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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