paradoxical vocal fold motion. goals background history history pathophysiology pathophysiology...

Paradoxical Vocal Fold Paradoxical Vocal Fold MotionMotion

GoalsGoalsBackgroundBackground historyhistory pathophysiologypathophysiology

Diagnostic approachDiagnostic approach differentialdifferential work-upwork-up

TreatmentTreatment

PVFM: SemanticsPVFM: SemanticsMunchausen’s stridorMunchausen’s stridor 19741974

pseudoasthmapseudoasthma 19761976

paradoxical movement of the vocal cordsparadoxical movement of the vocal cords 19781978

functional inspiratory stridorfunctional inspiratory stridor19801980

functional upper airway obstructionfunctional upper airway obstruction 19811981

factitious asthmafactitious asthma 19821982

paradoxical vocal fold motionparadoxical vocal fold motion 19821982

emotional wheezingemotional wheezing 19831983

psychogenic stridorpsychogenic stridor 19831983

spasmodic croupspasmodic croup 1983 1983

vocal cord dysfunctionvocal cord dysfunction 19831983

functional laryngospasmfunctional laryngospasm 19851985

psychogenic upper airway obstructionpsychogenic upper airway obstruction 19861986

episodic laryngeal dyskinesiaepisodic laryngeal dyskinesia 19861986

nonorganic stridornonorganic stridor 19871987

hysterical stridorhysterical stridor 19891989

laryngospasm of emotional originlaryngospasm of emotional origin 19901990

functional upper airway obstructionfunctional upper airway obstruction 19901990

functional stridorfunctional stridor 19921992

laryngeal asthmalaryngeal asthma 19951995

Vocal Cord DysfunctionVocal Cord Dysfunction

Christopher KL et al. Christopher KL et al. NEJM 1983; 308: 1566-70NEJM 1983; 308: 1566-70

in a recent editorial, he stated:in a recent editorial, he stated:

Chest 2006; 129: 842-43

PVFM: HistoryPVFM: History

Osler 1905Osler 1905“spasm of the muscles may occur with violent inspiratory “spasm of the muscles may occur with violent inspiratory

efforts and great distress, and may even lead to cyanosis”; efforts and great distress, and may even lead to cyanosis”; “remarkable inspiratory cry, somewhat like the whoop of whooping-“remarkable inspiratory cry, somewhat like the whoop of whooping-cough, but so intense it could be heard at long distance”cough, but so intense it could be heard at long distance”

Patterson 1974Patterson 1974Munchausen’s Stridor: non-organic laryngeal obstructionMunchausen’s Stridor: non-organic laryngeal obstruction

Newman 1995Newman 1995largest retrospective series (n=95)largest retrospective series (n=95)

PVFM: GnosticsPVFM: Gnostics

Diverse literature including:Diverse literature including: otolaryngologyotolaryngology pulmonologypulmonology allergy / immunology allergy / immunology pediatricspediatrics psychiatry / psychologypsychiatry / psychology speech therapy speech therapy physical therapyphysical therapy military medicine military medicine sports medicinesports medicine anesthesiaanesthesia

Why?Why? relatively rare entity with fleeting symptoms, mimics other dzsrelatively rare entity with fleeting symptoms, mimics other dzs heterogeneous disorderheterogeneous disorder

PVFM: DefinitionPVFM: Definition

Symptomatic, inspiratory, mobile VF Symptomatic, inspiratory, mobile VF adduction in the absence of specific VF adduction in the absence of specific VF pathologypathology

Traditionally defined by symptomatology Traditionally defined by symptomatology and exam findings rather than etiologyand exam findings rather than etiology

Manifestation of a variety of entities Manifestation of a variety of entities organic / non-organicorganic / non-organic associated / isolatedassociated / isolated

PVFM: The IssuePVFM: The Issue

Misdiagnosed, and inappropriately treated Misdiagnosed, and inappropriately treated patientspatients high-dose corticosteroids, methotrexatehigh-dose corticosteroids, methotrexate sedatives, anxiolyticssedatives, anxiolytics intubation, tracheostomyintubation, tracheostomy

Unrecognized underlying pathologyUnrecognized underlying pathology neurologic diseaseneurologic disease psychiatric diseasepsychiatric disease

PVFM: Normal PhysiologyPVFM: Normal Physiology

Laryngeal FunctionLaryngeal Function airway protectionairway protection regulation of airflowregulation of airflow phonationphonation

