Asthma Masqueraders:

Differential Diagnosis in Children and Teens

Ngoc Ly, MD, MPH

Assistant Professor of Pediatrics Pediatric Pulmonary Medicine UCSF Benioff Children’s Hospital

Diagnosing Asthma

No gold standard for diagnosis Clinical diagnosis Recurrent respiratory symptoms – Wheeze – Cough – Chest tightness – Breathlessness

www.european-lung-foundation.org

Symptoms History Exam Spirometric Results Treatment Response

No Treatment Response

Poor compliance or adherence Severe or difficult asthma Not asthma

Case #1

16 yo girl with 1 year history of recurrent severe dyspnea and wheezing Multiple ER visits due to severe respiratory distress Several hospitalizations Treated with various asthma therapy No consistent response to therapy

normal

Vocal cord dysfunction (VCD)

Trigger by exercise or emotional stress Young, female Dyspnea with exercise Sensation of tightness in throat rather than chest. Loud wheeze/stridor over larynx rather than chest. Respiratory distress Typically no bronchodilator response

Pediatrics 2007;120(4):860

Vocal cord dysfunction

Treatment: speech therapy Ipratropium may block exercise-induced vocal cord dysfunction Treat underlying conditions, including anxiety, depression, gastroesophageal reflux disease, and rhinosinusitis

Exercise-Induced Dyspnea

Outcomes from142 subjects Median age 14 years (range, 6-21) Average duration of symptoms of 30.2 months Male:Female 0.7:1.0

98 patients with prior asthma diagnosis 101 patients were treated including bronchodilators in 96, inhaled corticosteroids in 35, and systemic corticosteroids in 31. 82 had at least 1 urgent medical care visit Symptoms were reproduced during exercise testing in 117 patients Mutasim Abu-Hasan et al., ANNALS OF ALLERGY, ASTHMA & IMMUNOLOGY 2005

Etiology for EID

11 13 74 1 2 1 15

Mutasim Abu-Hasan et al., ANNALS OF ALLERGY, ASTHMA & IMMUNOLOGY 2005

EIB VCD Restrictive EIL EIH EISVtach Physiologic

Exercise Induced Dyspnea

EID not always asthma Treadmill exercise indicated when – other asthma symptoms not present – Bronchodilator doesn’t prevent EID – Normal baseline lung function Testing: – Pre- and post-exercise spirometry – Full exercise testing including gas exchange and EKG – Laryngoscopy when indicated

Case #2

10 yo girl who developed a cough along with her cousin after they went swimming at a public pool The following day, she was coughing so much at school that she failed her math test Kept out of school x2 weeks because the cough was disruptive Presented to the ED twice Treated with continuous albuterol, followed by inhaled corticosteroids and allergy medications- no improvement after 4 weeks Valium, codeine, then antibiotics were prescribed-none were effective Otherwise healthy, pertussis test negative Does not cough in her sleep

Habit Cough

Involuntary, repetitive cough Loud, dry, “barking” or “honking” Triggered by infection, irritants, or psychological stressors Can last for months to years Disruptive to others-affect school attendance Cough can be a source of secondary gain but not always

Cough ceases while asleep and usually improves with distraction

Habit Cough

Normal physical exam; normal lung function

Opiods, benzodiazepine, and asthma therapy are ineffective Treatment: suggestive therapy, self hypnosis, speech therapy

Case # 3

9 yo boy present with acute cough 1 week and fever x 2 days Treated with zithromax and albuterol for audible wheeze 2 weeks later, persistent wheeze, started on oral corticosteroids and inhaled corticosteroids 1 week later no response CXR taken

Foreign Body Aspiration

Case # 4

12 yo male Presenting with 5 days of worsening SOB when lying flat This progress and was waking him up from sleep Also wheeze at night Treated with albuterol and Flovent-no improvement No SOB during the day, physically active at school No other complaints Diagnosed with asthma 2 years prior , using albuterol MDI intermittently No prior hospitalization

Mediastinal Mass

Case # 5

7 yo boy with cough x 2 years Harsh, dry, barky cough Paroxysmal coughing with post-tussive emesis Awaken from sleep No response to bronchodilator, corticosteroids, theophylline Cushingoid from repeated prednisone therapy Multiple hospitalizations

Tracheomalacia

Wang CC et al. Ann Thorac Surg. 2010

Tracheomalacia

Aortopexy-stitching between aorta and sternum to reduce aortal tension on anterior wall of the trachea Behavioral-successful in mild cases Albuterol may worsen symptoms due to smooth muscle relaxation

Tracheomalacia

Most common tracheal defect Expiratory stridor/wheeze Worse during feeding, crying, or agitation Improve during sleep Persistent cough, dyspnea, or tachypnea Usually resolves by 12 months R/O vascular compression (ring, sling, anomalous innominate artery)

Tracheomalacia

Cardiac Wheeze

Cardiac conditions that result in pulmonary artery dilation and/or left atrial enlargement, including large left to right shunts, can compress large airways and cause wheezing. Left ventricular failure or pulmonary venous outflow obstruction can result in distension of the pulmonary vascular bed, bronchiolar wall edema, increased airway resistance, and wheezing.

