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N934 Adv Principles Fa 21 > Medical Conditions Influencing Management in Pediatric Anesthesia > Flashcards

Medical Conditions Influencing Management in Pediatric Anesthesia Flashcards

1
Q

most frequent chromosomal aberration

A

trisomy 21

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2
Q

HEENT/AW associated characteristics for down syndrome (trisomy 21)

A

short neck, epicanthic folds, brush fields spots, low set ears, macroglossia, mandibular hypoplasia, narrow nasopharynx, hypertrophic lymphatic tissue (tonsils and adenoids), subglottic stenosis

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3
Q

CV associated characteristics for down syndrome (trisomy 21)

A

40-50% of them get this. AV canal defect, ASD, VSD, TOF, PDA

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4
Q

neuromuscular associated characteristics for down syndrome (trisomy 21)

A

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

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5
Q

ortho associated characteristics for down syndrome (trisomy 21)

A

lax cervical ligaments can result in Atlanto-occipital (8.5%) or atlantoaxial(7-36%) instability and dislocation

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6
Q

GI/GU associated characteristics for down syndrome (trisomy 21)

A

duodenal atresia, increased incidence of hirschprungs disease

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7
Q

OTHER associated characteristics for down syndrome (trisomy 21)

A

leukemia, obesity, thyroid disease

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8
Q

anesthesia for down syndrome: aw considerations

A

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

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9
Q

anesthesia for down syndrome: CV considerations

A

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

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10
Q

anesthesia for down syndrome: hypothyroidism considerations

A

when present, can result in delayed gastric emptying and altered drug metabolism

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11
Q

anesthesia for down syndrome: induction considerations

A

challenging to sedate, premedical, caregiving present for induction

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12
Q

mucopolysaccharidosis (MPS) definition

A

genetic lysosomal storage disease. group of metabolic disorders that have absent or malfunction enzymes to break down glycosaminoglycans or GAGs (formally called mucopolysaccharides- a long chain CHO found in cells of bone, skin, connective tissue, corneas).
over time, GAGs will collect in cells and connective tissues which results in progressive and permanent damage

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13
Q

MPS features/sx general

A

often will have striking skeletal features. may or may not have behavior or cognitive difficulties

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14
Q

MPS subtype 1 eponym, enzyme deficiency, treatment

A

hurrler
alpha L iduronidase
stem cell txp, ERT, supportive

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15
Q

MPS subtype 2 eponym, enzyme deficiency, treatment

A

hunter
iduronate sulfatase
ERT, supportive

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16
Q

MPS subtype 3 eponym, enzyme deficiency, treatment

A

sanfilippo
multiple types
supportive

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17
Q

MPS subtype 4 eponym, enzyme deficiency, treatment

A

morquio
n acetylgalactosamine-6-sulfate
ERT, supportive

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18
Q

MPS subtype 6 eponym, enzyme deficiency, treatment

A

maroteaux-lamy
n-acetylgalactosamine-4-sulfatase
ERT, supportive

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19
Q

MPS subtype 7 eponym, enzyme deficiency, treatment

A

sly
beta glucoronidase
ERT, supportive

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20
Q

MPS 1: Hurler syndrome results from, sx, life expectancy

A

results in buildup of glycosaminoglycans due to a deficiency of alpha L iduronidase
sx appear during childhood and early death (usually by age 10) usually d/t organ damage, aw disease, respiratory infections or cardiac complications

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21
Q

Hurler syndrome associated features

A

hepatosplenomegaly, dawrfism, unique facial features, progressive mental decline

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22
Q

Hurler syndrome associated characteristics: HEENT/AW

A

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

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23
Q

Hurler syndrome associated characteristics: chest

A

CHEST: broad chest, spine deformities, recurrent respiratory infections

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24
Q

Hurler syndrome associated characteristics: CV

A

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

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25
Q

Hurler syndrome associated characteristics: neuro

A

intellectual disability, hydrocephalus

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26
Q

Hurler syndrome associated characteristics: ortho

A

small stature, hypo plastic odontoid with alantoaxial subluxation

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27
Q

Hurler syndrome anesthetic considerations

A 
obstruction of aw which worsens with age, especially after age 2
difficult intubation
odontoid hypoplasia and thick secretions
echo: possible cardiac impairment 
difficult IV access
28
Q

