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Airway and
Pulmonary Disorders
John Carl, MD
Pediatric Pulmonology
Objectives

Review clinical scenarios in pediatric respiratory
illnesses

Question and answer format

Considerations in :
- differential diagnoses
- diagnostic studies and
- treatment options
Question 1
6 month old term infant born without complications is seen at a
routine visit for immunization.
 She is alert, interactive, pink and well appearing.
 Weight is at 50th and length at the 25th percentile for age
 Has intermittent, audible inspiratory stridor
 No retractions or distress and clear lungs on auscultation.




Feeding well with occasional spit up. Is gaining weight.
Mother notes this noisy breathing which she calls
“wheezing”
wheezing” since about 2 weeks age
The “wheezing”
wheezing” had been louder a week ago when she had
a cold and upper respiratory symptoms for a few days.
Family history of asthma in both parents and 2 older
siblings.
The investigation you would consider at this point would be:
(A)
(B)
(C)
(D)
(E)
CXR to evaluate for hyperinflation, asthma
Upper GI study to screen for structural anomalies
Chest CT scan for structural abnormalities
Bronchoscopy to evaluate airways
No investigations now, continue to observe and
monitor clinically as she is thriving and doing well
The investigation you would consider at this point would be:
(A)
(B)
(C)
(D)
(E)
CXR to evaluate for hyperinflation, asthma
Upper GI study to screen for structural anomalies
Chest CT scan for structural abnormalities
Bronchoscopy to evaluate airways
No investigations now, continue to observe and
monitor clinically as she is thriving and doing well
Stridor

Definition: harsh, coarse grating sound with crowing
quality

Caused by airflow obstruction leading to turbulence

PHASE of breathing:
inspiratory
= extraextra-thoracic
expiratory
= intraintra-thoracic
biphasic
= fixed
variable/only in one phase = dynamic obstruction
Extrathoracic Airway Obstruction
Inhalation
0
-4
Normal
+2
Exhalation
0
Thoracic
Cage
-10
-7
Inhalation
-30
0
Extrathoracic
Laryngeal
-40
Obstruction
-37
-4
Airway
+4
Alveoli
Exhalation
0
+38
+32
+40
Causes of stridor in infancy

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


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

Laryngomalacia
Tracheomalacia
Subglottic stenosis
Airway lesionslesions- web, cyst, hemangioma
Vascular malformation: “ring/sling”
ring/sling” anomaly
Vocal cord paralysis
Infection: croup, epiglottitis, bacterial tracheitis
Foreign body aspiration: airway/esophageal
Laryngomalacia







Most common cause of stridor in infants (60%)
Cartilage not as firm
- collapsibility during inspiration
- prolapse of epiglottis, arytenoids during inspiration
Worsened by agitation, supine position
Usually normal voice/cry
Apnea can occur
Feeding dysfunction with severe cases, failure to thrive
(arching position)
GE reflux can worsen laryngomalacia
Laryngomalacia
Normal larynx
Laryngomalacia
Note prolapse of arytenoids
and “curling” of epiglottis
LaryngomalaciaLaryngomalacia-management

Close clinical observation, parental education of
natural history

Symptoms may worsen over first few months

Improvement by 1212-18 months in most cases

Barium swallow/UGI to screen for vascular anomalies

Bronchoscopy and visualization of airway indicated
IF atypical course, failure to thrive

Severe cases may require surgical intervention
Tracheomalacia


Weakness of tracheal walls
Dynamic intraintra-thoracic airway obstruction
- Airways stented open during inspiration




- Collapse during expiration, forceful exhalation, crying
- Worse after a bronchodilator treatment
Mild tracheomalacia
tracheomalacia expiratory noise
Moderate to severe
severe biphasic noise
Primary
Secondary causes: chronic inflammation, GE reflux,
positive pressure ventilation
Tracheomalacia
Antero-lateral flattening of
cartilage of tracheal rings
Airway fluoroscopy
Vascular rings/slings




Cause fixed but pulsatile compression of trachea in variety
of ways
Presentation:
- chronic cough
- inspiratory sounds/stridor,
- wheezing (homophonous)
Study of choice: barium esophagram/UGI, bronchoscopy
May need CT, MRI for detailed cardiovascular anatomy
Vascular compressive lesions





Complete vascular ring encircles trachea and esophagus
Biphasic sounds worse with swallowing
May have dysphagia and vomiting
Double aortic arch with
aberrant left subclavian artery 
Pulmonary sling :
- Left PA arises from Right PA
and not the main PA
- compresses right bronchus
Barium swallow: vascular ring
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Subglottic stenosis

