Medical Conditions Influencing Management of Pediatric Anesthesia Flashcards
What is the most frequent chromosomal aberration
trisomy 21
Down syndrome
HEENT/Airway Characteristics of T21
short neck, epicanthic folds, brushfield’s spots, low set ears, macroglossia, mandibular hypoplasia, narrow nasopharynx, hypertrophic lympathic tissue (tonsils and adenoids), subglottic stenosis
Cardiovascular characteristics of T21
40-50% have a CHD
AV canal defect, ASD VSD TOF PDA
What are neuromuscular characterstics of T21
hypotonia, ligamentous instability, dementia and parkinsonism in older adults, intellectual decline with age
What are ortho characteristics in T21
lax cervical ligaments can result in atlantooccipital or atlantoaxial instability and dislocation
GU/GI characteristics in T21
duodenal atresia, increased incidence of Hirschsprung disease
additional characteristics of T21
leukemia, obesity, thyroid disease
Anesthesia for down syndrome (airway)
assess atlanto-axial instability
inquire about changes in gross or fine motor function or head and neck pain care with laryngoscopy to minimize flexion or extension
assess for OSA
1/2 of these children will require a downsized ETT due to subglottic stenosis
Anesthesia for down syndrome (cardiovascular)
increased incidence of bradycardia on induction
CHD- bacterial endocarditis prophylaxis
assess for pulmonary HTN d/t to either CHD or OSA
Anesthesia for down syndrome
challenging vascular access
challenging to sedation, presmediate, caregiver present for induction
hypothyroidism when present can delay gastric emptying and alter drug metabolism
What is mucopolysaccharidosis?
genetic lyososomal storage disease
a group of metabolic disorders that have absent or malfunction enzymes to break down glycoaminoglycans or GAGs
which over time, the GAGs collect in cells and connective tissues resulting in progressive and permenant damage
What are the two prominent subtypes of mucopolysaccharidosis?
hurler and hunter
What is hurler syndrome?
genetic (autosomal recessive) mucopolysaccharidosis type 1 disease
deficiency of alpha L iduronidase
What are treatments for hurler syndrome?
enzyme replacement therapy and stem cell or umbilical cord blood transplant
What can be improved and halted in hurler’s syndrome?
abnormal physical characteristics (except skeleton and eyes) and neurologic degeneration
HEENT/Airway characteristics of Hurlers
macrocephaly, corneal opacities, hearing loss, large tongue, lips, tonsils, adenoids, copious nasal drainage, narrow trachea, OSA short neck, high epiglottis
What is the worst airway problem in pediatric anesthesia?
hurler syndrome d/t macrocephaly, short necks OSA, large tongues, tonsils, lips and adenoids, narrow trachea high epiglottis
Chest characteristics of hurlers
broad chest, spine deformities, recurrent respiratory infections
CV characteristics of hurlers
coronary artery narrowing and ischemic heart disease, mitral valve thickening, cardiomegaly
Neuro characteristics of hurlers
intellectual disability, hydropcephalus
Ortho characteristics of hurlers
small stature, hypoplastic odontoid with atlantoaxial subluxation
Anesthesia considerations for Hurler syndrome
one of the most difficult airways frequently encountered
obstruction of the airway and worsens with age, espeically after age 2
difficult intubation
odontoid hypoplasia and thick secretions
ECHO (possible cards impairment)
difficult IV access
What is hunter’s syndrome
x linked mucopolysaccharidosis type 2
presentation is variable, but often apparent by age 2-4 years
less intellectual disability, less joint disease, less organ involvement and slower progression than hurler
How is hunter’s syndrome treated?
