Medical Conditions Influencing Management in Pediatric Anesthesia

Question 1

most frequent chromosomal aberration

Answer 1

trisomy 21

Question 2

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

Answer 2

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

Question 3

CV associated characteristics for down syndrome (trisomy 21)

Answer 3

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

Question 4

neuromuscular associated characteristics for down syndrome (trisomy 21)

Answer 4

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

Question 5

ortho associated characteristics for down syndrome (trisomy 21)

Answer 5

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

Question 6

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

Answer 6

duodenal atresia, increased incidence of hirschprungs disease

Question 7

OTHER associated characteristics for down syndrome (trisomy 21)

Answer 7

leukemia, obesity, thyroid disease

Question 8

anesthesia for down syndrome: aw considerations

Answer 8

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

Question 9

anesthesia for down syndrome: CV considerations

Answer 9

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

Question 10

anesthesia for down syndrome: hypothyroidism considerations

Answer 10

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

Question 11

anesthesia for down syndrome: induction considerations

Answer 11

challenging to sedate, premedical, caregiving present for induction

Question 12

mucopolysaccharidosis (MPS) definition

Answer 12

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

Question 13

MPS features/sx general

Answer 13

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

Question 14

MPS subtype 1 eponym, enzyme deficiency, treatment

Answer 14

hurrler
alpha L iduronidase
stem cell txp, ERT, supportive

Question 15

MPS subtype 2 eponym, enzyme deficiency, treatment

Answer 15

hunter
iduronate sulfatase
ERT, supportive

Question 16

MPS subtype 3 eponym, enzyme deficiency, treatment

Answer 16

sanfilippo
multiple types
supportive

Question 17

MPS subtype 4 eponym, enzyme deficiency, treatment

Answer 17

morquio
n acetylgalactosamine-6-sulfate
ERT, supportive

Question 18

MPS subtype 6 eponym, enzyme deficiency, treatment

Answer 18

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

Question 19

MPS subtype 7 eponym, enzyme deficiency, treatment

Answer 19

sly
beta glucoronidase
ERT, supportive

Question 20

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

Answer 20

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

Question 21

Hurler syndrome associated features

Answer 21

hepatosplenomegaly, dawrfism, unique facial features, progressive mental decline

Question 22

Hurler syndrome associated characteristics: HEENT/AW

Answer 22

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

Question 23

Hurler syndrome associated characteristics: chest

Answer 23

CHEST: broad chest, spine deformities, recurrent respiratory infections

Question 24

Hurler syndrome associated characteristics: CV

Answer 24

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

Question 25

Hurler syndrome associated characteristics: neuro

Answer 25

intellectual disability, hydrocephalus

Question 26

Hurler syndrome associated characteristics: ortho

Answer 26

small stature, hypo plastic odontoid with alantoaxial subluxation

Question 27

Hurler syndrome anesthetic considerations

Answer 27

``` 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 ```

Question 28

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

Answer 28

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

Question 29

Hunter syndrome associated characteristics: HEENT/AW

Answer 29

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

Question 30

Hunter syndrome associated characteristics: CHEST

Answer 30

pectus excavatum or carinatum, frequent URI's

Question 31

Hunter syndrome associated characteristics: CV

Answer 31

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

Question 32

Hunter syndrome associated characteristics: Neuro

Answer 32

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

Question 33

Hunter syndrome associated characteristics: ortho

Answer 33

stiff joints, kyphosis

Question 34

Hunter syndrome anesthetic considerations

Answer 34

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

Question 35

CHARGE syndrome characterized by

Answer 35

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

Question 36

CHARGE syndrome associated characteristics: HEENT/AW

Answer 36

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

Question 37

CHARGE syndrome associated characteristics: chest

Answer 37

rib anomalies, precuts carinatum, respiratory insufficiency

Question 38

CHARGE syndrome associated characteristics: CV

Answer 38

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

Question 39

CHARGE syndrome associated characteristics: neuromuscular

Answer 39

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

Question 40

CHARGE syndrome associated characteristics: GI/GU

Answer 40

GERD, amphalocele, anal atresia, genital and retinal anomalies

Question 41

CHARGE syndrome associated characteristics: other

Answer 41

FTT and parathyroid hypoplasia

Question 42

CHARGE anesthetic considerations

Answer 42

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

Question 43

cystic fibrosis

Answer 43

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

Question 44

CF diagnosis

Answer 44

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

Question 45

CF diagnosis: neonate clinical manifestation includes

Answer 45

meconium ileus

Question 46

CF diagnosis: childhood clinical manifestation includes

Answer 46

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

Question 47

CF diagnosis: second decade clinical manifestation includes

Answer 47

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

Question 48

CF associated characteristics: HEENT/AW

Answer 48

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

Question 49

CF associated characteristics: chest

Answer 49

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

Question 50

CF associated characteristics: CV

Answer 50

chronic respiratory disease and hypoxemia can lead to cor pulmonale

Question 51

CF associated characteristics: GI/GU

Answer 51

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

Question 52

CF treatments and survival

Answer 52

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

Question 53

CF and PFT's

Answer 53

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

Question 54

CF surgeries most commonly include

Answer 54

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

Question 55

treatment of CF

Answer 55

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)

Question 56

management of anesthesia in CF

Answer 56

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)

Question 57

CP

Answer 57

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

Question 58

CP treatment

Answer 58

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

Question 59

anesthesia in CP

Answer 59

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

Question 60

pierre robin sequence

Answer 60

congenital condition or sequence/chain of developmental facial malformations

Question 61

3 main features of pierre robin

Answer 61

``` 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 ```

Question 62

when is pierre diagnosed

Answer 62

clinically shortly after birth due to respiratory difficulty

Question 63

pierre robin associated characteristics: HEENT/AW

Answer 63

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

Question 64

pierre robin associated characteristics: CV

Answer 64

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

Question 65

pierre robin associated characteristics: neuro

Answer 65

may have brainstem dysfunction and periods of central apnea

Question 66

pierre robin associated characteristics: GI/GU

Answer 66

feeding difficulties common due to anatomic abnormalities