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N934: Advanced Principles of Anesthesia > Medical Conditions Influencing Management of Pediatric Anesthesia > Flashcards

Medical Conditions Influencing Management of Pediatric Anesthesia Flashcards

(62 cards)

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1
Q

What is the most frequent chromosomal aberration

A

trisomy 21
Down syndrome

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2
Q

HEENT/Airway Characteristics of T21

A

short neck, epicanthic folds, brushfield’s spots, low set ears, macroglossia, mandibular hypoplasia, narrow nasopharynx, hypertrophic lympathic tissue (tonsils and adenoids), subglottic stenosis

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3
Q

Cardiovascular characteristics of T21

A

40-50% have a CHD
AV canal defect, ASD VSD TOF PDA

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4
Q

What are neuromuscular characterstics of T21

A

hypotonia, ligamentous instability, dementia and parkinsonism in older adults, intellectual decline with age

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5
Q

What are ortho characteristics in T21

A

lax cervical ligaments can result in atlantooccipital or atlantoaxial instability and dislocation

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6
Q

GU/GI characteristics in T21

A

duodenal atresia, increased incidence of Hirschsprung disease

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7
Q

additional characteristics of T21

A

leukemia, obesity, thyroid disease

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8
Q

Anesthesia for down syndrome (airway)

A

assess atlanto-axial instability
inquire about changes in gross or fine motor function or head and neck pain care with laryngoscopy to minimize flexion or extension
assess for OSA
1/2 of these children will require a downsized ETT due to subglottic stenosis

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9
Q

Anesthesia for down syndrome (cardiovascular)

A

increased incidence of bradycardia on induction
CHD- bacterial endocarditis prophylaxis
assess for pulmonary HTN d/t to either CHD or OSA

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10
Q

Anesthesia for down syndrome

A

challenging vascular access
challenging to sedation, presmediate, caregiver present for induction
hypothyroidism when present can delay gastric emptying and alter drug metabolism

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11
Q

What is mucopolysaccharidosis?

A

genetic lyososomal storage disease
a group of metabolic disorders that have absent or malfunction enzymes to break down glycoaminoglycans or GAGs
which over time, the GAGs collect in cells and connective tissues resulting in progressive and permenant damage

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12
Q

What are the two prominent subtypes of mucopolysaccharidosis?

A

hurler and hunter

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13
Q

What is hurler syndrome?

A

genetic (autosomal recessive) mucopolysaccharidosis type 1 disease
deficiency of alpha L iduronidase

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14
Q

What are treatments for hurler syndrome?

A

enzyme replacement therapy and stem cell or umbilical cord blood transplant

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15
Q

What can be improved and halted in hurler’s syndrome?

A

abnormal physical characteristics (except skeleton and eyes) and neurologic degeneration

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16
Q

HEENT/Airway characteristics of Hurlers

A

macrocephaly, corneal opacities, hearing loss, large tongue, lips, tonsils, adenoids, copious nasal drainage, narrow trachea, OSA short neck, high epiglottis

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17
Q

What is the worst airway problem in pediatric anesthesia?

A

hurler syndrome d/t macrocephaly, short necks OSA, large tongues, tonsils, lips and adenoids, narrow trachea high epiglottis

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18
Q

Chest characteristics of hurlers

A

broad chest, spine deformities, recurrent respiratory infections

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19
Q

CV characteristics of hurlers

A

coronary artery narrowing and ischemic heart disease, mitral valve thickening, cardiomegaly

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20
Q

Neuro characteristics of hurlers

A

intellectual disability, hydropcephalus

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21
Q

Ortho characteristics of hurlers

A

small stature, hypoplastic odontoid with atlantoaxial subluxation

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22
Q

Anesthesia considerations for Hurler syndrome

A

one of the most difficult airways frequently encountered
obstruction of the airway and worsens with age, espeically after age 2
difficult intubation
odontoid hypoplasia and thick secretions
ECHO (possible cards impairment)
difficult IV access

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23
Q

What is hunter’s syndrome

A

x linked mucopolysaccharidosis type 2
presentation is variable, but often apparent by age 2-4 years
less intellectual disability, less joint disease, less organ involvement and slower progression than hurler

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24
Q

How is hunter’s syndrome treated?

