Joan C. Arvedson, Ph.D.

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Airway Issues: Stability
Critical Basis for Oral Feeding
Joan C. Arvedson, PhD, CCC-SLP, BRS-S, ASHA Fellow
jcarved@aol.com & jarvedson@chw.org
Disclosures
I have the following financial relationships
relevant to the content of my presentation:
Cengage Learning: Royalties on sale of
books
Pearson: Royalties on sale of books
Northern Speech Services: Royalties on
sale of training manuals
I have no relevant nonfinancial relationships
to disclose
Education is the greatest
need of the people,
but first they must be fed
(Dantons Memorial, Paris)
Why Should We Try to Solve Complex
Feeding & Swallowing Issues?
Most infants & children have complex
issues even if it looks simple superficially
Underlying physiologic stability critical basis
for oral feeding airway most important
Evaluation & intervention decisions must
take into account respiratory/airway status
Care a lot about kids & their families
Primary Needs for All Humans
Respiration/Airway
Nutrition/Hydration
Feeding, Swallowing & Nutrition
Center at Childrens Hospital
Parents/caregivers + child
Physicians (Gastroenterologists)
Nurses
Dietitians
Speech-Language Pathologists
Psychologists
Occupational Therapists
Scheduler-Coordinator
Joan C. Arvedson, Ph.D.
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Additional Specialties
Social Work
Physical Therapy
Otolaryngology
Pulmonology
Physiatrists (Rehab)
Developmental Pediatrics
Radiology
Cardiology
Surgery
Range of Services
Outpatient clinic
Comprehensive evaluation
Follow-up based on individual needs
Outpatient intensive intervention 5 days
Inpatient intensive program
2 week admission parents with child
Psychologists initiate feedings, parents
observe, brought in after a few days, then
coached with bug in ear
Close monitoring by dietitians & MDs
Dysphagia: Health Considerations
Pulmonary/airway issues
Nutrition/hydration & undernutrition
Neurologic & neurodevelopmental issues
Gastrointestinal (GI) tract issues
Medication effects
Airway/Pulmonary Focuses
Upper airway issues
Aspiration focuses later
Airway evaluation by non-physicians
Upper airway etiologies
Diagnostic categories
Assessment
Intervention strategies
Bedside Airway Examination
Respiratory rate: at rest & feeding
Respiratory effort:
Stridor
Stertor
Retractions: suprasternal, substernal
Bedside Airway Examination
Vocal quality variables
Strong, clear phonation, appropriate pitch
Weak, breathy, husky to hoarse
Gurgly, wet
Velopharyngeal function inferences
(e.g., hypernasality, hyponasality)
Pharyngonasal backflow or reflux
Frequent burping or hiccups?
Joan C. Arvedson, Ph.D.
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Airway Stability for PO Feeding
Airway stability is prerequisite for successful PO
If airway concerns are noted during physical
exam, possible next steps:
Otolaryngology airway exam (FFL, DLB)
Bedside/clinical oral feeding evaluation
Combined FFL & FEES with ORL & SLP
Videofluoroscopic swallow study (VFSS)
Monitor status for a few days
Fixable Straight Forward Case:
What & Where is the Problem?
Term C-section, poor progression of labor
Respiratory distress at 1 hour of age
Transient tachypnea of newborn
Tonic-clonic seizures up to 30 sec, day 2
Head CT & EEG: normal
Feeding consult at 1 week of life
Upper Airway Obstruction
Feeding up to 1 hour for 1 oz with gagging,
choking, and gasping for air.
At rest, normal respirations
Neurodevelopmental exam normal
Feeding observation: good suck, inspiratory
stridor, & moderate chest retractions
Supraglottic airway obstruction suspected
What & where is the problem?