Laryngeal RegulationLaryngeal Regulation voluntary: cortical centers (CNS)voluntary: cortical centers (CNS) reflex arcs reflex arcs

supraglottic, distal airway, supraglottic, distal airway, esophageal sensorsesophageal sensors

medulla (CNS) via Vagus n. and medulla (CNS) via Vagus n. and branchesbranches

PVFM: Normal PhysiologyPVFM: Normal Physiology

active inspiratory ABductionactive inspiratory ABduction reduces glottic resistancereduces glottic resistance centrally coordinated with diaphragmcentrally coordinated with diaphragm

passive expiratory ADductionpassive expiratory ADduction relaxation of Abductors (PCA)relaxation of Abductors (PCA) activeactive in obstructive pulmonary disease (auto PEEP?) in obstructive pulmonary disease (auto PEEP?)

experimentally produced neg pressure across experimentally produced neg pressure across the glottisthe glottis awake: ABductors counteract Bernoulli effectawake: ABductors counteract Bernoulli effect anesthetized: glottic and supraglottic collapse (50%)anesthetized: glottic and supraglottic collapse (50%)

PVFM: PathophysiologyPVFM: Pathophysiology

Glottic closure reflexGlottic closure reflex e.g. aspiration, toxic inhalation, dive reflexe.g. aspiration, toxic inhalation, dive reflex

Hypersensitive reflex in PVFM? Hypersensitive reflex in PVFM? Lowered threshold for initiation?Lowered threshold for initiation? supported by reports of irritant exposuresupported by reports of irritant exposure

histologic changes differ from those of asthmahistologic changes differ from those of asthma glottic exposure to refluxateglottic exposure to refluxate preceding URIpreceding URI strong functional componentstrong functional component

PVFM: PathophysiologyPVFM: Pathophysiologyproposed mechanismsproposed mechanisms

Laryngeal hyper-responsivenessLaryngeal hyper-responsiveness Bucca et al. Bucca et al. Lancet.Lancet. 1995;346:791-95 1995;346:791-95

Altered autonomic balanceAltered autonomic balance Ayres, Gabbott. Ayres, Gabbott. Thorax.Thorax. 2002;57:284-5 2002;57:284-5

Stimulation of upper and/or distal airways Stimulation of upper and/or distal airways resulting reflex closureresulting reflex closure

Balkissoon. Balkissoon. Clin Chest MedClin Chest Med. 2002;23:717-25. 2002;23:717-25

Hyperventilation syndromeHyperventilation syndrome Parker, Berg. Parker, Berg. ChestChest. 2002;122(suppl):185-6. 2002;122(suppl):185-6

EvaluationEvaluation“it’s the history, stupid”“it’s the history, stupid”

HPIHPI pattern of attackspattern of attacks character of stridorcharacter of stridor precipitating factorsprecipitating factors associated symptomsassociated symptoms medications medications bronchodilator reliefbronchodilator relief

PMHPMH CHI CHI neurologic dz (CVA)neurologic dz (CVA) heartburnheartburn psychiatric hxpsychiatric hx perinatal hxperinatal hx occupational exposureoccupational exposure

PVFM: Clinical PresentationPVFM: Clinical Presentation

dyspnea a/w inspiratory wheezing / stridordyspnea a/w inspiratory wheezing / stridor focused over larynxfocused over larynx ““easier to get air out than in”easier to get air out than in”

cough: chronic, paroxysmalcough: chronic, paroxysmal

chest painchest pain

dysphonia, aphoniadysphonia, aphonia

choking, dysphagiachoking, dysphagia

post-exertion?post-exertion?

PVFM: Clinical PresentationPVFM: Clinical PresentationCharacter of StridorCharacter of Stridor

paroxysmalparoxysmal seconds –seconds –minutesminutes usuallyusually rarelyrarely

daytimedaytime hourshours rarelyrarely sometimessometimes

paroxysmalparoxysmal variablevariable nevernever rarelyrarely

continuouscontinuous continuouscontinuous sometimessometimes usuallyusually

pattern duration dysphoniaairway

intervention

reflux

laryngospasm

dystonias

functional

CNS

EvaluationEvaluationduring the during the acute episodeacute episode

Physical ExamPhysical Exam Laryngoscopy, flexibleLaryngoscopy, flexible

vegetative tasksvegetative tasks Auscultation to localize stridor / Auscultation to localize stridor /

wheezingwheezing Identify the etiology of PVCM:Identify the etiology of PVCM:

neurologic examneurologic exam

Lab Lab Spirometry, PFT – truncated Spirometry, PFT – truncated

insp limbinsp limb CXR – nlCXR – nl ABG – nlABG – nl

EvaluationEvaluationfor the for the asymptomaticasymptomatic patient patient

Physical examPhysical exam Resting Laryngoscopy – typically nlResting Laryngoscopy – typically nl

