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Advanced Principles 2 > Medical conditions influencing management in pediatric anesthesia > Flashcards

Medical conditions influencing management in pediatric anesthesia Flashcards

1
Q

HEENT/Airway characteristics of down syndrome include

A 
short neck
macroglossia
mandibular hypolasia
narrow nasopharynx 
hypertrophic lymphatic tissue 
subglottic stenosis- may have to downsize tube
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2
Q

Cardiovascular defects in patients with down syndrome may include

A

AV canal defect
ASD
VSD
TOF & PDA

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3
Q

Ortho defects in patients with down syndrome may include

A

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

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4
Q

Anesthesia considerations for patients with down syndrome related to the airway include

A

assess atlantoaxial instability- inquire about changes in gross or fine motor function or head/neck pain care with laryngoscopy to minimize flexion & extension
assess for OSA
1/4 of these children will require a downsized ETT due to subglottic stenosis

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5
Q

Anesthesia considerations for patients with down syndrome related to cardiac include

A

increased incidence of bradycardia on induction
CHD- bacterial endocarditis prophylaxis
assess for pulmonary hypertension either due to CHD or OSA

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6
Q

Anesthesia considerations for down syndrome include

A

challenging vascular access
challenging to sedate, pre-medicate, caregiving present for induction
hypothyroidism when present can result in delayed gastric emptying and altered drug metabolism

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7
Q

Mucopolysaccharidosis is a

A

genetic lysosomal storage disease
metabolic disorders that have absent or malfunction enzymes to break down glycosaminoglycans or GAGS
-found in cells of bone, skin, connective tissue, & corneas

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8
Q

Describe the treatment differences of Hurler vs. Hunter

A

Hurler is more responsive to SCT

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9
Q

Hurler syndrome results in the buildup of

A

glycosaminoglycans due to a deficiency of alpha-L iduronidase

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10
Q

Hurler syndrome is

A

genetic (autosomal recessive)

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11
Q

Symptoms of Hurler’s syndrome appear

A

during childhood & early death (frequently by age 10) occurs due to organ damage, airway disease, respiratory infections or cardiac complications

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12
Q

Hurler syndrome is characterized by

A

progressive deterioration
hepatosplenomegaly
dwarfism
and unique facial features with progressive mental decline

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13
Q

Describe associated characteristics of the HEENT/AIRWAY for hurler syndrome

A 
macrocephaly
large tongue, lips, tonsils & adenoids
copioius nasal discharge
narrow trachea
OSA
short neck
high epiglottis
considered worst airway problem in pediatric anesthesia
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14
Q

Describe associated characteristics of the chest for Hurler syndrome

A

broad chest
spine deformities
recurrent respiratory infections

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15
Q

Describe CV characteristics for Hurler syndrome

A

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

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16
Q

Describe neuro characteristics of Hurler syndrome

A

intellectual disability

hydrocephalus

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17
Q

Describe ortho characteristics of Hurler syndrome

A

small stature

hypoplastic odontoid with atlantoaxial subluxation

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18
Q

Describe anesthetic considerations for Hurler syndrome

A

One of the most difficult pediatric airways frequently encountered**
-obstruction of the airway and worsens with age, especially after age 2
-difficult intubation
-odontoid hypoplasia & thick secretions
ECHO-possible cardiac impairment
difficult IV access

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19
Q

Hunter syndrome is

A

X-linked mucopolysaccharidosis type II disease

presentation is variable but often apparent by age 2-4 years

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20
Q

Describe Hunter syndrome vs. Hurler syndrome

A

less intellectual disability, less joint disease, less organ involvement & slower progression than Hurler
death is often by late teens without treatment

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21
Q

Describe HEENT/airway characteristics of Hunter syndrome.

A

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

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22
Q

Describe characteristics of the chest of Hunter syndrome

A

pectus excavatum or carinatum, frequent URIs

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23
Q

Describe CV characteristics of Hunter syndrome

A

coronary artery disease

thickened valves

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24
Q

Describe neuro characteristics of Hunter syndrome

A

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

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25
Q

Anesthetic considerations with Hunter syndrome include

A

difficult laryngoscopy & tracheal intubation*****
OPA can worse airway (displaces epiglottis over alrynx)
positioning challenge with stiff joints
OSA & postoperative obstructive pulmonary edema
supraglottic airways have served as successful conduit for fiberoptic intubation
tracheotomy may be necessary
stem cell tx patients require special blood product considerations (leuko-reduced, irradiated)
sensitivity to opioids

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26
Q

Airway related anesthetic risks for MPS includes

A

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

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27
Q

Pulmonary related anesthetic risks for MPS includes

A

progressive restrictive disease

OSA leads to pHTN

28
Q

Spinal cord related anesthetic risks for MPS include

A

compression of the cervical spine
atlantoaxial instability & vertebral subluxation
progressive thickening & scarring of the meninges

29
Q

Cardiac related anesthetic risks for MPS include

A

valvular regurgitation and/or stenosis

systolic & diastolic dysfunction

30
Q

Neurological related anesthetic risks for MPS include

A

hydrocephalus

seizures

31
Q

CHARGE syndrome is characterized by:

A

colobomas of the eye
heart disease
atresia of the choanae
retarded growth or central nervous system anomalies
genital anomalies and ear anomalies or deafness
-at least four must be present for a diagnosis

32
Q

Describe HEENT/airway considerations for CHARGE syndrome

A 
microcephaly
cleft lip & palate
severe micrognathia
short neck
laryngomalacia
subglottic stenosis
TEF
33
Q