Congenital or acquired
 acquired more severe
 usually a result of endotracheal intubation

Clinical presentation
 Stridor, cough, often “barky”
barky”, recurrent croup
Bronchoscopy for definitive diagnosis and degree of
narrowing
Management based on degree of stenosis
 clinical observation
 tracheotomy
 surgical excision of scar tissue
 cricoid split or laryngolaryngo-tracheal reconstruction (LTR)


Subglottic Stenosis
Normal larynx
Subglottic stenosis
Vocal cord paralysis


Congenital anomaly
Stridor presenting symptom
Acquired: trauma, nerve injury
Obstruction, weak cry, hoarseness, dysphagia, aspiration

Diagnosis: flexible endoscopy at bedside

Etiology:
- Neurological conditions: CNS disease, Arnold Chiari,
birth trauma
- Idiopathic
Management:
Management: maintain adequate airway
Unilateral paralysisparalysis- rarely requires treatment
Bilateral paralysis – often tracheotomy
Idiopathic – high recovery rate



Laryngoscopic view of normal larynx
with vocal cords abducted and adducted
Unilateral vocal cord
paralysis
Bilateral vocal cord
paralysis
Key findings/discerning features
Laryngomalacia
Timing
Feature
Bronchoscopy
Diagnosis
Inspiratory
“Crowing”
Crowing”
Improves with
age,
Curled epiglottis
(“omega”
omega”)
Prolapse of
arytenoids
Clinical features,
natural course
Bronchoscopy
AnteroAntero-posterior
dynamic collapse
of trachea
Flexible
bronchoscopy
Airway
fluoroscopy
↑ with exertion
↑ supine, ↓ prone
Tracheomalacia
Wheezing,
biphasic
Prematurity,
PostPost-surgical,
inflammation
↑ agitation
↑ exertion
Subglottic
stenosis
Biphasic
High pitched
History of
intubation,
trauma
Narrowing of
subglottis
XRays airway,
Bronchoscopy
Vascular ring or
sling
Inspiratory
stridor
wheeze
Indentation of
esophagus on
barium swallow
May have
dysphagia,
reflux
Upper GI study
(indentation of
esophagus)
CT imaging
Vocal cord palsy
Dysphonia
Aphonia
Trauma, CNS
lesions ,cardiac
surgery
Immobile cords
Uni/bilateral
Direct
visualization by
endoscopy
Question 2
2 yr old boy is brought to the ED at midnight with difficulty
breathing and respiratory distress. There are multiple sick
contacts at home this past week




Has had a nasal discharge, cough and fever for 3 days.
Cough increased this evening since bring put to bed.
Appears anxious, tired and toxic.
He is febrile (104 F), HR= 150/min, RR= 40/minute
with lower intercostal and subcostal retractions.
Pulse oximetry is 93% on O2 via facemask at 6 L/min.
CXRay shows ragged appearance of tracheal air column
Your first step in managing this patient is to:
(A) Get a XRay lateral soft tissue neck and airway
(B) Order a chest CT with contrast
(C) Plan urgent bronchoscopy
(D) Intubate in controlled manner
(E) Obtain a stat arterial blood gas
Your first step in managing this patient is to:
(A) Get a XRay lateral soft tissue neck and airway
(B) Order a chest CT with contrast
(C) Plan urgent bronchoscopy
(D) Intubate in controlled manner
(E) Obtain a stat arterial blood gas
“Respiratory distress in a febrile,
tachypneic child with stridor”
stridor”
Differential diagnoses:





Epiglottitis
Laryngotracheobronchitis
Bacterial tracheitis
Acute suppurative pneumonia
Retropharyngeal abscess
Epiglottitis
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
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Most often due to Hemophilus influenzae type b infection
Rare now with immunization practice
Other etiologies: other H. flu species, Staph aureus,
aureus,
group A Strep
Classic:
Classic: muffled voice “hot potato”
potato”, respiratory distress,
toxic appearance
True medical emergency: dysphagia, drooling
“ tripod posture”
posture”
Keep patient calm, AVOID agitationagitation- which will increase
work of breathing
Airway stabilization in controlled setting
Epiglottitis
Normal epiglottis and
glottis
Swollen, inflamed,
beefy red epiglottis
Croup
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
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
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Laryngotracheobronchitis
Viral etiology: parainfluenzae types 1 & 3
RSV, Influenzae A & B, others
Distinguishing feature: viral prodrome
Airway edema: larynx, subglottis - marked narrowing
“barking cough”
cough”, “seal like cough”
cough”, increases at night
Temporary relief with cool mist, humidified air, steam
Responds quickly to adrenergic agonists and steroids
Racemic epinephrine, steroids: oral, IM, inhaled budesonide
Croup: “steeple sign”
sign”
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Bacterial tracheitis








