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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

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
Q

HEENT/AIrway characteristics of Hunter’s

A

macrocephaly, macroglossia, stiff soft tissues, cephalad and anterior larynx, hypertrophy tonsils and adenoids, OSA, copious secretions

26
Q

CHEST characteristics of Hunter’s

A

pectus excavatum or carinatum, frequent URIs

27
Q

CV characteristics of Hunter’s

A

coronary artery disease, thickened valves

28
Q

Neuro characteristics of Hunters

A

progressive intellectual disability to normal, hydrocephalus, cervical spine canal narrowing

29
Q

Ortho characteristics of Hunter’s

A

stiff joints, kyphosis

30
Q

What are anesthetic considerations for Hunter’s

A

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
Q

Airway risks with MPS features

A

upper airway obstruction
mandibular abnormalities, short neck, and high anterior larynx
abnormally thick secretions, tracheal distortion

32
Q

Pulmonary risks with MPS features

A

progressive restrictive disease
OSA leads to pHTN

33
Q

Spinal cord risks with MPS features

A

compression of the cervical spine
atlantoaxial instability and vertebral sublulxation
progressive thickening and scarring of the meninges

34
Q

Cardiac risks with MPS features

A

valvular regurgitation and or stenosis
systolic and diastolic dysfunction

35
Q

Neurological risk with MPS features

A

hydrocephalus
seizures

36
Q

CHARGE

A

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
Q

HEENT/Airway of CHARGE

A

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
Q

Chest in CHARGE

A

rib anormalies, pectus carinatum, respiratory insufficiency

39
Q

CV in CHARGE

A

TOF
PDA
ASD VSD DORV with AV canal defect and right sided aortic arch

40
Q

Neuromuscular in CHARGE

A

variable intellectual disability, developmental delay, facial nerve palsy, abnormal gag reflex, hearing loss

41
Q

GI/GU in CHARGE

A

GERD, omphalocele, anal atresia, genital and renal anomalies

42
Q

Additional characteristics of CHARGE

A

FFT and parathyroid hypoplasia

43
Q

Anesthesis implications of CHarge

A

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
Q

What syndrome can intubation become more difficult with increasing age?

A

CHARGE

45
Q

What is cystic fibrosis?

A

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
Q

In summary cystic fibrosis,

A

is a decrease in CI- accompanied by a decrease in Na and water which leads to dehydration, viscous secretion and electrolyte abnormalities

47
Q

HEENT/Airway of CF

A

chronic sinusitis, nasal polyps

48
Q

Chest of CF

A

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
Q

CV in CF

A

chronic respiratory disease + hypoxemia can lead cor pulmonale

50
Q

GI/GU of CF

A

pancreatic exocrine insufficiency leads to mucous plugging and. ductal obstructions and malabsorption

51
Q

What do PFTs look in like in CF?

A

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
Q

Anesthesia Management of CF

A

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
Q

Cerebral Palsy is

A

collective term for a variety of non-progressive conditions resulting from an insult early in life or leisons/anomalies of the brain

54
Q

What is the most common manifestation of CP?

A

skeletal muscle spasticity and contractures

55
Q

Anesthesia and CP

A

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
Q

What is pierre robin and the three main features?

A

congential condition or sequence/chain development facial malformations
hypoplastic mandible (micrognathia)
pseudo-macroglossia (posterior displacement of tongue)
high arched cleft palate

57
Q

what can improve airway movement in pierre robin?

A

prone position
by displacement of the tongue

58
Q

What do pierre robin kiddos often require?

A

tracheostomy placement, mandibular distraction, cleft palate repair

59
Q

HEENT/Airway for pierre robin

A

severe micrognathia, glossoptosis, U shaped cleft palate, obstructive apnea, airway obstruction usually improves with age

60
Q

CV for pierre robin

A

cor pulmonale can develope with severe chronic airway obstruction, may have vagal hyperactivity

61
Q

Neuro for pierre robin

A

may have brainstem dysfunction with periods of central apnea

62
Q

GI/GU for pierre robin

A

feeding difficulties common due to anatomic abnormalites

N934: Advanced Principles of Anesthesia (16 decks)

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