Pediatrics 2 Flashcards
Which congenital condition is associated with macroglossia?
a. glucose-6-dehydrogenase deficiency
b. Klippel-Feil
c. Treacher Collins
D. Trisomy 21
D. trisomy 21
Which conditions present with small/underdeveloped mandible?
Pierre Robin
Goldenhar
Treacher Collins
Cri du Chat
Please Get that chin
Which conditions present with cervical spine anomaly?
Klippel-Feil
Trisomy 21
Goldenhar
(Kids Try Gold)
Which conditions present with large tongue?
Beckwith syndrome
Trisomy 21
(Big Tongue)
Anesthetic considerations for cleft lip and palate include
airway obstruction
difficult laryngoscopy
difficult mask ventilation
aspiration
__________________ can reduce venous drainage and cause tongue engorgement
the Dingman-Dott mouth retractor
The Dingman-Dott mouth retractor can lead to
post-extubation airway obstruction
Neonates with Pierre Robin syndrome often require
intubation
Pierre robin syndrome is made up of
small/underdeveloped mandible
cleft palate
tongue that falls back and downwards (glossoptosis)
Treacher collins is composed of
small mouth
small/underdeveloped mandible
choanal atresia
ocular and auricular anomalies
Trisomy 21 is composed of
small mouth
large tongue
atlantoaxial instability
subglottic stenosis
Klippel-Feil is composed of
congenital fusion of cervical vertebrae–> neck rigidity
Beckwith syndrome is composed of
large tongue
Goldenhar syndrome is composed of
cervical spine abnormality
Small/underdeveloped mandible
Cri du Chat is composed of
small/underdeveloped mandible
laryngomalacia
stridor
Cleft lip repair is typically performed at
~1 month of age
Cleft palate repair is typically performed at
~12 months of age
What is the MOST common cardiac anomaly associated with Down syndrome?
a. atrioventricular septal defect
b. 1st degree heart block
c. bicuspid aortic valve
d. single ventricle
a. atrioventricular septal defect
_______________ is the most common chromosomal disorder.
Trisomy 21 (down syndrome)
The patient with down syndrome is at an increased risk for
difficult ventilation and intubation
Reasons the patient with down syndrome is at an increased risk of difficult ventilation and intubation include
small mouth
large tongue
midface hypoplasia
palate is narrow with a high arch
atlantoaxial instability
subglottic stenosis
obstructive sleep apnea
chronic pulmonary infection
Co-existing issues with trisomy 21 include
congenital heart disease
low muscle tone
GERD
intellectual disability
__________ are most likely to give birth to a child with down syndrome
Older mothers
Trisomy 21 results from
the addition of a 3rd copy of chromosome 21
What should be avoided during laryngoscopy of the patient with trisomy 21?
neck flexion
Due to their subglottic stenosis in patients with down syndrome,
use a smaller ETT
they have increased risk of postintubation croup
The most common co-existing congenital heart disease in patients with trisomy 21 is
AV septal defect
VSD is second most common
___________ is very common during induction with trisomy 21
bradycardia with sevoflurane induction (give anticholinergic)
Match each congenital condition with its MOST likely presentation.
Catch 22 (DiGeorge) syndrome
VACTERL Association
CHARGE association
Choanal atresia
renal dysplasia
hypocalcemia
Catch 22- hypocalcemia
CHARGE- Choanal atresia
VACTERL- Renal dysplasia
Describe VACTERL association.
Vertebral defects
imperforated anus
Cardiac anomalies
tracheoesophageal fistula
esophageal atresia
renal dysplasia
limb anomalies
Describe CHARGE association.
Coloboma
Heart defects
Choanal Atresia
Restriction of growth and development
Genitourinary problems
Ear anomalies
Describe CATCH 22
Cardiac defects
abnormal face
thymic hypoplasia
cleft palate
hypocalcemia
22q11.2 gene deletion (the cause of the syndrome)
What is coloboma?
a hole in one of the eye structures
What is choanal atresia?
back of nasal passage is obstructed
For the child with DiGeorge syndrome, if the thymus is absent
the child is at a high risk for infection
Treatment for an absent thymus consists of
thymus transplant or mature T cell infusion
Transfusion of ______________ for DiGeorge syndrome is best
leukocyte-depleted irradiated blood
What factors lower ionized Ca2+?
hyperventilation
albumin
citrated blood products
What is the MOST common coagulation disorder in children undergoing adenotonsillectomy?
a. Hemophilia A
b. Heparin-induced thrombocytopenia
c. Sickle cell disease
d. von Willebrand disease
d. Von willebrand disease
The most common pediatric surgical procedures are
tonsillectomy and adenoidectomy
Indications for tonsillectomy and adenoidectomy include
nocturnal upper airway obstruction and chronic and/or recurrent infections
The most common cause of OSA in kids is
adenotonsillar hypertrophy
The most common coagulation disorder in patients undergoing tonsillectomy is
Von Willebrand disease
_________ may reduce postoperative airway swelling, pain, and PONV with tonsillectomy.
Decadron 1 mg/kg up to 25 mg
With tonsillectomy and adenoidectomy there is a risk of
airway fire (maintain FiO2 <40% and avoid nitrous oxide)
Children with OSA undergoing adenotonsillectomy should be
admitted to the hospital for 23 hours and receive monitoring for airway obstruction
_________________ is a surgical emergency
Post-tonsillectomy bleeding
Key anesthetic risks for post-tonsillectomy bleeding include
hypovolemia
aspiration
Patients with post-tonsillectomy bleeding should receive
volume resuscitation & a RSI
Children with OSA undergoing adenotonsillectomy have a ______________________ & should receive
longer emergence from anesthesia; a lower intraoperative opioid dose
_____________ should not be given to children with OSA undergoing adenotonsillectomy because__________
codeine for postop pain d/t an increased risk of death d/t respiratory depression
Children with OSA undergoing adenotonsillectomy have an increased incidence of
postop airway obstruction
prolonged oxygen requirements
greater need for airway instrumentation
Patients who receive DDAVP are at risk for
hyponatremia (use isotonic crystalloids at 1/2-2/3rds calculated maintenance values)
Primary bleeding occurs within
the first 24 hours (post-tonsillectomy) with 75% occurring within the first 6 hours after surgery
Secondary bleeding typically occurs
5-10 days after tonsillectomy when the scar covering the tonsil bed contracts
____________ and ____________ are suggestive of a >20% loss of circulating volume
Dizziness and orthostatic hypotension
How should the post-tonsillectomy bleeding patient be induced
preoxygenated in the left-lateral and head down position helps drain blood from the airway
RSI
OGT after induction