Next Steps in Examination
Lateral neck X-ray: Mass anterior to, but
separate from, epiglottis
FFL: Normal vocal fold movement
Mass noted in valleculae,
Partially occluding supraglottic larynx,
especially with swallow
CT of nasopharynx: 1 cm
2
soft tissue mass
Intervention for Vallecular Cyst
Surgical excision
Bronchoscopy no further abnormalities
Pathology benign cyst
Feeding no problems at discharge
Common Airway Problems with
Feeding & Swallowing Difficulties
Choanal atresia or stenosis
Laryngomalacia
Midface & mandibular hypoplasia
Vocal fold paralysis or paresis
Laryngeal cleft
Subglottic stenosis
CNS & neuromuscular diagnoses
affecting airway protection
Joan C. Arvedson, Ph.D.
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Choanal Atresia
or Stenosis
Choanal Atresia
1 in 7,000 to 8,000 newborns, 50%-60%
unilateral
Usually presents with first feeding
Periods of cyanosis at rest, agitation,
begins to cry, & breathes through mouth
relieves airway obstruction
Some have little or no respiratory distress at
rest, but major distress at onset of feeding
Choanal Atresia
Test for airflow: wisp of cotton created at
end of a cotton-tipped applicator
FFNL + CT scan of nose & nasopharynx
needed to determine type (bony, mixed, &
membranous ) & extent of lesion
Surgery in neonatal period, transnasal or,
in some, transpalatal approach
Complication: GER with intermittent nasal
reflux (Beste, Conley, Brown, 1994)
Choanal or Nasal Stenosis
Deviated nasal septum rule out
Edema of nasal mucosa common with
nasal suction at birth clears when suction
stopped
Midnasal stenosis & anterior or nasal
aperture stenosis may cause obstructive
problems & feeding problems
Choanal Stenosis: SLP Role
Stertor (nasopharyngeal noise due to
obstruction) stethoscope not required
Unilateral stenosis can affect feeding:
incoordination of suck, swallow, breathe
Check breathing when infant is quiet or
asleep: Is mouth open? If yes, what
happens when you hold lips together?
Choanal Stenosis: Oral Feeding
Pacing may be sufficient with mild deficit
Nipple may have to be adjusted to allow
mouth breathing: best not to take it out
Try not to disrupt feeding
Monitor flow rate closely
Once nasal airway is sufficient & stable,
infant should feed well
Joan C. Arvedson, Ph.D.
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Beckwith-Wiedemann
Beckwith-Wiedemann Syndrome (BWS)
Macroglossia, omphalocele, ear creases,
& macrosomia
Development can be normal
Mild to moderate MR reported in some
Feeding problems secondary to large
tongue that can block airway
Management varies: conservative to
surgery
BWS: Outcomes
Surgical techniques variable
Modified keyhole technique (Kaufman et al 08)
Submucosal minimally invasive lingual excision
(SMILE) (Friedman et al, 2008)
Radiofrequency reduction of tongue base (RFBOT)
Outcomes: Variable
Best: Functioning tongue (normal mobility)
Worst: Tracheostomy (Kacker et al., 2000)
BWS: Outcomes
Decline in ability to detect salty & bitter
taste after tongue reduction (Matsune et al,
2006)
Reduction in apnea/hypopnea index
Improved speech in some
Laryngomalacia
Congenital Laryngomalacia (CLM)
Weak laryngeal tone
Prolapse of supraglottic tissue into airway
Common mechanisms
Cuneiforms drawn inward during inspiration
Exaggerated omega shaped epiglottis curls
on itself
Arytenoid cartilages collapse inward
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CLM: Incidence & Presentation
Most common laryngeal anomaly
Affects up to 45% to 75% of all infants with
congenital stridor
Typical presentation
Inspiratory stridor within 1
st
10 days of life
- over months & peaks at 6-8 months
Most cases benign & resolve 12-24 months
Up to 22% severe major upper airway
obstruction
Stridor in Severe CLM
Inspiratory
High pitched
Loudest when upset
More evident in supine
CLM: Complications
Airway obstruction with cyanosis
