The case presented below involves a pediatric patient with asthma, allergic rhinitis, and the possibility of vocal cord dysfunction (VCD).  The issue key to the case is whether or not it is necessary to confirm VCD through laryngoscopy for this pediatric patient.

2. Patient Presentation

“Annie” is a nine-year-old, Caucasian female with a previous diagnosis of asthma.  She is accompanied by her mother and two younger siblings when she presents for a visit at an allergy and asthma specialty clinic to be evaluated for shortness of breath.  The patient reported that her symptoms started a few hours ago during outdoor play at school, and her mother explained that implementation of the school’s asthma action plan did not resolve the current symptoms.

3. Chief Complaint

The patient complains of “shortness of breath” and feeling “unable to inhale.”

4. History of Present Illness

The patient complains of occasional moderate wheezing and chest tightness which began five days prior, an episode leading to an emergency room (ER) visit for an “asthma attack” after one day of outdoor play at school.  The patient currently denies cough, dizziness, headache, hoarseness, ear pain, nasal congestion/drainage, nausea, pharyngitis, postnasal drainage, reflux, sinus infection/pain, sneezing, eye redness/tearing, and urticaria.  The patient’s reports of “feeling funny” and her “heart beating faster” when a rescue medicine was used were interpreted by the clinician as heart palpitations.  The mother reports not giving the patient a bronchodilator before the current visit.

The patient’s medical history includes asthma and allergic rhinitis.  She has no known food or drug allergies, but previous allergy testing was positive for grass, weeds, and tree pollen.  Spirometric testing two years prior showed normal spirometric values indicating the absence of significant obstructive pulmonary impairment and/or restrictive ventilatory defect.

5. Social History

The patient is in 4th grade at a public school. She lives with both parents and two younger siblings.

6. Psychological/Behavioral History

The patient does well in school. She and mother deny being exposed to second-hand smoke.

7. Family History

 Non-contributory.

8. Current Medications

9. Recent ER Test Results

The clinic had copies of the patient’s laboratory findings from the recent ER visit.  Her complete blood count (CBC) was negative for anemia and a chest X-ray (CXR) was unremarkable, showing no radiographic evidence of acute cardiopulmonary disease. The CBC results were within normal ranges aside from elevated basophil (at 0.3 with a normal range of 0 to 0.1) and monocyte count (at 0.7 with normal range of 0 to 0.5) and low lymphocyte count (at 11.7% with a normal range of 27-57%).

10. Review of Relevant Systems

Respiratory:  The patient reports shortness of breath and difficulty inhaling, but denies cough and TB exposure.
CV:  The patient reports chest pressure, discomfort, and heart palpitations.
Neuro/Psych:  Denies headache, anxiety. No other symptoms reported. 

         
11. General and Focused Exam

Vital Signs:  HR 88, BP 86/60, Respirations 20/min, Temperature 98.3, SpO2 98%, BMI 17.9, 50th percentile for both height and weight.

Lungs: Bilateral expiratory wheezing with normal respiratory effort, rate and rhythm.

Cardiac:  Heart sounds are regular, S1, S2, normal rate and rhythm, no murmur, no extra sounds.

Psychiatric:  Oriented to time, place, person, situation, appropriate mood and affect.

Laboratory/Diagnostic Review: Patient is unable to perform a PFT due to respiratory distress. Recent CBC, PFT, CXR are unremarkable.

12. Diagnosis: Asthma, Acute Exacerbation, and Vocal Cord Dysfunction (VCD)

Review of the data suggests the strong possibility that “Annie’s” current visit is not an asthma attack and may indicate VCD. Possible indicators are: Shortness of breath upon inhalation. Implementation of asthma plan showed no relief of symptoms, and possible exacerbation. Patient has a history of normal spiro metric values during PFTs VCD is concurrent among a minimum of 33% and maximum of 75% patients diagnosed with asthma [1].
Many patients with VCD (42.4%) have been misdiagnosed previously as having asthma [2].

13. VCD Treatment

Diagnostics

Radiographic examinations are normal in patients with VCD in contrast to patients with asthma [3]. The clinician ordered tests to rule out acute cardio-pulmonary pathology and to rule out a fixed obstruction.

Pharmacological Intervention

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Systemic corticosteroids are an effective treatment for acute asthma [4]. The clinician affirms continued use of prescribed asthma and allergy medications.

Non-Pharmacological Interventions

Management of VCD requires a multidisciplinary team approach involving physician, pulmonologist, allergist, otolaryngologist, gastroenterologist, neurologist, psychiatrist or psychologist, and speech-language pathologist to successfully diagnose and treat VCD [5-7]. The clinician recommended the patient have a speech therapist train her in symptom-relieving breathing exercises (e.g., “quick inhalation”).

Education and Counseling

Patient education is a necessary part of any primary treatment of VCD [8]. The clinician:

• Explained that asthma treatment is usually ineffective for VCD and instructed patient and mother to carefully monitor bronchodilator effectiveness in episodes of shortness of breath upon inhalation.

• Explained the importance of the intake of calcium and vitamin D rich foods for patients receiving ICs [9]; the importance of a diet focused on food containing little sodium and yielding high amounts of water, calcium, magnesium and potassium to insure growth and development [10]; identified foods rich in calcium and vitamin D.

