Presenters DisclosuresRonald E. Bokulic D.O.

• Personal financial relationships with commercial interests relevant to medicine , within the past 3 years: None

• Personal financial support from a non-commercial source relevant to medicine, within the past 3 years: None

• Personal relationship with tobacco industry entities within the past 3 years : None

• Recognize and understand common and uncommon respiratory problems.

• Recognize that a common presentation of a respiratory illness may not represent a common respiratory illness

This is a 6 ½ year old male with recurrent pneumonias and wheezing

• First wheeze at 13 months of age

• Asthma diagnosed at 2 years of age

• Parents cannot describe a wheeze and the father disagrees that there is a wheeze

• Triggers : URI, fall – spring season, etc.

• Febrile to 101o F with ½ of the episodes

• Emergency room 3-4 times a year

• Exacerbations can last 2 weeks with daily to every other day “wheezing “

• Cough : tight, dry, barky, both day and night with an every 3 week cycle

• The cough may cause facial petechiae

• Exercise causes noisy breathing but he keeps up until he begins to cough

• Seasonal allergies but conflicting allergy testing less than 1 year apart

• Growth, birth history, and family history are unremarkable

• Therapy with montelukast, an antihistamine, and albuterol prn which helps

• He is treated with oral steroids and antibiotics at least twice a year

• No reflux symptoms• No choking, coughing or gagging while

eating• Carrot removed from the trachea at 13

months ( “pus” and narrowing of the trachea do to edema)

• Coin removed from the esophagus 6 months later

PHYSICAL• Weight – 22 kg

• Height – 110 cm

• Respiratory rate –20 b/min

• Heart rate – 80 b/min

• Blood pressure – 96/55 mmHg

• Oxygen saturation – 100% on room air

PHYSICAL• Well developed well nourished male

• Enlarged nasal turbinates 80% of nasal passage

• Mild tonsillar hypertrophy

• Trachea midline and thorax normal

• Lungs are without adventitial breath sounds

• No clubbing, edema, or cyanosis

Chest x- ray

Spirometry

DIFERENTIAL DIAGNOSIS

• Asthma

• Recurrent pneumonia

• Recurrent croup

• Immune deficiency

• Foreign body

THERAPY • Inhaled steroids at a high dose for

1- 2 months and then lower the dose

• Albuterol as needed with ipatropium bromide to be added if albuterol does not improve the acute symptoms

.He returns emergently 6 weeks later with a persistent cough and wheezing with minimal benefit from the medications. He was afebrile with a respiratory rate of 24 b/min and an oxygen saturation of 96%. Rhinorrhea, oropharyngeal cobblestoning and course upper and lower airway noise without wheezing.

TESTING• Bronchoscopy with BAL• Computerized Tomography of

the chest • MRI of the chest• Immune work up• Methacholine challenge

CHEST CT

DIFFERENTIAL DIANOSIS

• Asthma

• Mediastinal Tumor

• Bronchogenic cyst

• Intrathoracic meningocele

• Encapsulated abscess

• Tracheal stricture at the carina

BRONCHOGENIC CYSTS

.

CHEST MRI

THERAPY

SURGICAL RESECTION

BRONCHOSCOPY

BRONCHOALVEOLAR LAVAGE

• Cytology: 95% macrophages

3% lymphocytes

2% polymorphonuclear cells

• Viral, bacterial, and fungal cultures were no growth

• No lipid laden macrophages

BRONCHOGENIC CYSTS

• Foregut Duplication Cysts

Bronchogenic or Enterogenic

• Usually asymptomatic ( vague substernal discomfort, cough , noisy breathing , dyspnea and cyanosis )