Often in child with croup for several days
Staph aureus : direct bacterial infection of tracheal mucosa
High fever, toxic appearance, severe upper airway
obstruction
Bacterial cultures reveal organism
Endoscopy: thick mucomuco-pus, sloughed epithelium, sheet like
pseudomembrane
Lateral Xray neck: subglottic narrowing, ragged air column
Secure airway; intubation, mechanical ventilation, airway
hygiene, IV antibiotics (Staph
(Staph aureus coverage)
If effective early management
management complete recovery occurs
Airway foreign body

Age group: toddlers commonly


History of choking or aspiration event
RadioRadio-opaque versus radiolucent foreign body

Persistent cough, wheeze  refractory to treatment


Diagnosis: XRays, endoscopy
Airway foreign body: ball valve obstruction, hyperinflation
Esophageal foreign body may compress airway

Right bronchial mainmain-stem foreign body
Asymmetric hyperinflation of right lung
CXR: Right main-stem foreign body
lateral decubitus views
Left lateral decubitus
Right lateral decubitus
Key /discerning features
Age group
Etiological
agent
XRay
features
Treatment
Croup (LTB)
Infancy to
early
childhood
Viral,
Paraflu type 1
“Steeple sign”
sign”
on PA neck
film
Cool mist
Racemic epi
Steroids
Epiglottitis
2-7 years
Group A Strep “Thumb sign”
sign”
Staph aureus on lateral
neck film
Viral
Bacterial
tracheitis
Older children Staph aureus
(Trach: Gram
negatives)
-Subglottic
narrowing
-Ragged air
column
Secure airway
Intubation
Antibiotics,
steroids
Airway
foreign body
Toddlers,
Any age
-Persistent
atelectasis
-Localized
hyperinflation
Rigid
bronchoscopy
for removal
-
Secure airway
Intubate
Antibiotics
Question 3
12 year old girl is hit by a car while rollerblading. She falls
several feet and sustains multiple injuries including closed
head injury, facial trauma, bilateral rib, pelvic and extremity
fractures.
 She is transported by EMS to the nearest trauma center and
is drowsy but arousable.
 Heart rate is 140/minute, regular. Respirations are
24/minute with pulse oximetry saturations of > 94% on
oxygen via face mask. Initial blood pressure was low and
she received parenteral fluids.
 A head CT is done which his normal.
 On returning from CT scan, she is noted to have sudden
duskiness of lips, respiratory distress and cold, clammy
extremities. Blood pressure is low.
 Breath sounds are poorly heard over the right hemithorax
Your first step in managing this child is:
(A) Initiate 100% oxygen via a nonnon-rebreather mask, start
chest compressions and draw up ionotropic drugs
(B) Intubate the patient and start mechanical ventilatory
support
(C) Obtain a stat CXRay
(D) Immediate needle decompression of the right pleural
cavity
(E) Call for an urgent surgical consult for chest tube
placement
Your first step in managing this child is:
(A) Initiate 100% oxygen via a nonnon-rebreather mask, start
chest compressions and draw up ionotropic drugs
(B) Intubate the patient and start mechanical ventilatory
support
(C) Obtain a stat CXRay
(D) Immediate needle decompression of the right pleural
cavity
(E) Call for an urgent surgical consult for chest tube
placement
Pneumothorax

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



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Accumulation of gas in pleural space between parietal
and visceral pleura
“Tension”
Tension”: when pressure > atm. pressure
Spontaneous, asymptomatic in 1% of newborns
Barotrauma: mechanical ventilation
Penetrating injury
Diagnosis: CXR
Management:
- size
- extent of respiratory deterioration
- cardiorespiratory decompensation
Left apical pneumothorax
Patient with Cystic Fibrosis, chronic lung disease
Tension pneumothorax
• Hyperresonance of
affected hemithorax
• Decreased or
absent breath sounds
• Tracheal deviation
•Respiratory distress,
tachypnea
•Hypoxemia
•Hypotension
Diagnosis and management approach in
respiratory distress

Supplemental oxygen first


Presence of bronchobroncho-spasm, wheeze
Acute onset: suspect foreign body aspiration

Assess fatigue; respiratory failure:
- indication for intubation, mechanical ventilation

Intubated patient: “DOPE”
DOPE”

