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N934 Advanced Principles of Anesthesia II > Peds Medical Conditions Influencing Anesthesia > Flashcards

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
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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
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3
Q

CV characteristics of DS

A

40-50% of children have defect like AV canal, ASD, VSD, TOF and PDA

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4
Q

neuromuscular characteristics of DS

A
  • hypotonia
  • ligamentous instability
  • dementia
  • parkinsonism
  • intellecutal decline with age
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5
Q

ortho characteristics of DS

A

-lax cervical ligaments can result in alatoocipital or alantoaxial instability and dislocation

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6
Q

GI/GU characteristics of DS

A
  • duodenal atresia

- increased incidence of Hirschsprung disease

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7
Q

other characteristics of DS

A

-increased incidence of leukemia, obesity, and thyroid disease

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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
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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
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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
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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
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12
Q

MPS I

A
  • Hurler
  • alpha L iduronidase enzyme deficiency
  • treatment = stem-cell tx, ERT, supportive
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13
Q

MPS II

A
  • Hunter
  • iduronate sulfatase enzyme deficiency
  • treatment = ERT, supportive
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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
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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
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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

N934 Advanced Principles of Anesthesia II (17 decks)

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