• Subcarinal cysts and respiratory distress

BRONCHOGENIC CYSTS

• 7-15% of foregut cysts in infants/child

• More prevalent in adults

• Usually single and large

• 20% separate from bronchial tree

• Free in mediastinum or attached to pleura, esophagus, or pericardium

• Associated with other anomalies

BRONCHOGENIC CYSTS

• Cysts are thin walled

• Lined with ciliated respiratory epithelium and mucous glands

• Surrounded by muscle and fibrous tissue

• Rare anomalous arterial or venous supply

• Disrupted embryologically with bronchial tissue separating to form a cyst

BRONCHOGENIC CYSTS

• Five major locations• 51.5% carinal region (airway compression)

• 19% right paratracheal region

• 13.8% paraesophageal

• 8.6% hilar region (on/near large bronchi)

• 6.9%pericardial,retrosternal,or paravertebral

BRONCHOGENIC CYSTS

• CAT scan localizes lesion and defines the cyst

• Barium swallow may show compression of the esophagus

• Bronchoscopy and bronchograms are usually not necessary

BRONCHOGENIC CYSTS

•Surgery is indicated for symptomatic patients

Eight month old with a one month history of wheezing and

more difficulty breathing

HISTORY• Describe the wheeze

• How often and when did it start

• What medications give relief and how often do you use them

• Triggers

• Severity

• Dyspnea at rest and / or exercise

HISTORY• Growth and feeding ( CCG or diaphoresis)

• Cough (character and timing )

• Reflux symptoms

• Foreign body history

• Birth history

• Exposures (daycare or siblings)

• Character of voice and cry

PHYSICAL• HR - 150 RR - 70 O2 saturation 93%

• Distressed white male

• Head : NT / NC anterior fontanel flat

• Nares & Mouth : clear

• Trachea : midline without adenopathy

• Thorax : Hyperresonant on percussion with suprasternal and intercostal retractions

PHYSICAL• Lungs : Distant breath sound with both

polyphonic and monophonic wheezes

• RR after a bronchodilator(62) with minimal change in wheezing or work of breathing

• Cardiac : Tachycardia

• Abdomen : soft without masses

• Extremities : no clubbing, edema, or cyanosis

DIFFERENCIAL DIAGNOSIS

• Bronchiolitis

• Cystic fibrosis

• Aspiration Pneumonitis

• Airway anomaly

• Congenital heart disease

DIFFERENCIAL DIAGNOSIS

• Interstitial Lung Disease of Childhood

• Immunodeficiency with recurrent infections

• Pneumonia

• Foreign body

• Croup

• Environmental exposure to smoke

DIAGNOSTIC STUDIES

•Chest x-ray

DIAGNOSIS

• Esophageal foreign body

FOREIGN BODIES• 8% of accidental deaths less the 5 years old

• 65-70% of all foreign bodies less than 5 yoa

• Peak 1 - 2 years of age

• Male more common than female 2 : 1

• Complications of laryneotracheal foreign bodies are 4-5 time greater than other

FOREIGN BODIES

• 18% Laryngeal or tracheal

• 41.5% Right mainstem bronchus

• 34.5% Left mainstem bronchus

• No left to right dominance ( depends )

• Symptoms are nonspecific

• 57% symptom free period

• 54% referred in less than 3 days

• Greater than 4 weeks for 10 - 20 %

FOREIGN BODY• DIAGNOSIS• Chest x-ray (6 to 80% normal )

• Most foreign bodies are radiolucent

• Lateral neck if laryngeal symptoms

• Inspiratory, expiratory or lateral decubitus

• 33% of positive CXR have negative bronchoscopes

FOREIGN BODY• THERAPY• Preventive measures best

• Flexible vs. rigid bronchoscopy

Six month old male with a three month history of tachypnea and

wheezing

• Triggers - viral URI

• Characteristics and timing of the wheeze

• Hospitalized 2 months ago and treated with bronchodilators, steroids, and antibiotics

• Wheezing stopped 1 week later but tachypnea remained ( 60 - 70’s )

• Growing, happy and playful

• No reflux or feeding issues

Afebrile HR - 130 RR- 76 O2 sat. 99 %.