Describe chest considerations for CHARGE syndrome

A

rib anomalies, pectus carinatum, respiratory insufficiency

34
Q

Describe CV considerations for charge syndrome

A

TOF (most common), PDA, ASD, VSD, DORV with AV canal defect & right sided aortic arch

35
Q

Describe neuromuscular considerations for charge syndrome

A

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

36
Q

Describe GI/GU & other considerations for charge syndrome

A

GERD
renal anomalies
failure to thrive
parathyroid hypoplasia

37
Q

Anesthetic considerations for CHARGE association includes

A

interpreter for deaf patients
GERD & impaired gag reflex may place the patient at risk for aspiration
SBE prophylaxis in CHD
micrognathia may make tracheal intubation difficult- intubation can become more difficult with increase age**

38
Q

Cystic fibrosis is an

A

inherited autosomal recessive mutation on the long arm of chromosome 9

39
Q

Cystic fibrosis is characterized by

A

elevated sweat chloride concentrations viscous mucus production, lung disease, intestinal obstruction, pancreatic insufficiency, biliary cirrhosis, & congenital absence of the vas deferens

40
Q

With cystic fibrosis, there is a decrease in

A

Cl- accompanied by decrease of Na & water which leads to dehydration, viscous secretions & electrolyte abnormalities

41
Q

Diagnosis of CF is typically through

A

sweat chloride test >80 mEq/L plus clinical manifestations (cough, chronic purulent sputum, exertion dyspnea)
neonate- meconium ileus

42
Q

Childhood CF is diagnosed through

A

malabsorption & malnutrition due to pancreatic insufficiency
-pancreatic insufficiency
failure of enzyme secretion
impaired GI motility
abnormal bile circulation
increased caloric demand due to severe lung diseae

43
Q

HEENT/airway considerations for CF include

A

chronic sinusitis, nasal polyps

44
Q

Chest considerations for CF include

A

recurrent chest infections
viscoid mucus
chronic infections lead to lung inflammation and damage
bronchial hyperreactivity
spontaneous pneumothorax increases with age

45
Q

CV considerations for CF include

A

chronic respiratory disease & hypoxemia can lead to cor pulmonale

46
Q

GI/GU considerations for CF include

A

mucous plugging & ductal obstructions as well as malabsorption

47
Q

The main cause of morbidity & mortality in CF is

A

lung disease

48
Q

Lung disease in CF results in

A

increase secretions
viscous mucus
impaired ciliary clearance
prone to recurrent infections

49
Q

Surgery for CF patients most commonly includes

A

polypectomy
FESS
bronchoscopies
lung transplants

50
Q

PFTs in CF appear as

A 
obstructive:
increased FRC
decreased FEV1
decreased peak expiratory flow rate 
decreased vital capacity
51
Q

Lung disease in CF patients leads to

A

bronchiectasis
emphysema
V/Q mismatching & hypoxemia

52
Q

Treatment of CF includes

A

goal is to alleviate symptoms through
treatment of malnutrition
relief of airway obstruction

53
Q

Correction of organ dysfunction for CF includes

A 
clearance of airway secretions
bronchodilators
reduce visoelasticity of sputum
antibiotics for infections
organ transplant, cholecystectomy & treatment of pneumothorax
54
Q

Management of anesthesia in CF includes

A

postpone elective procedures until optimized
volatiles allow decreased airway resistance & smooth muscle tone- inhalation inductions are prolonged due to large FRC & hypoxia may develop rapidly
short-acting anesthetics to minimize postop respiratory depression
anticholinergics controversial, optimize hydration
humidify inspired gases
frequent tracheal suctioning
may require high ventilation pressures

55
Q

Cerebral palsy is a

A

symptom complex rather than a “disease”

includes non-progressive conditions resulting from an insult early in life or lesions/anomalies of the brain

56
Q

Cerebral palsy can vary from

A

mild local weakness to severe spastic quadriplegia- most common is muscle spasticity & contractures
seizure disorders

57
Q

CP patients often receive

A

seizure & muscle spasticity medications- avoid acute withdrawal, hepatic enzyme induction, lethargy/sedation

58
Q

Most children with CP undergo

A

elective orthopedic corrective procedures
dental restoration
anti-reflux operations

59
Q

Anesthesia for CP includes

A

determine patient’s baseline, understanding, and ability to communicate
tracheal intubation- at risk for aspiration (GERD is extremely common; increased secretions & impaired swallowing)
volatiles safe
children on anticonvulsants may be more resistant to NDMRs
susceptible to hypothermia
contractures may make positioning difficult
emergence may be slow
high incidence of pulmonary complications postop & may require ICU

60
Q

Pierre Robin sequence is a

A

congenital condition or sequence/chain of developmental facial malformations including

  • hypoplastic mandible (micronathia)
  • pseudo-macroglossia (posterior displacement of tongue)
  • high arched cleft palate
61
Q

Patients with Pierre Robin sequence are at risk for

A

Difficult intubation***

62
Q

Patient with Pierre Robin sequence may be placed

A

in the prone position as it may improve airway movement by displacement of the tongue

63
Q

Pierre robin sequence often requires

A

tracheostomy placement, mandibular distraction, & cleft palate repair

64
Q

HEENT/airway considerations of Pierre Robin includes

A

severe migrognathia, glossoptosis, U-shaped cleft palate, OSA, airway obstruction usually improves with age

65
Q

CV considerations of Pierre Robin includes

A

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

66
Q

Neuro considerations of Pierre Robin includes

A

may have brainstem dysfunction with periods of central apnea

67
Q

GI/GU considerations of Pierre Robin syndrome includes

A

feeding difficulties common due to anatomic abnormalities

Advanced Principles 2 (42 decks)

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