Acute respiratory decompensation
- suspect air leak
- emergency needle decompression first
Question 4
A healthy 10 year old boy, a soccer player has history of
wheezing with respiratory infections especially in winter;
fewer symptoms in summer.
 He has frequent sneezing, nasal congestion and
watering of eyes in spring and fall.
 Uses inhaled bronchodilators (Albuterol) with relief.
 His father notes that he coughs while running and
playing baseball and is visibly short of breath. He coughs
at night time, but this does not awaken him from sleep.
You decide to evaluate his symptoms with spirometry (pre and
post bronchodilator) which assesses:
(A)
(B)
(C)
(D)
(E)
Oxygenation and dede-saturation pre and post exercise
Diffusing capacity of the lungs
Expiratory flow rates and volumes
Maximal minute ventilation and exercise capacity
Total lung capacity, vital capacity, residual volume and
air trapping secondary to small airway obstruction
You decide to evaluate his symptoms with spirometry (pre and
post bronchodilator) which assesses:
(A)
(B)
(C)
(D)
(E)
Oxygenation and dede-saturation pre and post exercise
Diffusing capacity of the lungs
Expiratory flow rates and volumes
Maximal minute ventilation and exercise capacity
Total lung capacity, vital capacity, residual volume and
air trapping secondary to small airway obstruction
Spirometry: Flow volume loop
Measures flow rates in
asthma
 FVC, FEV1, FEF 2525-75%
as percentage predicted



Normal flow volume loop
Patterns in restrictive and
obstructive disease
Spirometry
Obstructive pattern
Restrictive pattern
Mixed pattern
Concave FV loop
↓ FEV1
↓ FEV1/FVC ratio
↓ FEF 25-75%
Normal shaped loop
↓ FVC and ↓ FEV1
Normal FEV1/FVC ratio
Normal FEF 25-75%
Concave FV loop
↓ FVC and ↓ FEV1
↓ FEV1/FVC ratio
↓ FEF 25-75%
Spirometry: air flow patterns in lung disease
Obstructive Restrictive
Mixed
FVC
↓
↓↓↓
↓↓
FEV1
↓↓↓
↓↓↓
↓↓
FEV1/FVC
ratio
↓↓
FEF 2525-75%
(MMEFR)
↓↓
normal or
↑↑
normal or
↑↑
↓↓
normal or
↓↓
NHLBI/NAEPP Asthma guidelines, 2007

“All about asthma control”
control”: new treatment approach:
focus on achieving and maintaining control

Takes into account variability of asthma symptoms
 Consider asthma severity when initiating treatment
 From then on focus on monitoring for asthma control

Monitor level of current impairment and future risk

Inhaled corticosteroids (ICS): preferred therapy across all
age groups
Add leukotriene inhibitor
Step up with combination therapy ICS + Long acting beta
agonist (LABA)


Question 5
9 month old Caucasian infant has daily cough since birth.
Cough is worse with upper respiratory infections and he has
had wheezing. The cough has never really cleared.
 He is active, feeds well and vigorously without choking.
 Does spit up frequently, but is playful and does not appear
distressed.
 Has had colic since the neonatal period with abdominal
bloating and frequent loose stools.
 Weight gain has been slow. Weight is below the 5th
percentile for age, while length is at the 25th percentile.
 There is a family history of asthma and GERD
Your clinical diagnosis at this point before further
investigations is :
(A)
(B)
(C)
(D)
(E)
Mild persistent asthma
Cystic fibrosis
Gastroesophageal reflux
Swallowing dysfunction with micromicro-aspiration
Bilateral vocal cord palsy, growth and developmental
delay
Your clinical diagnosis at this point before further
investigations is :
(A)
(B)
(C)
(D)
(E)
Mild persistent asthma
Cystic fibrosis
Gastroesophageal reflux
Swallowing dysfunction with micromicro-aspiration
Bilateral vocal cord palsy, growth and developmental
delay
Cystic fibrosis





Autosomal recessive
Caucasians: 1 in 3300 affected,1 in 25 carrier
More than 1000 known mutations on chromosome 7
Most common mutation: delta 508
(deletion of phenylalanine at position 508)
8585-90% are pancreatic insufficient
Diagnostic tests:
 Need high index of suspicion
 Sweat test: gold standard; high sweat chloride
 Newborn screen: elevated IRT level,
level, mutation screen
Cystic Fibrosis

Chronic, progressive, life limiting inherited disease

MultiMulti-system involvement

Hallmarks of CF :
-chronic respiratory disease
-pancreatic insufficiency, failure to thrive
-elevation of sweat chloride and
-male infertility
Multi-system manifestations of Cystic Fibrosis
CF: pancreatic exocrine insufficiency
Malabsorption,
steatorrhea
Malnutrition
Rectal prolapse
CF: pulmonary exacerbations