DIFFERENTIAL DIAGNOSIS

• Reactive airways ( asthma/ bronchiolitis )

• Chronic aspiration

• Congenital heart disease with CHF

• Airway anomaly ( ring, sling, etc. )

• Interstitial lung disease childhood

• Well child

• Restrictive lung process (neuromuscular)

DIAGNOSTIC TESTS

•CHEST X-RAY

ANY OTHER TESTS

• HRCT examination of the chest

• Video Swallowing Study

• Abdominal X-ray

• Metabolic profile with liver enzymes

• MRI of the brain

DIFFERENTIAL DIAGNOSIS

• Abdominal mass causing a restrictive lung process

• Wilms tumor, neuroblastoma, lymphoma , hepatic tumor or hepatomegally, stool impaction

WHAT NEXT ?CT OF THE ABDOMEN

LABORATORY TESTS

• Normal except for an elevated sedimentation rate

• Remove the mass

• Respiratory rate was 30 –40 B/min 36 hours after surgery

•WILMS TUMOR

•Stage I

14 YEAR OLD WHITE FEMALE WITH SEVERE WHEEZING AND UNCONTROLLABLE ASTHMA

HISTORY• Age when first diagnosed

• How often do you wheeze

• What medications give relief and how often do you use them

• Triggers

• Severity

• Dyspnea at rest and / or exercise

• Nighttime symptoms

HISTORY• Frequency of exacerbations

• Days missed of school

• Cough

• Nasal symptoms

• Reflux symptoms

PHYSICAL• HR- 83 RR- 14 BP- 120/73 O2 sat 96%

• Overweight white female slightly cushingoid in appearance

• Nares: See photo

• Mouth: post nasal drip and cobblestoning

• Trachea: midline, shoddy nodes

• Thorax: symmetric without retractions

PHYSICAL• Lungs: good breath sounds, without

wheezing, stridor or crackles

• Cardiac: RRR normal S1 and S2

• Abdomen: soft without masses

• Extremities : no clubbing, edema or cyanosis

• Skin : eczema

YOU HAVE EXAMINED THIS GIRL TWICE, BOTH

TIMES ONE WEEK AFTER HOSPITAL

DISCHARGE AND SHE HAD A NORMAL EXAM

.

DIFFERENTIAL DIAGNOSIS

• Hypersensitivity pneumonitis

• Asthma

• Psychogenic

• Congenital or acquired heart disease

• Immunodeficiency with recurrent viral and bacterial pneumonia

• Foreign body

DIAGNOSTIC STUDIES

• Chest x-ray

• Spirometry

• Immune work-up (screening)

• ABPA work-up

• Allergy testing

• Hypersensitivity work-up

• Better H&P

• An ICU nurse from a local hospital mentions that this patient seems to be in the ICU the same time almost every month

HISTORY• Next visit you ask onset of

menses and association with asthma exacerbations

• Present for 2 years since onset of menses and symptoms begin 3 - 4 days prior to menses

DIAGNOSIS

•CATAMENIAL or PREMENSRAL ASTHMA

CATAMENIAL ASTHMA

• 30 to 40% of women with asthma in childbearing years experience a premenstrual worsening of symptoms

• Peaks 2 - 3 days prior to menses

• Decrease in FEV1/FVC immediately prior and during menses

• Methacholine and histamine challenge increase AHR

CATAMENIAL ASTHMA

• A subgroup will have severe symptoms with a poor response to controller medications

• Controversial: others have not seen the associated changes in peak flows or provocation studies

FOLLOW UP• This patient was placed on suppressive

therapy and over a 2 year period was hospitalized once for 48 hours without an ICU admission

• She was treated with oral steroids twice and has decreased the dosage of inhaled steroids

CATAMENIAL ASTHMA

• Mechanism unknown

• Related to estrogen and progesterone

• Testosterone suppresses asthma in females

• Airway caliber, growth rates and BMI may play a factor

• Timing of menarche

CLINICAL CASEMale, 3 months of age, wheezing since the second

week of life

HISTORY• Circumstances at onset

• Frequency, duration, severity, triggers

• Birth history: antenatal and postnatal

• Parent needs to describe a wheeze / noise

• What makes it worse or better

• Feeding, growth, cyanosis, work of breathing

PHYSICAL• HR - 130 RR- 36 BP- 67/38 O2 sat 99%

• Weight 75% Height 50%

• Nares: patent ( 8 Fr catheter passes easily )