Cardiac Wheeze

Stridor with large airway compression Wheezing, tachypnea Recurrent respiratory infections with significant respiratory distress Apnea

Aspiration Syndromes

Chronic cough and wheeze Swallowing disorders related to neurologic or muscular dysfunction of the pharynx and/or larynx Chronic microaspiration can cause significant mucosal edema and inflammation Tracheoesophageal fistula

Case # 7

6 month old boy, term infant, presenting with chronic cough and wheeze only slightly better with asthma medications.

Reflux, gagging and choking with feeds.

Posterior laryngeal cleft

supraglottic partial cricoid cleft complete cricoid cleft laryngotracheal-esophageal clefts

Posterior laryngeal cleft

Failure of tracheoesophageal septum development Chronic cough and wheeze Respiratory distress with feeds, cyanosis May present at birth Aspiration, pneumonia 6% with TEF have PLC

Posterior laryngeal cleft

GERD must be controlled Type I clefts can sometimes be managed with speech and feeding therapy aimed towards decreasing aspiration Endoscopic or open repair Overall mortality 43% Type IV clefts: 93% mortality

Other Causes

Immune deficiency Cystic fibrosis Bronchiolitis Obliterans Interstitial lung diseases

Conclusions

Assess Treat Reassess if poor response to treatment

DIFFERENTIAL DIAGNOSTIC POSSIBILITIES FOR ASTHMA Upper airway diseases

􀀎 Allergic rhinitis and sinusitis

Obstructions involving large airways

􀀎 Foreign body in trachea or bronchus 􀀎 Vocal cord dysfunction 􀀎 Vascular rings or laryngeal webs 􀀎 Laryngotracheomalacia, tracheal stenosis, or bronchomalacia 􀀎 Enlarged lymph nodes or tumor

Obstructions involving small airways

􀀎 Viral bronchiolitis or bronchiolitis obliterans 􀀎 Cystic fibrosis 􀀎 Bronchopulmonary dysplasia 􀀎 Cardiac disease

Other causes

􀀎 Recurrent cough not due to asthma-habit cough 􀀎 Aspiration from swallowing mechanism dysfunction or gastroesophageal reflux

ASTHMA MANAGEMENT

Clinical Assessment Comorbid Conditions Pharmacologic Therapy Education

ICS +/ Other agents Spirometry & Allergy testing Triggers Techniques Action plan Continue Rx Close F/U Minimum effective dose

+

Response?

-

Consider further evaluation Referral Assess compliance/technique Step-up Therapy

-

History

Birth history Age of onset Progression Recurrent infections Relation to sleep Severe dyspnea, cyanosis, apnea Eating or feeding difficulties, reflux, FTT GI symptoms Cardiac abnormalities

Symptoms

Cough that goes away with sleep Persistent productive cough Prominent dizziness, light-headedness, peripheral tingling Dyspnea with exercise Persistent tachypnea

Exam

Normal exam when symptomatic Tachypnea without wheeze Severe dyspnea without wheeze Stridor or loud inspiratory wheeze Spirometry normal or inconsistent with airflow obstruction when symptomatic

Case # 5

3 month old girl with noisy breathing, worse when she is agitated or excited, and especially with colds. Normal birth history.

Growing and thriving

Tracheomalacia

Most common tracheal defect Expiratory stridor/wheeze Worse during feeding, crying, or agitation Improve during sleep Persistent cough, dyspnea, or tachypnea Usually resolves by 12 months R/O vascular compression (ring, sling, anomalous innominate artery)

Double aortic arch

Arches posterior to esophagus

and

anterior to trachea

Pulmonary Sling

Aberrant left pulmonary artery which arises from right pulmonary artery and crosses

between esophagus and trachea

Bronchomalacia

Wheezing can present at birth More commonly becomes apparent in the first two to three months after birth The wheezing usually becomes more pronounced with activity or in the setting of upper respiratory tract infections Albuterol may worsen symptoms due to smooth muscle relaxation

Case # 7

5 month old female infant with Pierre Robin Sequence, stridor, mild laryngomalacia, FTT, significant reflux Severe OSA on a sleep study Chronic cough x 2 months No gagging or choking with feeds Bilateral wheeze and crackles on exam