enzyme therapy and transplants have been limited
HEENT/AIrway characteristics of Hunter’s
macrocephaly, macroglossia, stiff soft tissues, cephalad and anterior larynx, hypertrophy tonsils and adenoids, OSA, copious secretions
CHEST characteristics of Hunter’s
pectus excavatum or carinatum, frequent URIs
CV characteristics of Hunter’s
coronary artery disease, thickened valves
Neuro characteristics of Hunters
progressive intellectual disability to normal, hydrocephalus, cervical spine canal narrowing
Ortho characteristics of Hunter’s
stiff joints, kyphosis
What are anesthetic considerations for Hunter’s
extraordinarily difficult laryngoscopy and tracheal intubation
OPA can worse airway as it displaces the epiglottis over larynx
OSa and postoperative obstruction pulmonary edema
supraglottic airways have served as successful conduit for fiberoptic intubation
trach may be neccessary
sensitivity to opioids
stem cell transplants require special blood product considerations (leuko-reduced, irradiated)
Airway risks with MPS features
upper airway obstruction
mandibular abnormalities, short neck, and high anterior larynx
abnormally thick secretions, tracheal distortion
Pulmonary risks with MPS features
progressive restrictive disease
OSA leads to pHTN
Spinal cord risks with MPS features
compression of the cervical spine
atlantoaxial instability and vertebral sublulxation
progressive thickening and scarring of the meninges
Cardiac risks with MPS features
valvular regurgitation and or stenosis
systolic and diastolic dysfunction
Neurological risk with MPS features
hydrocephalus
seizures
CHARGE
colobomas of the ete
heart diseaes
atresia of the choanae
retarded growth or CNS system abnormalities
genital anomalies and ear anomalies (deaf)
atleast 4 have to be present
HEENT/Airway of CHARGE
microcephaly, colobomas of the eye, upward slanting eyes, external ear abnormalites or hearing loss, choanal atresia, cleft lip and palate, severe micrognathia, short neck, laryngomalacia, subglottic stenosis, TEF
Chest in CHARGE
rib anormalies, pectus carinatum, respiratory insufficiency
CV in CHARGE
TOF
PDA
ASD VSD DORV with AV canal defect and right sided aortic arch
Neuromuscular in CHARGE
variable intellectual disability, developmental delay, facial nerve palsy, abnormal gag reflex, hearing loss
GI/GU in CHARGE
GERD, omphalocele, anal atresia, genital and renal anomalies
Additional characteristics of CHARGE
FFT and parathyroid hypoplasia
Anesthesis implications of CHarge
interpreter if deaf
GERD and impaired gag reflex may place the patient at risk for aspiration
SBE prophylaxis in CHD
micrognathia may make tracheal intubation difficult0- intubation can become more difficult with increasing age
laryngomalacia may prove difficult ventilation with LMA or mask airway
What syndrome can intubation become more difficult with increasing age?
CHARGE
What is cystic fibrosis?
inhertied autosomal recessive mutation on the long arm of chromosome 9
misfolding of CFTR protein that results in elevated sweat chloride concentrations viscous mucus production, lung disease, intestinal obstruction, pancreatic insufficiency, biliary cirrhosis, and congenital absence of vas defens
In summary cystic fibrosis,
is a decrease in CI- accompanied by a decrease in Na and water which leads to dehydration, viscous secretion and electrolyte abnormalities
HEENT/Airway of CF
chronic sinusitis, nasal polyps
Chest of CF
recurrent chest infections, viscis mucous due to electrolyte abnormalities and mucous gland cell hypertrophy, plugging and bacteria colonization
chronic infections lead to lung inflammation and damage bronchial hyperreactivity, decreased ciliary clearance and spontaneously pneumo
CV in CF
chronic respiratory disease + hypoxemia can lead cor pulmonale
GI/GU of CF
pancreatic exocrine insufficiency leads to mucous plugging and. ductal obstructions and malabsorption
What do PFTs look in like in CF?
obstructive, increased FRC, decreased FEV1, decreased peak expiratory flow rate, and decreased vital capactiy
but also can lead to restrictive disease (with chronic lung destruction)
Anesthesia Management of CF
optimize patient
volatiles allow decreased airway resistances and smooth muscle tone
long inhalation inductions due to large FRC, small tidal volumes and V/Q mismatch
hypoxia may develop due to V/Q mismatch
short acting anesthetics to minimize post op respiratory depression
anticholinergics are controversial, optimize hydration
humidifed inspired gases
frequent tracheal suctioning
may require high ventilation pressures (cuffed etts)
Cerebral Palsy is
collective term for a variety of non-progressive conditions resulting from an insult early in life or leisons/anomalies of the brain
What is the most common manifestation of CP?
skeletal muscle spasticity and contractures
Anesthesia and CP
determine patient’s baseline, understanding and ability to communicate
tracheal intubation- at risk for aspiration (GERD is common) increased secretions and impaired swallowing
volatiles are safe
children on anticonvulsants are more resistant to NMDRs
susceptible to hypothermia
contractures may positioning difficult
emergence may be slow
higher incidence of pulmonary complications postoperatively and may require ICU
What is pierre robin and the three main features?
congential condition or sequence/chain development facial malformations
hypoplastic mandible (micrognathia)
pseudo-macroglossia (posterior displacement of tongue)
high arched cleft palate
what can improve airway movement in pierre robin?
prone position
by displacement of the tongue
What do pierre robin kiddos often require?
tracheostomy placement, mandibular distraction, cleft palate repair
HEENT/Airway for pierre robin
severe micrognathia, glossoptosis, U shaped cleft palate, obstructive apnea, airway obstruction usually improves with age
CV for pierre robin
cor pulmonale can develope with severe chronic airway obstruction, may have vagal hyperactivity
Neuro for pierre robin
may have brainstem dysfunction with periods of central apnea
GI/GU for pierre robin
feeding difficulties common due to anatomic abnormalites