Life-threatening apnea
Feeding difficulty with failure to thrive
Developmental delay
Pectus excavatum
Cor pulmonale
Cardiac failure
Asphysiation
Death
CLM with Other Conditions
Neurologic disease
Cardiopulmonary disease
Congenital anomalies
Syndromes
Other medical co-morbidities
CLM: Diagnosis
Respiratory & swallow evaluation
Flexible endoscopy (laryngoscopy)
Flaccid epiglottis
Poorly supported arytenoids
Short aryepiglottic folds
Tracheobronchoscopy may be used
Indirect laryngoscopy not usually
used with infants
CLM: Etiologic Theories
First described 1843, etiology remains elusive
Anatomic: flaccid laryngeal tissue
Cartilaginous (chondromalacia): laryngeal
cartilage is abnormal, immature, & pliable
Neurologic: neuromuscular hypotonia most
commonly cited
GERD: implicated as causative factor, more
likely association
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CLM: Neuromuscular Etiology
Unclear whether alteration is neuromuscular or
located in PNS, CNS, or both
Sensorimotor integrative function or larynx
tested in 134 infants
Degree of alteration correlated with disease
severity
Sensorimotor integrative function improved as
symptoms resolved
Thompson, 2007, The Laryngoscope
CLM: Feeding Difficulties
Gastroesophageal reflux likely a major
factor
Secondary to upper airway obstruction
Incidence: 33% to 75%
Slow feeding, frequent emesis,
undernutrition
CLM: Range of Treatment
Mild: benign & self-limiting
Reassurance to parents
Follow-up
Severe: surgical intervention
Laser resection (Supraglottoplasty)
Management of GER
CLM: SLP Role for Feeding
Determine most efficient oral feeding:
position, liquid flow, pacing
Monitor inspiratory stridor & effect on PO
Effects of GER & nipple feeding?
Reassurance to parents re positive
prognosis in coming months
Spoon feeding & cup drinking may be
focus earlier than in typical infants
Midface & Mandibular
Hypoplasia
Midface Hypoplasia
Craniosynostoses most common
Crouzon
Apert
Autosomal dominant
Multiple anomalies: premature closure
of cranial sutures,ocular proptosis due
to small orbits, cleft palate, finger &
hand abnormalities in Apert
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Midface Hypoplasia: Management
Stridor at rest in severe forms (esp. no cleft)
Maxilla may be impacted against skull base
Less severe: oral feeding may stress a
marginally compensated airway
Tracheostomy required in some
Midface advancement as young as 3 years,
prefer to defer until after puberty
Mandibular Hypoplasia
Pierre Robin Sequence
Treacher Collins
Goldenhar
Hemifacial Microsomia
Mandibular Hypoplasia
All or part of mandible, bilateral or unilateral
Common malformation for infant respiratory
problems
Retrognathia/micrognathia
Glossoptosis
Cleft palate may exacerbate condition
Respiratory status improves with growth
Pierre-Robin Sequence
Mandibular Hypoplasia (micrognathia)
Glossoptosis (retroplaced tongue)
U-shaped cleft palate
80% per some current reports
Airway obstruction
PRS: Embryology
Abnormal mandible development
7 to 11 weeks PCA
High tongue position
Tongue cannot descend
Mechanisms variable
Pierre-Robin Sequence Types
True glossoptosis
Cleft portions of soft palate interposed
between tongue & PPW
Lateral pharyngeal wall hypotonia causing
hypopharyngeal collapse
Concurrent GERD/EERD may complicate
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Pierre-Robin Sequence
Associated Syndromes: 82%
Sticklers
22q11.2 deletion syndrome
(Velocardiofacial, Di George)
Treacher Collins
Miscellaneous
Non-syndromic: 18%
Stickler Syndrome
Pierre Robin Sequence
Severe myopia
Retinal detachment, cataracts
Arthropathy (joint disorder)
Musculoskeletal abnormalities
Pinna malformations (10-12%)
Genetics Evaluation
Encouraged as part of newborn workup
Important to define possible syndrome or
additional anomalies as early as possible
Intervention strategies often depend on
