Post on 04-Jun-2020
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