Treole (1999) noted motion abnormalities in 50/50Treole (1999) noted motion abnormalities in 50/50Patel (2003) found edema, anatomic and neuroPatel (2003) found edema, anatomic and neurominimize topical anestheticminimize topical anesthetic

Provocative laryngoscopyProvocative laryngoscopy Post-exercise laryngoscopyPost-exercise laryngoscopy Provocative agentsProvocative agents

LabLab Spirometry – may have truncated insp limb (23%)Spirometry – may have truncated insp limb (23%) Bronchoprovocative spirometry / PFTBronchoprovocative spirometry / PFT CXR, ABG – generally not usefulCXR, ABG – generally not useful

hypoxemia, hypercapnea both reported in PVFMhypoxemia, hypercapnea both reported in PVFM eosinophilia, abnl sinus radiographs in asthmaeosinophilia, abnl sinus radiographs in asthma

Differential DiagnosesDifferential Diagnosesto considerto consider withwith PVFM PVFM

SpontaneousSpontaneous Exercise-associatedExercise-associated

Differential DiagnosesDifferential Diagnosesto considerto consider withwith PVFM PVFM

OrganicOrganic Brainstem compressionBrainstem compression Cortical/UMN injuryCortical/UMN injury Nuclear/LMN injuryNuclear/LMN injury Laryngopharyngeal Laryngopharyngeal

refluxreflux Movement disordersMovement disorders

(dystonia, dyskinesia)(dystonia, dyskinesia) Asthma-associatedAsthma-associated Irritant-inducedIrritant-induced

Non-OrganicNon-Organic Conversion or Conversion or

Somatization d/oSomatization d/o Malingering or Malingering or

Factitious d/oFactitious d/o

Spontaneous

Exercise-

Associated

NEURO DZBrainstem compressionCortical/UMN injuryNuclear/LMN injuryMovement disorders

asthma-associated

LPR

irritant-induced

PSYCH

Conversion/ Somatization

Malingering/Factitious

PVFMPVFM

Some Differential DiagnosesSome Differential Diagnosesto considerto consider withoutwithout PVFM PVFM

LaryngospasmLaryngospasm

Pseudo Pseudo PVFMPVFM BVFP, CA joint abnormalities, interarytenoid web, hereditary BVFP, CA joint abnormalities, interarytenoid web, hereditary

ABD paralysisABD paralysis

Upper airway obstructionUpper airway obstruction obst tracheal, bronchial, laryngeal lesion or FBobst tracheal, bronchial, laryngeal lesion or FB tracheal / subglottic stenosistracheal / subglottic stenosis

Obstructive pulmonary diseaseObstructive pulmonary disease

Pulmonary Function Testing in PVFMPulmonary Function Testing in PVFM

SpirometrySpirometry Exp / Insp flow ratio >1 (at 50% VC) c/w variable Exp / Insp flow ratio >1 (at 50% VC) c/w variable

extrathoracic airway obstructionextrathoracic airway obstruction produces “truncated inspiratory loop”produces “truncated inspiratory loop”

for for asympasymp pts: 23% PVFM, 13% with both, 2% asthma alone pts: 23% PVFM, 13% with both, 2% asthma alone

role of provocationrole of provocation what exactly did you provoke? what exactly did you provoke?

PFVM vs distal airway obstructionPFVM vs distal airway obstruction what do you evaluate?what do you evaluate?

Flow volume loop, laryngeal examFlow volume loop, laryngeal exam

optionsoptionshistaminehistamine 0/20/2 0%0%methacholinemethacholine 12/3412/34 35%35%isocapnic hyperisocapnic hyper 1/3 1/3 33% 33% allergenallergen 3/43/4 75%75%exerciseexercise 24/4624/46 54%54%

TOTALTOTAL 40/8940/89 44%44%

Laryngoscopy is the gold standard; provoke the sx and scopeLaryngoscopy is the gold standard; provoke the sx and scope