Abnormal, viscous sputum

Productive cough, weight loss,
decrease in pulmonary function with
airflow obstruction

Respiratory microbiology :
- Infancy: Staph aureus, H. influenzae
- eventually: Pseudomonas aeruginosa
(80% colonized by age 8 -10 yrs)
Signs and Symptoms of Pulmonary
Exacerbation

Increased cough

New chest exam findings
- crackles, wheeze

Increased volume of
sputum, change in
sputum color

Weight loss

School/work absenteeism
Increased dyspnea


Decreased FEV1
(interval decrease of 10%)

Decreased exercise
tolerance

New radiographic
findings, lung infiltrate
Diagnosis: CF pulmonary exacerbation




History – increase or change in sputum quality,volume
Physical examination: tachypnea, retractions, decrease in
oxygen saturation, crackles, wheeze
Pulmonary function teststests- decline in FEV1, airflow obstruction
Sputum culture and sensitivity

Radiology : CXRCXR- hyperinflation, infiltrates
High resolution chest CT scanscan-bronchiectasis

Flexible bronchoscopy and bronchobroncho-alveolar lavage
CF mild disease:
hyperinflation,
increased markings
CF advanced disease:
with bronchiectasis
High yield facts for the boards on CF







Newborn screening for CF
- screens with IRT (immuno(immuno-reactive trypsinogen) level and
genetic mutation screen
- positive newborn screen
screen need to obtain sweat test
Sweat chloride test : elevated ClCl- level, gold standard
Suspect CF in infant with chronic cough, wheeze,
malabsorption, diarrhea, failure to thrive
Sputum/respiratory culture with Pseudomonas aeruginosa pathognomonic of CF
CF: risk for dehydration with hyponatremic, hypochloremic,
metabolic alkalosis
PFTS:
PFTS: obstructive defect (small airway obstruction)
Infertility in males: obstruction of vas deferens
Additional review questions
on Cystic fibrosis for the boards
CF: question 1
Newborn screening for cystic fibrosis as conducted in
several states in the U.S. tests for
(A) Elevation in sweat chloride level
(B) Blood immunoimmuno-reactive trypsinogen (IRT) level
(C) Stool: fecal elastase level
(D) Genetic mutations of CF including delta 508
CF: question 1
Newborn screening for CF conducted in several states in the
U.S. tests for
(A) Elevation in sweat chloride level
(B) Blood immunoreactive trypsinogen level
(IRT) *
(C) Stool fecal elastase level
(D) Genetic mutations of CF including delta 508
* Viscid pancreatic secretions, pancreatic ductular
inflammation and obstruction in utero result in elevation in
blood immuno-reactive trypsinogen level
CF: question 2
In order to definitively diagnose and inform parents that
their child has CF you must have:
(A) A positive newborn screen (high IRT) and at least one
parent is a known carrier for CF
(B) A positive newborn screen in a child with meconium
ileus, steatorrhea and elevated fecal elastase level
(C) Elevated sweat chloride on sweat test
(D) Genetic testing positive for two mutations for CF
CF: question 2
In order to definitively diagnose and inform parents that
their child has CF you must have:
(A) A positive newborn screen (high IRT) and at least one
parent is a known carrier for CF
(B) A positive newborn screen in a child with meconium
ileus, steatorrhea and elevated fecal elastase level
(C) Elevated sweat chloride on sweat test
(D) Genetic testing positive for two mutations for CF
* Sweat test remains “gold standard” for diagnosis of CF
CF: question 3
The most common reason for a false negative sweat
test is:
(A) Age less than 1 month
(B) Patient’
Patient’s weight less than 10 lbs
(C) Acute illness with dehydration
(D) Inadequate volume of sweat collected
(E) Laboratory error in chloride estimation
CF: question 3
The most common reason for a false negative sweat
test is:
(A) Age less than 1 month
(B) Patient’
Patient’s weight less than 10 lbs
(C) Acute illness with dehydration
(D) Inadequate volume of sweat collected
(E) Laboratory error in chloride estimation
* NEED at least 75 mg of sweat for a valid test
Cystic Fibrosis Screening Algorithm
IRT Screening Test
(Immunoreactive Trypsinogen)
“Elevated” IRT
Top 5%
“Normal” IRT
Bottom 95%
CF mutation screening
1 CF mutation
Negative Screen
2 CF mutations
no CF mutations
>170 mg/mL
Sweat test
at CF center
-
+
Sweat test
at CF center
+
<170 mg/mL
Sweat test at CF center
+
CF Diagnosis
CF carrier
Further work-up,
as indicated.
Sweat test


