presentation1 april 2017 - copy · 2017-04-10 · 3uhvhqwhuv 'lvforvxuhv 5rqdog ( %rnxolf...
TRANSCRIPT
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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
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• Recognize and understand common and uncommon respiratory problems.
• Recognize that a common presentation of a respiratory illness may not represent a common respiratory illness
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This is a 6 ½ year old male with recurrent pneumonias and wheezing
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• 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
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• 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
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• 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
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• 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
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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
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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
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Chest x- ray
Spirometry
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DIFERENTIAL DIAGNOSIS
• Asthma
• Recurrent pneumonia
• Recurrent croup
• Immune deficiency
• Foreign body
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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
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.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.
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TESTING• Bronchoscopy with BAL• Computerized Tomography of
the chest • MRI of the chest• Immune work up• Methacholine challenge
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CHEST CT
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DIFFERENTIAL DIANOSIS
• Asthma
• Mediastinal Tumor
• Bronchogenic cyst
• Intrathoracic meningocele
• Encapsulated abscess
• Tracheal stricture at the carina
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BRONCHOGENIC CYSTS
.
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CHEST MRI
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THERAPY
SURGICAL RESECTION
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BRONCHOSCOPY
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BRONCHOALVEOLAR LAVAGE
• Cytology: 95% macrophages
3% lymphocytes
2% polymorphonuclear cells
• Viral, bacterial, and fungal cultures were no growth
• No lipid laden macrophages
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BRONCHOGENIC CYSTS
• Foregut Duplication Cysts
Bronchogenic or Enterogenic
• Usually asymptomatic ( vague substernal discomfort, cough , noisy breathing , dyspnea and cyanosis )
• Subcarinal cysts and respiratory distress
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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
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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
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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
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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
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BRONCHOGENIC CYSTS
•Surgery is indicated for symptomatic patients
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Eight month old with a one month history of wheezing and
more difficulty breathing
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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
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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
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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
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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
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DIFFERENCIAL DIAGNOSIS
• Bronchiolitis
• Cystic fibrosis
• Aspiration Pneumonitis
• Airway anomaly
• Congenital heart disease
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DIFFERENCIAL DIAGNOSIS
• Interstitial Lung Disease of Childhood
• Immunodeficiency with recurrent infections
• Pneumonia
• Foreign body
• Croup
• Environmental exposure to smoke
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DIAGNOSTIC STUDIES
•Chest x-ray
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DIAGNOSIS
• Esophageal foreign body
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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
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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 %
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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
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FOREIGN BODY• THERAPY• Preventive measures best
• Flexible vs. rigid bronchoscopy
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Six month old male with a three month history of tachypnea and
wheezing
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• 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
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Afebrile HR - 130 RR- 76 O2 sat. 99 %.
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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)
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DIAGNOSTIC TESTS
•CHEST X-RAY
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ANY OTHER TESTS
• HRCT examination of the chest
• Video Swallowing Study
• Abdominal X-ray
• Metabolic profile with liver enzymes
• MRI of the brain
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DIFFERENTIAL DIAGNOSIS
• Abdominal mass causing a restrictive lung process
• Wilms tumor, neuroblastoma, lymphoma , hepatic tumor or hepatomegally, stool impaction
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WHAT NEXT ?CT OF THE ABDOMEN
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LABORATORY TESTS
• Normal except for an elevated sedimentation rate
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• Remove the mass
• Respiratory rate was 30 –40 B/min 36 hours after surgery
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•WILMS TUMOR
•Stage I
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14 YEAR OLD WHITE FEMALE WITH SEVERE WHEEZING AND UNCONTROLLABLE ASTHMA
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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
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HISTORY• Frequency of exacerbations
• Days missed of school
• Cough
• Nasal symptoms
• Reflux symptoms
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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
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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
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YOU HAVE EXAMINED THIS GIRL TWICE, BOTH
TIMES ONE WEEK AFTER HOSPITAL
DISCHARGE AND SHE HAD A NORMAL EXAM
.
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DIFFERENTIAL DIAGNOSIS
• Hypersensitivity pneumonitis
• Asthma
• Psychogenic
• Congenital or acquired heart disease
• Immunodeficiency with recurrent viral and bacterial pneumonia
• Foreign body
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DIAGNOSTIC STUDIES
• Chest x-ray
• Spirometry
• Immune work-up (screening)
• ABPA work-up
• Allergy testing
• Hypersensitivity work-up
• Better H&P
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• An ICU nurse from a local hospital mentions that this patient seems to be in the ICU the same time almost every month
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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
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DIAGNOSIS
•CATAMENIAL or PREMENSRAL ASTHMA
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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
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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
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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
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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
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CLINICAL CASEMale, 3 months of age, wheezing since the second
week of life
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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
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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
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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
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DIAGNOSIS
• LARYNGOMALACIA
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MECHANISM• Medial collapse of aryepiglottic
folds
• Anterior collapse of arytenoids
• Posterior collapse of an omega shaped epiglottis
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DIAGNOSTIC TESTING
• None
• Oximetry
• Polysomnography
• Nasopharyngeal laryngoscopy
• Bronchoscopy
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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
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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
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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
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LARNGOMALACIA
• Diagnosis is make on clinical exam
• Atypical cases bronchoscopy or laryngoscopy
• Sleep disturbed patients may need polysomnography
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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
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This is a three year old with recurrent
pneumonias.
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• 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
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• 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
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• 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
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• Chest x-ray done 2 weeks prior to the office visit when she was diagnosed with a pneumonia
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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)
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• Chest x- ray PA and Lateral
• Immune testing ( screening )
• Sweat test
• Bronchoscopy
• MRI of the chest
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CT of the chest or MRI
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PULMONARY SEQUESTRATION
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Surgical removal
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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
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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
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SEQUESTRATION
• Usually no problems until it is infected - mid childhood early adult
• Fever, cough, suppuration, hemoptysis, rarely cardiovascular
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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.
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• .
An 8 month white male with recurrent
pneumonias
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HISTORY• Cough began at 2 months
• No wheezing
• Frank regurgitation
• Afebrile
• Mild tachypnea without retractions or distress
• Growing with a good oral intake
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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
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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
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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
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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%
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DIFFERENTIAL DIAGNOSIS
• Aspiration pneumonia
• Immunodeficiency
• Foreign body aspiration
• Community acquired pneumonia
• Sequestration
• Atelectasis
• Agenesis of the left upper lobe
• Agenesis of the left lung
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OTHER TESTING
CT EXAMINATION OF THE CHEST
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OTHER TESTING
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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
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Agenesis of the left upper lobe
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• Supportive
• Treat the associated anomalies
• Prevent respiratory infections
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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
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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
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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
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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
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• Decreased chest wall movement
• Decreased air movement• Scoliosis• Dyspnea with exertion
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PULMONARY AGENESIS
PROGNOSIS • Right sided 75% (mortality)
• Left sided 25%
• Pulmonary hypertension
• Associated heart defects
• Scoliosis