MPS: Hunter syndrome (MPS subtype 2) results from, life expectancy, compared to MPS1

A

x linked MPS disease. presentation is variable, but often apparent by 2-4 years. generally less intellectual disability, less joint disease, less organ involvement and slower progression than hurler
death is often by late teens without treatment, but some mild forms have lived into 40’s

29
Q

Hunter syndrome associated characteristics: HEENT/AW

A

macrocephaly, macroglossia, stiff soft tissues, cephalad and anterior larynx, hypertrophy tonsils and adenoids, OSA (leads to pHTN), copious secretions that are abnormally thick, tracheal distortion

30
Q

Hunter syndrome associated characteristics: CHEST

A

pectus excavatum or carinatum, frequent URI’s

31
Q

Hunter syndrome associated characteristics: CV

A

coronary artery disease, thickened valves, regurgitation, systolic and diastolic dysfunction

32
Q

Hunter syndrome associated characteristics: Neuro

A

progressive intellectual disability to normal, progressive thickening and scarring of meninges, hydrocephalus, cervical spine canal narrowing/compression, atlantoaxial instability and vertebral subluxation, seizures

33
Q

Hunter syndrome associated characteristics: ortho

A

stiff joints, kyphosis

34
Q

Hunter syndrome anesthetic considerations

A

OPA can worsen airway (displaces epiglottis over larynx)
positioning challenging with stiff joints
OSA and postoperative obstructive pulmonary edema
supraglottic airways have served as a successful conduit for fiberoptic intubation
tracheostomy may be necessary
stem cell transplant patients require special blood product considerations (leuko reduced, irradiated)
sensitivity to opioids

35
Q

CHARGE syndrome characterized by

A

colobomas of the eye, heart disease, atresia of the choanae, retarded growth or CNS anomalies, genital anomalies, and ear anomalies or deafness
at least 4 must be present of dx
all of organ systems involved are at critical development during 2nd month of gestation

36
Q

CHARGE syndrome associated characteristics: HEENT/AW

A

microcrephaly, colobomas of the eye (a hole in the structure of the eye), upward slanting eyes, external ear abnormalities or hearing loss, choanal atresia, cleft lip and palate, severe micrognathia, short neck, laryngomalacia, subglottic stenosis, TEF

37
Q

CHARGE syndrome associated characteristics: chest

A

rib anomalies, precuts carinatum, respiratory insufficiency

38
Q

CHARGE syndrome associated characteristics: CV

A

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

39
Q

CHARGE syndrome associated characteristics: neuromuscular

A

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

40
Q

CHARGE syndrome associated characteristics: GI/GU

A

GERD, amphalocele, anal atresia, genital and retinal anomalies

41
Q

CHARGE syndrome associated characteristics: other

A

FTT and parathyroid hypoplasia

42
Q

CHARGE anesthetic considerations

A

interpreter for deaf patients
GERD and impaired gag reflex may place patient at risk for aspiration
SBE prophylaxis in CHD
micrognathia may make tracheal intubation difficult
laryngomalacia may prove difficult ventilation with LMA or mask airway. subglottic stenosis may require a smaller ETT, choanal atresia may cause severe respiratory distress in the newborn and precludes the use of nasal airways or nasogastric tubes

43
Q

cystic fibrosis

A

inherited autosomal recessive mutation on long arm of chromosome 9, membrane glycoprotein chloride channel that contributes to regulation of ion flux at various epithelial surfaces. results in misfolding of CFTR protein.
elevated sweat chloride concentrations viscous mucous production, lung disease, intestinal obstruction, pancreatic insufficiency, biliary cirrhosis, congenital absence of vas deferens

44
Q

CF diagnosis

A

> 80mEq/L plus clinical manifestations (cough, chronic purulent sputum, exertion dyspnea)