“Gold standard”
standard” for diagnosis of CF
Carefully performed quantitative pilocarpine iontophoresis
test

Generally should be done in duplicate, repeated at least
once to confirm

Sweat chloride level: normal < 40 mEq/L
borderline 4040-60 mEq/L
positive > 60 mEq/L

False positive sweat test: malnutrition, adrenal insufficiency,
nephrogenic diabetes insipidus, hypohypo-gammaglobulinemia

False negative sweat test: edema with hypoproteinemia
CF: question 4
The major cause of death in Cystic Fibrosis is:
( A) Malnutrition,cachexia due to fat malabsorption and
steatorrhea
(B) Respiratory infections and their complications
(C) Cystic fibrosis related diabetes (CFRD) and its
complications
(E) Septicemia and shock due to multimulti-drug resistant
bacterial strains
CF: question 4
The major cause of death in Cystic Fibrosis is:
( A) Malnutrition,cachexia due to fat malabsorption and
steatorrhea
(B) Respiratory infections and their complications
(C) Cystic fibrosis related diabetes (CFRD) and its
complications
(E) Septicemia and shock due to multimulti-drug resistant
bacterial strains
CF: question 5
A 6 year old has chronic nasal discharge, moist cough, nasal
obstruction and snoring. The nasal exam is as below, your next
step in diagnosis is :
(A) CBC diff fpr eosinophila,
total IgE level
(B) Skin testing for
environmental allergens
(C) Sweat chloride test
(E) CT scan of the sinuses,
refer to ENT for surgery
CF: question 5
A 6 year old has chronic nasal discharge, moist cough, nasal
obstruction and snoring. The nasal exam is as below, your next
step in diagnosis is :
(A) CBC diff fpr eosinophila,
total IgE level
(B) Skin testing for
environmental allergens
(C) Sweat chloride test
(E) CT scan of the sinuses,
refer to ENT for surgery
* Nasal polyps in children – ALWAYS rule out CF:
CF:
polyps rarely due to allergies in children, unlike adults
CF: question 6
A 12 yr old Asian boy, recently migrated to the U.S. has
history of recurrent pneumonia, chronic productive cough
and poor weight gain with loose foul smelling stools.
He has bronchiectasis on chest CT and undergoes flexible
bronchoscopy; the organism you are most likely to recover
from his respiratory cultures is:
(A) Mycobacterium tuberculosis
(B) Fungus: Aspergillus species
(C) Pseudomonas aeruginosa
(D) Pneumocystis jiroveci
CF: question 6
A 12 yr old Asian boy, recently migrated to the U.S. has
history of recurrent pneumonia, chronic productive cough
and poor weight gain with loose foul smelling stools.
He has bronchiectasis on chest CT and undergoes flexible
bronchoscopy; the organism you are most likely to recover
from his respiratory cultures is:
(A) Mycobacterium tuberculosis
(B) Fungus: Aspergillus species
(C) Pseudomonas aeruginosa
(D) Pneumocystis jiroveci
Question 6
18 yr old girl has chronic cough since childhood, frequent
ear and sinus infections, recurrent pneumonia and
asthma. Has had frequent antibiotic courses,
hospitalizations for pneumonia less frequent as she has
grown older.
 She uses an Advair inhaler twice daily, takes Singulair at
bedtime and Albuterol as needed.
 Has a daily cough productive of yellowishyellowish-green sputum.
She is able to tolerate moderate activity, but reports
shortness of breath with exertion. Denies smoking or use
of recreational drugs.
You review her recent CXRay:
Question 6: CXRay
R
Your diagnosis based on her history and CXRay is
(A) Bronchiectasis with acute exacerbation
(B) Acute asthma exacerbation
(C) Cystic Fibrosis
(D) Kartagener’
Kartagener’s syndrome
(E) Common variable immune deficiency (CVID) with
frequent infections
Your diagnosis based on her history and CXRay is
(A) Bronchiectasis with acute exacerbation
(B) Acute asthma exacerbation
(C) Cystic Fibrosis
(D) Kartagener’
Kartagener’s syndrome
(E) Common variable immune deficiency (CVID) with
frequent infections
Kartagener’
Kartagener’s syndrome

