Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Cardiovascular defects in patients with down syndrome may include

A

AV canal defect
ASD
VSD
TOF & PDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Ortho defects in patients with down syndrome may include

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe the treatment differences of Hurler vs. Hunter

A

Hurler is more responsive to SCT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hurler syndrome results in the buildup of

A

glycosaminoglycans due to a deficiency of alpha-L iduronidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Hurler syndrome is

A

genetic (autosomal recessive)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Hurler syndrome is characterized by

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Describe associated characteristics of the chest for Hurler syndrome

A

broad chest
spine deformities
recurrent respiratory infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe CV characteristics for Hurler syndrome

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe neuro characteristics of Hurler syndrome

A

intellectual disability

hydrocephalus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Describe ortho characteristics of Hurler syndrome

A

small stature

hypoplastic odontoid with atlantoaxial subluxation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Hunter syndrome is

A

X-linked mucopolysaccharidosis type II disease

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Describe HEENT/airway characteristics of Hunter syndrome.

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Describe characteristics of the chest of Hunter syndrome

A

pectus excavatum or carinatum, frequent URIs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Describe CV characteristics of Hunter syndrome

A

coronary artery disease

thickened valves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Describe neuro characteristics of Hunter syndrome

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Anesthetic considerations with Hunter syndrome include
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
26
Airway related anesthetic risks for MPS includes
upper airway obstruction mandibular abnormalities, short neck & high anterior larynx abnormally thick secretions, tracheal distortion
27
Pulmonary related anesthetic risks for MPS includes
progressive restrictive disease | OSA leads to pHTN
28
Spinal cord related anesthetic risks for MPS include
compression of the cervical spine atlantoaxial instability & vertebral subluxation progressive thickening & scarring of the meninges
29
Cardiac related anesthetic risks for MPS include
valvular regurgitation and/or stenosis | systolic & diastolic dysfunction
30
Neurological related anesthetic risks for MPS include
hydrocephalus | seizures
31
CHARGE syndrome is characterized by:
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
Describe HEENT/airway considerations for CHARGE syndrome
``` microcephaly cleft lip & palate severe micrognathia short neck laryngomalacia subglottic stenosis TEF ```
33
Describe chest considerations for CHARGE syndrome
rib anomalies, pectus carinatum, respiratory insufficiency
34
Describe CV considerations for charge syndrome
TOF (most common), PDA, ASD, VSD, DORV with AV canal defect & right sided aortic arch
35
Describe neuromuscular considerations for charge syndrome
variable intellectual disability, developmental delay, facial nerve palsy, abnormal gag reflex, hearing loss
36
Describe GI/GU & other considerations for charge syndrome
GERD renal anomalies failure to thrive parathyroid hypoplasia
37
Anesthetic considerations for CHARGE association includes
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
Cystic fibrosis is an
inherited autosomal recessive mutation on the long arm of chromosome 9
39
Cystic fibrosis is characterized by
elevated sweat chloride concentrations viscous mucus production, lung disease, intestinal obstruction, pancreatic insufficiency, biliary cirrhosis, & congenital absence of the vas deferens
40
With cystic fibrosis, there is a decrease in
Cl- accompanied by decrease of Na & water which leads to dehydration, viscous secretions & electrolyte abnormalities
41
Diagnosis of CF is typically through
sweat chloride test >80 mEq/L plus clinical manifestations (cough, chronic purulent sputum, exertion dyspnea) neonate- meconium ileus
42
Childhood CF is diagnosed through
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
HEENT/airway considerations for CF include
chronic sinusitis, nasal polyps
44
Chest considerations for CF include
recurrent chest infections viscoid mucus chronic infections lead to lung inflammation and damage bronchial hyperreactivity spontaneous pneumothorax increases with age
45
CV considerations for CF include
chronic respiratory disease & hypoxemia can lead to cor pulmonale
46
GI/GU considerations for CF include
mucous plugging & ductal obstructions as well as malabsorption
47
The main cause of morbidity & mortality in CF is
lung disease
48
Lung disease in CF results in
increase secretions viscous mucus impaired ciliary clearance prone to recurrent infections
49
Surgery for CF patients most commonly includes
polypectomy FESS bronchoscopies lung transplants
50
PFTs in CF appear as
``` obstructive: increased FRC decreased FEV1 decreased peak expiratory flow rate decreased vital capacity ```
51
Lung disease in CF patients leads to
bronchiectasis emphysema V/Q mismatching & hypoxemia
52
Treatment of CF includes
goal is to alleviate symptoms through treatment of malnutrition relief of airway obstruction
53
Correction of organ dysfunction for CF includes
``` clearance of airway secretions bronchodilators reduce visoelasticity of sputum antibiotics for infections organ transplant, cholecystectomy & treatment of pneumothorax ```
54
Management of anesthesia in CF includes
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
Cerebral palsy is 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
Cerebral palsy can vary from
mild local weakness to severe spastic quadriplegia- most common is muscle spasticity & contractures seizure disorders
57
CP patients often receive
seizure & muscle spasticity medications- avoid acute withdrawal, hepatic enzyme induction, lethargy/sedation
58
Most children with CP undergo
elective orthopedic corrective procedures dental restoration anti-reflux operations
59
Anesthesia for CP includes
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
Pierre Robin sequence is 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
Patients with Pierre Robin sequence are at risk for
Difficult intubation*******
62
Patient with Pierre Robin sequence may be placed
in the prone position as it may improve airway movement by displacement of the tongue
63
Pierre robin sequence often requires
tracheostomy placement, mandibular distraction, & cleft palate repair
64
HEENT/airway considerations of Pierre Robin includes
severe migrognathia, glossoptosis, U-shaped cleft palate, OSA, airway obstruction usually improves with age
65
CV considerations of Pierre Robin includes
cor pulmonale can develop with severe chronic airway obstruction, may have vagal hyperactivity
66
Neuro considerations of Pierre Robin includes
may have brainstem dysfunction with periods of central apnea
67
GI/GU considerations of Pierre Robin syndrome includes
feeding difficulties common due to anatomic abnormalities

Advanced Principles 2 (42 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions