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Paradoxical Vocal Cord Motion (PVCM) also termed Vocal Cord Dysfunction (VCD) or Episodic Laryngeal Breathing Disorder (ELBD)
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Definition: Inappropriate adduction (closure) of vocal cords
- Classically occurs with inspiration constricting the airway causing stridor
- May occur throughout respiratory cycle
- VCD defined as "a paradoxical movement of the vocal cords' - with a hyperfunctional laryngeal reflex which results in adduction (coming together) of the vocal cords with resultant airflow limitation (Porsbjerg 2017, Kenn 2012)
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Differential diagnosis
- Bilateral vocal cord paralysis, abductor paralysis or unilateral paralysis with a narrow glottic aperture(see: Bilateral Vocal Cord Paralysis) - Bernoulli effect during inspiration (passive adduction) may confuse diagnosis; need to see abduction to rule out either
- Posterior glottic stenosis (interarytenoid web,scar or arytenoid fixation -see: Posterior Glottic Stenosis)
- Subglottic or tracheal stenosis (see: Subglottic stenosis)
- Association with asthma - "VCD' reported to be present in 32-50% of patients with difficult asthma (Tay 2016, Low 2011)
- Irritable larynx -with conditions leading to increased laryngeal sensitivity such as
- a. Postnasal drip Medical Management of Sinusitis
- b. Gastroesophageal reflux Esophageal Reflux Precautions
- c. Respiratory infections
- As per Porsbjerg et al (Porsbjerg2017) "a large proportion of patients appear to have a functional component, with pathopsychological traits such as signs of conversion disorder"
- As per Shembel et al (Shembel 2017) "'consensus lacks among clinicians regarding clinical criteria for diagnosis" of ELBD = episodic laryngeal breathing disorder
- As per Forrest et al (Forrest 2012) Although PVCM (paradoxical vocal cord motion) may be a psychological disorder, there are different forms which they divided into "primary, or psychological, and secondary" - with the secondary form consists of medical disorders divided into irritable larynx syndrome and neurologic disorders.
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Standard testing protocols not universally adopted as yet (Forrest 2012)
- General Features test
- Normal complete abduction of vocal cords should be seen at some point in exam to rule out vocal cord paresis/paralysis
- Flexible fiberoptic laryngoscopy at bedside (adults) vs direct laryngoscopy in operating room under spontaneous ventilation (consider for pediatric patients to rule out other causes of obstruction)
- Dr. Bastian demonstrates a simulated period of laryngospasm on a website accessible by searching for "laryngospasm and bastian"
- Classically presents with intermittent shortness of breath
- Often provoked by exertion or chemical exposures
- Commonly misdiagnosed as asthma
- Attacks typically do not respond to medical therapy targetted to reactive airway disease (beta antagonists)
- Rarely may be severe enough to provoke treatment with intubation or tracheotomy
- Normal complete abduction of vocal cords should be seen at some point in exam to rule out vocal cord paresis/paralysis
- Flexible fiberoptic transanasal laryngoscopy (see:Flexible Fiberoptic Laryngoscopy (written instruction) )
- Exercise laryngoscopy
- General Features test
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Key findings from a key prospective study including psychologic evaluation (Forrest 2012)
- Primary (non-organic) PVCM
- Generally not life-threatening
- 75% of patients with PVCM are "primary" - represent a somatoform disorder
- Somatoform disorders are psychological difficulties with symptoms of a physical disorder unexplained by a medical condition
- Conversion or somatization disorder (more common)
- unconsciously produced deficit in motor or sensory functioning, always preceded by a psychological stressor; females>males
- Conversion - common; may only present with neurological (sensory/motor) symptoms
- Somatization - less common; diagnosis requires a mix of pain symptoms, gastroenterological symptoms, sexual/reproductive symptoms and neurological symptoms
- Factitious or malignering (less common)
- consciously produced deficit in motor or sensory functioning; males>females
- Factitious - feigning of symptoms to simulate a disease with a goal for attainment of patient role without external incentive
- Malingering - feigning of symptoms to simulate a disease with external incentive as motivation (eg. monetary gain, drug seeking), symptoms improve once objective attained
- Psychological testing (including MMPI)by Forest et at 2012
- Abnormal in 75% with PVCM
- Abnormalities: highly elevated hypochondriasis scale, highly elevated hysterical scale. (not elevated on anxiety scale)
- After initial evaluation and exam, recommend referral to speech and language therapist (SLP) who can provide various methods to counsel patients, such as:
- Sniff in through the nose - mandates vocal cord abduction moreso than mouth-breathing
- "Pant like a pup"
- Diaphragmatic breathing
- Education on function and importance of larynx
- Consider heli-ox to abort acute episodes
- Secondary (organic) PVCM
- Less common
- Potentially life-threatening
- 25% of PVCM patients have normal psychological testing - divide this group into Neurologic / Hyperreactively
- Laryngeal Hyperreactivity
- Irritable larynx syndrome
