|Year : 2015 | Volume
| Issue : 1 | Page : 17-20
Paradoxical vocal cord motion disorder - An unexpected mimicker of bronchial asthma
Abdul Wadood Mohammed, Pearl Sara George, Kasim Kolakkadan, Jishnu Narayanan, Deepak Raj
Departments of Otolaryngology-Head and Neck Surgery, KIMS Al-Shifa Hospital Private Limited, Perinthalmanna, Kerala, India
|Date of Web Publication||17-Dec-2015|
Abdul Wadood Mohammed
Department of Otolaryngology-Head and Neck Surgery, KIMS Al-Shifa Hospital Private Limited, Perinthalmanna - 679 322, Kerala
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Paradoxical vocal cord motion (PVCM) disorder, otherwise called vocal cord dysfunction is a very peculiar disorder often misdiagnosed and mistreatment. The awareness of such a condition is very little between practicing pulmonologist and otolaryngologist and often patients are treated as bronchial asthma or get tracheostomized misdiagnosed as bilateral abductor cord palsy. We present a case of a 25-year-old lady, who was being treated for bronchial asthma in the form of oral and inhaled steroids. She presented to the emergency with a supposed acute exacerbation of asthma. However, the patient did not respond to intravenous and nebulized steroids. The patient was referred to the otolaryngologist and fiber optic laryngoscopy was done and a diagnosis of bilateral abductor cord palsy. The patient was subsequently tracheostomized and decannulated once the vocal cord motion recovered. However, the patient again presented to the emergency with stridor, but this time a detailed fibreoptic laryngoscopy helped us to diagnose PVCM disorder. The patient was treated with nothing other than psychotherapy and speech therapy and the patient recovered never to get a further episode. We discuss the important aspects of clinical features, diagnosis, and management of this unique condition.
Keywords: Bronchial asthma, fiber optic laryngoscopy, paradoxical vocal cord motion
|How to cite this article:|
Mohammed AW, George PS, Kolakkadan K, Narayanan J, Raj D. Paradoxical vocal cord motion disorder - An unexpected mimicker of bronchial asthma. J Laryngol Voice 2015;5:17-20
| Introduction|| |
The paradoxical vocal cord motion (PVCM) disorder is characterized by paroxysmal periods of vocal cord adduction during inspiration and/or expiration, causing reduced airway leading to episodic dyspnea, wheezing, and stridor.  The condition is precipitated by exercise or emotional stress and is often misdiagnosed as more common conditions like bronchial asthma which have a similar presentation. This usually puts the patient to take unnecessary treatment in the form of steroids and suffer the complications related to it. This may even lead the patient to undergo emergency tracheostomy or intubation to relieve the stridor. Paradoxical vocal cord motion disorder can be easily diagnosed with a proper laryngoscopy and save the patient from unnecessary ordeals. The treatment involves removal of the offending trigger which is usually emotional disorders. Our aim of this article is to present a case who presented to us with the disorder and the difficulties we faced to diagnose the condition and treatment and to discuss the present understanding on this unique disorder.