45
Q

CF diagnosis: neonate clinical manifestation includes

A

meconium ileus

46
Q

CF diagnosis: childhood clinical manifestation includes

A

malabsorption and malnutrition due to pancreatic insufficiency. failure of enzyme secretion, impaired GI motility, abnormal bile circulation, increased caloric demand due to severe lung disease

47
Q

CF diagnosis: second decade clinical manifestation includes

A

malabsorption superseded by increasing pulmonary problems. respiratory failure and chronic infections

48
Q

CF associated characteristics: HEENT/AW

A

chronic sinusitis, nasal polyps, chronic neutrophil inflammatory response, recurrent infections, bronchiectasis, emphysema, VQ mismatching, hypoxemia

49
Q

CF associated characteristics: chest

A

recurrent chest infections, viscid mucous due to electrolyte abnormalities and mucous gland cell hypertrophy, viscous plugging and bacteria colonization typically pseudomonas or staph. spontaneous pneumothorax increases with age due to decreased ciliary clearance and bronchial hyperreactibity

50
Q

CF associated characteristics: CV

A

chronic respiratory disease and hypoxemia can lead to cor pulmonale

51
Q

CF associated characteristics: GI/GU

A

85% have a pancreatic exocrine insufficiency leading to mucous plugging and ductal obstructions as well as malabsorption.

52
Q

CF treatments and survival

A

advances in gene therapy, global registry, and surgical treatments. cystic fibrosis transmembrane conductance regulator (CFTR) modulators
35-40y in US and UK

53
Q

CF and PFT’s

A

obstructive, increased FRC, decreased FEV1, decreased peak expiratory flow rate, decreased VC. however, chronic lung obstruction leads to restrictive disease

54
Q

CF surgeries most commonly include

A

polypectomy, functional endoscopic sinus surgery (FESS), bronchoscopies, lung transplants

55
Q

treatment of CF

A

goal to alleviate symptoms (malnutrition, relieve aw obstruction). correct organ dysfunction (clearance of aw secretions, bronchodilators, reduce viscoelasticity of sputum, abx for infections, organ transplant, cholecystectomy, tx of pneumothorax)

56
Q

management of anesthesia in CF

A

postpone elective procedures until optimized
volatiles allow decreased AW resistances and smooth muscle tone. however, inhalation inductions are prolonged due to large FRC, small Vt, and VQ mismatch. hypoxia may develop rapidly due to VQ mismatch.
short acting anesthetics to minimize postop respiratory depression
anticholinergics controversial, optimize hydration
humidify inspired gases
frequent tracheal suctioning
may require high ventilation pressures (cuffed ETT’s)

57
Q

CP

A

symptom complex rather than a disease and the exact cause is still unknown. collective term for variety of non progressive conditions resulting from an insult early in life or lesions/anomalies of the brain. varying degrees of severe developmental delay to normal intelligence. varying degrees of mild local weakness to severe spastic quadriplegia. most commonly spasticity and contractures. seizure disorders

58
Q

CP treatment

A

most children undergo elective orthopedic corrective procedures including achilles tendon lengthening, hip adductor release, scoliosis correction, etc
dental restoration
anti reflux operations (nissen)
often receive seizure and muscle spasticity meds. avoid acute withdrawal, hepatic enzyme induction, lethargy/sedation

59
Q

anesthesia in CP

A

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

60
Q

pierre robin sequence

A

congenital condition or sequence/chain of developmental facial malformations

61
Q

3 main features of pierre robin

A 
hypoplastic mandible (micronathia)
pseudo macroglossia (posterior displacement of tongue. placement in prone position may improve aw movement by displacement of tongue.)
high arched cleft palate
62
Q

when is pierre diagnosed

A

clinically shortly after birth due to respiratory difficulty

63
Q

pierre robin associated characteristics: HEENT/AW

A

severe micrognathia, glossoptosis, U shaped cleft palate, OSA, aw obstruction. usually improves with age

64
Q

pierre robin associated characteristics: CV

A

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

65
Q

pierre robin associated characteristics: neuro

A

may have brainstem dysfunction and periods of central apnea

66
Q

pierre robin associated characteristics: GI/GU

A

feeding difficulties common due to anatomic abnormalities

N934 Adv Principles Fa 21 (23 decks)

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