Medical Conditions Influencing Management in Pediatric Anesthesia Flashcards
most frequent chromosomal aberration
trisomy 21
HEENT/AW associated characteristics for down syndrome (trisomy 21)
short neck, epicanthic folds, brush fields spots, low set ears, macroglossia, mandibular hypoplasia, narrow nasopharynx, hypertrophic lymphatic tissue (tonsils and adenoids), subglottic stenosis
CV associated characteristics for down syndrome (trisomy 21)
40-50% of them get this. AV canal defect, ASD, VSD, TOF, PDA
neuromuscular associated characteristics for down syndrome (trisomy 21)
hypotonia, ligamentous instability, dementia and parkinsonism in older adults. intellectual decline with age
ortho associated characteristics for down syndrome (trisomy 21)
lax cervical ligaments can result in Atlanto-occipital (8.5%) or atlantoaxial(7-36%) instability and dislocation
GI/GU associated characteristics for down syndrome (trisomy 21)
duodenal atresia, increased incidence of hirschprungs disease
OTHER associated characteristics for down syndrome (trisomy 21)
leukemia, obesity, thyroid disease
anesthesia for down syndrome: aw considerations
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
anesthesia for down syndrome: CV considerations
increased incidence of bradycardia on induction
CHD: bacterial endocarditis prophylaxis
assess for pulmonary HTN either due to CHD or OSA
anesthesia for down syndrome: hypothyroidism considerations
when present, can result in delayed gastric emptying and altered drug metabolism
anesthesia for down syndrome: induction considerations
challenging to sedate, premedical, caregiving present for induction
mucopolysaccharidosis (MPS) definition
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
MPS features/sx general
often will have striking skeletal features. may or may not have behavior or cognitive difficulties
MPS subtype 1 eponym, enzyme deficiency, treatment
hurrler
alpha L iduronidase
stem cell txp, ERT, supportive
MPS subtype 2 eponym, enzyme deficiency, treatment
hunter
iduronate sulfatase
ERT, supportive
MPS subtype 3 eponym, enzyme deficiency, treatment
sanfilippo
multiple types
supportive
MPS subtype 4 eponym, enzyme deficiency, treatment
morquio
n acetylgalactosamine-6-sulfate
ERT, supportive
MPS subtype 6 eponym, enzyme deficiency, treatment
maroteaux-lamy
n-acetylgalactosamine-4-sulfatase
ERT, supportive
MPS subtype 7 eponym, enzyme deficiency, treatment
sly
beta glucoronidase
ERT, supportive
MPS 1: Hurler syndrome results from, sx, life expectancy
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
Hurler syndrome associated features
hepatosplenomegaly, dawrfism, unique facial features, progressive mental decline
Hurler syndrome associated characteristics: HEENT/AW
macrocephaly, corneal opacities, hearing loss, large tongue, lips, tonsils and adenoids, copious nasal discharge, narrow trachea, OSA, short neck, high epiglottis
Hurler syndrome associated characteristics: chest
CHEST: broad chest, spine deformities, recurrent respiratory infections
Hurler syndrome associated characteristics: CV
coronary artery narrowing, ischemic heart disease, mitral valve thickening, cardiomegaly
Hurler syndrome associated characteristics: neuro
intellectual disability, hydrocephalus
Hurler syndrome associated characteristics: ortho
small stature, hypo plastic odontoid with alantoaxial subluxation
Hurler syndrome anesthetic considerations
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
MPS: Hunter syndrome (MPS subtype 2) results from, life expectancy, compared to MPS1
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
Hunter syndrome associated characteristics: HEENT/AW
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
Hunter syndrome associated characteristics: CHEST
pectus excavatum or carinatum, frequent URI’s
Hunter syndrome associated characteristics: CV
coronary artery disease, thickened valves, regurgitation, systolic and diastolic dysfunction
Hunter syndrome associated characteristics: Neuro
progressive intellectual disability to normal, progressive thickening and scarring of meninges, hydrocephalus, cervical spine canal narrowing/compression, atlantoaxial instability and vertebral subluxation, seizures
Hunter syndrome associated characteristics: ortho
stiff joints, kyphosis
Hunter syndrome anesthetic considerations
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
CHARGE syndrome characterized by
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
CHARGE syndrome associated characteristics: HEENT/AW
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
CHARGE syndrome associated characteristics: chest
rib anomalies, precuts carinatum, respiratory insufficiency
CHARGE syndrome associated characteristics: CV
TOF (most common), PDA, ASD, VSD, DORV with AV canal defect and right sided aortic arch
CHARGE syndrome associated characteristics: neuromuscular
variable intellectual disability, developmental delay, facial nerve palsy, abnormal gag reflex, hearing loss
CHARGE syndrome associated characteristics: GI/GU
GERD, amphalocele, anal atresia, genital and retinal anomalies
CHARGE syndrome associated characteristics: other
FTT and parathyroid hypoplasia
CHARGE anesthetic considerations
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
cystic fibrosis
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
CF diagnosis
> 80mEq/L plus clinical manifestations (cough, chronic purulent sputum, exertion dyspnea)
CF diagnosis: neonate clinical manifestation includes
meconium ileus
CF diagnosis: childhood clinical manifestation includes
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
CF diagnosis: second decade clinical manifestation includes
malabsorption superseded by increasing pulmonary problems. respiratory failure and chronic infections
CF associated characteristics: HEENT/AW
chronic sinusitis, nasal polyps, chronic neutrophil inflammatory response, recurrent infections, bronchiectasis, emphysema, VQ mismatching, hypoxemia
CF associated characteristics: chest
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
CF associated characteristics: CV
chronic respiratory disease and hypoxemia can lead to cor pulmonale
CF associated characteristics: GI/GU
85% have a pancreatic exocrine insufficiency leading to mucous plugging and ductal obstructions as well as malabsorption.
CF treatments and survival
advances in gene therapy, global registry, and surgical treatments. cystic fibrosis transmembrane conductance regulator (CFTR) modulators
35-40y in US and UK
CF and PFT’s
obstructive, increased FRC, decreased FEV1, decreased peak expiratory flow rate, decreased VC. however, chronic lung obstruction leads to restrictive disease
CF surgeries most commonly include
polypectomy, functional endoscopic sinus surgery (FESS), bronchoscopies, lung transplants
treatment of CF
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)
management of anesthesia in CF
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)
CP
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
CP treatment
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
anesthesia in CP
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
pierre robin sequence
congenital condition or sequence/chain of developmental facial malformations
3 main features of pierre robin
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
when is pierre diagnosed
clinically shortly after birth due to respiratory difficulty
pierre robin associated characteristics: HEENT/AW
severe micrognathia, glossoptosis, U shaped cleft palate, OSA, aw obstruction. usually improves with age
pierre robin associated characteristics: CV
cor pulmonale can develop with severe chronic aw obstruction, may have vagal hyperactivity
pierre robin associated characteristics: neuro
may have brainstem dysfunction and periods of central apnea
pierre robin associated characteristics: GI/GU
feeding difficulties common due to anatomic abnormalities
