Peds Medical Conditions Influencing Anesthesia Flashcards
1
Q
Down Syndrome/Trisomy 21
A
- most frequent chromosomaal aberration
- 1.5 per 1000 live births
- DD present but varies in severity
2
Q
HEENT/Airway characteristics of DS
A
- short neck
- epicanthic folds
- Brushfield’s spots
- low set ears
- macroglossia
- mandibular hypoplasia
- narrow nasopharynx
- hypertrophic lymphatic tissue (tonsils/adenoids)
- subglottic stenosis
3
Q
CV characteristics of DS
A
40-50% of children have defect like AV canal, ASD, VSD, TOF and PDA
4
Q
neuromuscular characteristics of DS
A
- hypotonia
- ligamentous instability
- dementia
- parkinsonism
- intellecutal decline with age
5
Q
ortho characteristics of DS
A
-lax cervical ligaments can result in alatoocipital or alantoaxial instability and dislocation
6
Q
GI/GU characteristics of DS
A
- duodenal atresia
- increased incidence of Hirschsprung disease
7
Q
other characteristics of DS
A
-increased incidence of leukemia, obesity, and thyroid disease
8
Q
airway anesthesia considerations for DS
A
- assess alantoaxial instability
- inquire about changes in gross or fine motor function or head/neck pain
- care with laryngoscopy to minimize flexion and extension
- assess for OSA
- 1/4 of these children will need downsized ETT due to subglottic stenosis
9
Q
CV anesthesia considerations for DS
A
- increased incidence of bradycardia on induction
- CHD = may need SBE prophylaxis
- assess for pulmonary HTN either due to CHD or OSA
10
Q
other anesthesia considerations for DS
A
- challenging vascular acess
- challenging to sedate, premed, caregiver may need to be present for induction
- hypothyroidism when present can result in delayed gastric emptying and altered drug metabolism
11
Q
mucopolysaccharidosis (MPS)
A
- genetic lysosomal storage disease
- a group of metabolic disorders that have absent or malfunction enzymes to break down glycosaminoglycans or GAGs
- 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
- several distinct sub-types of MPS
- often will have striking skeletal features
- may or may not have behavior or cognitive difficulties
12
Q
MPS I
A
- Hurler
- alpha L iduronidase enzyme deficiency
- treatment = stem-cell tx, ERT, supportive
13
Q
MPS II
A
- Hunter
- iduronate sulfatase enzyme deficiency
- treatment = ERT, supportive
14
Q
Hurler syndrome
A
- MPS type I
- result = build up of glycosaminoglycans due to deficiency of alpha-L iduronidase
- symptoms appear during childhood and early death (frequently by age 10) occurs due to organ damage, airway disease, respiratory infections or cardiac complications
- progressive deterioration, hepatosplenomegaly, dwarfism, and unique facial features with progressive mental decline
15
Q
hurler syndrome treatments
A
- treatments include enzyme replacement therapy and stem cell or umbilical cord blood transplant
- abnormal physical characteristics (except for those affecting the skeletal and eyes) can be improved and neurologic degeneration can often be halted
16
Q
hurler syndrome associated characteristics
A
- AIRWAY - short neck, narrow trachea, high epiglottis; extremely difficult if not the WORST airway problem in pediatric anesthesia
- CHEST - broad chest, spine deformities, recurrent resp infections
- CV - coronary artery narrowing, mitral valve thickening, cardiomegaly
- NEURO - intellectual disability, hydrocephalus
- ORTHO - small stature, hypoplastic odontoid with alantoaxial subluxation
17
Q
hurler anesthestic considerations
A
- one of the MOST difficult pediatric airways frequently encountered
- obstruction of the airway worsens with age, especially after age 2
- difficult intubation
- odontoid hypoplasia and thick secretions
- ECHO - possible cardiac impairment
- difficult IV access
18
Q
Hunter syndrome
A
- X-linked MPS type II
- presentation variable but often apparent by age 2-4 years
- generally less intellectual disability, less joint disease, less organ involvement, and slower progression than hurler
- death often by late teens without treatment, but some mild forms have lived into 40s
- treatment = enzyme therapy and transplants have been limited
19
Q
Hunter syndrome associated characteristics
A
- HEENT/AIRWAY - macrocephaly, macroglossia, stiff tissues, cephalad anterior larynx, OSA, lots of secretions
- CHEST - pectus, frequent URIs
- CV - CAD, thickened valves
- NEURO - progressive intellectual disability to normal, hydrocephalus, cervical spine canal narrowing
- ORTHO - stiff joints, kyphosis
20
Q
Hunter syndrome anesthetic considerations
A
- extraordinarily difficult laryngoscopy and tracheal intubation
- OPA can worsen airway (displaces epiglottis over larynx)
- positioning challenging with stiff joints
- OSA and postop obstructive pulmonary edema
- supraglottic airways have served as successful conduit for fiberoptic intubation
- trach may be necessary
- stem cell transplant patients require special blood product considerations
- sensitivity to opioids
21
Q