• Informed patient and mother that the best way to diagnose VCD is with laryngoscopy but that may be best as a “last resort” for young patients because it is invasive and sometimes traumatic. Clinician recommended speech therapy instead.

Vocal Cord Dysfunction (VCD): Pathophysiology

When functioning normally, vocal cords open during inspiration and close slightly during expiration, but with VCD, they close when breathing in and out. Vocal cord dysfunction is an abnormal closure of the vocal cords leaving only a small opening, especially during inspiration, though both cycles can be affected [11]. The condition is reported to be predominant in females; studies vary, with 3:1 or 2:1 ratio of females to males [12].

• Vocal cord dysfunction can present clinically as mild dyspnea, but more often presents with fairly sudden, acute breathing difficulty, almost always upon inhalation. Patients sometimes complain of chest tightness, throat tightness, difficulty swallowing, or a sensation of choking, which can be frightening [6].

• The causes of VCD are not well understood [11-14]. Some experts claim it is possible that VCD is identical with laryngomalacia (LM) [15]. Initially, VCD was thought to have a psychological etiology, but a definite link with a psychological condition has not been established [6]. Still, VCD patients have psychological manifestations (e.g., anxiety) present 40% of the time, but there is little certainty as to whether these psychological factors are causes or effects of VCD and its treatment [14].

• Vocal cord dysfunction can be challenging to diagnose, largely because of how it “mimics” similar disorders-particularly asthma [2,5,7,14,15]. As many as 75% of patients previously diagnosed with asthma had a diagnosis of VCD upon laryngoscopy [1].

• Misdiagnosis of VCD (e.g., as asthma) can lead to misdirected (e.g., exclusion of speech therapy vocal cord relaxation techniques) or unnecessary (e.g., steroid use) treatment plans, either of which might have notable or serious side-effects [5,13,16].

• Triggers of VCD:

• The triggers of VCD are often not identified. At best, there are some consistent triggers to VCD onset including: exercise, upper respiratory tract infections and gastroesophageal reflux disease (GERD) [2,6-8]. Other posited triggers include: rhinitis or allergic rhinitis; chemical or occupational irritants, and postnasal drip [5,12,13,17]. Previous traumatic events involving breathing, severe emotional stress, playing a wind instrument, and competitive athletics have also been mentioned [13].

• Management of VCD:

• Sound management of VCD requires identification of any underlying triggers to the condition and a multidisciplinary approach to addressing them. This may include general internists, allergists, speech therapists, otolaryngologists, psychiatrists, vocational counselors, and others. Speech and language therapists play a key role, teaching techniques to counter throat tightness, cough, and continuous throat-clearing urges. Quick inhalation techniques also help force abduction of the vocal cords [5,6].

Differential Diagnosis

• Differential diagnoses which are important to be considered when suspecting VCD include [18].

• Asthma Extrinsic Acute Exacerbation

• Bronchitis, Acute

• GERD

• Anaphylaxis

• Tracheal Obstruction

• Myocardial Infarction

• Pulmonary Embolus

• Aspiration

• Anxiety

​Treatment Options

• Differentiating VCD from asthma or recognizing its co-existence with asthma early on are important for improvements in long-term prognosis and in the reduction of possible negative psychological effects [2,5]. Also, identification, evaluation, and possible eradication of the triggers are important to lessen or avoid VCD exacerbation episodes. Treatment continued-care plans should involve:

• An otolaryngologist and a speech-language pathologist to help diagnose and treat VCD, the latter teaching relaxed throat breathing techniques [5].

• Well-guided and regularly re-evaluated family involvement (e.g., careful monitoring of frequency and quality of episodes), as well as community resource involvement (e.g., school nurse education

• Flexible laryngoscopy, the “gold standard” of VCD diagnosis [6], should be considered, but recommendation should be weighed against possible traumatic effects on a patient as young as “Annie.”

“Annie” continued to improve with the cooperation of the team and with the well-guided efforts of her family. Symptoms were eased and less frequent. To date, no laryngoscopy has been recommended.

Summary and Conclusion

Vocal cord dysfunction occurs when there is abnormal adduction of the vocal cords which leads to acute signs of airway obstruction. It is a condition that can often be misclassified because it “mimics” symptoms of other conditions, often asthma, and this can lead to misdiagnosis and mistreatment. Diagnostic criteria for VCD are based on clinical symptoms such as shortness of breath, especially while inhaling; pulmonary function flow volume curves; and, most definitively, flexible laryngoscopy. Management of VCD should be multidisciplinary. In this case study, asthma with the co-presence of VCD was assumed, speech therapy was recommended, and well-guided complementary family care helped to relieve VCD symptoms.   “Annie’s” eased symptoms as a result of the team treatment strategy suggest strongly that she did present with VCD even though no laryngoscopic confirmation has been obtained. Confirming the presence of VCD is still important, but, because of the age of the patient, flexible laryngoscopy should be recommended only in the case of reversion to symptom patterns prior to the current VCD treatment plan. Especially in the case of pediatric patients suspected of having VCD, confirmation of VCD via laryngoscopy is less important than relief of the patient’s symptoms based on a treatment and management plan which assumes the presence of VCD.

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