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Transcript
Paradoxical Vocal Fold
Motion
Goals
Background


history
pathophysiology
Diagnostic approach


differential
work-up
Treatment
PVFM: Semantics
Munchausen’s stridor
pseudoasthma
paradoxical movement of the vocal cords
functional inspiratory stridor
functional upper airway obstruction
factitious asthma
paradoxical vocal fold motion
emotional wheezing
psychogenic stridor
spasmodic croup
vocal cord dysfunction
functional laryngospasm
psychogenic upper airway obstruction
episodic laryngeal dyskinesia
nonorganic stridor
hysterical stridor
laryngospasm of emotional origin
functional upper airway obstruction
functional stridor
laryngeal asthma
1974
1976
1978
1980
1981
1982
1982
1983
1983
1983
1983
1985
1986
1986
1987
1989
1990
1990
1992
1995
Vocal Cord Dysfunction
Christopher KL et al.
NEJM 1983; 308: 1566-70
in a recent editorial, he stated:
Chest 2006; 129: 842-43
PVFM: History
Osler 1905
“spasm of the muscles may occur with violent inspiratory efforts
and great distress, and may even lead to cyanosis”; “remarkable
inspiratory cry, somewhat like the whoop of whooping-cough, but so
intense it could be heard at long distance”
Patterson 1974
Munchausen’s Stridor: non-organic laryngeal obstruction
Newman 1995
largest retrospective series (n=95)
PVFM: Gnostics
Diverse literature including:










otolaryngology
pulmonology
allergy / immunology
pediatrics
psychiatry / psychology
speech therapy
physical therapy
military medicine
sports medicine
anesthesia
Why?

relatively rare entity with fleeting symptoms, mimics other dzs

heterogeneous disorder
PVFM: Definition
Symptomatic, inspiratory, mobile VF
adduction in the absence of specific VF
pathology
Traditionally defined by symptomatology
and exam findings rather than etiology
Manifestation of a variety of entities


organic / non-organic
associated / isolated
PVFM: The Issue
Misdiagnosed, and inappropriately treated
patients



high-dose corticosteroids, methotrexate
sedatives, anxiolytics
intubation, tracheostomy
Unrecognized underlying pathology


neurologic disease
psychiatric disease
PVFM: Normal Physiology
Laryngeal Function



airway protection
regulation of airflow
phonation
Laryngeal Regulation


voluntary: cortical centers (CNS)
reflex arcs
supraglottic, distal airway,
esophageal sensors
medulla (CNS) via Vagus n. and
branches
PVFM: Normal Physiology
active inspiratory ABduction


reduces glottic resistance
centrally coordinated with diaphragm
passive expiratory ADduction


relaxation of Abductors (PCA)
active in obstructive pulmonary disease (auto PEEP?)
experimentally produced neg pressure across
the glottis


awake: ABductors counteract Bernoulli effect
anesthetized: glottic and supraglottic collapse (50%)
PVFM: Pathophysiology
Glottic closure reflex

e.g. aspiration, toxic inhalation, dive reflex
Hypersensitive reflex in PVFM?
Lowered threshold for initiation?

supported by reports of irritant exposure
histologic changes differ from those of asthma



glottic exposure to refluxate
preceding URI
strong functional component
PVFM: Pathophysiology
proposed mechanisms
Laryngeal hyper-responsiveness

Bucca et al. Lancet. 1995;346:791-95
Altered autonomic balance

Ayres, Gabbott. Thorax. 2002;57:284-5
Stimulation of upper and/or distal airways
resulting reflex closure

Balkissoon. Clin Chest Med. 2002;23:717-25
Hyperventilation syndrome

Parker, Berg. Chest. 2002;122(suppl):185-6
Evaluation
“it’s the history, stupid”
HPI






pattern of attacks
character of stridor
precipitating factors
associated symptoms
medications
bronchodilator relief
PMH