Situs inversus, “mirror image”
image”
- reversal thoracic and abdominal organs

Chronic sinusitis

Bronchiectasis
R
Bronchiectasis


Dilated, damaged airways
Due to infection, airway obstruction, inflammation

Sequela of insult : infection, obstruction, mucus stasis,
pathogen colonization

Types:
- Saccular/cystic
- Cylindrical/tubular
Causes of bronchiectasis








Cystic fibrosis
Primary ciliary dysfunction (PCD)
Kartagener’
Kartagener’s syndrome
Immune deficiency: recurrent infection
Tuberculosis
ABPA
(allergic bronchopulmonary
aspergillosis)
Bronchial stenosis ,obstruction, airway
compression
Injury: chronic aspiration or infection
Bronchiectasis
•Chronic productive cough, crackles on auscultation
•Failure to thrive, clubbing
Radiology :
- bronchial wall thickening
and dilatation
- honeycomb pattern
- mucus impaction
- CXR: “tramtram-track”
track” sign
- HRCT: “signet ring”
ring”
Bronchiectasis: management


Airway clearance of mucus
Aggressive use of antibiotics

Daily chest physical therapy:
- percussion, clapping, postural drainage
- mechanical percussor vest
- Acapella, flutter devices

Regular care: serial pulmonary function tests, sputum
cultures can be used to guide antibiotic therapy
Question 7
12 year old girl with trisomy 21 with asthma is on inhaled
steroids (Flovent) as daily medication and receives
immunotherapy for allergic rhinitis.
 She is not very physically active and has gained 15 lbs
weight over the past year.
 Had been doing well in special education until recently.
Has been irritable in class with poor attention span and
not been learning well.
 Parents admit that they have not been regular with her
inhaled asthma medications and that she has a daily
cough.
 She has always had snoring but this has increased in
recent months with restless sleep and frequent
awakenings.
Your priority in her management at this time is:
(A) Refer her for a sleep study
(B) Increase inhaled asthma medication regimen including
a long acting beta agonist for better control
(C) Referral for screening and evaluation for ADHD
(D) Nutrition counseling for weight gain
(E) Repeat skin testing for environmental triggers, intensify
treatment for allergic rhinitis and nasal obstruction
causing snoring
Your priority in her management at this time is:
(A) Refer her for a sleep study
(B) Increase inhaled asthma medication regimen including
a long acting beta agonist for better control
(C) Referral for screening and evaluation for ADHD
(D) Nutrition counseling for weight gain
(E) Repeat skin testing for environmental triggers, intensify
treatment for allergic rhinitis and nasal obstruction
causing snoring
Sleep disordered breathing
Considerations:
 Age, developmental stage
 Craniofacial malformations
 Neuromuscular disorders, hypotonia
 Obesity, recent weight gain
 Snoring, mouth breathing, choking, pauses in breathing
 Restless thrashing in sleep, bruxism
 Difficult to awaken in morning, daytime fatigue, somnolence
 Learning difficulties,
difficulties, poor school performance, behavioral
issues
Obstructive sleep apnea (OSA)

Recurrent events of partial or upper airway obstruction
during sleep that result in disruption of ventilation and
normal sleep patterns

Primary OSAS:
- children 22-6 yrs age with adenoadeno-tonsillar hypertrophy

Secondary OSAS:
- craniofacial anomalies,
- neurological disorders affecting upper airway shape,
configuration and collapsibility during sleep

Diagnosis: polysomnographypolysomnography- gold standard
OSA

Physiological consequences:

hypoxemia : SpO2 < 90%

hypoventilation
hypoventilation hypercarbia, right heart strain,
cor pulmonale

sleep fragmentation, altered sleep architecture
Effect on growth, neurologic and cardiac function

Risk factors:
 adenotonsillar hypertrophy
 obesityobesity- upper airway adipose tissue
 craniofacial, neurologic : affect upper airway patency
 trisomy 21: large tongue, obesity, hypotonia

Management:
Management: adenotonsillectomy, CPAP/BIPAP
Question 8
17 yr old league football player had an ankle fracture last year
which prevented him from playing an entire season.
 Is anxious to get back on the team this year.
 Has exercise induced asthma and seasonal allergies, and
uses Albuterol before sport with benefit



Presents now with shortness of breath when playing and
reduced exercise tolerance
He finds it extremely hard to breathe, feels like his “chest is
closing and he cannot catch a breath”
breath” within few minutes of
playing.
Also reports palpitations and severe chest pain and has to
stop playing until symptoms subside. He is very anxious as
this has affected his game and performance on the team.
Of the conditions listed below, the most likely diagnosis at
this time would be:
(A) Inadequately controlled asthma that is exercise
induced
(B) Chronic sinusitis
(C) Suboptimal/poor physical conditioning
(D) Vocal cord dysfunction
(E) Undiagnosed cardiac condition unmasked during
exercise
Of the conditions listed below, the most likely diagnosis at
this time would be:
(A) Inadequately controlled asthma that is exercise
induced
(B) Chronic sinusitis
(C) Suboptimal/poor physical conditioning
(D) Vocal cord dysfunction
(E) Undiagnosed cardiac condition unmasked during
exercise
Vocal cord dysfunction








