sign in sign up
<<
Home , Heliox

What’s Your Diagnosis? A Perplexing Presentation of New Onset Wheezing

Esan O. Simon, MD, MBA; and Jesse Bracamonte, DO

Can you guess the underlying condition causing this patient’s episodic wheezing and difficulty ?

17-year-old girl presented to the At the primary care provider’s office, supplements; she also denied any inha- emergency department (ED) re- the patient received multiple albuterol lation exposures or sexual activity. Her porting difficulty breathing and treatments for the wheezing prescribed medications at the time in- A wheezing, which had begun the and difficulty breathing. When the pa- cluded escitalopram 10 mg/day, for her previous day. The patient had no history tient showed no appreciable response to disorder, and the birth control of or other breathing difficulties. the treatments, she was transferred via pill, for regulating her menses. During the physician-patient interview, ambulance to the ED for further evalua- Upon physical examination, the pa- the patient was having such significant tion and treatment. During the transfer, tient’s vital signs included heart rate, difficulty breathing that she was unable she again received additional albuterol 123 beats per minute; respiratory rate, to provide a medical history apart from nebulizer treatments. 24 breaths per minute; blood , head-nod responses to yes or no ques- In the ED, the patient received mul- 139/77 mm Hg; , 99.7°F; tions. tiple 2.5 mg and 5.0 mg albuterol and , 126 lbs. Examinations of According to her father, the patient nebulizer treatments, intravenous meth- her abdomen, all 4 extremities, head, was at school when she experienced 4 ylprednisolone 125 mg, nebulized ra- ears, eyes, nose, and throat were unre- to 5 episodes of wheezing with short- cemic epinephrine 2.25% (.75 ml), and markable. Cardiac auscultation revealed ness of breath—each episode lasting ap- heliox (/ mixture) treat- tachycardia with a regular rhythm, and proximately 20 minutes—followed by ments. While the patient did not seem to no evidence of murmurs, rubs, gallops, asymptomatic periods with no wheezing respond to the repeated albuterol treat- or jugular venous distension. or difficulty breathing. She was treated ments, the heliox and racemic epineph- Examination of her pulmonary func- with nebulized albuterol by the school rine appeared to provide a fair amount tions revealed moderate respiratory dis- nurse, which did not result in any signif- of symptom relief. tress with tachypnea, increased work of icant improvement. The wheezing epi- The patient’s medical history was sig- breathing with the use of accessory mus- sodes recurred throughout the evening at nificant for an anxiety disorder, which cles of , and pronounced head home and, when the symptoms persisted manifested a year ago, after surviving bobbing back and forth, particularly the following day, she was taken to see a motor vehicle accident in which her with inspiration. Breath were her primary care provider. friend’s father died. Otherwise, she had minimal throughout the fields, and no history of asthma, allergic rhinitis, there was intermittent and inspi- cardiac disease, exposure to any air- ratory wheezing, but no rhonchi or rales CDR Simon is an active-duty family phy- sician and regional practice director at the Samuel way irritants (such as paint products, were auscultated. J. Call Health Services Center at the U.S. Coast allergens, or tobacco), gastroesophageal Results of laboratory studies in- Guard Health, Safety and Work-Life Regional Prac- reflux disease (GERD), posttraumatic cluded a normal complete blood count, tice in Cape May, New Jersey. Dr. Bracamonte is a family medicine physician at Desert Hills Family stress disorder, drug allergies, prior sur- a normal electrolyte panel, a normal Medicine in Glendale, Arizona. At the time this geries, involvement in sports, or regu- urinalysis, and urine negative for beta article was submitted, CDR Simon was a resident lar exercise. She denied using tobacco, human chorionic gonadotropin. A chest and Dr. Bracamonte was an attending physician, both in the Department of Family Medicine at the alcohol, illicit drugs, over-the-counter roentgenogram showed no evidence of Mayo Clinic in Scottsdale, Arizona. medications, herbal remedies, or other hyperinflation, infiltrate, pneumotho-

MAY 2011 • FEDERAL PRACTITIONER • 25 WHAT’S YOUR DIAGNOSIS?