- Contributing factors: GERD, allergic laryngitis, laryngeal sicca, recent intubation, laryngeal candidiatsis, untreated OSAS, tobacco, in halation exposure
- Rx: Behavioral and medical management designed to reduce laryngeal irritation
- Irritable larynx syndrome
- Neurologic Disorders
- Focal respiratory dystonia (rare) / Multiple sclerosis flares / autonomic dysfunction (Shy Drager syndrome)
- Laryngeal sensory neuropathy (possibly a factor in PVCM)
- Brainstem compression
- suspect in infants
- suspect in presence other intracranial processes/symptoms (Arnold-Chiari, aqueduct stenosis, hydrocephalus),
- suspect when associated with vagal dysfunction (VPI, nasal regurgitation, dysphagia, aspiration, GERD)
- Good results with surgical decompression
- Cortical or UMN injury
- stroke
- static encephalopathy - older children (developmental delay, sialorrhea, hypertonia)
- LMN injury affecting vagal nuclei or RLN
- eg. ALS, myasthenia gravis, medullary infarction
- Movement disorders
- eg. dystonia, tremors, hyporeflexia
- Parkinsonism
- Select candidates may benefit from botox to thyroarytenoids
- eg. dystonia, tremors, hyporeflexia
- Laryngeal Hyperreactivity
- Primary (non-organic) PVCM
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Management
- Attention to avoid irritants by managing reflux, sino-nasal complaints and the environment
- Intervention by speech pathology
- Identified by Kramer et al (Kramer 2017) as helpful not only for the vocal cord closure problems, but also in decreasing need for asthma medications in those diagnosed with asthma
- "Laryngeal control therapy" coupled with educating the patient about anatomy and physiology in the course of performing transnasal flexible laryngoscopy was employed in this study
- Severe airway obstruction as in the case depicted below of a laryngeal dystonia may be associated with life-threatening airway obstruction and may warrant tracheotomy
- Botulinum toxin injection to the TA muscle (see ) has been reported as useful treatment in the small subset of refractory cases identified by deSilva et a (2019) in treating 13 patients over a 10 year period
- Mean dose of 2.55 units of 'botulinum toxin'per vocal fold (range 1.75-5.5 units)
- 11/13 patients (84.6%) reported improvement in dyspnea; 2 of these 11 patients reported complete resolution of symptoms with one injection
- 2/13 pateitns did not have improvement following botulinum toxin injection underwent tracheotomy
- Other measures for treating this refractory group were identified by deSilva et al to include use of benzodiapines and heliox -reported as useful for short-term but not long-term therapy
- These investigators conclude that PVFMD is a highly variable disorder both in presentation and severity of symptoms - "further complicating the ability to make standardized recommendations for how to best treat a small subset of refractory PVFMD patients."
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References:
- Shembel AC, Sandage MJ, Verdolini Abbott K: Episodic Laryngeal Breathing Disorders: Literature Review and Proposal of Preliminary Theoretical Framework J Voice. 2017 Jan;31(1):125.e7-125.e16. doi: 10.1016/j.jvoice.2015.11.027. Epub 2016 Feb 24.
- Porsbjerg C, Menzies-Gow A:Co-morbidities in severe asthma: Clinical impact and management.Respirology. 2017 May;22(4):651-661. doi: 10.1111/resp.13026. Epub 2017 Mar 22
- Kenn K, Balkissoon R. Vocal cord dysfunction: what do we know? Eur. Respir. J. 2011; 37: 194–200.
- Tay TR, Radhakrishna N, Hore-Lacy F, Smith C, Hoy R, Dabscheck E, Hew M. Comorbidities in difficult asthma are independent risk factors for frequent exacerbations, poor control and diminished quality of life. Respirology 2016; 21: 1384–90.
- Low K, Lau KK, Holmes P, Crossett M, Vallance N, Phyland D, Hamza K, Hamilton G, Bardin PG. Abnormal vocal cord function in difficult-to-treat asthma. Am. J. Respir. Crit. Care Med. 2011; 184: 50–6.
- Patel RR, Venediktov R, Schooling T, Wang B. Evidence-based systematic review: effects of speech-language pathology treatment for individuals with paradoxical vocal fold motion. Am. J. Speech Lang. Pathol. 2015; 24: 566.
- Kramer S1, deSilva B1, Forrest LA1, Matrka L1.Does treatment of paradoxical vocal fold movement disorder decrease asthma medication use?Laryngoscope. 2017 Jul;127(7):1531-1537. doi: 10.1002/lary.26416. Epub 2016 Nov 15.
- Maschka DA, Bauman NM, McCray PB,et al. A classification system for paradoxical vocal cord motion. Laryngoscope 1997;107:1429-1435
- Forrest LA, Husein T, and Husein O: Paradoxical Vocal Cord Motion: Classification and Treatment. Laryngoscope, 122:844-853,2012
- Forrest LA, Husein T, Husein O. Paradoxical vocal cord motion: classification and treatment. Laryngoscope2012;122:844–853
- Bastian RW, Vaidya AM, Delsupehe KG: Sensory neuropathic cough: a common and treatable cause of chronic cough. Otolaryngol Head and and Neck Surg 2006; 135:17-21
- Lee B, Woo P. Chronic cough as a sign of laryngeal sensory neuropathy: diagnosis and treatment. Ann Otol Rhinol Laryngol 2005;114:253-257
- Mintz S, Lee JK. Gabapentin in treatment of intractable idiopathic chrnoic cough: case reports. Am J Med 2006;119:
- deSilva B, Crenshaw D, Matrka L, and Forrest LA: Vocal Fold Botlinum Toxin Injection for Refractory Paradoxical Vocal Fold Motion Disorder Laryngoscope, 129:808-811, 2019
- Marcinow AM, Thompson J, Chiang T, Forrest LA, deSilva BW. Paradoxical vocal fold motion disorderi n the elite athlete: experience ata large division I university. Laryngoscope 2014;124:1425-1430