accurate diagnosis
Prognosis may be related directly to
underlying etiology
PRS: Upper Airway Obstruction
Most acute finding in infants
Not always due to glossoptosis
Usually immediate, may be delayed
Feeding difficulties related
Pulse oximetry monitoring
Feeding: Pierre Robin Sequence
Needs relate to degree of airway obstruction
PRS +/- Stickler syndrome
Feeding difficulties pre-intervention
Oral, pharyngeal, & esophageal phases
Non-oral supplements needed for prolonged
periods
Esophageal manometry: 50% abnormalities
Suggest brain stem control dysregulation
Baujat et al., 2001
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Feeding: Pierre Robin Sequence
Tests related to swallowing efficiency
Electromyography:Sucking-
swallowing incoordination
Esophageal manometry: Multiple
disturbances in function
Interpretation: Dysfunction in motor
organization of tongue, pharynx, &
esophagus
Baudon et al., 2002
Growth Data
Deficient growth in PRS (Laitinen et al, 1994)
Premature birth
Associated anomalies
PRS with hypercaloric diet (Marques et al, 2004)
Improved nutrition status
Improved respiratory conditions (shorter
nasopharyngeal intubation)
Management of Airway for PRS
Nasopharyngeal Airway (NPA)
Mandibular Distraction Osteogenesis (MDO)
External distractors
Internal distractors
Tongue-Lip Adhesion (TLA)
Periosteal release
Tracheostomy
Swallowing After MDO
Success relates to airway status, neurologic
status, & other medical/health variables
Timing of surgery (neonatal period vs
several months of age)
Gastrostomy tube for those who need
supplements for > 30 days
Prognosis: good for long term PO
PR Swallowing Disorders with MDO
18 patients, before MDO & 4 months after
Bilateral corticotomies, followed by distraction
External devices
Achieved 7 to 19 mm elongation (mean=12 mm)
GER in 83% associated with apnea episodes
Disappeared after MDO
Oxygen saturation 72% pre-op, 93% after
Apnea & hypopnea disappeared after distraction
Monasterio et al. 2004
PRS: Emphases in Treatment
Positioning
Feeding alterations
Intraoral prostheses not helpful
Thickened liquid be careful
If non-oral feeding for nutrition
Oral tastes as tolerated & safe
Not likely to be via nipple
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Vocal Fold Paralysis/Paresis
Vocal Fold Paralysis
Second most common congenital
abnormality of pediatric airway
VF in midline (adducted) in most cases
Good voice or cry, poor airway
Surgical goal: improve airway, while
maintaining normal voice & intact swallow
Vocal Fold Paralysis: Etiology
Bilateral abducted
Idiopathic or iatarogenic
Small subset: autosomal dominant
linked to chromosome 6q16.
Unilateral:
S/p surgical procedures that may injure
recurrent laryngeal nerve
S/p traumatic event
Unilateral VF Paralysis: Management
May be asymptomatic or with signs related
to laryngeal incompetence, e.g., aspiration
& dysphonia
High % recover spontaneously
Collagen injection risk of aspiration &
improves vocal quality may avoid
tracheostomy or GT
Patel, Kerschner, & Merati (2003).
Vocal Fold Paralysis: SLP Role
Voice evaluation
Breathy, dysphonic, excessive effort
FFNL for visual inspection
Feeding/swallowing evaluation
Instrumental examination
FEES
VFSS
Laryngeal Cleft
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Laryngeal Cleft: Epidemiology
Rare midline defect of posterior larynx &
membranous trachea
Usually present in newborn period
Incidence: 1/10,000 to 20,000 live births
No consistent pattern of inheritance
Typically present with airway obstruction
due to extra mucosa
Laryngeal Cleft: Basic Types
Type I: Above level of vocal folds involves
failure of interarytenoid muscle development
Type II: Extends into upper cricoid
cartilaginous ring remains intact
Type III: Entire cricoid cartilage with or
without extension into cervical trachea
Type IV: Extensive affecting
laryngotracheoesophageal structures.