Jamilla et al. Clin Pulm Med 2000

Christenson KL. Chest 2006; 129: 842-43

Morris et al. Clin Pulm Med 2006;13:73-86

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 in 105 AD with exertional dyspnea referred to pulmonary (2002)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, Looked at a series of screening tests; ABG, CXR, lung volume, DLCO, EKG,

echocardiography, which were all “not useful” without specific indicationsechocardiography, which were all “not useful” without specific indications If remained undiagnosed after MTC, laryngoscopy had higher yield than theseIf remained undiagnosed after MTC, laryngoscopy had higher yield than these

PVFM: Differential, OrganicPVFM: Differential, Organic

Brainstem CompressionBrainstem Compression EpidemiologyEpidemiology young kidsyoung kids suggestive PMHsuggestive PMH

PathophysiologyPathophysiology compression of nuc. compression of nuc.

ambiguus (vagal nuc)ambiguus (vagal nuc)

Associated findingsAssociated findings VPIVPI dysphagiadysphagia GER from LES incompGER from LES incomp no central apneano central apnea

Work-upWork-up CNS imagingCNS imaging

Airway interventionAirway intervention intubation / trach intubation / trach

TreatmentTreatment surgical surgical

decompressiondecompression


Severe Cortical / UMN InjurySevere Cortical / UMN Injury EpidemiologyEpidemiology adults with CVAadults with CVA kids with static kids with static

encephalopathyencephalopathy CHICHI

Pathophys - variablePathophys - variable

Assoc findings (SE)Assoc findings (SE) a/w upper airway obs a/w upper airway obs global delayglobal delay hypertoniahypertonia spastic diplegiaspastic diplegia sialorrheasialorrhea

Work-upWork-up CNS imagingCNS imaging

Airway interventionAirway intervention CPAPCPAP trach trach

TreatmentTreatment surgery for obstructionsurgery for obstruction GERGER


Nuclear or LMN InjuryNuclear or LMN Injury EpidemiologyEpidemiology adults withadults with

ALSALS

myesthenia gravismyesthenia gravis

medullary infarctmedullary infarct

PathophysiologyPathophysiology abnormality of vagal abnormality of vagal

nuclei, or RLN (without nuclei, or RLN (without brainstem comp)brainstem comp)

Associated symptomsAssociated symptoms dysphagiadysphagia hypophoniahypophonia

Work-upWork-up CNS imagingCNS imaging Neurology evalNeurology eval

Airway interventionAirway intervention variablevariable

TreatmentTreatment supportivesupportive myesthenic rxmyesthenic rx


Movement DisordersMovement Disorders EpidemiologyEpidemiology Adductor laryngeal Adductor laryngeal

breathing dystoniabreathing dystonia Laryngeal dyskinesiaLaryngeal dyskinesia

Parkinsonism (1,2)Parkinsonism (1,2)

Parkinsonism “Plus”Parkinsonism “Plus” progressive SN palsyprogressive SN palsy Shy-Drager syndShy-Drager synd

MyoclonusMyoclonus Drug-induced laryngeal Drug-induced laryngeal

dystoniadystonia

Associated findingsAssociated findings

Work-upWork-up neurologic evalneurologic eval

Airway interventionAirway intervention variable variable

TreatmentTreatment ALBD: BOTOX, surgALBD: BOTOX, surg Parkinson’s: Parkinson’s:

1:L-dopa1:L-dopa

2: BOTOX2: BOTOX Myoclonus: rxMyoclonus: rx


Laryngopharyngeal RefluxLaryngopharyngeal Reflux EpidemiologyEpidemiology associatedassociated with PVFM with PVFM pediatricpediatric

PathophysiologyPathophysiology microaspiration microaspiration esophago-laryngeal reflex?esophago-laryngeal reflex?

Associated findingsAssociated findings dysphonia, globus, dysphonia, globus,

dysphagia, coughdysphagia, cough heartburn in only 1/3heartburn in only 1/3 laryngospasmlaryngospasm

Work-upWork-up dual sensor pH probedual sensor pH probe

Airway interventionAirway intervention not required not required

TreatmentTreatment BID-QID PPIBID-QID PPI Nissen fundoplicationNissen fundoplication


Asthma AssociatedAsthma Associated

EpidemiologyEpidemiology a/w asthmaa/w asthma unrecognizedunrecognized

PathophysiologyPathophysiology vagal reflex vagal reflex

dysfunction?dysfunction?