Vocal cords close paradoxically in inspiration
inspiration airflow
obstruction at larynx
larynx audible wheeze, stridor
Acute presentation dramatic, “throat tightness”
tightness”
Symptoms often start and cease abruptly
Oxygenation, arterial blood gases normal
Does not respond to adequate asthma therapy
Majority in females ; high IQ, high achiever
Normal spirometry;
spirometry; avoid unnecessary treatment with
steroids
Diagnosis: visualization of vocal cords on endoscopy,
classic “glottic chink”
chink”
Normal and abnormal laryngoscopy
results
Perkner JJ et al, J Occup Environ Med 1998; 40: 136-143
Spirometry: Vocal cord dysfunction
Normal flow volume
loop on spirometry
Truncated or flattened
inspiratory curve is
suggestive of VCD
Question 9
A 4 yr old who recently migrated to the US from Southeast
Asia, has nasal discharge, dry cough and intermittent
fever. Now has decreased oral intake, fever of 102 F,
abdominal pain and recent weight loss.
 He is grunting, mildly tachypneic with intercostal
retractions. Breath sounds are diminished and harsh,
tubular over the right chest, with scattered crackles.
There is no wheeze.
 Labs show an white count of 22,000 with shift to the left,
ESR of 64mm/hour and decreased serum sodium of
130mEq/L
 A PPD is placed, blood culture is drawn and CXR is
done.
Question 9: CXR
The most likely etiology/pathogen is:
(A) Mycoplasma pneumoniae
(B) Adenovirus pneumonia
(C) Mycobacterium tuberculosis
(D) Streptococcus pneumoniae
(E) Methicillin resistant Staphylococcus aureus
(MRSA)
The most likely etiology/pathogen is:
(A) Mycoplasma pneumoniae
(B) Adenovirus pneumonia
(C) Primary tuberculosis
(D) Streptococcus pneumoniae
(E) Methicillin resistant Staphylococcus aureus
(MRSA)
Pneumonia: etiology according to age

Neonate:
Group B streptococci (GBS)
Escherichia coli and other fecal coliforms
Chlamydia trachomatis

Young infant:
Perinatally acquired pathogens, Listeria monocytogenes
Community acquired: Strep pneumoniae,Staph aureus,
Hemophilus influenzae.

Young children
Viral:
Viral: RSV, Influenza, Adenovirus, other
Community acquired as above

Older children:
Atypical infection:
infection: Mycoplasma, Chlamydia
Viral:
Viral: as above
Community acquired : Pneumococcus common
Bacterial pneumonia

Classic: Pneumococcal , fever, chills rusty sputum,
defervescence with antibiotics

Radiological:
lobar pattern: alveolar/air space disease with

consolidation, air bronchograms
bronchopneumonia: infection of airways, obstruction

and spread to air spaces
interstitial pattern: affects tissue spaces between

alveoli and vasculature, linear, reticular, nodular

XRays lag behind clinical findings:
findings: take 44- 6 weeks to
resolve

Complications:
Complications: effusion, empyema, abscess,
bronchobroncho-pleural fistula
Viral pneumonia





Cause majority of pneumonia in children
Difficult to distinguish from bacterial
Usually prodrome, URI  progresses to cough 
worsening tachypnea, malaise, lethargy
Clinically: more tachypneic, more hypoxemic
CXR : interstitial markings, peribronchial thickening
hyperinflation, infiltrates
Viral pneumonia:
Interstitial pattern on CXR
Atypical or “walking”
walking” pneumonia





“Atypical”
Atypical”: did not respond to “usual”
usual” antibiotics
Clinically not as sick or debilitated
Mycoplasma, Chlamydia
Insiduous onset: malaise, low grade fever, cough
Clinical signs: crackles, wheeze, coarse breath sounds

CXRay: bilateral interstitial filtrates, patchy consolidation,
effusion in up to 20%

Cold agglutinins: antibodies against RBC antigen
(may be absent in 50% cases however)
Mycoplasma pneumonia
Bronchopneumonia with airway obstruction (hyper inflation)
Aspiration pneumonia


Clinical syndrome of aspiration of oral or gastric content
into the bronchial tree
(Other: foreign body/material, near drowning)

Acidity of aspirate
aspirate chemical pneumonitis
Oropharyngeal bacterial pathogens  anaerobes add to
inflammation

Fever, cough, wheezing, hypoxemia

Typical locations:
 Right lower lobe, Right middle lobe
 Upper lobes in infants, supine position