CHI
neurologic dz (CVA)
heartburn
psychiatric hx
perinatal hx
occupational exposure
PVFM: Clinical Presentation
dyspnea a/w inspiratory wheezing / stridor


focused over larynx
“easier to get air out than in”
cough: chronic, paroxysmal
chest pain
dysphonia, aphonia
choking, dysphagia
post-exertion?
PVFM: Clinical Presentation
Character of Stridor
dysphonia
airway
intervention
pattern
duration
reflux
laryngospasm paroxysmal
seconds –
minutes
usually
rarely
dystonias
daytime
hours
rarely
sometimes
functional
paroxysmal
variable
never
rarely
CNS
continuous
continuous
sometimes
usually
Evaluation
during the acute episode
Physical Exam

Laryngoscopy, flexible
vegetative tasks


Auscultation to localize stridor /
wheezing
Identify the etiology of PVCM:
neurologic exam
Lab



Spirometry, PFT – truncated
insp limb
CXR – nl
ABG – nl
Evaluation
for the asymptomatic patient
Physical exam

Resting Laryngoscopy – typically nl
Treole (1999) noted motion abnormalities in 50/50
Patel (2003) found edema, anatomic and neuro
minimize topical anesthetic



Provocative laryngoscopy
Post-exercise laryngoscopy
Provocative agents
Lab



Spirometry – may have truncated insp limb (23%)
Bronchoprovocative spirometry / PFT
CXR, ABG – generally not useful
hypoxemia, hypercapnea both reported in PVFM

eosinophilia, abnl sinus radiographs in asthma
Differential Diagnoses
to consider with PVFM
Spontaneous
Exercise-associated
Differential Diagnoses
to consider with PVFM
Organic







Brainstem compression
Cortical/UMN injury
Nuclear/LMN injury
Laryngopharyngeal
reflux
Movement disorders
(dystonia, dyskinesia)
Asthma-associated
Irritant-induced
Non-Organic


Conversion or
Somatization d/o
Malingering or
Factitious d/o
PVFM
Spontaneous
asthma-associated
NEURO DZ
LPR
Brainstem
compression
Cortical/UMN
injury
Nuclear/LMN
injury
Movement
disorders
ExerciseAssociated
PSYCH
Conversion/ Somatization
Malingering/Factitious
irritant-induced
Some Differential Diagnoses
to consider without PVFM
Laryngospasm
Pseudo PVFM

BVFP, CA joint abnormalities, interarytenoid web, hereditary
ABD paralysis
Upper airway obstruction


obst tracheal, bronchial, laryngeal lesion or FB
tracheal / subglottic stenosis
Obstructive pulmonary disease
Pulmonary Function Testing in PVFM
Spirometry


Exp / Insp flow ratio >1 (at 50% VC) c/w variable
extrathoracic airway obstruction
produces “truncated inspiratory loop”
for asymp pts: 23% PVFM, 13% with both, 2% asthma alone
role of provocation

what exactly did you provoke?
PFVM vs distal airway obstruction

what do you evaluate?
Flow volume loop, laryngeal exam
options
histamine
methacholine
isocapnic hyper
allergen
exercise
TOTAL
0/2
12/34
1/3
3/4
24/46
40/89
0%
35%
33%
75%
54%
44%
Laryngoscopy is the gold standard; provoke the sx and scope
Jamilla et al. Clin Pulm Med 2000
Christenson KL. Chest 2006; 129: 842-43
But goes on to say: “Truncation of inspiratory limb on FVL is extremely helpful
in suggesting the diagnosis of VCD in symptomatic patients”
Morris et al. Clin Pulm Med 2006;13:73-86
Morris et al in 105 AD with exertional dyspnea referred to pulmonary (2002)


Looked at a series of screening tests; ABG, CXR, lung volume, DLCO, EKG,
echocardiography, which were all “not useful” without specific indications
If remained undiagnosed after MTC, laryngoscopy had higher yield than these
PVFM: Differential, Organic
Brainstem Compression
Epidemiology


young kids
suggestive PMH
Pathophysiology

compression of nuc.
ambiguus (vagal nuc)
Associated findings




VPI
dysphagia
GER from LES incomp
no central apnea
Work-up

CNS imaging
Airway intervention

intubation / trach
Treatment

surgical
decompression
PVFM: Differential, Organic
Severe Cortical / UMN Injury
Epidemiology



adults with CVA
kids with static
encephalopathy
CHI
Pathophys - variable
Assoc findings (SE)





a/w upper airway obs
global delay
hypertonia
spastic diplegia
sialorrhea
Work-up

CNS imaging
Airway intervention


CPAP
trach
Treatment


surgery for obstruction
GER
PVFM: Differential, Organic
Nuclear or LMN Injury
Epidemiology

adults with
ALS
myesthenia gravis
medullary infarct
Pathophysiology

abnormality of vagal
nuclei, or RLN (without
brainstem comp)
Associated symptoms


dysphagia
hypophonia
Work-up


CNS imaging
Neurology eval
Airway intervention

variable
Treatment


supportive
myesthenic rx
PVFM: Differential, Organic
Movement Disorders
Epidemiology