MPS Features + anesthetic risk –> AIRWAY
A
- upper airway obstruction
- mandibular abnormalities, short neck, and high anterior larynx
- abnormally thick secretions
- tracheal distortion
22
Q
CHARGE syndrome/association
A
- Colobomas of the eye
- Heart disease
- Atresia of the Choanae
- Retarded growth or CNS anomalies
- Genital anomalies
- Ear anomalies or deafness
- at least four must be present for diagnosis
- all organ systems involved are at critical development during the 2nd month of gestation
23
Q
CHARGE syndrome associated characteristics
A
- HEENT/AIRWAY - microcephaly, colombas of eye, slanting eyes, ear/hearing abnormalities, cleft lip/palate, severe micrognathia, short neck, laryngomalacia, subglottic stenosis, TEF
- CHEST - rib abnormalities, pectus, resp insufficiency
- CV - TOF (most common), PDA, ASD, VSD, DORV with AV canal defect and R sided aortic arch
- NEURO - intellectual disability, DD, facial nerve palsy, abnormal gag reflex
- GI/GU - GERD, omphalocele, anal atresia, gental/renal anomalies
- OTHER - FTT, parathyroid hypoplasia
24
Q
CHARGE anesthetic considerations
A
- interpreter for deaf patients
- GERD and impaired gag = aspiration risk
- SBE prophylaxis in CHD
- micrognathia may make tracheal intubation difficult and intubation can become more difficult with increasing age
- laryngomalacia may prove difficult ventilation with LMA or mask airway
- subglottic stenosis may require smaller ETT
- choanal atresia may cause severe resp distress in newborn; precludes use of nasal airways and NGTs
25
Q
Cystic Fibrosis
A
- inherited autosomal recessive mutation on the long arm of chromosome 9
- membrane glycoprotein chloride channel that contributes to regulation of ion flux at various epithelial surfaces (sweat ducts, airway, pancreatic duct, intestines, biliary tree, and vas deferns)
- misfolding of CTFR gene
- 1 in 2000 live births
- elevated sweat chloride –> viscous mucus, lung disease, intestinal obstruction, pancreatic insufficiency, biliary cirrhosis, congenital absence of vas deferens
26
Q
diagnosis of CF
A
sweat chloride test > 80 mEq/L plus clinical manifestations (cough, chronic purulent sputum, exertion dyspnea
27
Q
CF Anesthestic Considerations
A
- HEENT/AIRWAY - chronic sinusitis, nasal polyps
- CHEST - recurrent chest infections, viscous mucus, plugging and bacterial colonization pseudomonas or staph; lung inflammation and damage
- CV - chronic respiratory disease and hypoxemia can lead to cor pulmonale
- GI/GU - 85% have pancreatic exocrine insufficiency leading to mucus pluging and ductal obstructions as well as malabsorption
28
Q
lung disease in CF
A
- lung disease is main cause of morbidity and mortality
- increase secretions, viscous mucus, and impaired ciliary clearance
- prone to recurrent infections
- chronic neutrophil inflammatory response
- bronchiectasis, emphysema, V/Q mismatch, hypoxemia
- PFTs are obstructive –> increased FRC, decreased FEV1, decreased peak exp flow rate, decreased vital capacity
29
Q
common surgeries for CF
A
- polypectomy
- functional endoscopic sinus surgery (FESS)
- bronchoscopies
- lung transplants
30
Q
treatment of CF
A
- goal is to alleviate symptoms
- treatment of malnutrition
- relief of airway obstruction
- correct organ dysfunction –> clearance of airway secretions, bronchodilators, reduce viscoelasticity of sputum, abx for infections, organ transplant, chole, treatment of pneumo
31
Q
anesthesia management of CF
A
- postpone elective procedures until optimized
- volatiles allow decreased airway resistance and smooth muscle tone
- short-acting anesthetics to minimize postop resp depression
- anticholinergics controversial, optimize hydration
- humidify inspired gases
- frequent tracheal suctioning
- may require high ventilation pressures (def cuffed ETTs are your friend)
32
Q
cerebral palsy
A
- symptom complex rather that disease
- cause = unknown
- collective term for variety non-progressive condition resulting from an insult early in life
- varying degrees of severe DD to normal intelligence
- varying degrees of mild local weakness to severe spastic quadriplegia
- seizure disorder
33
Q
CP treatment
A
- most children undergo ortho procedures, dental restoration, anti-reflux operations (Nissen)
- often receive seizure and muscle spasticity meds - avoid acute withdrawal, hepatic enzyme induction, lethargy/sedation
34
Q
anesthesia for CP
A
- determine patient’s baseline, understanding and ability to communicate
- tracheal intubation = at risk for aspiration (d/t GERD)
- 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 pulm complications post op and may require ICU
35
Q
Pierre Robin Sequence
A
- congenital condition of sequence/chain of developmental facial malformations
- 3 main features = hypoplastic mandible, pseudo-macroglossia, high arched cleft palate
- DIFFICULT intubation
- usually diagnosed shortly after birth due to resp difficulty
- often requires trach, mandibular distraction, and cleft palate repair