rax, foreign body, or any other abnor- tory distress during the initial 2 hours mality. Analysis of arterial blood gas of hospitalization. For symptom re- (ABG) on supplemental oxygen re- lief, she was given a brief trial of bi- vealed a normal pH level of 7.42, a nor- level positive airway pressure, which mal carbon dioxide (PCO2) level of was discontinued after approximately 38.3 mm Hg, an elevated oxygen (PO2) 5 minutes because of patient discom- level of 450 mm Hg (reference range, fort. A trial of continuous positive air- 80 mm Hg to 100 mm Hg), a normal way pressure was provided for several serum bicarbonate level of 23.8 mEq/L, hours, which did result in some relief and a normal base deficit of +1 mEq/L. and was well tolerated by the patient. The patient’s initial presentation of A speech therapist was consulted marked stridor and wheezing resolved and provided the patient with inter- after approximately 8 minutes. Then, she ventions to ameliorate the PVCM was calm and able to speak in complete symptoms, including breathing pat- sentences, without any difficulty. With Figure 1. Normal vocal cord abduction tern exercises, tactile feedback, and the clinical picture suggesting an upper during inspiration. visualization; these techniques re- airway component, she was evaluated sulted in significant relief of the pa- further with a computerized tomography tient’s symptoms. A psychiatrist also scan of the upper airway, which noted a was consulted, who concurred with normal epiglottis, normal aryepiglottic the speech therapist and recom- folds, normal paratracheal soft tissues, mended continuing the escitalopram and no evidence of retropharyngeal ab- for generalized anxiety disorder. scess or other soft-tissue abnormality. Outpatient follow-up included With the continued episodic stridor evaluation by a psychologist, with a and wheezing, the patient was prepared biofeedback trial of respiration pac- for transfer to the intensive care unit, ing. Additionally, videostroboscopic and aggressive treatments were ordered, evaluation was performed by the including intravenous methylpredniso- speech therapist, which noted the lone 125 mg every 6 hours, oral zileuton complete abduction of her vocal 600 mg every 6 hours, nebulized race- cords with sniff breathing as well as mic epinephrine 2.25% (.75 ml) every “S” breathing. When asked to imi- Figure 2. Paradoxical vocal cord move- tate an episode of respiratory distress, hour, and continued heliox treatments. ment, with vocal cord adduction during inspiration. vocal cord adduction was noted on What’s your diagnosis? the videostroboscope. Outpatient follow-up with the otolaryngologist OUR DIAGNOSIS With the diagnosis of PVCM con- concurred with the assessment and An otolaryngologist was consulted in firmed on endoscopic evaluation, the treatment recommendations of the the ED and a nasopharyngeal endo- patient was admitted to the hospital psychologist and speech therapist. scopic examination was performed for overnight observation, largely to at bedside. The examination revealed allay patient and parental anxiety, and ABOUT THE CONDITION that the oropharynx (including the to provide them with further edu- Located in the , the vocal cords base of the tongue and the epiglot- cation on this condition. Once the are comprised of a muscular compo- tis), nasopharynx, hypopharynx, and diagnosis was confirmed, the meth- nent (the 2 thyroarytenoid muscles, larynx were unremarkable in appear- ylprednisolone, zileuton, racemic epi- forming the “body”) and a non­ ance, with no evidence of erythema nephrine, and heliox treatments were muscular component (the inner and or edema. The patient exhibited ob- discontinued. outer layers and lamina propria, form- vious evidence of paradoxical vocal ing the “cover”).1 At rest, the vocal cord motion (PVCM), however, with Treatment cords form a v-shaped space, known as the vocal folds adducting upon inspi- The patient proceeded to have several the glottis, and during normal inspira- ration. additional episodes of acute respira- tion, they abduct (Figure 1). PVCM

26 • FEDERAL PRACTITIONER • MAY 2011 WHAT’S YOUR DIAGNOSIS?