Adductor laryngeal
breathing dystonia
Laryngeal dyskinesia
Parkinsonism (1,2)
Parkinsonism “Plus”




progressive SN palsy
Shy-Drager synd
Myoclonus
Drug-induced
laryngeal dystonia
Associated findings
Work-up

neurologic eval
Airway intervention

variable
Treatment


ALBD: BOTOX, surg
Parkinson’s:
1:L-dopa
2: BOTOX

Myoclonus: rx
PVFM: Differential, Organic
Laryngopharyngeal Reflux
Epidemiology


associated with PVFM
pediatric
Pathophysiology


microaspiration
esophago-laryngeal
reflex?
Associated findings



dysphonia, globus,
dysphagia, cough
heartburn in only 1/3
laryngospasm
Work-up

dual sensor pH probe
Airway intervention

not required
Treatment


BID-QID PPI
Nissen fundoplication
PVFM: Differential, Organic
Asthma Associated
Epidemiology


a/w asthma
unrecognized
Pathophysiology

vagal reflex
dysfunction?
Work-up

PFT
Airway intervention

variable
Treatment

for asthma
Assoc sx / findings vs
asthma controls
Newman et al 1995: 95 pts, 56% with VCD and Asthma
O’Connell et al 1995: 20 pts, 35% with VCD and Asthma
PVFM: Differential, Organic
Irritant Induced
Epidemiology

occupational exposure
Pathophysiology
Associated findings



chest pain
similar hx in
malingering
decreased coincidence of psych d/o
Work-up

irritant/allergen
bronchoprovocation
Airway intervention

not required
Treatment

identify and remove
irritant
PVFM: Differential, Non-organic
Conversion / Somatization Disorder
Epidemiology



young female athlete
female health care
worker
psychologic stressors
Pathophysiology

unintentional,
unconscious
Associated findings

well-motivated,
compliant with tx
Work-up


dx of exclusion
psych eval
Airway intervention

not required
Treatment

to follow
PVFM: Differential, Non-organic
Factitious / Malingering Disorder
Epidemiology


50-73% psych hx
18% prior factitious d/o
Pathophysiology


Factitious: “sick role”
Malingering: sec gain
Associated findings

Malingering
poorly cooperative
inciting event
assoc axis II d/o
Work-up


dx of exclusion
psych eval
Airway intervention

not required
Treatment

to follow
PCFM: Management
Short-term Strategies for Most
Breathing techniques




sniffing, panting
humming
diaphragmatic
talking
Distraction
Reassurance
Sedation


benzodiazepams
propofol
Airway adjuvants




Heliox
CPAP / IPPV / BiPAP
intubation
tracheostomy
PCFM: Management
Long-term Strategies are “multidisciplinary”
Speech therapy



relaxation techniques
breathing retraining
biofeedback (video)
Psychotherapy



identifying stressors
biofeedback
hypnosis
Pharmacologic



dx specific, e.g.
ipratropium in
exercise-associated
BOTOX
PPI
Surgical

tracheostomy
PCFM: Management
Long-term Strategies for Non-organic
PVFM: Prognosis
Distinguished exercise-induced vs spontaneous
Contacted 28/49 pts
Median time until resolution


Exercise-associated: 4 mos
Spontaneous: 5 mos
and ranged from 1 wk to 5 years, irrespective of
intervention
PVFM: Summary
Product of diverse etiologies
Prompt and accurate diagnosis facilitates
appropriate management


hx / laryngoscopy / provocation are keys to dx
multidisciplinary therapy essential in nonorganic cases
Prognosis varies with cause
Get the pulmonologists and neurologists
involved early, they’re really smart