Medical conditions influencing management in pediatric anesthesia Flashcards
HEENT/Airway characteristics of down syndrome include
short neck macroglossia mandibular hypolasia narrow nasopharynx hypertrophic lymphatic tissue subglottic stenosis- may have to downsize tube
Cardiovascular defects in patients with down syndrome may include
AV canal defect
ASD
VSD
TOF & PDA
Ortho defects in patients with down syndrome may include
lax cervical ligaments can result in atlantooccipital or atlantoaxial instability & dislocation
Anesthesia considerations for patients with down syndrome related to the airway include
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
Anesthesia considerations for patients with down syndrome related to cardiac include
increased incidence of bradycardia on induction
CHD- bacterial endocarditis prophylaxis
assess for pulmonary hypertension either due to CHD or OSA
Anesthesia considerations for down syndrome include
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
Mucopolysaccharidosis is 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
Describe the treatment differences of Hurler vs. Hunter
Hurler is more responsive to SCT
Hurler syndrome results in the buildup of
glycosaminoglycans due to a deficiency of alpha-L iduronidase
Hurler syndrome is
genetic (autosomal recessive)
Symptoms of Hurler’s syndrome appear
during childhood & early death (frequently by age 10) occurs due to organ damage, airway disease, respiratory infections or cardiac complications
Hurler syndrome is characterized by
progressive deterioration
hepatosplenomegaly
dwarfism
and unique facial features with progressive mental decline
Describe associated characteristics of the HEENT/AIRWAY for hurler syndrome
macrocephaly large tongue, lips, tonsils & adenoids copioius nasal discharge narrow trachea OSA short neck high epiglottis considered worst airway problem in pediatric anesthesia
Describe associated characteristics of the chest for Hurler syndrome
broad chest
spine deformities
recurrent respiratory infections
Describe CV characteristics for Hurler syndrome
coronary artery narrowing & ischemic heart disease, mitral valve thickening, cardiomegaly
Describe neuro characteristics of Hurler syndrome
intellectual disability
hydrocephalus
Describe ortho characteristics of Hurler syndrome
small stature
hypoplastic odontoid with atlantoaxial subluxation
Describe anesthetic considerations for Hurler syndrome
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
Hunter syndrome is
X-linked mucopolysaccharidosis type II disease
presentation is variable but often apparent by age 2-4 years
Describe Hunter syndrome vs. Hurler syndrome
less intellectual disability, less joint disease, less organ involvement & slower progression than Hurler
death is often by late teens without treatment
Describe HEENT/airway characteristics of Hunter syndrome.
macrocephaly, macroglossia, stiff soft tissues, cephalad & anterior larynx, hypertrophy tonsils & adenoids, OSA, copious secretions
Describe characteristics of the chest of Hunter syndrome
pectus excavatum or carinatum, frequent URIs
Describe CV characteristics of Hunter syndrome
coronary artery disease
thickened valves
Describe neuro characteristics of Hunter syndrome
progressive intellectual disability to normal, hydrocephalus, cervical spine canal narrowing
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
Airway related anesthetic risks for MPS includes
upper airway obstruction
mandibular abnormalities, short neck & high anterior larynx
abnormally thick secretions, tracheal distortion
Pulmonary related anesthetic risks for MPS includes
progressive restrictive disease
OSA leads to pHTN
Spinal cord related anesthetic risks for MPS include
compression of the cervical spine
atlantoaxial instability & vertebral subluxation
progressive thickening & scarring of the meninges
Cardiac related anesthetic risks for MPS include
valvular regurgitation and/or stenosis
systolic & diastolic dysfunction
Neurological related anesthetic risks for MPS include
hydrocephalus
seizures
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
Describe HEENT/airway considerations for CHARGE syndrome
microcephaly cleft lip & palate severe micrognathia short neck laryngomalacia subglottic stenosis TEF
Describe chest considerations for CHARGE syndrome
rib anomalies, pectus carinatum, respiratory insufficiency
Describe CV considerations for charge syndrome
TOF (most common), PDA, ASD, VSD, DORV with AV canal defect & right sided aortic arch
Describe neuromuscular considerations for charge syndrome
variable intellectual disability, developmental delay, facial nerve palsy, abnormal gag reflex, hearing loss
Describe GI/GU & other considerations for charge syndrome
GERD
renal anomalies
failure to thrive
parathyroid hypoplasia
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**
Cystic fibrosis is an
inherited autosomal recessive mutation on the long arm of chromosome 9
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
With cystic fibrosis, there is a decrease in
Cl- accompanied by decrease of Na & water which leads to dehydration, viscous secretions & electrolyte abnormalities
Diagnosis of CF is typically through
sweat chloride test >80 mEq/L plus clinical manifestations (cough, chronic purulent sputum, exertion dyspnea)
neonate- meconium ileus
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
HEENT/airway considerations for CF include
chronic sinusitis, nasal polyps
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
CV considerations for CF include
chronic respiratory disease & hypoxemia can lead to cor pulmonale
GI/GU considerations for CF include
mucous plugging & ductal obstructions as well as malabsorption
The main cause of morbidity & mortality in CF is
lung disease
Lung disease in CF results in
increase secretions
viscous mucus
impaired ciliary clearance
prone to recurrent infections
Surgery for CF patients most commonly includes
polypectomy
FESS
bronchoscopies
lung transplants
PFTs in CF appear as
obstructive: increased FRC decreased FEV1 decreased peak expiratory flow rate decreased vital capacity
Lung disease in CF patients leads to
bronchiectasis
emphysema
V/Q mismatching & hypoxemia
Treatment of CF includes
goal is to alleviate symptoms through
treatment of malnutrition
relief of airway obstruction
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
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
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
Cerebral palsy can vary from
mild local weakness to severe spastic quadriplegia- most common is muscle spasticity & contractures
seizure disorders
CP patients often receive
seizure & muscle spasticity medications- avoid acute withdrawal, hepatic enzyme induction, lethargy/sedation
Most children with CP undergo
elective orthopedic corrective procedures
dental restoration
anti-reflux operations
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
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
Patients with Pierre Robin sequence are at risk for
Difficult intubation***
Patient with Pierre Robin sequence may be placed
in the prone position as it may improve airway movement by displacement of the tongue
Pierre robin sequence often requires
tracheostomy placement, mandibular distraction, & cleft palate repair
HEENT/airway considerations of Pierre Robin includes
severe migrognathia, glossoptosis, U-shaped cleft palate, OSA, airway obstruction usually improves with age
CV considerations of Pierre Robin includes
cor pulmonale can develop with severe chronic airway obstruction, may have vagal hyperactivity
Neuro considerations of Pierre Robin includes
may have brainstem dysfunction with periods of central apnea
GI/GU considerations of Pierre Robin syndrome includes
feeding difficulties common due to anatomic abnormalities