occurs when the vocal cords inappro- associated with underlying psychi- tients frequently have a decrease of priately adduct during inspiration and atric diagnoses, social stresses, and > 25% in the maximum inspiratory abduct on expiration (Figure 2). sexual abuse,4 a psychogenic compo- flow during histamine inhalation The resultant airway obstruction nent also is thought to play a key role challenge.13 can manifest as stridor and often is in the condition. Methacholine challenge testing mistaken for asthmatic wheezing, Additional patient demographic typically induces PVCM during la- with a diagnosis of asthma or “re- descriptions and epidemiologic asso- ryngoscopic evaluation.14 Despite fractory asthma” being made, and ciations include a 2:1 female-to-male overt respiratory distress, ABG analy- patients receiving bronchodila- ratio,10 with 63.6% of PVCM cases sis typically is normal; however, pa- tor treatments, intubation, and even reported in females and 36.4% re- tient breath-holding can result in a tracheostomy.2-4 While other such ported in males.8 While the reported decreased PO2, while hyperventila- 10 conditions as , epiglottitis, la- age range of the affected population tion can result in a decreased PCO2. ryngospasm, and angioedema may is broad (from < 1 year to 82 years), Chest roentgenograms are not helpful mimic the symptoms of PVCM, lack PVCM is more prevalent in children in the diagnosis of PVCM.4 of awareness of the condition, com- and young adults, with a median pe- With various possible mechanisms bined with misdiagnosis, are reasons diatric age of 14 years and a median of disease, treatment of PVCM is mul- PVCM is thought to be an underdiag- adult age of 36.5 years.10 tifactorial and multidisciplinary in nosed condition.4 Even though psychosocial factors nature. In the acute setting, reassur- Published description of laryngeal may play a role in PVCM, there are a ance, benzodiazepines, heliox treat- muscle disorders dates back to 1842, number of other etiologies and asso- ments, and even botulinum toxin when a medical textbook termed ciations that the evaluating clinician injections and sedation have been a condition known as “hysterical should consider in the differential utilized.8,10 Long-term management croup,” as it was postulated that the diagnosis. While some patients may involves speech therapy, biofeedback, disorder was brought on by hysteria.5 report dyspnea and exercise-induced psychotherapy, and hypnosis, all of With the advent of in asthma-like symptoms, 4 such con- which have been shown to relieve 1854,6 the following decade noted the ditions as GERD,11 irritant-induced symptoms.4 Speech therapy, which first visualization of PVCM in patients PVCM (caused by inhaling smoke, teaches proper breathing techniques, symptomatic with stridor.7 Since then, gases, vapors, dust, airborne pollut- with a focus on laryngeal-area control, various names have been ascribed to ants, or odors),4 and even extubation plays a vital role in the treatment of the condition, including “irritable lar- following general have PVCM, and is considered to be the ynx syndrome,” “Munchausen’s stri- been linked to PVCM. Also included cornerstone of long-term manage- dor,” and “pseudoasthma.”8,9 Medical in the differential diagnosis are neu- ment.8,10 Although the medical litera- literature review is noteworthy for rologic diseases, such as Parkinson ture demonstrates inconclusive results over 70 terms used to describe this disease, Arnold-Chiari malformation, regarding the long-term prognosis of disorder, such as paradoxical vocal cerebral aqueduct stenosis, and amy- patients with PVCM, there are some fold motion, paradoxical vocal fold otrophic lateral sclerosis.12 data that suggest spontaneous resolu- movement disorder, paradoxical vocal Although the gold standard for di- tion of symptoms may be common.15 cord movement, episodic paroxysmal agnosis is direct laryngoscopy,8 with Additionally, for patients with GERD- , and vocal cord dys- a broad differential, patients often associated PVCM, acid-suppression function.10 will have additional diagnostic evalu- therapy potentially may provide relief Although the precise pathogen- ations. Pulmonary function testing of PVCM symptoms.16 esis of PVCM is not entirely under- often is used to support the diagno- stood, it has been hypothesized that sis of PVCM and typically reveals a IN CONCLUSION the underlying mechanisms for the highly variable pattern of inspiratory The patient was seen by her primary condition may include laryngeal hy- flow configurations, as well as a ratio care provider 2 weeks after the ini- perresponsiveness, altered autonomic between the forced expiratory flow tial presentation and noted almost balance, direct stimulation of the sen- (FEF) at 50% of the exhaled vital complete resolution of her PVCM sory nerve endings in the upper and capacity and the forced inspiratory symptoms after utilizing the speech lower , and hyper- flow (FIF) at 50% (FEF50/FIF50) that and biofeedback techniques. She did ventilation.8 With PVCM frequently often is > 1.10 Additionally, these pa- report that she had some increasing