| Case report|| |
A 25-year-old unmarried lady presented to the emergency department with complaints of breathing difficulty and choking sensation. She gave history of similar episodes for the past 10 years and was under treatment for bronchial asthma in the form of regular inhaled steroids, bronchodilators, and oral steroids during the acute exacerbations. She had cushionoid features, which she alleged to have after starting treatment for breathlessness. She had stridor, the intensity of which increased during inspiration. Her phonation was normal. She showed oxygen saturation of 92%. Her pulmonary function test during her previous visits showed a reduced forced expiratory volume in 1 (FEV1)/FEV ratio of 70% and flattening of both inspiratory and expiratory limbs. An emergency fiber optic laryngoscopy was done which showed bilateral abductor cord palsy. A tracheostomy was performed which relieved the stridor. The patient was planned for observation for a period of 6 months for any spontaneous resolution of cord function and if no recovery a definitive procedure in the form of laser cordectomy was advised. The patient was followed up with flexible laryngoscopies, which showed recovery of the cord movement within 2 weeks and the patient was successfully decannulated at the end of 2 nd week. After 10 days, the patient again presented to the emergency with breathlessness. On examination, the patient was in stridor but the patient was relatively calm and composed. This time, a thorough flexible laryngoscopy was done which revealed paradoxical vocal cord movements. The vocal cords were completely adducting during inspiration [Figure 1] and expiration [Figure 2], whereas abduction during rest. This allowed us to make the diagnosis of PVCM disorder. The patient was started on speech therapy and psychiatry evaluation was done. History revealed depressed mood, anhedonia, anergia, disinterest, lack of drive, decreased sleep and appetite, and death wishes. Her mental state examination (MSE) was corroborative. A diagnosis of severe depression was made. She was put on escitalopram 10 mg and clonazepam 1 mg and latter was tapered in 3 weeks. She was then put on escitalopram 10 mg. Her serial MSEs were done which showed good improvement as per Montgomery-Asberg depression scale scores. The improvement in her mood state was coinciding with her resolution of dysphonia and dyspnea. This suggests a causal relation of depression toward PVCM. Speech therapy sessions included respiratory training, breathing exercises, and inhalation phonation. In respiratory training, the patient is trained to practice diaphragmatic breathing. This helps to divert the patient attention from the larynx and to reduce tension on laryngeal musculature during respiration. The breathing exercise included open throat breathing to reduce tension on the laryngeal musculature.
The patient completely recovered within 2 days of starting speech and psychotherapy without any additional need for bronchodilators or steroids. A repeat fiber optic laryngoscopy after 2 weeks showed normal abduction of cords during inspiration [Figure 3] and expiration [Figure 4]. After 6 months of follow-up, a pulmonary function test showed only partial improvement of the FEV1/FEV ratio up to 85% and the expiratory and inspiratory limbs of normal morphology. This signified a coexistent bronchial asthma for which treatment had to be continued. However, the patient did not have any further episodes of acute exacerbation until 6 months of follow-up indicating that the exacerbations were due to PVCM.
| Discussion|| |
The earliest definite description of PVCM disorder was given by Mackenzie in 1869 when he visualized the paradoxical movement of the vocal cords during inspiration causing stridor.  In 1842, Dunglison had appropriately described the disorder as a "hysteric croup." Patterson et al. in 1974 termed the condition "Munchausen's strider" describing it in a 33-year-old lady who presented to the hospital seeking treatment for stridor.  Due to its peculiar presentation this disorder has been nicknamed by many throughout the history like "episodic laryngeal dyskinesis," "Munchausen's stridor," "pseudoasthma", "functional upper airway obstruction", "spasmodic croup", "emotional laryngeal wheezing", "irritable larynx syndrome", "vocal cord dysfunction" etc. 
PVCM is mainly seen in children and young adults  with a definite female predominance of 2:1. However, the true incidence of PVCM could be under-reported as this condition is misdiagnosed in the majority of cases. The pathogenesis of PVCM is not certain. However, there are possible theories of explaining the presentation. Both organic triggers like reflux, irritants etc., or inorganic triggers like emotional stress can bring about sudden laryngeal narrowing through four proposed mechanisms in PVCM namely, laryngeal hyperresponsiveness, altered autonomic balance, direct stimulation of the sensory nerve endings in the upper or lower respiratory tract, and hyperventilation. The most accepted mechanism behind PVCM is that by Ayers and Gabbott  who combines all these theories to postulate that laryngeal hyperresponsiveness initiated by an initial inflammatory insult and resulting in altered autonomic balance, which may be transient or persistent. If persistent, subsequent stimuli will induce a local presynaptic reflex causing airway narrowing, either at the level of glottis or the lower airway. Psychogenic factors have been implicated as an etiological factor in many studies. In a study by Gavin et al.,  12 patients with PVCM, had the episodes occurring only at times of anxiety and not related to exercise. Newman et al. noted that a previous history of psychiatric illness was seen in 73% of patients with PVCM. Powell et al. reported that 55% of 20 adolescent females with PVCM suffered from severe social stresses like competitive sports. Our patient had multiple stress factors which lead to depression. Among psychiatric illnesses, conversion disorder seems to predominate in patients with PVCM. However, mood disturbances such as depression and anxiety are known to coexist in patients with conversion disorders.  Whether PVCM is a part of the symptom spectrum of conversion disorder is not clearly understood. The association of PVCM with asthma is still confusing as many patients would be under treatment of asthma at the time of diagnosis. Newman et al.  found that 56% of the 95 patients with laryngoscopically proved PVCM had coexistent asthma. However, they also noted that patients with PVCM only (without asthma) were misdiagnosed as asthma and had used prednisone on a regular basis before correct diagnosis. Some studies also mention gastroesophageal reflux disease as an etiological factor for PVCM.
