosis: | an increase in what ABG parameter signals severe disease

PaCO2

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

PVR (peripheral vascular resistance)

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

ABG PFTs EKG