Laryngeal Cleft: Diagnosis
Vital factors for diagnosis
High index of suspicion during clinical
exam or by history
Direct endoscopic exam under anesthesia
with specific palpation of arytenoid
cartilage & interarytenoid space
Laryngeal Cleft: Clinical Signs
Coughing or choking
Cyanosis
Tachypnea
Recurrent pneumonia
Respiratory infection
Respiratory distress with oral feeding
Clinical Signs in Infants
GER may accompany & sometimes
adds confusion for diagnosis
Abnormal cry or stridor similar to
laryngomalacia
Laryngeal Cleft
in Some Genetic Syndromes
Opitz (Autosomal dominant)
Hypertelorism, hernias, cardiac anomalies,
Laryngotracheal malformations + others
Pallister Hall (Single-gene malformation)
Bifid epiglottis
Central polydactyly (extra digits)
Hypothalamic hamartoma (benign tumor like
nodule
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VFSS Aid to Diagnosis
Suspicious, but not definitive
Pattern of aspiration not accounted for by
other oral or pharyngeal phase problems
Penetration appears between arytenoids
Definitive diagnosis
Direct laryngoscopy & bronchoscopy
Laryngeal Cleft: Management
Type I: may not need intervention
Injection (Cymetra) for some infants
Other types: Surgical correction
G-tube for feeding
Reconstruction after 1 year of age
Sooner if aspiration pneumonia with GT
feeds
Laryngeal Cleft Early Management
Usually NPO pre-op
Op: laryngotomy anterior approach
through thyroid cartilage
Expose area of cleft
Excise redundant mucosa
Two-to-three layer closure of cleft
Re-approximate anterior larynx
SLP Intervention
Non-oral stimulation to facilitate interest
in tastes & minimize defensiveness
Oral sensory issues
Oral motor skill development
Transition tube to oral feeding
Diet modifications, e.g., thickened liquid
may help short time with cautions!!
Postural changes
Laryngeal Cleft: Outcomes
Total PO variable time s/p surgery; some
in 6 months
Possible complications
Fistula along suture line may need
surgery
Long-term tracheostomy that may affect
voice & speech output (speaking valve
helps some)
Subglottic Stenosis
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Subglottic Stenosis
Management of severe subglottic stenosis
Laryngotracheal reconstruction
Cricotracheal resection
Posterior costal cartilage graft (with or
without vascular pedicle) usually preferred
over anterior approaches
Tracheostomy & Ventilator Issues
Related to Swallowing
Tracheostomy: Indications
Craniofacial abnormalities (13%)
Upper airway obstruction (19%)
Prolonged intubation(26%)
Neurologic impairment (27%)
Trauma (7%)
Vocal fold paralysis (7%)
Carron et al., 2000
Tracheostomy: Inflated Cuffs
Prefer no cuff in pediatrics, if possible
Breathing in & out through tube only
No airflow through upper airway
Lack of phonation (no voicing)
Decreased sense of smell/taste
Risk of tissue necrosis
Esophageal impingement may cause GER
Tracheostomy: - Aspiration Risk
Cuff of trach tube can tether larynx
Not elevate, epiglottis not tilt down
Lack of airflow through upper airway
Lack sensation in oropharynx
Lack sensation of pooled secretions
Vocal folds remain in open position
Tracheostomy: - Aspiration Risk
Lack of subglottic pressure
swallow efficiency
effectiveness of cough resulting in more
frequent suction
physiologic PEEP (positive end-expiratory
pressure)
gas exchange due to surface area of alveoli
oxygenation
Atelectasis possible
Joan C. Arvedson, Ph.D.
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Tracheostomy: Speaking Valves
Normalizes pharyngeal, glottic
& subglottic pressures
May facilitate
Improved pharyngeal sensation
Humidification, taste, & smell
Tracheostomy: Open Position Valve
Person must exhale to close the diaphragm
of a speaking valve
Secretions travel up the tube & may
occlude the valve
Used for communication only
Patient Criteria for Use
Medically stable
Awake, responsive
Toleration of cuff deflation (partial?)