A

enzyme therapy and transplants have been limited

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25
HEENT/AIrway characteristics of Hunter's
macrocephaly, macroglossia, stiff soft tissues, cephalad and anterior larynx, hypertrophy tonsils and adenoids, OSA, copious secretions
26
CHEST characteristics of Hunter's
pectus excavatum or carinatum, frequent URIs
27
CV characteristics of Hunter's
coronary artery disease, thickened valves
28
Neuro characteristics of Hunters
progressive intellectual disability to normal, hydrocephalus, cervical spine canal narrowing
29
Ortho characteristics of Hunter's
stiff joints, kyphosis
30
What are anesthetic considerations for Hunter's
extraordinarily difficult laryngoscopy and tracheal intubation OPA can worse airway as it displaces the epiglottis over larynx OSa and postoperative obstruction pulmonary edema supraglottic airways have served as successful conduit for fiberoptic intubation trach may be neccessary sensitivity to opioids stem cell transplants require special blood product considerations (leuko-reduced, irradiated)
31
Airway risks with MPS features
upper airway obstruction mandibular abnormalities, short neck, and high anterior larynx abnormally thick secretions, tracheal distortion
32
Pulmonary risks with MPS features
progressive restrictive disease OSA leads to pHTN
33
Spinal cord risks with MPS features
compression of the cervical spine atlantoaxial instability and vertebral sublulxation progressive thickening and scarring of the meninges
34
Cardiac risks with MPS features
valvular regurgitation and or stenosis systolic and diastolic dysfunction
35
Neurological risk with MPS features
hydrocephalus seizures
36
CHARGE
colobomas of the ete heart diseaes atresia of the choanae retarded growth or CNS system abnormalities genital anomalies and ear anomalies (deaf) atleast 4 have to be present
37
HEENT/Airway of CHARGE
microcephaly, colobomas of the eye, upward slanting eyes, external ear abnormalites or hearing loss, choanal atresia, cleft lip and palate, severe micrognathia, short neck, laryngomalacia, subglottic stenosis, TEF
38
Chest in CHARGE
rib anormalies, pectus carinatum, respiratory insufficiency
39
CV in CHARGE
TOF PDA ASD VSD DORV with AV canal defect and right sided aortic arch
40
Neuromuscular in CHARGE
variable intellectual disability, developmental delay, facial nerve palsy, abnormal gag reflex, hearing loss
41
GI/GU in CHARGE
GERD, omphalocele, anal atresia, genital and renal anomalies
42
Additional characteristics of CHARGE
FFT and parathyroid hypoplasia
43
Anesthesis implications of CHarge
interpreter if deaf GERD and impaired gag reflex may place the patient at risk for aspiration SBE prophylaxis in CHD micrognathia may make tracheal intubation difficult0- intubation can become more difficult with increasing age laryngomalacia may prove difficult ventilation with LMA or mask airway
44
What syndrome can intubation become more difficult with increasing age?
CHARGE
45
What is cystic fibrosis?
inhertied autosomal recessive mutation on the long arm of chromosome 9 misfolding of CFTR protein that results in elevated sweat chloride concentrations viscous mucus production, lung disease, intestinal obstruction, pancreatic insufficiency, biliary cirrhosis, and congenital absence of vas defens
46
In summary cystic fibrosis,
is a decrease in CI- accompanied by a decrease in Na and water which leads to dehydration, viscous secretion and electrolyte abnormalities
47
HEENT/Airway of CF
chronic sinusitis, nasal polyps
48
Chest of CF
recurrent chest infections, viscis mucous due to electrolyte abnormalities and mucous gland cell hypertrophy, plugging and bacteria colonization chronic infections lead to lung inflammation and damage bronchial hyperreactivity, decreased ciliary clearance and spontaneously pneumo
49
CV in CF
chronic respiratory disease + hypoxemia can lead cor pulmonale
50
GI/GU of CF
pancreatic exocrine insufficiency leads to mucous plugging and. ductal obstructions and malabsorption
51
What do PFTs look in like in CF?
obstructive, increased FRC, decreased FEV1, decreased peak expiratory flow rate, and decreased vital capactiy but also can lead to restrictive disease (with chronic lung destruction)
52
Anesthesia Management of CF
optimize patient volatiles allow decreased airway resistances and smooth muscle tone long inhalation inductions due to large FRC, small tidal volumes and V/Q mismatch hypoxia may develop due to V/Q mismatch short acting anesthetics to minimize post op respiratory depression anticholinergics are controversial, optimize hydration humidifed inspired gases frequent tracheal suctioning may require high ventilation pressures (cuffed etts)
53
Cerebral Palsy is
collective term for a variety of non-progressive conditions resulting from an insult early in life or leisons/anomalies of the brain
54
What is the most common manifestation of CP?
skeletal muscle spasticity and contractures
55
Anesthesia and CP
determine patient's baseline, understanding and ability to communicate tracheal intubation- at risk for aspiration (GERD is common) increased secretions and impaired swallowing volatiles are safe children on anticonvulsants are more resistant to NMDRs susceptible to hypothermia contractures may positioning difficult emergence may be slow higher incidence of pulmonary complications postoperatively and may require ICU
56
What is pierre robin and the three main features?
congential condition or sequence/chain development facial malformations hypoplastic mandible (micrognathia) pseudo-macroglossia (posterior displacement of tongue) high arched cleft palate
57
what can improve airway movement in pierre robin?
prone position by displacement of the tongue
58
What do pierre robin kiddos often require?
tracheostomy placement, mandibular distraction, cleft palate repair
59
HEENT/Airway for pierre robin
severe micrognathia, glossoptosis, U shaped cleft palate, obstructive apnea, airway obstruction usually improves with age
60
CV for pierre robin
cor pulmonale can develope with severe chronic airway obstruction, may have vagal hyperactivity
61
Neuro for pierre robin
may have brainstem dysfunction with periods of central apnea
62
GI/GU for pierre robin
feeding difficulties common due to anatomic abnormalites

N934: Advanced Principles of Anesthesia (16 decks)

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