MAY 2011 • FEDERAL PRACTITIONER • 27 WHAT’S YOUR DIAGNOSIS?

phia, PA: Lea and Blanchard; 1842:257-258. anxiety symptoms related to her pre- Disclaimer 6. Bailey B. Laryngoscopy and laryngoscopes–who’s first?: The forefathers/four fathers of laryngology. vious motor vehicle accident, as the The opinions expressed herein are those of Laryngoscope. 1996;106(8):939-943. 1-year anniversary of the accident the authors and do not necessarily reflect 7. Mackenzie M. Use of Laryngoscopy in Diseases of the Throat. Philadelphia, PA: Lindsay and Blakiston; was less than 2 weeks away. As such, those of Federal Practitioner, Quadrant 1869:246-250. she was referred to a pediatric psy- HealthCom Inc., the U.S. Government, 8. Morris MJ, Allan PF, Perkins PJ. Vocal cord dys- function: Etiologies and treatment. Clin Pulm Med. chologist for outpatient evaluation or any of its agencies. This article may 2006;13(2):73-86. and further treatment of her anxiety discuss unlabeled or investigational use 9. Mathers-Schmidt BA. Paradoxical vocal fold motion: A tutorial on a complex disorder and the speech- disorder. of certain drugs. Please review complete language pathologist’s role. Am J Speech Lang Pathol. On follow-up assessment 2 years prescribing information for specific drugs 2001;10:111-125. 10. Hicks M, Brugman SM, Katial R. Vocal cord dys- following her initial presentation, or drug combinations—including indica- function/paradoxical vocal fold motion. Prim Care. the patient reported experiencing tions, contraindications, warnings, and 2008;35(1):81-103, vii. only mild, brief (few seconds dura- adverse effects—before administering 11. Powell DM, Karanfilov BI, Beechler KB, Treole K, Trudeau MD, Forrest LA. Paradoxical vocal dord tion), sporadic episodes of respira- pharmacologic therapy to patients. dysfunction in juveniles. Arch Otolayngol Head Neck tory symptoms, typically coinciding Surg. 2000;126(1):29-34. 12. Maschka DA, Bauman NM, McCray PB Jr, Hoffman with excessively stressful situations. REFERENCES HT, Karnell MP, Smith RJ. A classification scheme The episodes occurred 2 to 4 times a 1. Izdebski K. Clinical voice assessment: The role & for paradoxical vocal cord motion. Laryngoscope. value of the phonatory function studies. In: Lalwani 1997;107(11 Pt 1):1429-1435. year and were easily resolved with the AK, ed. Diagnosis & Treatment in Otolaryn- 13. Bucca C, Rolla G, Scappaticci E, Baldi S, Caria E, breathing techniques the patient had gology–Head & Neck . 2nd ed. New York, NY: Oliva A. Histamine hyperresponsiveness of the ex- l McGraw-Hill Companies, Inc; 2008:416-429. trathoracic airway in patients with asthmatic symp- learned previously. 2. Thomas PS, Geddes DM, Barnes PJ. Pseudo-steroid toms. Allergy. 1991;46(2):147-153. resistant asthma. Thorax. 1999;54(4):352-356. 14. Balkissoon R. Occupational upper airway disease. 3. Murray DM, Lawler PG. All that wheezes is not Clin Chest Med. 2002;23(4):717-725. Author disclosures asthma. Anaesthesia. 1998;53(10):1006-1011. 15. Doshi DR, Weinberger MM. Long-term outcome of The authors report no actual or poten- 4. Ibrahim WH, Gheriani HA, Almohamed AA, Raza T. . Ann Allergy Asthma Immu- Paradoxical vocal cord motion disorder: Past, present nol. 2006;96(6):794-799. tial conflicts of interest with regard to and ruture. Postgrad Med J. 2007;83(977):164-172. 16. Koufman J. Paradoxical vocal cord movement. The this article. 5. Dunglison RD. The Practice of Medicine. Philadel- Visible Voice. 1994;3(3):49-53, 70-71.

28 • FEDERAL PRACTITIONER • MAY 2011