Assoc sx / findings vs Assoc sx / findings vs asthma controlsasthma controls

Work-upWork-up PFTPFT

Airway interventionAirway intervention variable variable

TreatmentTreatment for asthmafor asthma

Newman et al 1995: 95 pts, 56% with VCD and Asthma

O’Connell et al 1995: 20 pts, 35% with VCD and Asthma


Irritant InducedIrritant Induced

EpidemiologyEpidemiology occupational exposureoccupational exposure

PathophysiologyPathophysiology

Associated findingsAssociated findings chest painchest pain similar hx in similar hx in

malingeringmalingering decreased coinci-decreased coinci-

dence of psych d/odence of psych d/o

Work-upWork-up irritant/allergen irritant/allergen

bronchoprovocationbronchoprovocation

Airway interventionAirway intervention not required not required

TreatmentTreatment identify and remove identify and remove

irritantirritant

PVFM: Differential, Non-organicPVFM: Differential, Non-organic

Conversion / Somatization DisorderConversion / Somatization Disorder

EpidemiologyEpidemiology young female athleteyoung female athlete female health care female health care

workerworker psychologic stressorspsychologic stressors

PathophysiologyPathophysiology unintentional, unintentional,

unconsciousunconscious

Associated findingsAssociated findings well-motivated, well-motivated,

compliant with txcompliant with tx

Work-upWork-up dx of exclusiondx of exclusion psych evalpsych eval

Airway interventionAirway intervention not requirednot required

TreatmentTreatment to followto follow

PVFM: Differential, Non-organicPVFM: Differential, Non-organic

Factitious / Malingering DisorderFactitious / Malingering Disorder

EpidemiologyEpidemiology 50-73% psych hx50-73% psych hx 18% prior factitious d/o18% prior factitious d/o

PathophysiologyPathophysiology Factitious: “sick role”Factitious: “sick role” Malingering: sec gainMalingering: sec gain

Associated findingsAssociated findings MalingeringMalingering

poorly cooperativepoorly cooperative

inciting eventinciting event

assoc axis II d/oassoc axis II d/o

Work-upWork-up dx of exclusiondx of exclusion psych evalpsych eval

Airway interventionAirway intervention not requirednot required

TreatmentTreatment to followto follow

PCFM: ManagementPCFM: ManagementShort-term Strategies for MostShort-term Strategies for Most

Breathing techniquesBreathing techniques sniffing, pantingsniffing, panting humminghumming diaphragmaticdiaphragmatic talkingtalking

DistractionDistractionReassuranceReassuranceSedationSedation benzodiazepamsbenzodiazepams propofolpropofol

Airway adjuvantsAirway adjuvants HelioxHeliox CPAP / IPPV / BiPAPCPAP / IPPV / BiPAP intubationintubation tracheostomytracheostomy

PCFM: ManagementPCFM: ManagementLong-term Strategies are “multidisciplinary”Long-term Strategies are “multidisciplinary”

Speech therapySpeech therapy relaxation techniquesrelaxation techniques breathing retrainingbreathing retraining biofeedback (video)biofeedback (video)

PsychotherapyPsychotherapy identifying stressorsidentifying stressors biofeedbackbiofeedback hypnosishypnosis

PharmacologicPharmacologic dx specific, e.g. dx specific, e.g.

ipratropium in ipratropium in exercise-associatedexercise-associated

BOTOX BOTOX PPIPPI

SurgicalSurgical tracheostomytracheostomy

PCFM: ManagementPCFM: ManagementLong-term Strategies for Non-organicLong-term Strategies for Non-organic

PVFM: PrognosisPVFM: Prognosis

Distinguished exercise-induced vs spontaneousDistinguished exercise-induced vs spontaneousContacted 28/49 ptsContacted 28/49 ptsMedian time until resolutionMedian time until resolution Exercise-associated: 4 mosExercise-associated: 4 mos Spontaneous: 5 mosSpontaneous: 5 mos

and ranged from 1 wk to 5 years, irrespective of and ranged from 1 wk to 5 years, irrespective of interventionintervention

PVFM: SummaryPVFM: Summary

Product of diverse etiologiesProduct of diverse etiologiesPrompt and accurate diagnosis facilitates Prompt and accurate diagnosis facilitates appropriate managementappropriate management hx / laryngoscopy / provocation are keys to dxhx / laryngoscopy / provocation are keys to dx multidisciplinary therapy essential in non-multidisciplinary therapy essential in non-

organic cases organic cases

Prognosis varies with causePrognosis varies with causeGet the pulmonologists and neurologists Get the pulmonologists and neurologists involved early, they’re really smartinvolved early, they’re really smart

paradoxical vocal fold motion. goals background history history pathophysiology pathophysiology...

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