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Advanced Anesthesia II > Peds Medical Conditions > Flashcards

Peds Medical Conditions Flashcards

1
Q

What is the most frequent chromosomal aberration?

A

Down Syndrome @ 1.5 per 1000 live births

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2
Q

Major airway characteristics associated w/down syndrome

A 
short neck, macroglossia, mandibular hypoplasia
narrow nasopharynx
hypertrophic T&A
subglottic stenosis
c-spine instability
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3
Q

major CV problems w/down’s

A

CV d/o 40 - 50 % (ASD, VSD, TOF, PDA, AV Canal Defect)

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4
Q

down’s & AAI

A

characterized by excessive movement at the junction between the atlas (C1) and axis (C2) as a result of either a bony or ligamentous abnormality. Neurologic symptoms can occur when the spinal cord or adjacent nerve roots are involved.

7 - 36%

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5
Q

AOI

A

Atlanto-occipital dislocation involves complete disruption of all ligamentous relationships between the occiput and the atlas. Death usually occurs immediately

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6
Q

airway considerations for ds

A

assess OSA
downsize ETT
video blad

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7
Q

cardiac considerations for ds

A

bradycarda on induction
CHD? bacteria endocarditis prophylaxis
pulm. HTN d/t CHD or OSA

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8
Q

ds & hypothyroidism

A

delayed GI, altered drug metabolism

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9
Q

MPS

A

genetic lysosomal storage disease that has malfunctioning enzymes. they normally break down glycosaminoglycans or ‘gags’

MPS or GAGs are long chain CHO found in the cells of bone, skin, CT, corneas

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10
Q

MPS s/s

A

striking skeletal features

behavior

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11
Q

MPS I

A

hurler

tx: stem cell, ERT, supportive
enzyme: alpha-L-iduronidase

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12
Q

MPS II

A

hunter

tx: ERT, supportive
enzyme: iduronate sulfatase

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13
Q

hurler syndrome

A

genetic (autosomal recessive)
glycosaminoglycan buildup
dead by 10 d/t organ damage

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14
Q

hurler s/s (5)

A 
hepatosplenomegaly
dwarf + unique facial features
broad chest, spine deformities
recurrent respiratory infections
CAD, mitral valve thickening, CM
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15
Q

worst airway in peds (8)

A

hurler b/c

  1. big head
  2. large tongue, lips, T&A
  3. snotty
  4. narrow trachea
  5. OSA
  6. short neck
  7. high epiglottis
  8. atlantoaxial subluxation

WORSE WITH AGE

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16
Q

hunter

A

x-linked mucopolysaccharidosis type II

variable presentation, apparent by age 2 - 4

17
Q

hunter compared to hurler

A

less ID, less joint dx, less organ stuff & slower
death by late teens w/o treatment (mild form maybe 40)
ERT not as helpful

18
Q

hunter s/s (3)

A

stiff soft tissue, cephalad + anterior larynx
pectus excavatum
cspine narrowing

19
Q

hunter + anesthesia

A

DAW
OPA worsens airway
positioning sucks
sensitive to opioids

20
Q

hurler + prbcs

A

irradiated, leuko-reduced b/c stem cell tx

21
Q

neuro + MPS

A

hydrocephalus and seizures

22
Q

CHARGE

A

must have four of the below

Colobomas
Heart disease
Atresia chonae (no nasal airway/ngt)
Retarded growth/cns abnormalities
Genital anomalies
Ear anomalies or death
23
Q

CHARGE & gestation

24
Q

other s/s CHARGE (4)

A

cleft lip
micrognathia & short neck
laryngomalacia
subglottic stenosis

25
Q

anesthesia & charge

A

deaf pt - interpreter
GERD + impaired gag = aspiration
SBE prophylaxis for CHD kids
DAW that increases with age

26
Q

CF definition

A

inherited - autosomal recessive mutation of long arm of chromosome 9

membrane GP chloride channel that contributes to the regulation of ion flux at various epithelial surfaces

1 in 2000

27
Q

CF & systems

A 
mucus = thick
intestine obstruction
pancreas sucks
biliary cirrhosis
congenital absence of vas deferens
28
Q

CF dx

A

sweat chloride >80 mEq/L + clinical s/s (cough, chronic purulent sputum, exertion dyspnea)

29
Q

resp. dx + CF

A

pseudomonas or staph
mucous gland hypertrophy
impaired ciliary clearance
spontaneous pneumo risk increases with age

30
Q

PFT & CF

A 
obstructive - eventually leads to restrictive
FRC increased
decreased FEV1
decreased PEFR
decreased VC
31
Q

anesthesia & CF

A

volatiles decrease airway resistance and smooth muscle tone. however, inhalational inductions are prolonged

  • short-acting anesthetics are important
  • hydrate + suction
  • cuffed ETT b/c high ventilation preessures
32
Q

CP

A

1/3 have mental impairment - which means 2/3 don’t

varying degrees of weakness - spastic quad
1/3 epilepsy

33
Q

CP treatment

A
  • ortho procedures
  • dental
  • nissen
34
Q

CP & anesthesia

A
  • hepatic inducer d/t epilepsy drugs
  • GERD = high risk, no LMA
  • contractures
  • slow emergence
35
Q

pierre robin

A

congenital condition of developmental fascial malformations including

  1. hypoplastic mandible
  2. pseudo-macroglossia (posterior tongue placement)
  3. high cleft palate

clearly DAW

36
Q

pierre robin s/s

A

OSA, improves w/age
cor pulmonale, vagal hyperreactivity
central apnea

Advanced Anesthesia II (9 decks)

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