Peds- Congenital Abnormalities Flashcards
Omphalocele vs gastroschisis:
which one has a sac or covering?
Omphalocele
cOvering = Omphalcele
Omphalocele vs gastroschisis:
which one has no covering? just intestines poping out
gastroschisis
G-sausage = Gastroschisis
Omphalocele:
where is defect
base of umbilicus, within the umbilical cord
the covering
gastroschisis:
where is the defect
lateral to the umbilicus, periumbilical
gastroschisis:
has no amnion or covering what does this mean it is important for the anesthetist to do?
prevent hypothermia
Prevent infection
prevent dehydration
Omphalocele vs gastroschisis:
which one needs a cardiac eval prior to proceeding to sx
omphalocele
Omphalocele vs gastroschisis:
which one is NOT associated with other anomalies
gastroschesis
Omphalocele
is associated with what sysndrome
beckwith-woideman syndrome (giantism)
Omphalocele vs gastroschisis:
which one is associated with high gastric pressures post op and usually do not have primary closure
omphalocele
Omphalocele
with increases gastric pressures what 3 things can occur as a complication
dusky bowel
dead bowel
dead baby
Omphalocele vs gastroschisis:
which one has a better survival rate
gastroschisis
Omphalocele vs gastroschisis:
is anesthesia concerns the same or different for both
same
Omphalocele vs gastroschisis: Anesthesia concerns
what do you want to do before induction?
decompress stomach w/ NG tube
Omphalocele vs gastroschisis: Anesthesia concerns
what do you want to do with induction?
intubate awake/asleep
with or without paralysis
(basically any indiction works)
Omphalocele vs gastroschisis: Anesthesia concerns
should you use N2O?
Nope
Omphalocele vs gastroschisis: Anesthesia concerns
what is required to replace bowel into the and cavity?
muscle relaxation
Omphalocele vs gastroschisis: Anesthesia concerns
keep intubated how long post op
1-2 days
Omphalocele vs gastroschisis: Anesthesia concerns
should you be aggressive with fluid replacements?
yes
Omphalocele vs gastroschisis: Anesthesia concerns
what should you do with the ambient temperature of the OR
keep it warm ( > 24C or 75F)
Prune Belly Syndrome:
is associated with complications of what systems in the infant?
urinary tract
Bladder
Prune Belly Syndrome:
how to remember?
prUne Belly
Urinary tract
Bladder
Prune Belly Syndrome:
why is 95% of incidences associated w/ male babies?
b/c it is usually associated with bilateral undescended testicles
Prune Belly Syndrome:
you should always treat these pts as what
full stomachs
Intestinal malrotation and vulvulus:
what is it
spont abnormal rotation of the midgut around the mesentery
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