• Trachea : midline

• Thorax : symmetric, no dullness

• Lungs : upper airway noise, mild stridor

• Extremities : no clubbing, edema, or cyanosis

DIFFERENTIAL DIAGNOSIS

• “RAD” asthma or bronchiolitis

• Recurrent URI’s ( immune deficiency )

• Congenital Subglottic Stenosis

• Laryngomalacia / Tracheomalacia

• Foreign body

• Laryngeal web vs. paralyzed vocal cord

• Normal child

DIAGNOSIS

• LARYNGOMALACIA

MECHANISM• Medial collapse of aryepiglottic

folds

• Anterior collapse of arytenoids

• Posterior collapse of an omega shaped epiglottis

DIAGNOSTIC TESTING

• None

• Oximetry

• Polysomnography

• Nasopharyngeal laryngoscopy

• Bronchoscopy

LARYNGOMALACIA• Most common congenital upper airway

anomaly

• Usually mild & diagnosed clinically

• Not a true anomaly but a delayed maturation of support structures

• Generally noticed in neonatal period frequently after first URI

• Almost all present by 6 weeks and a few outgrow it by 2 -3 months, all by 17 - 24 months

LARYNGEOMALACIA

• Primarily inspiratory stridor that varies with inspiratory force

• Louder with crying, feeding & URI

• Usually worse supine than prone

• Quiet to minimal noise when sleeping

• Significant obstructive symptoms rare

• 10% have a mild expiratory component

LARYNGOMALACIA• 15% of laryngomalacia can coexist with

other laryngeal and tracheal anomalies

• Tracheoesophageal fistulas• Esophageal atresia• Congenital webs or subglottic stenosis• Airway hemangiomas• Rings or slings

LARNGOMALACIA

• Diagnosis is make on clinical exam

• Atypical cases bronchoscopy or laryngoscopy

• Sleep disturbed patients may need polysomnography

LARYNGOMALACIA

• Therapy is observation and reassurance

• If the child has a decreased ability to eat, poor growth, cyanotic episodes or life threatening events then therapy

• Oxygen therapy or CPAP

• Epiglottoplasty

• Tracheostomy

This is a three year old with recurrent

pneumonias.

• Five episodes of pneumonia clinically diagnosed.

• Febrile to 104o F • Tachypnea, lethargy, increased work of

breathing and pallor• Two to three weeks to recover after

antibiotic therapy• One hospitalization at 6 months of age

• Well in between episodes • No wheezing and normal growth• Eats well without choking, coughing or

gagging• No foreign body history • No regurgitation • No other infections, rashes, blisters, boils• Ill exposures at daycare

• Well developed well nourished female in no distress

• Heart rate 97 b/min

• Respiratory rate 23 b/min

• Blood pressure 95/56 mmHg

• Oxygen saturation 99% on room air

• Normal examination

• Chest x-ray done 2 weeks prior to the office visit when she was diagnosed with a pneumonia

DIFFERENTIAL DIAGNOSIS

• Day care and repeated exposure • Immune deficiency• Foreign body aspiration• Congenital anomaly ( sequestration,

CCAM, etc.)• Immotile cilia syndrome • Airway anomaly ( stenosis, agenesis,

bronchiectasis)

• Chest x- ray PA and Lateral

• Immune testing ( screening )

• Sweat test

• Bronchoscopy

• MRI of the chest

CT of the chest or MRI

PULMONARY SEQUESTRATION

Surgical removal

SEQUESTRATION

• Ectopic or dislocated bronchopulmonary mass with anomalous systemic arterial supply