Gross aspiration/ventilator may prevent
Must be able to exhale sufficiently past
tracheostomy tube
Upper airway must be patent
Contraindications for Valve Use
Tube size that fills the trachea
Degree of stenosis &/or granulation tissue
(consideration of inhalation vs exhalation)
Foam-filled cuff
Edema
Secretions
Physiologic Benefits of P-M Valves
Improved voice production
Improved sense of smell/taste
Prevent aspiration (deflated cuff allows for
- laryngeal elevation/excursion
Vocal fold closure airflow moves over
baroreceptors
Improved sensation of pooled secretions
Trial Use of Speaking Valve
Baseline measurements of oxygenation,
vital signs, breath sounds, color, work of
breathing, patient responsiveness
Assess upper airway patency
Deflate cuff (variable MD recommendations)
Vocalize on exhalation
Cough
Joan C. Arvedson, Ph.D.
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Treatment Strategies to Facilitate
Vocal Fold Function
Older child who can follow directions
Hard glottal attack exercises
Vocal fold exercises to strengthen vfs
Verbal communication
Swallow evaluation & treatment
PT & OT exercises for overall strengthening
Tracheostomy: Feeding Problems
Restricted movement
Discomfort from tube
Incomplete glottic closure
Structural abnormalities of larynx
Reduced cough effectiveness
Feeding with Tracheostomy
Restricted laryngeal elevation
Infants less difficulty than older children
Blue dye testing why not?
Reason for tracheostomy may be factor
Degree of difficulty
Type of difficulty
Swallow Gains with Valve
Cuff must be deflated to aid laryngeal
elevation (Unless cuff not tight?)
Vocal folds closed
Restored airflow = improved sensation
Restored subglottic pressure
Improved secretion management (cough
improved, suction less, reduced risk of
tracheal damage)
Valve Aids Tracheostomy Weaning
Physiologic benefits stated previously aid
in setting stage for weaning
Uses expiratory muscles
Restores normal physiology
Decannulation may occur sooner than
with no valve
Tracheostomy: Outcomes
Time to decannulation longer with
neurologic impairment & prolonged
intubation
Mortality 19% overall
(3.6% tracheostomy related)
Deaths usually related to underlying
disease, not tracheostomy itself
Carron et al., 2000
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Ventilator Management in NICU
Neurally adjusted ventilatory assist (NAVA)
FDA approved therapy
Practical experience & data in this area
lacking
Carefully prepared & organized plan is
essential for successful implementation
NAVA: How it works
Allows synchronization of spontaneous
respiratory effort with mechanical
ventilation
Electrodes embedded within NG catheter
detect electrical activity of diaphragm &
transmit information to ventilator
Ventilator breath is triggered & terminated
by changes in this electrical activity
NAVA: How it works (cont)
Ventilator determines inspiratory pressure
in proportion to this electrical signal
Thus, patient determines respiratory rate,
tidal volume, peak inspiratory pressure,
mean airway pressure, & inspiratory &
expiratory times
Valve Placement with Ventilator
Record ventilator settings (mode, rate, tidal
volume [VT], F1O2, PEEP, peak inspiratory
pressure [PIP], alarms)
Deflate cuff gradually
Monitor PIP for possible changes to VT
Ventilator Adjustments
Alarms (volume & pressure)
Compensate for loss of airflow
through vocal folds if necessary
PEEP on/off
Pressure vs flow trigger
Pressure support/pressure control
Ventilator Adjustments
PEEP
Pressure support
Humidification
Transition & troubleshooting
Anxiety & depression
Airway patency
Breathing pattern changes
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Valve Removal with Ventilator
Replace original circuit set-up
Return ventilator settings & alarms
to pre-valve parameters
Re-inflate cuff if that was baseline
condition
SUMMARY
Stable airway is a prerequisite for safe
oral feeding
Pulmonary deficits may cause or
exacerbate dysphagia
Dysphagia may cause or exacerbate
pulmonary deficits/compromise
Professionals must understand airway
implications to facilitate oral feeding