Patients with PVCM almost exclusively present with symptoms of shortness of breath and asthma-like symptoms during exercise or intense emotion, which does not respond to bronchodilators or steroids. This misdiagnosis has often lead patients to undergo unnecessary morbidities including intubation, tracheostomy, high-dose corticosteroid use with attendant adverse effects, multiple hospitalizations and long-term psychological and behavioral dysfunctions.  In our case, the patient was on long-term corticosteroid because of which she had developed cushinoid features and had to undergo tracheostomy once. Features of PVCM, which distinguishes it from asthma can be nonresponse to bronchodilators or corticosteroids, symptoms that are precipitated by stress, emotional factors or anxiety, athletes with chocking sensation during exercise etc., Furthermore, it may be noted that symptoms of PVCM can sometimes be abated by distraction.  Fiber optic laryngoscopy and direct visualization of the vocal cords during the episode of breathlessness is the gold standard for diagnosing PVCM. Complete or partial adduction of the vocal cords during inspiration with the formation of a small posterior diamond-shaped glottal chink is diagnostic of PVCM.  If the patient is asymptomatic asking the patient to pant, breathe deeply, and phonate may sometimes elicit symptoms.  In the study by Newman et al. he reported laryngoscopic findings to be diagnostic of PVCM in 100% of symptomatic patients and 60% of asymptomatic patients.  Chest X-ray is not recommended for diagnosis as they may be normal in both PVCM and asthma. Findings such as hyperinflation and peribronchial thickening could suggest asthma, but does not rule out PVCM.  Airway fluoroscopy and ultrasound of vocal cords may be helpful, but do not add any advantage to fiber optic laryngoscopy. Pulmonary function tests may also help to support the diagnosis. However, in an asymptomatic patient, the flow volume loop is usually normal. If the patient is symptomatic, a flattening of the inspiratory limb or expiratory limb is seen suggestive of extra-thoracic obstruction.
Treatment mainly can be divided into two those for terminating the attack and preventing relapse. The mainstay of treatment is speech therapy and psychotherapy. Since the psychological factor is a predominant contributor, these methods have a very high success rate. Reassuring the patient and psychologically supporting the patient have found to have a definite advantage to abort an attack. Asking the patient to focus on making a soft "s" sound while exhaling helps by diverting attention during inhalation and giving auditory feedback on air movement.  Panting and coughing are also effective during an acute attack. Benzodiazepines are anxiolytics used to sedate patients and relieve their anxiety during the attack and are found to be effective. Heliox and nebulized lignocaine have also shown to have short-term benefit in managing acute attacks. Speech therapy is considered to be the mainstay of the treatment of PVCM. The method involves teaching the patient to increase the airway dimensions during respiration. Sullivan et al. reports almost 95% success in controlling symptoms in patients treated by speech therapy.  Psychotherapy and hypnosis are also important pillars of treatment of PVCM. The objective of psychotherapy includes relaxation therapy to reduce distress during the attacks, identification of stressors that contribute to PVCM, and developing techniques to counter the stressors. Other moralities of doubtful benefit are inhaled anticholinergics, botulinum toxins, inspiratory valve, and continuous positive pressure ventilation.
| Conclusion|| |
We consider that PVCM is not a rare disorder. With the actual incidence of the disorder unknown due to misdiagnosis, the seriousness of the condition should not be overlooked. As the incidence of stress and other psychological disorders are increasing, more patients with PVCM would be presenting to us. In such instances knowledge of the biomechanics of the condition and a simple fiber optic laryngoscopy would save the patient from the burdens on long-term steroids and tracheostomy. Chest physician should be made aware of this disorder and keep PVCM as a differential diagnosis in prolonged nonresponsive asthma.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]