• May connect to bronchial tree

• Normal pulmonary artery with normal or anomalous venous drainage

SEQUESTRATION

• Intrapulmonary - 90% of sequestrations

• 60% posterior basal of left lower lobe

• May be an accessory segment at the expense of a bronchial segment

• Usually segmental or less

• Bronchial communication is absent or abnormal

SEQUESTRATION

• Usually no problems until it is infected - mid childhood early adult

• Fever, cough, suppuration, hemoptysis, rarely cardiovascular

EXTRAPULMONARY SEQUESTRATION

• 90% in the left lower lung

• 90% with aberrant systemic artery

• Almost always from thoracic aorta

• Usually small with low blood flow

• Venous drainage to right atrium, vena cava, or azygos v.

• .

An 8 month white male with recurrent

pneumonias

HISTORY• Cough began at 2 months

• No wheezing

• Frank regurgitation

• Afebrile

• Mild tachypnea without retractions or distress

• Growing with a good oral intake

HISTORY• Birth history is unremarkable

• Two hospital admissions at 2 and 4 months of age for pneumonia

• Parents feel he was unchanged after both hospital admissions

• Good cry

• Happy and playful

PHYSICAL EXAMINATION

• Respiratory rate – 36 b/min

• Heart rate - 122 b/min

• Blood pressure - 87/50 mmHg

• Afebrile

• Well developed well nourished white male in no acute distress

PHYSICAL EXAMINATION

• HEENT - Normal

• Trachea - deviated slightly to the left

• Thorax - Very subtle change of the left anterior thorax

• Lungs – no adventitial breath sounds but a decrease in the left upper lobe

• No clubbing edema or cyanosis

PREVIOUS STUDIES

• Laboratory studies were normal

• Capillary blood gas pH – 7.38 and a CO2

of 41mmHg

• Chest x-rays suggested left upper lobe pneumonia

• Bariums swallow demonstrated reflux

• A impedance study was abnormal at 10%

DIFFERENTIAL DIAGNOSIS

• Aspiration pneumonia

• Immunodeficiency

• Foreign body aspiration

• Community acquired pneumonia

• Sequestration

• Atelectasis

• Agenesis of the left upper lobe

• Agenesis of the left lung

OTHER TESTING

CT EXAMINATION OF THE CHEST

OTHER TESTING

DIFFERENTIAL DIAGNOSIS

• Pulmonary sequestration• Bronchial atresia• Atelectasis• Unilateral emphysema with

compression of the contralateral lung• Intraluminal or extraluminal mass

compressing the airway• Agenesis of the lung

Agenesis of the left upper lobe

• Supportive

• Treat the associated anomalies

• Prevent respiratory infections

PULMONARY AGENESIS

DIVIDED IN 3 SUBGROUPS• Agenesis – no formed carina with

absent lung tissue and vessels

• Aplasia – bronchial stub (rudimentary)

• Hypoplasia – decreased number and size of airways, alveoli, and vessels

PULMONARY AGENESIS

• Lobar agenesis and aplasia is rarer than agenesis of the lung

• Usually the loss of the right upper and middle lobes

• Agenesis: no neural plexus, pulmonary artery, or parietal pleura

• Left sided 70%• Male equal to female• 50% die in the first year of life

PULMONARY AGENESIS

• Unilateral agenesis occurs because of a lack of one of the lung buds to form

• Congenital heart disease in 50%

• Right lung agenesis has an increased incidence of heart disease, morbidity, and mortality

• Vitamin A deficiency

• Association with Trisomy 18

CLINICAL• Varies depending on severity of the lesion

• Tachypnea, dyspnea, and cyanosis

• Mediastinal shift with compression of aorta and or tracheal compression

• Airway obstruction

• Unilateral lung expansion to fill chest

• No narrowing of the intercostal space or elevation of the diaphragm early on

• Decreased chest wall movement

• Decreased air movement• Scoliosis• Dyspnea with exertion

PULMONARY AGENESIS

PROGNOSIS • Right sided 75% (mortality)

• Left sided 25%

• Pulmonary hypertension

• Associated heart defects

• Scoliosis